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Engstrand Lilja, Helene
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Publications (10 of 28) Show all publications
Kassa, A.-M., Engstrand Lilja, H. & Engvall, G. (2019). From crisis to self-confidence and adaptation; Experiences of being a parent of a child with VACTERL association: A complex congenital malformation. PLoS ONE, 14(4), Article ID e0215751.
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
Hambraeus, M., Al-Mashhadi, A., Wester, T., Svensson, P.-J., Stenstrom, P. & Engstrand Lilja, H. (2019). Functional outcome and health-related quality of life in patients with sacrococcygeal teratoma - a Swedish multicenter study. Journal of Pediatric Surgery, 54(8), 1638-1643
Open this publication in new window or tab >>Functional outcome and health-related quality of life in patients with sacrococcygeal teratoma - a Swedish multicenter study
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2019 (English)In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 54, no 8, p. 1638-1643Article in journal (Refereed) Published
Abstract [en]

Background/Purpose: The aims of this study were to evaluate bowel and urinary tract function, to identify predictors for dysfunctional outcome and to evaluate health-related quality-of life (QoL) in patients treated for sacrococcygeal teratomas (SCT). Methods: Medical records of patients with SCT born between 1985 and 2015 treated at three Swedish pediatric surgical centers were reviewed. Questionnaires regarding urinary tract function, bowel function and QoL were sent to patients and parents. Different QoL instruments were used for the different age groups. Results: Totally 85 patients were identified. Four patients died in the neonatal period. Forty-nine patients answered the questionnaires (60%). Median age at follow-up was 8.9 years (range 3.6-28.8). Bowel dysfunction was reported by 36% and urinary tract dysfunction by 46% of the patients. Univariate analysis revealed that urinary tract dysfunction correlated with gestational age (p = 0.018) and immature histology (p = 0.008), and bowel dysfunction correlated with gestational age (p = 0.016) and tumor size (p = 0.042). Low gestational age was an independent predictor for both urinary tract and bowel dysfunction. Good or very good QoL was reported by 56% of children aged 4-7 years, 90% of children aged 8-17 years and 67% of the adults. Conclusion: Although a considerable proportion of bowel and urinary tract dysfunction was found, the reported QoL was good in a majority of the patients with SCT. Low gestational age was found to be a predictor for bowel-and urinary tract dysfunction. (C) 2018 Elsevier Inc. All rights reserved.

Place, publisher, year, edition, pages
W B SAUNDERS CO-ELSEVIER INC, 2019
Keywords
Sacrococcygeal teratoma, Outcome, Quality of life
National Category
Pediatrics
Identifiers
urn:nbn:se:uu:diva-393744 (URN)10.1016/j.jpedsurg.2018.10.044 (DOI)000481622100022 ()30420172 (PubMedID)
Available from: 2019-11-11 Created: 2019-11-11 Last updated: 2019-11-11Bibliographically approved
Al-Mashhadi, A. N., Dukic, M. & Engstrand Lilja, H. (2019). Rhabdomyomatous mesenchymal hamartoma presenting in a child as a perineal mass. Journal of Pediatric Surgery Case Reports, 47, Article ID 101242.
Open this publication in new window or tab >>Rhabdomyomatous mesenchymal hamartoma presenting in a child as a perineal mass
2019 (English)In: Journal of Pediatric Surgery Case Reports, ISSN 0022-3476, E-ISSN 2213-5766, Vol. 47, article id 101242Article in journal (Refereed) Published
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.

Place, publisher, year, edition, pages
ELSEVIER, 2019
Keywords
Rhabdomyomatous, Mesenchymal, Hamartoma, Perianal
National Category
Pediatrics
Identifiers
urn:nbn:se:uu:diva-391282 (URN)10.1016/j.epsc.2019.101242 (DOI)000477705900021 ()
Available from: 2019-08-22 Created: 2019-08-22 Last updated: 2019-11-11Bibliographically approved
Stadil, T., Koivusalo, A., Pakarinen, M., Mikkelsen, A., Emblem, R., Svensson, J. F., . . . Qvist, N. (2019). Surgical repair of long-gap esophageal atresia: A retrospective study comparing the management of long-gap esophageal atresia in the Nordic countries. Journal of Pediatric Surgery, 54(3), 423-428
Open this publication in new window or tab >>Surgical repair of long-gap esophageal atresia: A retrospective study comparing the management of long-gap esophageal atresia in the Nordic countries
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2019 (English)In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 54, no 3, p. 423-428Article in journal (Refereed) Published
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.

Keywords
Esophageal atresia, Long gap, Gross type A, Gross type B, Surgical repair, Postoperative complication
National Category
Surgery Pediatrics
Identifiers
urn:nbn:se:uu:diva-379033 (URN)10.1016/j.jpedsurg.2018.07.023 (DOI)000458782300009 ()30220451 (PubMedID)
Available from: 2019-03-11 Created: 2019-03-11 Last updated: 2019-03-11Bibliographically approved
Fredriksson, F. & Engstrand Lilja, H. (2019). Survival rates for surgically treated necrotising enterocolitis have improved over the last four decades. Acta Paediatrica, 108(9), 1603-1608
Open this publication in new window or tab >>Survival rates for surgically treated necrotising enterocolitis have improved over the last four decades
2019 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 108, no 9, p. 1603-1608Article in journal (Refereed) Published
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.

Place, publisher, year, edition, pages
WILEY, 2019
Keywords
Intestinal failure, Mortality, Necrotising enterocolitis, Short bowel syndrome, Surgery
National Category
Pediatrics
Identifiers
urn:nbn:se:uu:diva-393650 (URN)10.1111/apa.14770 (DOI)000479320100010 ()30825252 (PubMedID)
Available from: 2019-09-25 Created: 2019-09-25 Last updated: 2019-09-25Bibliographically approved
Kassa, A.-M., Dahl, M., Strinnholm, M. & Engstrand Lilja, H. (2018). Attention difficulties and physical dysfunction common in children with complex congenital malformations:: a study of preschool children with VACTERL association.. Acta Paediatrica
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
Markasz, L., Wanders, A., Szekely, L. & Engstrand Lilja, H. (2018). Diminished DEFA6 Expression in Paneth Cells Is Associated with Necrotizing Enterocolitis. Gastroenterology Research and Practice, Article ID 7345426.
Open this publication in new window or tab >>Diminished DEFA6 Expression in Paneth Cells Is Associated with Necrotizing Enterocolitis
2018 (English)In: Gastroenterology Research and Practice, ISSN 1687-6121, E-ISSN 1687-630X, article id 7345426Article in journal (Refereed) Published
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.

Place, publisher, year, edition, pages
Hindawi Publishing Corporation, 2018
National Category
Gastroenterology and Hepatology
Identifiers
urn:nbn:se:uu:diva-369436 (URN)10.1155/2018/7345426 (DOI)000449320500001 ()30420878 (PubMedID)
Available from: 2018-12-14 Created: 2018-12-14 Last updated: 2019-01-22Bibliographically approved
Wester, T., Engstrand Lilja, H., Stenström, P. & Pakarinen, M. (2017). Absent ileocecal valve predicts the need for repeated STEP in children. Surgery, 161(3), 818-822
Open this publication in new window or tab >>Absent ileocecal valve predicts the need for repeated STEP in children
2017 (English)In: Surgery, ISSN 0039-6060, E-ISSN 1532-7361, Vol. 161, no 3, p. 818-822Article in journal (Refereed) Published
Abstract [en]

Background. Serial transverse enteroplasty facilitates weaning from parenteral support in selected patients with short bowel syndrome, although repeated procedure is frequently required. Our aim was to evaluate the outcome of a series of patients after serial transverse enteroplasty and define predictors of repeated serial transverse enteroplasty and weaning off parenteral support. Methods. All children who underwent serial transverse enteroplasty at 4 Nordic pediatric surgery centers from 2004-2015 were included in this observational study. Data were collected from the patient records. The study was approved by the local ethics review boards. Results. Twenty-seven children with short bowel with initial median small bowel length of 26 cm (range, 10-100 cm) were included. Eleven patients had the ileocecal valve remaining. Serial transverse enteroplasty was performed at median age of 7.5 months (range, 0.9-224 months). Serial transverse enteroplasty made the small bowel 46% (0-233%) longer. Eleven patients (41 %) underwent a repeated serial transverse enteroplasty 12 months (1.0-72 months) later; 7 patients required additional operative procedures, but none were transplanted. At follow-up, 45.1 months (1.8-126 months) after the first serial transverse enteroplasty, 11 (41 %) patients needed parenteral support. The remaining 16 patients had been weaned off parenteral support. One patient had died. Absence of the ileocecal valve was the only factor, which predicted the need for a repeated serial transverse enteroplasty (odds ratio 16.7, 95 % confidence interval, 1.7-164.8, P =.007). No factor was identified predicting need for parenteral support at follow-up. Conclusion. A majority of children with short bowel syndrome can be weaned from parenteral support after serial transverse enteroplasty. The absence of the ileocecal valve predicts the need for a repeated serial transverse enteroplasty, which was required by 40% of the patients.

National Category
Surgery
Identifiers
urn:nbn:se:uu:diva-304714 (URN)10.1016/j.surg.2016.09.011 (DOI)000394729100035 ()27816208 (PubMedID)
Available from: 2016-10-07 Created: 2016-10-07 Last updated: 2017-04-20Bibliographically approved
Pammi, M., Cope, J., Tarr, P. I., Warner, B. B., Morrow, A. L., Mai, V., . . . Neu, J. (2017). Intestinal dysbiosis in preterm infants preceding necrotizing enterocolitis: a systematic review and meta-analysis. Microbiome, 5, Article ID 31.
Open this publication in new window or tab >>Intestinal dysbiosis in preterm infants preceding necrotizing enterocolitis: a systematic review and meta-analysis
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2017 (English)In: Microbiome, ISSN 0026-2633, E-ISSN 2049-2618, Vol. 5, article id 31Article, review/survey (Refereed) Published
Abstract [en]

Background: Necrotizing enterocolitis (NEC) is a catastrophic disease of preterm infants, and microbial dysbiosis has been implicated in its pathogenesis. Studies evaluating the microbiome in NEC and preterm infants lack power and have reported inconsistent results. Methods and results: Our objectives were to perform a systematic review and meta-analyses of stool microbiome profiles in preterm infants to discern and describe microbial dysbiosis prior to the onset of NEC and to explore heterogeneity among studies. We searched MEDLINE, PubMed, CINAHL, and conference abstracts from the proceedings of Pediatric Academic Societies and reference lists of relevant identified articles in April 2016. Studies comparing the intestinal microbiome in preterm infants who developed NEC to those of controls, using cultureindependent molecular techniques and reported a and beta-diversity metrics, and microbial profiles were included. In addition, 16S ribosomal ribonucleic acid (rRNA) sequence data with clinical meta-data were requested from the authors of included studies or searched in public data repositories. We reprocessed the 16S rRNA sequence data through a uniform analysis pipeline, which were then synthesized by meta-analysis. We included 14 studies in this review, and data from eight studies were available for quantitative synthesis (106 NEC cases, 278 controls, 2944 samples). The age of NEC onset was at a mean +/- SD of 30.1 +/- 2.4 weeks post-conception (n = 61). Fecal microbiome from preterm infants with NEC had increased relative abundances of Proteobacteria and decreased relative abundances of Firmicutes and Bacteroidetes prior to NEC onset. Alpha-or beta-diversity indices in preterm infants with NEC were not consistently different from controls, but we found differences in taxonomic profiles related to antibiotic exposure, formula feeding, and mode of delivery. Exploring heterogeneity revealed differences in microbial profiles by study and the target region of the 16S rRNA gene (V1-V3 or V3-V5). Conclusions: Microbial dysbiosis preceding NEC in preterm infants is characterized by increased relative abundances of Proteobacteria and decreased relative abundances of Firmicutes and Bacteroidetes. Microbiome optimization may provide a novel strategy for preventing NEC.

Place, publisher, year, edition, pages
BIOMED CENTRAL LTD, 2017
Keywords
Microbiome, Intestinal, Preterm, Neonate, NEC, 16S rRNA sequencing
National Category
Pediatrics Microbiology in the medical area
Identifiers
urn:nbn:se:uu:diva-320962 (URN)10.1186/s40168-017-0248-8 (DOI)000397308600001 ()28274256 (PubMedID)
Funder
NIH (National Institute of Health), UH3AI083265 R01 HD059140
Available from: 2017-04-28 Created: 2017-04-28 Last updated: 2018-01-13Bibliographically approved
Stenström, P., Brautigam, M., Borg, H., Graneli, C., Engstrand Lilja, H. & Wester, T. (2017). Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis.. Journal of Pediatric Surgery, 52(8), 1302-1307
Open this publication in new window or tab >>Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis.
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2017 (English)In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 52, no 8, p. 1302-1307Article in journal (Refereed) Published
Abstract [en]

BACKGROUND: The aim of this study was to evaluate the nationwide outcome of children with total colonic aganglionosis (TCA) during the last 20years.

METHODS: This was an observational, cross-sectional study where all patients with TCA, including aganglionosis of 0-50cm of ileum, born in Sweden 1995-2014 were included. Data were collected from the medical records. Patients >4years old without stoma answered a questionnaire regarding bowel function (bowel function score, BFS, score 1-20), medical treatment and nutrition.

RESULTS: Twenty-seven children were included. Twenty-five children were reconstructed at median age of 56 (4-236) weeks. Reconstruction procedures included Swenson (6), Soave (5), mucosectomy with short muscular cuff with or without J-pouch (9), Duhamel (3) and Rehbein (2). There was no mortality. The median follow-up time was 9.5years (8months-20years). At follow-up 7 (26%) patients had an ileostomy, 4 with a syndrome. Eight patients required parenteral support, until a median age of 11 (2-24) months. Oral energy support was used by 5/27 (15%), still 5/22 (23%) were underweighted. Obstructive symptoms were reported by 7/20 (31%). All 17 patients >4years old completed the BFS questionnaire at median age of 10 (4-20) years. Median stool frequency/24h was 5 (1-30). Fecal accidents at least once per week was reported by 4 (24%), and social problems by 8 (47%). The median BFS was 15 (11-19) without any gender differences.

CONCLUSION: One-third of patients with TCA report obstructive symptoms, one-third need additional nutrition and one-fifth require a permanent stoma. TCA have a negative impact on social life. Subsequently, children with TCA need a careful lifelong follow-up of specialized teams.

Keywords
Bowel function, Hirschsprung disease, Nutrition, Total colonic aganglionosis
National Category
Pediatrics
Identifiers
urn:nbn:se:uu:diva-319274 (URN)10.1016/j.jpedsurg.2016.11.033 (DOI)000406421300015 ()27912975 (PubMedID)
Available from: 2017-04-03 Created: 2017-04-03 Last updated: 2017-11-06Bibliographically approved
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