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  • 1.
    Al-Mashhadi, Ammar
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Häggman, Michael
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Urology.
    Läckgren, Göran
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Ladjevardi, Sam
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Urology.
    Nevéus, Tryggve
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Paediatric Inflammation Research.
    Stenberg, Arne
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Persson, A. Erik G.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Cell Biology.
    Carlstrom, Mattias
    Karolinska Inst, Dept Physiol & Pharmacol, Stockholm, Sweden.
    Changes of arterial pressure following relief of obstruction in adults with hydronephrosis2018In: Upsala Journal of Medical Sciences, ISSN 0300-9734, E-ISSN 2000-1967, Vol. 123, no 4, p. 216-224Article in journal (Refereed)
    Abstract [en]

    Background: As much as 20% of all cases of hypertension are associated with kidney malfunctions. We have previously demonstrated in animals and in pediatric patients that hydronephrosis causes hypertension, which was attenuated by surgical relief of the ureteropelvic junction (UPJ) obstruction. This retrospective cohort study aimed to investigate: (1) the proposed link between hydronephrosis, due to UPJ obstruction, and elevated arterial pressure in adults; and (2) if elevated blood pressure in patients with hydronephrosis might be another indication for surgery.

    Materials and methods: Medical records of 212 patients undergoing surgical management of hydronephrosis, due to UPJ obstruction, between 2000 and 2016 were assessed. After excluding patients with confounding conditions and treatments, paired arterial pressures (i.e. before/after surgery) were compared in 49 patients (35 years old; 95% CI 29–39). Split renal function was evaluated by using mercaptoacetyltriglycine (MAG3) renography before surgical management of the hydronephrotic kidney.

    Results: Systolic (−11 mmHg; 95% CI 6–15 mmHg), diastolic (−8 mmHg; 95% CI 4–11 mmHg), and mean arterial (-9 mmHg; 95% CI 6–12) pressures were significantly reduced after relief of the obstruction (p < 0.001). Split renal function of the hydronephrotic kidney was 39% (95% CI 37–41). No correlations were found between MAG3 and blood pressure level before surgery or between MAG3 and the reduction of blood pressure after surgical management of the UPJ obstruction.

    Conclusions: In adults with hydronephrosis, blood pressure was reduced following relief of the obstruction. Our findings suggest that elevated arterial pressure should be taken into account as an indication to surgically correct hydronephrosis.

  • 2.
    Al-Mashhadi, Ammar Nadhom Farman
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Dukic, Milena
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Rhabdomyomatous mesenchymal hamartoma presenting in a child as a perineal mass2019In: Journal of Pediatric Surgery Case Reports, ISSN 0022-3476, E-ISSN 2213-5766, Vol. 47, article id 101242Article in journal (Refereed)
    Abstract [en]

    Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare hamartomatous lesion in the dermis and subcutaneous tissue. It is mostly found in the face and neck region of children. We report a case of solitary RMH located in the perineum of an 8-month-old boy. Microscopic examination of specimen showed a disordered collection of mature adipose tissue, skeletal muscle, adnexal elements and nerve bundles, and immunohistochemistry confirmed a RMH. This case emphasizes the possibility of RMH in the perineum of the children. Even if RMH is a rare condition in the perineum it should be considered as a differential diagnosis of a perineal mass in children.

  • 3.
    Angsten, Gertrud
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Gustafson, Elisabet
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Dahl, Niklas
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Medicinsk genetik och genomik.
    Christofferson, Rolf H.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Resolution of infantile intestinal pseudo-obstruction in a boy2017In: Journal of Osteoporosis and Physical Activity, ISSN 2052-3211, E-ISSN 2213-5766, Vol. 24, p. 28-34Article in journal (Refereed)
    Abstract [en]

    A term boy with spontaneous passage of meconium exhibited episodes of abdominal distension and diarrhea. Due to failure to thrive and suspicion of Hischsprung's disease he was referred to our university hospital at five months of age. Rectal biopsies were normal. Laparotomy revealed dilation of the small bowel and colon without any mechanical obstruction. Full thickness bowel biopsies were taken and a loop ileostomy was constructed. Histopathology revealed fibrosing myopathy, Cajal cell hypertrophy, and neuronal degeneration in both the large and small bowel. The small bowel showed mastocytosis without inflammation. A central venous catheter was placed for vascular access, replaced three times and later switched to a subcutaneous venous port. Catheters were locked after use with vancomycin-heparin and later taurolidine. The individually tailored home parenteral nutrition contained unsaturated fatty acid lipids to reduce cholestasis. Initial insufficient growth was improved after correction of partial parenteral nutrition based on a metabolic balance study. The ileostomy was revised once and finally taken down at 11 years of age following one year without parenteral support. At follow-up at 13 years of age he has episodes of moderate abdominal pain and has entered puberty and reports a high quality of life. (C) 2017 The Authors. Published by Elsevier Inc.

  • 4.
    Danielson, Johan
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Karlbom, Urban
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Colorectal Surgery.
    Graf, Wilhelm
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Colorectal Surgery.
    Wester, Tomas
    Karolinska Univ Hosp, Dept Pediat Surg, Stockholm, Sweden;Karolinska Inst, Stockholm, Sweden.
    Persistent fecal incontinence into adulthood after repair of anorectal malformations2019In: International Journal of Colorectal Disease, ISSN 0179-1958, E-ISSN 1432-1262, Vol. 34, no 3, p. 551-554Article in journal (Refereed)
    Abstract [en]

    Purpose: Persistent fecal incontinence beyond childhood is common in ARM patients. The aim of this study was to analyze a consecutive series of adult patients with persistent incontinence, establish the causes, and evaluate whether further treatment could be offered. Methods: Forty-four adult ARM patients with reported incontinence were invited. Eighteen patients (11 males, median age 40.5 years, range 18-50 years) accepted and underwent clinical examination, rectoscopy, and 3D-ultrasound. Five had previously been treated with secondary surgery to improve continence. Results: Seventeen of the 18 patients had abnormal findings at examination. Eight patients had obstruction of the reconstructed anus. Eleven patients had sacral deformities. Nine patients had a defect in the external anal sphincter and nine patients could not contract the sphincter on demand. Five patients had significant prolapse of mucosa. In one patient, the neoanus was totally misplaced, one patient had a rectovaginal fistula, and one patient had short bowel syndrome due to several small bowel resections. Ten patients were offered conservative and five surgical treatment. Conclusions: This case series of adults shows that a majority of the patients can be offered further treatment. This indicates a need for structured follow-up of ARM patients into adulthood.

  • 5.
    Danielson, Johan
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Karlbom, Urban
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences.
    Wester, Tomas
    Astrid Lindgren Childrens Hosp, Dept Pediat Surg, Stockholm, Sweden.
    Graf, Wilhelm
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences.
    Long-Term Outcome after Dynamic Graciloplasty for Treatment of Persistent Fecal Incontinence in Patients with Anorectal Malformations2019In: European journal of pediatric surgery, ISSN 0939-7248, E-ISSN 1439-359X, Vol. 29, no 3, p. 276-281Article in journal (Refereed)
    Abstract [en]

    Purpose Dynamic graciloplasty (DGP) has been used to treat severe fecal incontinence since the 1980s. Previous studies have shown an inferior outcome in patients with anorectal malformations (ARMs). Our experience has been that DGP has been appreciated by ARM -patients. The objective of the study was to evaluate the long-term outcome of DGP in our patients with ARM compared with patients with other underlying conditions. Materials and Methods Twenty-three patients operated with DGP at our institution from 1996 to 2010 were sent validated bowel function and quality of life questionnaires. Eighteen of 23 responded. Seven had ARM and 11 had other etiologies of fecal incontinence. The mean follow-up time was 11.6 years (range, 5-17). Results Four of 7 of the patients with ARM and 8 of 11 of patients with other etiologies used their implants at follow-up. The Miller incontinence score was slightly higher for patients with ARMs, but they had less constipation and higher Fecal Incontinence Quality of Life (FIQL)- and 36-Item Short Form Health Survey (SF-36) scores. None of the differences were statistically significant. Conclusion This study cannot confirm earlier reports in which DGP has an inferior outcome in patients with ARM. We therefore believe that the procedure should remain a treatment option for selected patients.

  • 6.
    Fredriksson, Fanny
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Survival rates for surgically treated necrotising enterocolitis have improved over the last four decades2019In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 108, no 9, p. 1603-1608Article in journal (Refereed)
    Abstract [en]

    Aim Improved survival rates for premature infants have also increased the population at risk of necrotising enterocolitis (NEC). This study evaluated the outcomes of surgically treated NEC and identified risk factors for mortality, intestinal failure (IF) and IF associated liver disease (IFALD). Methods This was a retrospective observational study of 131 infants with surgically treated NEC from 1976 to 2016 in a Swedish tertiary referral centre: 20 in 1976-1996, 33 in 1997-2006 and 78 in 2007-2016. Data were extracted from medical records, and the Cox regression model was used to identify risk factors. Results When the first and last periods were compared, they showed decreases in both gestational age, from 30 to 26 weeks, and mortality rates, from 45% to 29%. IF was found in 67 patients (56%), IFALD in 41 patients (34%) and short bowel syndrome (SBS) in 13 (19%). The incidence of IF was high, even in infants without SBS. Low gestational age was an independent risk factor for mortality. No risk factors were identified for IF or IFALD. Conclusion Survival rates for NEC improved from 1976-2016, despite a decrease in gestational age. Clinicians should be particularly aware of the risk of infants without SBS developing IF.

  • 7.
    Fredriksson, Fanny
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Sellberg, Felix
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Bowden, Tim
    Uppsala University, Disciplinary Domain of Science and Technology, Chemistry, Department of Chemistry - Ångström, Polymer Chemistry.
    Engstrand, T.
    Karolinska Univ Hosp, Dept Reconstruct Plast Surg, SE-17176 Stockholm, Sweden.;Karolinska Inst, SE-17176 Stockholm, Sweden..
    Berglund, David
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Sutures impregnated with carbazate-activated polyvinyl alcohol reduce intraperitoneal adhesions2017In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 52, no 11, p. 1853-1858Article in journal (Refereed)
    Abstract [en]

    Background: Intraperitoneal adhesions cause significant morbidity. They occur after peritoneal trauma, which induces oxidative stress with production of inflammatory cytokines, peroxidized proteins (carbonyls) and lipids (aldehydes). This study aimed to investigate if carbazate-activated polyvinyl alcohol (PVAC), an aldehyde-carbonyl inhibitor, can reduce intraperitoneal adhesions in an experimental model.

    Material and methods: Male Sprague-Dawley rats (n = 110) underwent laparotomy, cecal abrasion and construction of a small bowel anastomosis. They either were treated with intraperitoneal instillation of PVAC or were sutured with PVAC-impregnated sutures. Thromboelastography analysis was performed using human blood and PVAC. The lipid peroxidation product malondialdehyde (MDA) and inflammatory cytokines IL-1 beta and IL-6 were quantified in peritoneal fluid. At day 7, bursting pressure of the anastomosis was measured and adhesions were blindly scored.

    Results: PVAC in human blood decreased the production of the fibrin-thrombocyte mesh without affecting the coagulation cascade. MDA, IL-1 beta and IL-6 were increased after 6 h without significant difference between the groups. PVAC-impregnated sutures reduced intraperitoneal adhesions compared to controls (p = 0.0406) while intraperitoneal instillation of PVAC had no effect. Anastomotic bursting pressure was unchanged.

    Conclusions: Intervention with an aldehyde-carbonyl inhibitor locally in the wound by PVAC-impregnated sutures might be a new strategy to reduce intraperitoneal adhesions.

  • 8.
    Georgantzi, Kleopatra
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology. University Children's Hospital, Uppsala, Sweden.
    Sköldenberg, Erik
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. University Children's Hospital, Uppsala, Sweden.
    Stridsberg, Mats
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Biochemical endocrinology. University Hospital, Uppsala, Sweden.
    Kogner, Per
    Department of Women´s and Children´s Health, Karolinska University Hospital, Solna, Stockholm, Sweden.
    Jakobson, Åke
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology. University Children's Hospital, Uppsala, Sweden.
    Tiensuu Janson, Eva
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Endocrin Oncology.
    Christofferson, Rolf. H.B.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. University Children's Hospital, Uppsala, Sweden.
    Chromogranin A and neuron-specific enolase in neuroblastoma: Correlation to stage and prognostic factors.2018In: Pediatric Hematology & Oncology, ISSN 0888-0018, E-ISSN 1521-0669, Vol. 35, no 2, p. 156-165Article in journal (Refereed)
    Abstract [en]

    Chromogranin A (CgA) and neuron specific enolase (NSE) are important markers in adult neuroendocrine tumors (NET). Neuroblastoma (NB) has certain neuroendocrine properties. The aim of this study was to correlate blood concentrations of CgA, chromogranin B (CgB), and NSE to prognostic factors and outcome in children with NB. Blood samples from 92 patients with NB, 12 patients with benign ganglioneuroma (GN), 21 patients with non-NB solid tumors, 10 patients with acute leukemias, and 69 healthy children, were analyzed. CgA concentrations were higher in neonates vs. children older than one month in the control group (p < 0.0001), and in neonates with NB vs. the control group (p < 0.01). CgA and NSE concentrations were higher in patients with stages 3 and 4 disease (p < 0.05 and p < 0.05), in patients having tumors with amplification of MYCN (p < 0.05 and p < 0.001), or chromosome 1 p deletion (p < 0.05 and p < 0.05). NSE correlated to the tumor size at diagnosis (p < 0.001) and to tumor related death (p < 0.01) in NB. CgA and NSE concentrations were elevated in patients with NB and especially in those with advanced disease. Both CgA and NSE correlated to genetic markers, while only NSE correlated to primary tumor size and outcome in NB. We found that CgA and NSE are clinically valuable tumor markers in NB and they merit prospective clinical evaluations as such.

  • 9.
    Georgantzi, Kleopatra
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
    Sköldenberg, Erik
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Tiensuu Janson, Eva
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Endocrine Oncology.
    Jakobson, Åke
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
    Christofferson, Rolf
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Diagnostic Ultrasound-Guided Cutting Needle Biopsies in Neuroblastoma: a safe and efficient procedure2019In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 54, no 6, p. 1253-1256Article in journal (Refereed)
    Abstract [en]

    Background

    Neuroblastoma (NB) is the most common extracranial solid tumor of childhood and accounts for 15% of deaths in pediatric oncology. Apart from the clinical stage at diagnosis, molecular factors are important for the characterization of the tumor and for decision on adequate treatment. Pretreatment diagnosis and molecular profiling are based on analysis of a tumor sample, obtained either by fine needle aspiration cytology (FNAC), cutting needle biopsy or open surgical biopsy. The method used depends on local tradition and routines. Ultrasound-guided cutting needle biopsy (UCNB) has been used at the Uppsala University Hospital since 1988 for diagnosis of pediatric solid tumors.

    Procedures

    Medical records of 29 patients with NB who underwent pretreatment, diagnostic, ultrasound-guided needle biopsy were reviewed. Information extracted from the patients’ records included: age at diagnosis, gender, tumor site, clinical stage, molecular profiling made on biopsies (e.g. MYCN status, ploidy and chromosomal aberrations), and UCNB complications (i.e. bleeding, pain, or anesthesiologic complications).

    Results

    A total of 34 UCNBs were performed in the 29 patients. Repeated biopsies were done in three patients. UCNB was diagnostic in 90% (26/29). A complete molecular profiling was obtained in all UCNBs after 2008. Two patients (7%) developed a significant bleeding and two (7%) needed analgesics following UCNB. Neither infection nor tumor growth in the needle tract was observed. There were no anesthesiologic complications.

    Conclusions

    UCNB is reasonably safe in patients with NB and usually gives a sufficient amount of tumor tissue for a histological diagnosis, molecular profiling, and biobank storage.

  • 10.
    Georgantzi, Kleopatra
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
    Tsolakis, Apostolos V.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Endocrine Tumor Biology. Karolinska Inst, Dept Oncol & Pathol, Stockholm, Sweden;Karolinska Univ Hosp Solna, CCK, Stockholm, Sweden.
    Jakobson, Åke
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
    Christofferson, Rolf
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Tiensuu Janson, Eva
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Endocrine Oncology.
    Grimelius, Lars
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Experimental and Clinical Oncology.
    Synaptic Vesicle Protein 2 and Vesicular Monoamine Transporter 1 and 2 Are Expressed in Neuroblastoma2019In: Endocrine pathology, ISSN 1046-3976, E-ISSN 1559-0097, Vol. 30, no 3, p. 173-179Article in journal (Refereed)
    Abstract [en]

    Neuroblastoma (NB), the most common extracranial cancer in childhood, exhibits neuroendocrine (NE) differentiation. Two well-established NE markers, chromogranin A (CgA) and synaptophysin (syn), are used in the histopathological diagnostics. Our aims were to explore if the NE markers synaptic vesicle protein 2 (SV2) and vesicular monoamine transporter 1 (VMAT1) and 2 (VMAT2) also are expressed in human NB and if so, evaluate their usefulness in NB histopathological diagnostics. Tumor specimens from 21 NB patients, before and/or after chemotherapy, were immunostained for CgA, syn, SV2, VMAT1, and VMAT2. Clinical data was extracted from patients' records. SV2 was highly expressed in NB, as was CgA while syn was less frequently expressed compared to the other two. Both VMATs were expressed in several NB, VMAT2 in more cases than VMAT1 and its expression was similar to syn. Chemotherapy did not affect the immunoreactivity in an obvious way. SV2 was highly expressed in NB and can thus be useful marker in NB diagnostics. VMAT1 and VMAT2 were also expressed in NB but similar to syn less reliable as tumor markers.

  • 11.
    Gustafson, Elisabet K.
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Hamad, Osama A.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Deckmyn, Hans
    Katholieke Univ Leuven, IRF Life Sci, Lab Thrombosis Res, Campus Kulak Kortrijk, Kortrijk, Belgium.
    Barbu, Andreea R
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology.
    Ekdahl, Kristina N.
    Linnaeus Univ, Linnaeus Ctr Biomaterials Chem, Kalmar, Sweden.
    Nilsson, Bo
    Uppsala University, Science for Life Laboratory, SciLifeLab. Uppsala Univ, Dept Immunol Genet & Pathol, Uppsala, Sweden.
    Exposure of von Willebrand Factor on Isolated Hepatocytes Promotes Tethering of Platelets to the Cell Surface2019In: Transplantation, ISSN 0041-1337, E-ISSN 1534-6080, Vol. 103, no 8, p. 1630-1638Article in journal (Refereed)
    Abstract [en]

    Background. Hepatocyte transplantation (Hctx) is a potentially attractive method for the treatment of acute liver failure and liver-based metabolic disorders. Unfortunately, the procedure is hampered by the instant blood-mediated inflammatory reaction (IBMIR), a thromboinflammatory response elicited by the vascular innate immune system, causing activation of the coagulation and complement systems and clearance of transplanted cells. Observations have also revealed platelets adhered to the surface of the hepatocytes (Hc). To establish Hctx as a clinical treatment, all factors that trigger IBMIR need to be identified and controlled. This work explores the expression of von Willebrand factor (VWF) on isolated Hc resulting in tethering of platelets. Methods. VWF on Hc was studied by flow cytometry, confocal microscopy, immunoblot, and real-time polymerase chain reaction. Interaction between Hc and platelets was studied in a Chandler loop model. Adhesion of platelets to the hepatocyte surface was demonstrated by flow cytometry and confocal microscopy. Results. Isolated Hc constitutively express VWF on their cell surface and mRNA for VWF was found in the cells. Hc and platelets, independently of coagulation formed complexes, were shown by antibody blocking studies to be dependent on hepatocyte-associated VWF and platelet-bound glycoprotein Ib alpha. Conclusions. VWF on isolated Hc causes, in contact with blood, adhesion of platelets, which thereby forms an ideal surface for coagulation. This phenomenon needs to be considered in hepatocyte-based reconstitution therapy and possibly even in other settings of cell transplantation.

  • 12.
    Gustafson, Elisabet K.
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Larsson, Therese
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Danielson, Johan
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Controlled outcome of Hirschsprung's disease beyond adolescence: a single center experience2019In: Pediatric surgery international (Print), ISSN 0179-0358, E-ISSN 1437-9813, Vol. 35, no 2, p. 181-185Article in journal (Refereed)
    Abstract [en]

    PurposeThe aim of this study was to assess the function and quality of life of Hirschsprung's Disease (HD) beyond adolescence and relate it to matched controls.MethodsAll 203 patients diagnosed with HD at our department from 1961 to 1995 were identified. 21 had died, 43 had unclear diagnosis and 16 could not be traced. The remaining 123 patients were sent bowel function and SF-36 quality of life questionnaires. 69 patients (mean age 37.8, range 22-58, 13 female) responded and were matched with 138 age and sex-matched controls.ResultsFunction: HD-patients had significantly higher number of bowel movements per week, higher incidence of soiling, urgency, permanent stomas, use of laxatives, enemas and loperamide. HD-patients also scored significantly lower in their satisfaction with their bowel function. There was, however, no significant difference in Miller Incontinence score.QOL: HD-patients reported a significantly higher incidence of negative impact by their bowel function on daily life, social interaction and ability to go on vacation. There were no significant differences in SF-36-scores.ConclusionsBowel function has a lifelong negative impact on the lives of patients with HD. This strongly indicates a need for structured follow-up beyond adolescence.

  • 13.
    Kassa, Ann-Marie
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Living with VACTERL association: From the perspectives of children, adolescents and their parents2019Doctoral thesis, comprehensive summary (Other academic)
    Abstract [en]

    VACTERL association is a rare and complex congenital condition often requiring repeated surgery and entailing various physical sequelae. Knowledge is scarce regarding experiences of the health condition and health care, need of support at school, health-related quality of life (HRQoL), and psychological well-being in children, adolescents and their parents.

    This thesis aims to investigate various aspects of living with VACTERL association, from the perspectives of children, adolescents and their parents.

    Ten children aged five to eight years were interviewed using the computer-assisted interview technique In My Shoes (Study I). They expressed awareness of their health history and felt proud but also different due to physical dysfunction. While happy to meet familiar staff in the hospital they voiced worries about medical procedures.

    The nineteen parents interviewed described crisis reactions on the discovery of malformations in their child (Study II). Parental involvement in care was reported from the initial hospital admission until taking responsibility for treatments at home. Eventually the health condition became integrated in everyday life. Insufficient emotional and limited medical support were reported. Various levels of professionalism among healthcare professionals and discrepancies concerning knowledge and experience between the tertiary and local hospitals were described.

    In ten evaluated pre-school children (Study III), intelligence measured by Wechsler-scales was within the normal range. Eight children had attention difficulties and two were later diagnosed with attention deficit hyperactivity disorder (ADHD). All children had physical dysfunctions affecting their nutrition, bowel or bladder functions. All needed extra support and adjustments at school.

    Forty children and adolescents responded to validated questionnaires of DISABKIDS and Beck inventories (Study IV). The HRQoL was comparable to European children with chronic conditions. Their psychological well-being was similar to that of Swedish school children and significantly better than that of a clinical sample. Self-reported anxiety and depression in 38 mothers and 33 fathers were comparable to non-clinical samples.

    In conclusion, regular follow-up by multi-professional team with continuity is crucial to optimise the physical function in children with VACTERL, to identify those in need of extra support at school and to detect reduced psychological well-being in children and parents. Fear of medical procedures may be reduced by carefully providing information and individual care strategies. For the parents psychological processing, support from medical experts and peers is essential to achieve self-confidence and adaptation. Transfer of knowledge and information between multi-professional teams at the local and tertiary hospitals could be improved by the use of video sessions.

    List of papers
    1. Young children with severe congenital malformations (VACTERL) expressed mixed feelings about their condition and worries about needles and anaesthesia
    Open this publication in new window or tab >>Young children with severe congenital malformations (VACTERL) expressed mixed feelings about their condition and worries about needles and anaesthesia
    2017 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 106, no 10, p. 1694-1701Article in journal (Refereed) Published
    Abstract [en]

    Aim: Our knowledge of the perceptions that children with severe congenital malformations have of their health, treatment and how to improve hospital care is limited. This study focused on patients with vertebral defects, anal atresia, cardiac defects, tracheo-oesophageal fistula, renal anomalies and limb abnormalities (VACTERL).

    Methods: We interviewed 10 children aged five to eight years with VACTERL association who were treated in a Swedish tertiary paediatric surgical centre, using a computer-assisted technique called In My Shoes. The interviews were analysed by qualitative content analysis.

    Results: The children described their awareness of their health history and said they felt proud but different due to their physical dysfunction. They were happy to visit the hospital to meet familiar staff, but expressed negative feelings about missing normal life. They were afraid of needle-related procedures and not wakening up after anaesthesia. Various ways of coping with difficult situations were expressed, and suggestions to improve hospital care were voiced.

    Conclusion: Careful follow-up of these children by multidisciplinary teams is crucial to optimise their health and functional status. Fear of medical procedures may be reduced by carefully delivered information, listening to the children, providing continuity of care and creating individual care strategies.

    Place, publisher, year, edition, pages
    WILEY, 2017
    Keywords
    Care strategies, Computer-assisted interview Fear of anaesthesia, Needle-related procedures, Severe congenital malformations
    National Category
    Medical and Health Sciences
    Identifiers
    urn:nbn:se:uu:diva-334743 (URN)10.1111/apa.13973 (DOI)000409348400024 ()28672091 (PubMedID)
    Available from: 2017-11-29 Created: 2017-11-29 Last updated: 2019-08-21Bibliographically approved
    2. From crisis to self-confidence and adaptation; Experiences of being a parent of a child with VACTERL association: A complex congenital malformation
    Open this publication in new window or tab >>From crisis to self-confidence and adaptation; Experiences of being a parent of a child with VACTERL association: A complex congenital malformation
    2019 (English)In: PLoS ONE, ISSN 1932-6203, E-ISSN 1932-6203, Vol. 14, no 4, article id e0215751Article in journal (Refereed) Published
    Abstract [en]

    Aim Knowledge is scarce regarding mothers' and fathers' experiences of being a parent of a child with VACTERL association-a complex malformation. The aim of the study was to describe experiences of being a parent of a child with VACTERL association. Method Semi-structured interviews were performed with ten mothers and nine fathers face-to-face or by telephone and analyzed by using Qualitative content analysis. Results The parents described crisis reactions at the discovery of malformations in their child. Involvement in care was reported from the initial hospital admission until actively taking responsibility for treatments at home. Eventually the health condition became an integrated part of everyday life. The parents expressed the importance of meeting other families with a child with VACTERL. Descriptions were given of more or less professionalism with perceived discrepancies of knowledge and experience between the healthcare professionals in the tertiary hospital and those in the local hospital. Difficulties in receiving medical support during the initial period at home were described. Furthermore, emotional support and practical arrangements regarding parental accommodation and transportation varied. Conclusion Being a parent of a child with VACTERL association involves crisis, mixed emotional reactions and shared responsibility for the child ' s treatment and care with the professional care providers. Psychological processing, good medical care and support from experts, and peer support from other parents is essential in the parents' struggle to reach self-confidence and adaptation. A care plan with individualized tailored care for each child including a training and support plan for the parents is warranted. To reduce the described discrepancies in knowledge and experience between the local and tertiary hospital, video sessions with the parents and responsible professionals at the local and tertiary hospital could be an appropriate mode of transferring information at discharge and follow up of the child.

    Place, publisher, year, edition, pages
    PUBLIC LIBRARY SCIENCE, 2019
    National Category
    Nursing
    Identifiers
    urn:nbn:se:uu:diva-383190 (URN)10.1371/journal.pone.0215751 (DOI)000465019900049 ()31002700 (PubMedID)
    Available from: 2019-07-24 Created: 2019-07-24 Last updated: 2019-08-21Bibliographically approved
    3. Attention difficulties and physical dysfunction common in children with complex congenital malformations:: a study of preschool children with VACTERL association.
    Open this publication in new window or tab >>Attention difficulties and physical dysfunction common in children with complex congenital malformations:: a study of preschool children with VACTERL association.
    2018 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227Article in journal (Refereed) Epub ahead of print
    Abstract [en]

    AIM: Knowledge on the neurodevelopmental and physical function in children with vertebral defects, anorectal malformations, cardiac defects, tracheo-oesophageal fistula, renal and limb malformations (VACTERL) is scarce. We evaluated Swedish preschool children with VACTERL and identified whether they would need extra support in school.

    METHODS: From 2015 to 2017, we recruited children aged 5-7 with VACTERL association from the paediatric surgical centre at the University Children's Hospital at Uppsala. Neurodevelopmental function was assessed by age-appropriate intelligence and visual and auditory attention tests, and the children's behaviour and attention were observed by an experienced psychologist. Physical function was evaluated through parental interviews and examinations. Data on patient characteristics, including any surgery and anaesthesia, were extracted from medical records.

    RESULTS: Of the 13 eligible families, 10 agreed to participate. Intelligence was within the normal range for all children, but attention difficulties were found in eight of the children, requiring adjustments at school, and two of these were later diagnosed with attention deficit hyperactivity disorder. All children had physical dysfunctions that affected their daily nutrition, bowel or bladder functions.

    CONCLUSION: Attention difficulties and physical dysfunction were common in Swedish preschool children aged 5-7 with VACTERL and they would need support and adjustments when they started school.

    Keywords
    Attention difficulties, Comorbidities, Congenital malformations, Neurodevelopmental function, Physical dysfunction
    National Category
    Pediatrics
    Identifiers
    urn:nbn:se:uu:diva-371838 (URN)10.1111/apa.14566 (DOI)30187514 (PubMedID)
    Available from: 2019-01-02 Created: 2019-01-02 Last updated: 2019-08-21Bibliographically approved
    4. Children and adolescents with VACTERL association: health-related quality of life and psychological well-being in children and adolescents and their parents
    Open this publication in new window or tab >>Children and adolescents with VACTERL association: health-related quality of life and psychological well-being in children and adolescents and their parents
    Show others...
    (English)In: Article in journal (Refereed) Accepted
    Abstract [en]

    Purpose: VACTERL association is a rare and complex condition of congenital malformations, often requiring repeated surgery and entailing various physical sequelae. Due to scarcity of knowledge, the study aim was to investigate self-reported Health-Related Quality of Life (HRQoL), anxiety, depression and self-concept in children and adolescents with VACTERL association and self-reported anxiety and depression in their parents.

    Methods: Patients aged 8-17 years with VACTERL association and their parents were recruited from three of four Swedish paediatric surgical centres during 2015-2019. The well-established validated questionnaires DISABKIDS, Beck Youth Inventories, Beck Anxiety Inventory and Beck Depression Inventory were sent to the families. Data were analysed using descriptives, t-tests and multivariable analysis. Results were compared with norm groups and reference samples.

    Results: The questionnaires were returned by 40 patients, 38 mothers and 33 fathers. The mean HRQoL was M=80.4, comparable to children with asthma (M=80.2) and diabetes (M=79.5). Self-reported psychological well-being was comparable to the norm group of Swedish school children, and was significantly higher than a clinical sample. Factors negatively influencing children’s HRQoL and psychological well-being were identified. The parents’ self-reports of anxiety and depression were comparable to non-clinical samples.

    Conclusions: Although children and adolescents with VACTERL association reported similar HRQoL to those of European children with chronic conditions, their psychological well-being was comparable to Swedish school children in general. Nevertheless, some individuals among both children and parents were in need of extra support. This attained knowledge is valuable when counselling parents regarding the prognosis for children with VACTERL association.

    Keywords
    Congenital malformations, VACTERL association, Health-Related Quality of Life, psychological well-being, children and adolescents, parents
    National Category
    Medical and Health Sciences
    Identifiers
    urn:nbn:se:uu:diva-390576 (URN)
    Available from: 2019-08-16 Created: 2019-08-16 Last updated: 2019-09-04
  • 14.
    Kassa, Ann-Marie
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Akademiska sjukhuset, sektionen för barnkirurgi.
    Dahl, Margareta
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Strinnholm, Margareta
    Akademiska sjukhuset, Folke Bernadotte regionhabilitering.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Akademiska sjukhuset, sektionen för barnkirurgi.
    Attention difficulties and physical dysfunction common in children with complex congenital malformations:: a study of preschool children with VACTERL association.2018In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227Article in journal (Refereed)
    Abstract [en]

    AIM: Knowledge on the neurodevelopmental and physical function in children with vertebral defects, anorectal malformations, cardiac defects, tracheo-oesophageal fistula, renal and limb malformations (VACTERL) is scarce. We evaluated Swedish preschool children with VACTERL and identified whether they would need extra support in school.

    METHODS: From 2015 to 2017, we recruited children aged 5-7 with VACTERL association from the paediatric surgical centre at the University Children's Hospital at Uppsala. Neurodevelopmental function was assessed by age-appropriate intelligence and visual and auditory attention tests, and the children's behaviour and attention were observed by an experienced psychologist. Physical function was evaluated through parental interviews and examinations. Data on patient characteristics, including any surgery and anaesthesia, were extracted from medical records.

    RESULTS: Of the 13 eligible families, 10 agreed to participate. Intelligence was within the normal range for all children, but attention difficulties were found in eight of the children, requiring adjustments at school, and two of these were later diagnosed with attention deficit hyperactivity disorder. All children had physical dysfunctions that affected their daily nutrition, bowel or bladder functions.

    CONCLUSION: Attention difficulties and physical dysfunction were common in Swedish preschool children aged 5-7 with VACTERL and they would need support and adjustments when they started school.

  • 15.
    Kassa, Ann-Marie
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Dellenmark-Blom, Michaela
    Insitutionen för kliniska vetenskaper, avd för pediatrik, Sahlgrenska akademin, Göteborgs universitet.
    Thorsell Cederberg, Jenny
    Institutionen för klinisk neurovetenskap, Karolinska Institutet, Stockholm.
    Engvall, Gunn
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Public Health and Caring Sciences. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Children and adolescents with VACTERL association: health-related quality of life and psychological well-being in children and adolescents and their parentsIn: Article in journal (Refereed)
    Abstract [en]

    Purpose: VACTERL association is a rare and complex condition of congenital malformations, often requiring repeated surgery and entailing various physical sequelae. Due to scarcity of knowledge, the study aim was to investigate self-reported Health-Related Quality of Life (HRQoL), anxiety, depression and self-concept in children and adolescents with VACTERL association and self-reported anxiety and depression in their parents.

    Methods: Patients aged 8-17 years with VACTERL association and their parents were recruited from three of four Swedish paediatric surgical centres during 2015-2019. The well-established validated questionnaires DISABKIDS, Beck Youth Inventories, Beck Anxiety Inventory and Beck Depression Inventory were sent to the families. Data were analysed using descriptives, t-tests and multivariable analysis. Results were compared with norm groups and reference samples.

    Results: The questionnaires were returned by 40 patients, 38 mothers and 33 fathers. The mean HRQoL was M=80.4, comparable to children with asthma (M=80.2) and diabetes (M=79.5). Self-reported psychological well-being was comparable to the norm group of Swedish school children, and was significantly higher than a clinical sample. Factors negatively influencing children’s HRQoL and psychological well-being were identified. The parents’ self-reports of anxiety and depression were comparable to non-clinical samples.

    Conclusions: Although children and adolescents with VACTERL association reported similar HRQoL to those of European children with chronic conditions, their psychological well-being was comparable to Swedish school children in general. Nevertheless, some individuals among both children and parents were in need of extra support. This attained knowledge is valuable when counselling parents regarding the prognosis for children with VACTERL association.

  • 16.
    Kassa, Ann-Marie
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Univ Childrens Hosp, Dept Pediat Surg, Uppsala, Sweden.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Univ Childrens Hosp, Dept Pediat Surg, Uppsala, Sweden.
    Engvall, Gunn
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
    From crisis to self-confidence and adaptation; Experiences of being a parent of a child with VACTERL association: A complex congenital malformation2019In: PLoS ONE, ISSN 1932-6203, E-ISSN 1932-6203, Vol. 14, no 4, article id e0215751Article in journal (Refereed)
    Abstract [en]

    Aim Knowledge is scarce regarding mothers' and fathers' experiences of being a parent of a child with VACTERL association-a complex malformation. The aim of the study was to describe experiences of being a parent of a child with VACTERL association. Method Semi-structured interviews were performed with ten mothers and nine fathers face-to-face or by telephone and analyzed by using Qualitative content analysis. Results The parents described crisis reactions at the discovery of malformations in their child. Involvement in care was reported from the initial hospital admission until actively taking responsibility for treatments at home. Eventually the health condition became an integrated part of everyday life. The parents expressed the importance of meeting other families with a child with VACTERL. Descriptions were given of more or less professionalism with perceived discrepancies of knowledge and experience between the healthcare professionals in the tertiary hospital and those in the local hospital. Difficulties in receiving medical support during the initial period at home were described. Furthermore, emotional support and practical arrangements regarding parental accommodation and transportation varied. Conclusion Being a parent of a child with VACTERL association involves crisis, mixed emotional reactions and shared responsibility for the child ' s treatment and care with the professional care providers. Psychological processing, good medical care and support from experts, and peer support from other parents is essential in the parents' struggle to reach self-confidence and adaptation. A care plan with individualized tailored care for each child including a training and support plan for the parents is warranted. To reduce the described discrepancies in knowledge and experience between the local and tertiary hospital, video sessions with the parents and responsible professionals at the local and tertiary hospital could be an appropriate mode of transferring information at discharge and follow up of the child.

  • 17.
    Kassa, Ann-Marie
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Univ Childrens Hosp, Dept Paediat Surg, SE-75185 Uppsala, Sweden..
    Engvall, Gunn
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Univ Childrens Hosp, Dept Paediat Surg, SE-75185 Uppsala, Sweden..
    Young children with severe congenital malformations (VACTERL) expressed mixed feelings about their condition and worries about needles and anaesthesia2017In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 106, no 10, p. 1694-1701Article in journal (Refereed)
    Abstract [en]

    Aim: Our knowledge of the perceptions that children with severe congenital malformations have of their health, treatment and how to improve hospital care is limited. This study focused on patients with vertebral defects, anal atresia, cardiac defects, tracheo-oesophageal fistula, renal anomalies and limb abnormalities (VACTERL).

    Methods: We interviewed 10 children aged five to eight years with VACTERL association who were treated in a Swedish tertiary paediatric surgical centre, using a computer-assisted technique called In My Shoes. The interviews were analysed by qualitative content analysis.

    Results: The children described their awareness of their health history and said they felt proud but different due to their physical dysfunction. They were happy to visit the hospital to meet familiar staff, but expressed negative feelings about missing normal life. They were afraid of needle-related procedures and not wakening up after anaesthesia. Various ways of coping with difficult situations were expressed, and suggestions to improve hospital care were voiced.

    Conclusion: Careful follow-up of these children by multidisciplinary teams is crucial to optimise their health and functional status. Fear of medical procedures may be reduced by carefully delivered information, listening to the children, providing continuity of care and creating individual care strategies.

  • 18.
    Lundin, Johanna
    et al.
    Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm, Sweden;Karolinska Univ Hosp, Dept Clin Genet, Stockholm, Sweden.
    Markljung, Ellen
    Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm, Sweden.
    Korberg, Izabella Baranowska
    Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm, Sweden.
    Hofmeister, Wolfgang
    Karolinska Inst, Dept Mol Med & Surg, Stockholm, Sweden.
    Cao, Jia
    Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm, Sweden.
    Nilsson, Daniel
    Karolinska Univ Hosp, Dept Clin Genet, Stockholm, Sweden;Karolinska Inst, Dept Mol Med & Surg, Stockholm, Sweden;Karolinska Inst, Sci Life Lab, Sci Pk, Stockholm, Sweden.
    Holmdahl, Gundela
    Sahlgrens Acad, Dept Pediat Surg, Gothenburg, Sweden.
    Barker, Gillian
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Anderberg, Magnus
    Univ Hosp Lund, Dept Pediat Surg, Lund, Sweden.
    Vukojevic, Vladana
    Karolinska Inst, Ctr Mol Med, Dept Clin Neurosci, Stockholm, Sweden.
    Lindstrand, Anna
    Karolinska Univ Hosp, Dept Clin Genet, Stockholm, Sweden;Karolinska Inst, Dept Mol Med & Surg, Stockholm, Sweden.
    Nordenskjold, Agneta
    Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm, Sweden;Karolinska Univ Hosp, Astrid Lindgren Children Hosp, Pediat Surg, Stockholm, Sweden.
    Further support linking the 22q11.2 microduplication to an increased risk of bladder exstrophy and highlighting LZTR1 as a candidate gene2019In: Molecular Genetics & Genomic Medicine, ISSN 2324-9269, Vol. 7, no 6, article id UNSP e666Article in journal (Refereed)
    Abstract [en]

    Background

    The bladder exstrophy‐epispadias complex (BEEC) is a congenital malformation of the bladder and urethra. The underlying causes of this malformation are still largely unknown; however, aside from environment, genetics is thought to play an essential role. The recurrent 22q11.2 microduplication is the most persistently detected genetic aberration found in BEEC cases.

    Methods

    We performed array comparative genomic hybridization (array‐CGH) analysis of 76 Swedish BEEC patients. Statistical analysis was performed on current dataset pooled with previously published data on the 22q11.2 microduplication in BEEC patients. We performed massive parallel sequencing (MPS) of the 22q11.2 region in 20 BEEC patients without the 22q11.2 microduplication followed by functional studies.

    Results

    We identified three additional cases with the 22q11.2 microduplication. Pooling data from this study with previously published reports showed a statistically significant enrichment of the 22q11.2 microduplication in BEEC patients (2.61% in cases vs. 0.08% in controls; OR = 32.6; p = 8.7 × 10−4). MPS of the 22q11.2 region in 20 BEEC patients without the 22q11.2 microduplication identified a novel variant in LZTR1 (p.Ser698Phe) in one patient. Functional evaluation of the LZTR1 p.Ser698Phe variant in live NIH 3T3 cells showed that the concentration and cytoplasmic mobility differ between the Lztr1wt and Lztr1mut, indicating a potential functional effect of the LZTR1mut.

    Conclusion

    Our study further emphasizes the involvement of the 22q11.2 region in BEEC development and highlights LZTR1 as a candidate gene underlying the urogenital malformation.

  • 19.
    Markasz, Laszlo
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Perinatal, Neonatal and Pediatric Cardiology Research.
    Wanders, Alkwin
    Umea Univ, Dept Biomed Sci, Umea, Sweden.
    Szekely, Laszlo
    Karolinska Inst, Dept Lab Med, Div Pathol, Stockholm, Sweden.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Diminished DEFA6 Expression in Paneth Cells Is Associated with Necrotizing Enterocolitis2018In: Gastroenterology Research and Practice, ISSN 1687-6121, E-ISSN 1687-630X, article id 7345426Article in journal (Refereed)
    Abstract [en]

    Background. Necrotizing enterocolitis (NEC) is the most common gastrointestinal disorder in premature infants with a high morbidity and mortality. Paneth cell dysfunction has been suggested to be involved in the pathogenesis of NEC. Defensin alpha-6 (DEFA6) is a specific marker for Paneth cells acting as part of the innate immunity in the human intestines. The aim of this study was to investigate the expression of DEFA6 in infants with NEC. Materials and Methods. Infants who underwent bowel resection for NEC at level III NICU in Sweden between August 2004 and September 2013 were eligible for the study. Macroscopically vital tissues were selected for histopathological evaluation. All infants in the control group underwent laparotomy and had ileostomy due to dysmotility, and samples were taken from the site of the stoma. DEFA6 expression was studied by immunohistochemistry. Digital image analysis was used for an objective and precise description of the samples. Results. A total of 12 infants were included in the study, eight with NEC and four controls. The tissue samples were taken from the colon (n = 1), jejunum (n = 1), and ileum (n = 10). Both the NEC and control groups consisted of extremely premature and term infants (control group: 25-40 gestational weeks, NEC group: 23-39 gestational weeks). The postnatal age at the time of surgery varied in both groups (control group: 4-47 days, NEC group: 4-50 days). DEFA6 expression in the NEC group was significantly lower than that in the control group and did not correlate with gestational age. Conclusion. The diminished DEFA6 expression in Paneth cells associated with NEC in this study supports the hypothesis that alpha-defensins are involved in the pathophysiology of NEC. Future studies are needed to elucidate the role of alpha-defensins in NEC aiming at finding preventive and therapeutic strategies against NEC.

  • 20.
    Nilsson Ekdahl, Kristina
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology. Linnæus Center of Biomaterials Chemistry, Linnæus University, Kalmar, Sweden.
    Teramura, Yuji
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology. Department of Bioengineering, The University of Tokyo, Tokyo, Japan.
    Hamad, Osama A.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Asif, Sana
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Dührkop, Claudia
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Fromell, Karin
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Gustafson, Elisabet K.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Hong, Jaan
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Kozarcanin, Huda
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Magnusson, Peetra
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Huber-Lang, Markus
    Department of Orthopedic Trauma, Hand, Plastic and Reconstructive Surgery, University of Ulm, Ulm, Germany.
    Garred, Peter
    Laboratory of Molecular Medicine, Department of Clinical Immunology, Section 7631, Faculty of Health and Medical Sciences, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
    Nilsson, Bo
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Dangerous liaisons: complement, coagulation, and kallikrein/kinin cross-talk act as a linchpin in the events leading to thromboinflammation2016In: Immunological Reviews, ISSN 0105-2896, E-ISSN 1600-065X, Vol. 274, no 1, p. 245-269Article in journal (Refereed)
    Abstract [en]

    Innate immunity is fundamental to our defense against microorganisms. Physiologically, the intravascular innate immune system acts as a purging system that identifies and removes foreign substances leading to thromboinflammatory responses, tissue remodeling, and repair. It is also a key contributor to the adverse effects observed in many diseases and therapies involving biomaterials and therapeutic cells/organs. The intravascular innate immune system consists of the cascade systems of the blood (the complement, contact, coagulation, and fibrinolytic systems), the blood cells (polymorphonuclear cells, monocytes, platelets), and the endothelial cell lining of the vessels. Activation of the intravascular innate immune system in vivo leads to thromboinflammation that can be activated by several of the system's pathways and that initiates repair after tissue damage and leads to adverse reactions in several disorders and treatment modalities. In this review, we summarize the current knowledge in the field and discuss the obstacles that exist in order to study the cross-talk between the components of the intravascular innate immune system. These include the use of purified in vitro systems, animal models and various types of anticoagulants. In order to avoid some of these obstacles we have developed specialized human whole blood models that allow investigation of the cross-talk between the various cascade systems and the blood cells. We in particular stress that platelets are involved in these interactions and that the lectin pathway of the complement system is an emerging part of innate immunity that interacts with the contact/coagulation system. Understanding the resulting thromboinflammation will allow development of new therapeutic modalities.

  • 21.
    Ogasawara, Hiroyuki
    et al.
    Tohoku Univ, Grad Sch Med, Dept Surg, Sendai, Miyagi, Japan.
    Teramura, Yuji
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology. Univ Tokyo, Dept Bioengn, Tokyo, Japan.
    Imura, Takehiro
    Tohoku Univ, Div Transplantat & Regenerat Med, Sendai, Miyagi, Japan.
    Inagaki, Akiko
    Tohoku Univ, Div Transplantat & Regenerat Med, Sendai, Miyagi, Japan.
    Saito, Yoshikatsu
    Tohoku Univ, Grad Sch Med, Dept Surg, Sendai, Miyagi, Japan.
    Matsumura, Muneyuki
    Tohoku Univ, Grad Sch Med, Dept Surg, Sendai, Miyagi, Japan.
    Fukuoka, Kengo
    Tohoku Univ, Grad Sch Med, Dept Surg, Sendai, Miyagi, Japan.
    Fathi, Ibrahim
    Tohoku Univ, Div Transplantat & Regenerat Med, Sendai, Miyagi, Japan.
    Miyagi, Shigehito
    Tohoku Univ, Grad Sch Med, Dept Surg, Sendai, Miyagi, Japan.
    Nilsson, Kristina
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology.
    Ohashi, Kazuo
    Osaka Univ, Grad Sch Pharmaceut Sci, Osaka, Japan.
    Unno, Michiaki
    Tohoku Univ, Grad Sch Med, Dept Surg, Sendai, Miyagi, Japan.
    Kamei, Takashi
    Tohoku Univ, Grad Sch Med, Dept Surg, Sendai, Miyagi, Japan.
    Satomi, Susumu
    Tohoku Univ, Grad Sch Med, Dept Surg, Sendai, Miyagi, Japan.
    Nilsson, Bo
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Gustafson, Elisabet K.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Goto, Masafumi
    Tohoku Univ, Grad Sch Med, Dept Surg, Sendai, Miyagi, Japan;Tohoku Univ, Div Transplantat & Regenerat Med, Sendai, Miyagi, Japan.
    The Optimization of the Hepatocyte Surface Modification Procedures in Terms of Heparin and Apyrase for Improving Hepatocyte Engraftment2018In: Transplantation, ISSN 0041-1337, E-ISSN 1534-6080, Vol. 102, p. S727-S727Article in journal (Other academic)
  • 22.
    Peleli, Maria
    et al.
    Karolinska Inst, Dept Physiol & Pharmacol, Stockholm, Sweden..
    Flacker, Peter
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Karolinska Inst, Dept Physiol & Pharmacol, Stockholm, Sweden.
    Zhuge, Zhengbing
    Karolinska Inst, Dept Physiol & Pharmacol, Stockholm, Sweden..
    Gomez, Cristina
    Karolinska Inst, Dept Med Biochem & Biophys, Div Physiol Chem 2, Stockholm, Sweden..
    Wheelock, Craig E.
    Karolinska Inst, Dept Med Biochem & Biophys, Div Physiol Chem 2, Stockholm, Sweden..
    Persson, Erik
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Cell Biology. Karolinska Inst, Dept Physiol & Pharmacol, Stockholm, Sweden.
    Carlstrom, Mattias
    Karolinska Inst, Dept Physiol & Pharmacol, Stockholm, Sweden..
    Renal denervation attenuates hypertension and renal dysfunction in a model of cardiovascular and renal disease, which is associated with reduced NADPH and xanthine oxidase activity2017In: Redox Biology, ISSN 0090-7324, E-ISSN 2213-2317, Vol. 13, p. 522-527Article in journal (Refereed)
    Abstract [en]

    Oxidative stress is considered a central pathophysiological event in cardiovascular disease, including hypertension. Early age reduction in renal mass is associated with hypertension and oxidative stress in later life, which is aggravated by increased salt intake. The aim of the present study was to examine if renal sympathetic denervation can exert blood pressure lowering effects in uninephrectomized (UNX) rats (3-week old) fed with high salt (HS, 4%; w/w) diet for 4 weeks. Moreover, we investigated if renal denervation is associated with changes in NADPH and xanthine oxidase-derived reactive oxygen species. Rats with UNX + HS had reduced renal function, elevated systolic and diastolic arterial pressures, which was accompanied by increased heart weight, and cardiac superoxide production compared to sham operated Controls. UNX + HS was also associated with higher expression and activity of NADPH and xanthine oxidase in the kidney. Renal denervation in rats with UNX + HS attenuated the development of hypertension and cardiac hypertrophy, but also improved glomerular filtration rate and reduced proteinuria. Mechanistically, renal de nervation was associated with lower expression and activity of both NADPH oxidase and xanthine oxidase in the kidney, but also reduced superoxide production in the heart. In conclusion, our study shows for the first time that renal denervation has anti-hypertensive, cardio- and reno-protective effects in the UNX + HS model, which can be associated with decreased NADPH oxidase- and xanthine oxidase-derived reactive oxygen species (i.e., superoxide and hydrogen peroxide) in the kidney.

  • 23.
    Stadil, Tatjana
    et al.
    Odense Univ Hosp, Surg Dept A, Sdr Blvd 29, DK-5000 Odense C, Denmark.
    Koivusalo, Antti
    Univ Helsinki, Childrens Hosp, Dept Pediat Surg, Helsinki, Finland.
    Pakarinen, Mikko
    Univ Helsinki, Childrens Hosp, Dept Pediat Surg, Helsinki, Finland.
    Mikkelsen, Audun
    Oslo Univ Hosp, Rikshosp, Dept Gastr & Pediat Surg, Oslo, Norway;Oslo Univ Hosp, Ulleval, Dept Gastr & Pediat Surg, Oslo, Norway.
    Emblem, Ragnhild
    Oslo Univ Hosp, Rikshosp, Dept Gastr & Pediat Surg, Oslo, Norway;Oslo Univ Hosp, Ulleval, Dept Gastr & Pediat Surg, Oslo, Norway.
    Svensson, Jan F.
    Karolinska Univ Hosp, Dept Pediat Surg, Stockholm, Sweden;Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm, Sweden.
    Ehren, Henrik
    Karolinska Univ Hosp, Dept Pediat Surg, Stockholm, Sweden;Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm, Sweden.
    Jonsson, Linus
    Queen Silvia Childrens Hosp, Dept Pediat Surg, Gothenburg, Sweden.
    Backstrand, Jakob
    Queen Silvia Childrens Hosp, Dept Pediat Surg, Gothenburg, Sweden.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Donoso, Felipe
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Thorup, Jorgen Mogens
    Copenhagen Univ Hosp, Rigshosp, Dept Pediat Surg, Copenhagen, Denmark.
    Saeter, Thorstein
    Trondhe Univ Hosp, St Olavs Hosp, Dept Pediat Surg, Trondheim, Norway.
    Rasmussen, Lars
    Odense Univ Hosp, Surg Dept A, Sdr Blvd 29, DK-5000 Odense C, Denmark.
    Pedersen, Rikke Neess
    Odense Univ Hosp, Hans Christian Andersen Childrens Hosp, Odense, Denmark.
    Stenstrom, Pernilla
    Lund Univ, Childrens Hosp, Dept Pediat, Lund, Sweden.
    Arnbjornsson, Einar
    Lund Univ, Childrens Hosp, Dept Pediat, Lund, Sweden.
    Oskarsson, Kristjan
    Univ Hosp, Childrens Hosp, Reykjavik, Iceland.
    Qvist, Niels
    Odense Univ Hosp, Surg Dept A, Sdr Blvd 29, DK-5000 Odense C, Denmark;Odense Univ Hosp, Hans Christian Andersen Childrens Hosp, Odense, Denmark;Odense Univ Hosp, Odense Patient Data Explorat Network, OPEN, Odense, Denmark.
    Surgical repair of long-gap esophageal atresia: A retrospective study comparing the management of long-gap esophageal atresia in the Nordic countries2019In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 54, no 3, p. 423-428Article in journal (Refereed)
    Abstract [en]

    Background:

    Several surgical procedures have been described in the reconstruction of long-gap esophageal atresia (LGEA). We reviewed the surgical methods used in children with LGEA in the Nordic countries over a 15-year period and the postoperative complications within the first postoperative year.

    Methods:

    Retrospective multicenter medical record review of all children born with Gross type A or B esophageal atresia between 01/01/2000 and 12/31/2014 reconstructed within their first year of life.

    Results:

    We included 71 children; 56 had Gross type A and 15 type B LGEA. Delayed primary anastomosis (DPA) was performed in 52.1% and an esophageal replacement procedure in 47.9%. Gastric pull-up (GPU) was the most frequent procedure (25.4%). The frequency of chromosomal abnormalities, congenital heart defects and other anomalies was significantly higher in patients who had a replacement procedure. The frequency of gastroesophageal reflux (GER) was significantly higher after DPA compared to esophageal replacement (p = 0.013). At 1-year follow-up the mean body weight was higher after DPA than after organ interposition (p = 0.043).

    Conclusion:

    DPA and esophageal replacement procedures were equally applied. Postoperative complications and follow-up were similar except for the development of GER and the body weight at 1-year follow-up. Long-term results should be investigated.

    Type of study:

    Treatment study.

  • 24.
    Teramura, Yuji
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology. Department of Bioengineering, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8656, Japan.
    Asif, Sana
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Nilsson Ekdahl, Kristina
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology. Linnæus Center of Biomaterials Chemistry, Linnæus University, SE-391 82 Kalmar, Sweden.
    Gustafson, Elisabet K.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Nilsson, Bo
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Cell Adhesion Induced Using Surface Modification with Cell-Penetrating Peptide-Conjugated Poly(ethylene glycol)-Lipid: A New Cell Glue for 3D Cell-Based Structures2017In: ACS Applied Materials and Interfaces, ISSN 1944-8244, E-ISSN 1944-8252, Vol. 9, no 1, p. 244-254Article in journal (Refereed)
    Abstract [en]

    We synthesized a novel material, cell-penetrating peptide-conjugated poly(ethylene glycol)-lipid (CPP-PEG-lipid), that can induce the adhesion of floating cells. Firm cell adhesion with spreading could be induced by cell surface modification with the CPP-PEG-lipids. Cell adhesion was induced by CPPs but not by any other cationic short peptides we tested. Here, we demonstrated adherence using the floating cell line CCRF-CEM as well as primary human T cells, B cells, erythrocytes, and hepatocytes. As compared to cells grown in suspension, adherent cells were more rapidly induced to attach to substrates with the cell-surface modification. The critical factor for attachment was localization of CPPs at the cell membrane by PEG-lipids with PEG > 20 kDa. These cationic CPPs on PEG chains were able to interact with substrate surfaces such as polystyrene (PS) surfaces, glass surfaces, and PS microfibers that are negatively charged, inducing firm cell adhesion and cell spreading. Also, as opposed to normal cationic peptides that interact strongly with cell membranes, CPPs were less interactive with the cell surfaces because of their cell-penetrating property, making them more available for adhering cells to the substrate surface. No effects on cell viability or cell proliferation were observed after the induction of cell adhesion. With this technique, cells could be easily immobilized onto PS microfibers, an important step in fabricating 3D cell-based structures. Cells immobilized onto 3D PS microfibers were alive, and human hepatocytes showed normal production of urea and albumin on the microfibers. This method is novel in inducing firm cell adhesion via a one-step treatment.

  • 25.
    Visuri, Sofia
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Univ Helsinki, Helsinki, Finland; Helsinki Univ Hosp, Dept Pediat Surg, Helsinki, Finland.
    Jahnukainen, Timo
    Univ Helsinki, Helsinki, Finland;Helsinki Univ Hosp, Dept Pediat Nephrol & Transplantat, Helsinki, Finland.
    Kivisaari, Reetta
    Helsinki Univ Hosp, Dept Pediat Radiol, Helsinki, Finland.
    Taskinen, Seppo
    Helsinki Univ Hosp, Dept Pediat Surg, Helsinki, Finland.
    Reduced differential renal function in scintigraphy predicted high-grade vesicoureteral reflux in children with antenatal hydronephrosiss2019In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 108, no 4, p. 751-756Article in journal (Refereed)
    Abstract [en]

    Aim: A top-down approach is widely used for detecting vesicoureteral reflux (VUR) in children with febrile urinary tract infections. We evaluated the diagnostic value of renal scintigraphy in predicting VUR in children with antenatal hydronephrosis (AHN).

    Methods: The voiding cystourethrogram (VCUG) and renal scintigraphy results of 125 AHN patients (76% male) admitted to the Children's Hospital of Helsinki University, Finland, from 2003 to 2013 were analysed. Of those, 94 had nonrefluxing hydronephrosis, nine had low-grade VUR and 22 had high-grade VUR.

    Results: Scintigraphy was performed at a median age of 1.4 (0.8-15.6) months. In patients with high-grade VUR, the differential renal function (DRF) of the worse kidney was significantly lower than in patients without VUR, with a median of 35% and interquartile range (IQR) of 20-45 versus 47% (IQR: 44-49), (p < 0.001). There was no difference between patients with low-grade VUR and patients without VUR (p = 0.181). DRFs below 44% showed a sensitivity of 73% and specificity of 79% and predicted significantly high-grade VUR (odds ratio 9.82, 95% confidence interval 3.44-28.05, p < 0.001) in the univariate analysis.

    Conclusion: A DRF below 44% predicted high-grade VUR in patients with AHN and supported the decision to perform VCUG.

  • 26.
    Visuri, Sofia
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Helsinki Univ Hosp, Dept Pediat Surg, Helsinki, Finland.
    Kivisaari, Reetta
    Helsinki Univ Hosp, Dept Pediat Radiol, Helsinki, Finland.
    Jahnukainen, Timo
    Helsinki Univ Hosp, Dept Pediat Nephrol & Transplantat, Helsinki, Finland;Univ Helsinki, Helsinki, Finland.
    Taskinen, Seppo
    Helsinki Univ Hosp, Dept Pediat Surg, Helsinki, Finland.
    Postnatal imaging of prenatally detected hydronephrosis - when is voiding cystourethrogram necessary?2018In: Pediatric nephrology (Berlin, West), ISSN 0931-041X, E-ISSN 1432-198X, Vol. 33, no 10, p. 1751-1757Article in journal (Refereed)
    Abstract [en]

    Objective: To evaluate whether grade 4-5 vesicoureteral reflux (VUR) can be predicted from renal ultrasound (RUS) findings and perform voiding cystourethrograms (VCUGs) only on high-risk patients.

    Methods: The RUS and VCUG images of infants with prenatally detected hydronephrosis admitted to our institution between 2003 and 2013 were re-evaluated. The UTI episodes were collected retrospectively from patient journals. Patients with complex urinary tract anomalies were excluded.

    Results: One hundred eighty, 44 female and 136 male, patients (352 renal units (RU)), 23 (30 RU) of them having grade 4-5 VUR, were included. The median age of the patients at the time of the RUS was 1.3 (0.1-3.0) months and the median follow-up time was 2.0 (0.1-11.2) years. In multivariate analysis, a visible ureter (OR 12.72; CI 5.33-32.04, p < 0.001) and shorter renal length (OR 2.67; CR 1.504.86, p < 0.001) in RUS predicted grade 4-5 VUR while a visible ureter predicted UTIs (OR 5.75; CI 2.59-12.66, p < 0.001). A three-grade risk score for high-grade VUR was developed based on the RUS findings and the patients were categorized into low-, intermediate-, and high-risk groups. The incidence of grade 4-5 VUR was 2.9% in the low-risk, 12.2% in the intermediaterisk, and 52.2% in the high-risk group. The sensitivity and specificity for detecting grade 4-5 VUR were 79 and 82%, respectively.

    Conclusions: In patients with antenatally detected hydronephrosis, a visible ureter and reduced renal length in RUS are significant risk factors for high-grade VUR. A RUS-based risk scoring would probably reduce the proportion of unnecessary VCUGs.

  • 27.
    Vivier, Pierre-Hugues
    et al.
    Hop Prive Estuaire, Radiol, Ramsay Gen Sante, Le Havre, France;Univ Hosp Charles Nicolle, Pediat Radiol, Rouen, France.
    Augdal, Thomas A.
    Univ Hosp North Norway, Pediat Radiol, Tromso, Norway..
    Avni, Fred E.
    Lille Univ Hosp, Jeanne de Flandre Hosp, Pediat Radiol, Lille, France..
    Bacchetta, Justine
    Hop Femme Mere Enfant, Pediat Nephrol, Bron, France..
    Beetz, Rolf
    Univ Med Clin, Ctr Paediat & Adolescent Med, Pediat Nephrol, Mainz, Germany..
    Bjerre, Anna K.
    Natl Hosp Norway, Oslo Univ Hosp, Pediat Nephrol, Oslo, Norway..
    Blickman, Johan
    Golisano Childrens Hosp, Pediat Radiol, Rochester, NY USA..
    Cochat, Pierre
    Hop Femme Mere Enfant, Pediat Nephrol, Bron, France..
    Coppo, Rosana
    Regina Margherita Hosp, Pediat Nephrol, Turin, Italy..
    Damasio, Beatrice
    Ist Giannina Gaslini, Pediat Radiol, Genoa, Italy..
    Darge, Kassa
    Univ Penn, Perelman Sch Med, Childrens Hosp Philadelphia, Pediat Radiol, Philadelphia, PA 19104 USA..
    El-Ghoneimi, Alaa
    Univ Paris Diderot, Univ Hosp Robert Debre, AP HP, Pediat Surg & Urol, Paris, France..
    Hoebeke, Piet
    Ghent Univ Hosp, Urol, Ghent, Belgium..
    Läckgren, Göran
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Leclair, Marc-David
    Children Univ Hosp, Pediat Surg & Urol, Nantes, France..
    Lobo, Maria-Luisa
    Univ Hosp, Hosp Santa Maria, Radiol, Lisbon, Portugal..
    Manzoni, Gianantonio
    Osped Maggiore Policlin, Fdn IRCCS Ca Granda, Pediat Urol, Milan, Italy..
    Marks, Stephen D.
    Great Ormond St Hosp Children NHS Fdn Trust, Paediat Nephrol, London, England..
    Mattioli, Girolamo
    Dinogmi Univ Genova, Pediat Surg & Urol, Gaslini Inst, Genoa, Italy..
    Mentzel, Hans-Joachim
    Univ Hosp Jena, Pediat Radiol, Diagnost & Intervent Radiol, Jena, Germany..
    Mouriquand, Pierre
    Hosp Civils Lyon, Hop Mere Enfant, Pediat Urol, Lyon 1, France.;Claude Bernard Univ, Lyon 1, France..
    Nevéus, Tryggve
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Paediatric Inflammation Research.
    Ntoulia, Aikaterini
    Univ Penn, Perelman Sch Med, Childrens Hosp Philadelphia, Pediat Radiol, Philadelphia, PA 19104 USA.;Kings Coll Hosp London, Paediat Radiol, London, England..
    Ording-Muller, Lil-Sofie
    Oslo Univ Hosp, Paediat Radiol, Oslo, Norway..
    Oswald, Josef
    Hosp Sisters Char, Pediat Urol, Linz, Austria..
    Papadopoulou, Frederica
    Ioannina Univ, Radiol, Ioannina, Greece..
    Porcellini, Gabriella
    Regina Margherita Hosp, Pediat Nephrol, Turin, Italy..
    Ring, Ekkehard
    Univ Hosp LKH Graz, Dept Pediat, Graz, Austria..
    Rösch, Wolfgang
    Univ Med Ctr Regensburg, Pediat Urol, Regensburg, Germany..
    Teixeira, Ana F.
    Ctr Hosp Sao Joao, Pediat Nephrol, Oporto, Portugal..
    Riccabona, Michael
    Univ Hosp LKH Graz, Pediat Radiol, Graz, Austria..
    Standardization of pediatric uroradiological terms: A multidisciplinary European glossary2017In: Journal of Pediatric Urology, ISSN 1477-5131, E-ISSN 1873-4898, Vol. 13, no 6, p. 641-650Article in journal (Other academic)
    Abstract [en]

    To promote the standardization of nephro-uroradiological terms used in children, the European Society of Pediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication among different clinicians involved in pediatric urology and nephrology.

  • 28.
    Vivier, Pierre-Hugues
    et al.
    Hop Prive Estuaire, Serv Radiol, Ramsay Gen Sante, Radiol, 505 Rue Irene Joliot Curie, F-76620 Le Havre, France.;Univ Hosp Charles Nicolle, Pediat Radiol, Rouen, France..
    Augdal, Thomas A.
    Univ Hosp North Norway, Pediat Radiol, Tromso, Norway..
    Avni, Fred E.
    Lille Univ Hosp, Jeanne de Flandre Hosp, Pediat Radiol, Lille, France..
    Bacchetta, Justine
    Hop Femme Mere Enfant, Pediat Nephrol, Bron, France..
    Beetz, Rolf
    Univ Med Clin, Ctr Paediat & Adolescent Med, Pediat Nephrol, Mainz, Germany..
    Bjerre, Anna K.
    Natl Hosp Norway, Oslo Univ Hosp, Pediat Nephrol, Oslo, Norway..
    Blickman, Johan
    Golisano Childrens Hosp, Pediat Radiol, Rochester, NY USA..
    Cochat, Pierre
    Hop Femme Mere Enfant, Pediat Nephrol, Bron, France..
    Coppo, Rosana
    Regina Margherita Hosp, Pediat Nephrol, Turin, Italy..
    Damasio, Beatrice
    Ist Giannina Gaslini, Pediat Radiol, Genoa, Italy..
    Darge, Kassa
    Univ Penn, Childrens Hosp Philadelphia, Perelman Sch Med, Pediat Radiol, Philadelphia, PA 19104 USA..
    El-Ghoneimi, Alaa
    Univ Paris Diderot, Univ Hosp Robert Debre, APHP, Pediat Surg & Urol, Paris, France..
    Hoebeke, Piet
    Ghent Univ Hosp, Urol, Ghent, Belgium..
    Läckgren, Göran
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Leclair, Marc-David
    Children Univ Hosp, Pediat Surg & Urol, Nantes, France..
    Lobo, Maria-Luisa
    Univ Hosp, Hosp Santa Maria, Radiol, Lisbon, Portugal..
    Manzoni, Gianantonio
    Osped Maggiore Policlin, Fdn IRCCS Ca Granda, Pediat Urol, Milan, Italy..
    Marks, Stephen D.
    Great Ormond St Hosp Children NHS Fdn Trust, Paediat Nephrol, London, England..
    Mattioli, Girolamo
    Dinogmi Univ Genova, Gaslini Inst, Pediat Surg & Urol, Genoa, Italy..
    Mentzel, Hans-Joachim
    Univ Hosp Jena, Pediat Radiol Diagnost & Intervent Radiol, Jena, Germany..
    Mouriquand, Pierre
    Hosp Civils Lyon, Hop Mere Enfant, Pediat Urol, Lyon 1, France.;Claude Bernard Univ, Lyon 1, France..
    Nevéus, Tryggve
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Paediatric Inflammation Research.
    Ntoulia, Aikaterini
    Univ Penn, Childrens Hosp Philadelphia, Perelman Sch Med, Pediat Radiol, Philadelphia, PA 19104 USA.;Kings Coll Hosp London, Paediat Radiol, London, England..
    Ording-Muller, Lil-Sofie
    Oslo Univ Hosp, Paediat Radiol, Oslo, Norway..
    Oswald, Josef
    Hosp Sisters Char, Pediat Urol, Linz, Austria..
    Papadopoulou, Frederica
    Ioannina Univ, Radiol, Ioannina, Greece..
    Porcellini, Gabriella
    Regina Margherita Hosp, Pediat Nephrol, Turin, Italy..
    Ring, Ekkehard
    Univ Hosp LKH Graz, Dept Pediat, Graz, Austria..
    Rösch, Wolfgang
    Univ Med Ctr Regensburg, Pediat Urol, Regensburg, Germany..
    Teixeira, Ana F.
    Ctr Hosp Sao Joao, Pediat Nephrol, Oporto, Portugal..
    Riccabona, Michael
    Univ Hosp LKH Graz, Pediat Radiol, Graz, Austria..
    Standardization of pediatric uroradiological terms: a multidisciplinary European glossary2018In: Pediatric Radiology, ISSN 0301-0449, E-ISSN 1432-1998, Vol. 48, no 2, p. 291-303Article in journal (Refereed)
    Abstract [en]

    To promote the standardization of nephro-uroradiological terms used in children, the European Society of Paediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication between different clinicians involved in pediatric urology and nephrology.

  • 29.
    Zachar, Rikke
    et al.
    Univ Southern Denmark, Inst Mol Med, Dept Cardiovasc & Renal Res, JB Winslosvej 21,3, DK-5000 Odense, Denmark.
    Al-Masbhadi, Ammar
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Dimke, Henrik
    Univ Southern Denmark, Inst Mol Med, Dept Cardiovasc & Renal Res, JB Winslosvej 21,3, DK-5000 Odense, Denmark.
    Svenningsen, Per
    Univ Southern Denmark, Inst Mol Med, Dept Cardiovasc & Renal Res, JB Winslosvej 21,3, DK-5000 Odense, Denmark.
    Jensen, Boye L.
    Univ Southern Denmark, Inst Mol Med, Dept Cardiovasc & Renal Res, JB Winslosvej 21,3, DK-5000 Odense, Denmark.
    Carlström, Mattias
    Karolinska Inst, Dept Physiol & Pharmacol, Stockholm, Sweden.
    Hydronephrosis is associated with elevated plasmin in urine in pediatric patients and rats and changes in NCC and gamma-ENaC abundance in rat kidney2018In: American Journal of Physiology - Renal Physiology, ISSN 1931-857X, E-ISSN 1522-1466, Vol. 315, no 3, p. F547-F557Article in journal (Refereed)
    Abstract [en]

    Obstruction of urine flow at the level of the pelvo-ureteric junction (UPJO) and subsequent development of hydronephrosis is one of the most common congenital renal malformations. UPJO is associated with development of salt-sensitive hypertension, which is set by the obstructed kidney, and with a stimulated renin-angiotensin-aldosterone system (RAAS) in rodent models. This study aimed at investigating the hypothesis that 1) in pediatric patients with UPJO the RAAS is activated before surgical relief of the obstruction; 2) in rats with UPJO the RAAS activation is reflected by increased abundance of renal aldosterone-stimulated Na transporters; and 3) the injured UPJO kidney allows aberrant filtration of plasminogen, leading to proteolytic activation of the epithelial Na channel gamma-subunit (gamma-ENaC). Hydronephrosis resulting from UPJO in pediatric patients and rats was associated with increased urinary plasminogen-to-creatinine ratio. In pediatric patients, plasma renin, angiotensin II, urine and plasma aldosterone, and urine soluble prorenin receptor did not differ significantly before or after surgery, or compared with controls. Increased plasmin-to-plasminogen ratio was seen in UPJO rats. Intact gamma-ENaC abundance was not changed in UPJO kidney, whereas low-molecular cleavage product abundance increased. The Na-Cl cotransporter displayed significantly lower abundance in the UPJO kidney compared with the nonobstructed contralateral kidney. The Na-K-ATPase alpha-subunit was unaltered. Treatment with an angiotensin-converting enzyme inhibitor (8 days, captopril) significantly lowered blood pressure in UPJO rats. It is concluded that the RAAS contributes to hypertension following partial obstruction of urine flow at the pelvo-ureteric junction with potential contribution from proteolytic activation of ENaC.

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