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1.
BMC Pediatr ; 23(1): 336, 2023 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-37393233

RESUMO

BACKGROUND: Impaired intestinal microcirculation seems to play an important role in the pathogenesis of necrotizing enterocolitis (NEC). A previous study showed that a SrSO2 < 30% is associated with an increased risk of developing of NEC. We aimed to determine the clinical usefulness of the cut off < 30% for SrSO2 in predicting NEC in extremely preterm neonates. METHODS: This is a combined cohort observational study. We added a second cohort from another university hospital to the previous cohort of extremely preterm infants. SrSO2 was measured for 1-2 h at days 2-6 after birth. To determine clinical usefulness we assessed sensitivity, specificity, positive and negative predictive values for mean SrSO2 < 30. Odds ratio to develop NEC was assessed with generalized linear model analysis, adjusting for center. RESULTS: We included 86 extremely preterm infants, median gestational age 26.3 weeks (range 23.0-27.9). Seventeen infants developed NEC. A mean SrSO2 < 30% was found in 70.5% of infants who developed NEC compared to 33.3% of those who did not (p = 0.01). Positive and negative predictive values were 0.33 CI (0.24-0.44) and 0.90 CI (0.83-0.96), respectively. The odds of developing NEC were 4.5 (95% CI 1.4-14.3) times higher in infants with SrSO2 < 30% compared to those with SrSO2 ≥ 30%. CONCLUSIONS: A mean SrSO2 cut off ≥ 30% in extremely preterm infants between days 2-6 after birth may be useful in identifying infants who will not develop NEC.


Assuntos
Enterocolite Necrosante , Doenças Fetais , Doenças do Recém-Nascido , Lactente , Feminino , Recém-Nascido , Humanos , Lactente Extremamente Prematuro , Enterocolite Necrosante/diagnóstico , Estudos de Coortes , Idade Gestacional
2.
J Pediatr Surg ; 56(2): 239-244, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32829881

RESUMO

PURPOSE: Assessing quality of life (QoL) after esophageal replacement (ER) for long gap esophageal atresia (LGEA). METHODS: All patients after ER for LGEA with gastric pull-up (GPU n = 9) or jejunum interposition (JI n = 14) at the University Medical Center Groningen and Utrecht (1985-2007) were included. QoL was assessed with 1) gastrointestinal-related QoL using the Gastrointestinal Quality of Life Index (GIQLI)), 2) general QoL (Child Health questionnaire CHF87-BREF (children)/World Health Organization questionnaire WHOQOL-BREF (adults)), and 3) health-related QoL (HRQoL) (TNO AZL TACQoL/TAAQoL). Association of morbidity (heartburn, dysphagia, dyspnea on exertion, recurrent cough) and (HR)QoL was evaluated. RESULTS: Six patients after GPU (75%) and eight patients after JI (57%) responded to the questionnaires (mean age 15.7, SD 5.9, 12 male, two female). Mean gastrointestinal, general and health-related QoL total scores of the patients were comparable to healthy controls. However, young adults reported a worse physical functioning (p = 0.02) but better social functioning compared to peers (p = 0.01). Morbidity was not associated with significant differences in (HR)QoL. CONCLUSIONS: With the current validated QoL most patients after ER with GPU and JI for LGEA have normal generic and disease specific QoL scores. Postoperative morbidity does not seem to influence (HR)QoL. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: III.


Assuntos
Atresia Esofágica , Esofagoplastia , Adolescente , Anastomose Cirúrgica , Criança , Atresia Esofágica/cirurgia , Feminino , Humanos , Masculino , Qualidade de Vida , Inquéritos e Questionários , Adulto Jovem
3.
Semin Pediatr Surg ; 29(4): 150942, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32861446

RESUMO

Much about the aetiology, pathophysiology, natural course and optimal treatment of choledochal malformation remains under debate. Surgeons continuously strive to optimize their roles in the management of choledochal malformation. Nowadays the standard treatment is complete cyst excision followed by Roux-en-Y hepaticojejunostomy, be it via a laparotomy, laparoscopy or robot-assisted procedure. Whatever surgical endeavor is undertaken, it will be a major operation, with significant morbidity. It is important to realize that especially in asymptomatic cases, this is considered prophylactic surgery, aimed at preventing symptoms but even more important the development of malignancy later in life. A clear overview of long-term outcomes is therefore necessary. This paper aims to review the long-term outcomes after surgery for choledochal malformation. We will focus on biliary complications such as cholangitis, the development of malignancy and quality of life. We will try and identify factors related to a worse outcome. Finally, we make a plea for a large scale study into quality and course of life after resection of a choledochal malformation, to help patients, parents and their treating physicians to come to a well-balanced decision regarding the treatment of a choledochal malformation.


Assuntos
Procedimentos Cirúrgicos do Sistema Biliar , Colangiocarcinoma , Colangite , Cisto do Colédoco/cirurgia , Avaliação de Resultados em Cuidados de Saúde , Qualidade de Vida , Procedimentos Cirúrgicos do Sistema Biliar/efeitos adversos , Colangiocarcinoma/etiologia , Colangite/etiologia , Humanos
4.
Early Hum Dev ; 147: 105091, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32492527

RESUMO

BACKGROUND: Necrotizing enterocolitis (NEC) is associated with poorer neurodevelopment. It is, however, unclear which factors besides surgery affect neurodevelopment in preterm-born children surviving NEC. AIMS: We determined whether time to full enteral feeding (FEFt) and post-NEC complications after NEC were associated with neurodevelopment. STUDY DESIGN: Prospective observational cohort study. SUBJECTS: Two to three year old preterm-born children who survived NEC (Bells stage ≥ 2). We categorized children in two groups, one group shorter and equal and one group longer than the group's median FEFt. Post-NEC complications included recurrent NEC and/or post-NEC stricture. OUTCOME MEASURES: Bayley Scales of Infants and Toddler Development III (Bayley-III) and Child Behavior Checklist (CBCL). Associations between Bayley-III and CBCL scores with FEFt and Post-NEC complications were determined using linear regression analyses, adjusted for severity of illness and potential confounders. RESULTS: We included 44 children, median gestational age of 27.9 [IQR: 26.7-29.3] weeks, birth weight 1148 [IQR: 810-1461] grams. Median FEFt after NEC was 20 [IQR: 16-30] days. Median follow-up age was 25.7 [IQR: 24.8-33.5] months. FEFt > 20 days was associated with lower cognitive and lower motor composite scores of the Bayley-III (B: -8.6, 95% CI -16.7 to -0.4, and B: -9.0, 95% CI, -16.7 to -1.4). FEFt was not associated with CBCL scores. Post-NEC complications (n = 11) were not associated with Bayley-III scores nor with CBCL scores. CONCLUSIONS: Prolonged FEFt after NEC in preterm-born children surviving NEC is associated with lower cognitive and lower motor composite scores at the age of 2-3 years. These results show the importance of limiting the duration of the nil per mouth regimen if and when possible.


Assuntos
Deficiências do Desenvolvimento/epidemiologia , Nutrição Enteral , Enterocolite Necrosante/complicações , Recém-Nascido Prematuro/fisiologia , Desenvolvimento Infantil , Pré-Escolar , Enterocolite Necrosante/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro/crescimento & desenvolvimento , Masculino
5.
J Pediatr Surg ; 54(9): 1755-1760, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30635129

RESUMO

BACKGROUND: Necrotizing enterocolitis (NEC) predominantly occurs in preterm infants (PT-NEC). In term neonates, NEC occurs more frequently when a congenital heart disease is present (CHDNEC). Our aim was to evaluate differences and similarities in disease characteristics of PT-NEC versus CHD-NEC. METHODS: In this retrospective case-control study we identified all CHD infants who developed NEC Bell's stage ≥2 in our center from 2004 to 2014. We randomly selected (1:2 ratio) PT-NEC infants from the same period. Biochemical and clinical variables were retrieved from patient files. RESULTS: We found 18 CHD-NEC infants and selected 36 PT-NEC infants (gestational age 28.3 [25-35.6] weeks vs. 38.6 [31.7-40.7] weeks). Postnatal age at onset was significantly lower in CHD-NEC patients (4 [2-24] vs. 11 [4-41] days, p < 0.001). Lowest pH levels were lower (7.21 [7.01-7.47] vs. 7.27 [6.68-7.39], p = 0.02), and highest CRP levels were higher (112.5 mg/L [5.0-425.0] vs. 66.0 [5.2-189.0], p = 0.05) in PT-NEC vs. CHD-NEC. Anatomic localisation of the disease differed: the colon was significantly more often involved in CHD-NEC versus PT-NEC (86% vs. 33%, p = 0.03). Mortality caused by NEC was not different (22% vs. 11%, p = 0.47). CONCLUSION: While outcome of NEC in both groups is similar, the predominant NEC localisation differed between CHD-NEC and PT-NEC patients. This suggests that both variants of the disease have a different underlying pathophysiological mechanism that predisposes different intestinal regions to develop NEC. TYPE OF STUDY: Retrospective Case-Control Study. LEVEL OF EVIDENCE: Level III.


Assuntos
Enterocolite Necrosante , Cardiopatias Congênitas , Doenças do Recém-Nascido/epidemiologia , Estudos de Casos e Controles , Enterocolite Necrosante/complicações , Enterocolite Necrosante/epidemiologia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Estudos Retrospectivos
6.
Br J Surg ; 105(5): 482-490, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29480528

RESUMO

BACKGROUND: Choledochal malformations comprise various congenital cystic dilatations of the extrahepatic and/or intrahepatic biliary tree. Choledochal malformation is generally considered a premalignant condition, but reliable data on the risk of malignancy and optimal surgical treatment are lacking. The objective of this systematic review was to assess the prevalence of malignancy in patients with choledochal malformation and to differentiate between subtypes. In addition, the risk of malignancy following cystic drainage versus complete cyst excision was assessed. METHODS: A systematic review of PubMed and Embase databases was performed in accordance with the PRISMA statement. A meta-analysis of the risk of malignancy following cystic drainage versus complete cyst excision was undertaken in line with MOOSE guidelines. Prevalence of malignancy was defined as the rate of biliary cancer before resection, and malignant transformation as new-onset biliary cancer after surgery. RESULTS: Eighteen observational studies were included, reporting a total of 2904 patients with a median age of 36 years. Of these, 312 in total developed a malignancy (10·7 per cent); the prevalence of malignancy was 7·3 per cent and the rate of malignant transformation was 3·4 per cent. Patients with types I and IV choledochal malformation had an increased risk of malignancy (P = 0·016). Patients who underwent cystic drainage had an increased risk of developing biliary malignancy compared with those who had complete cyst excision, with an odds ratio of 3·97 (95 per cent c.i. 2·40 to 6·55). CONCLUSION: The risk of developing malignancy among patients with choledochal malformation was almost 11 per cent. The malignancy risk following cystic drainage surgery was four times higher than that after complete cyst excision. Complete surgical resection is recommended in patients with choledochal malformation.


Assuntos
Neoplasias do Sistema Biliar , Cisto do Colédoco , Ducto Colédoco/anormalidades , Neoplasias do Sistema Biliar/diagnóstico , Neoplasias do Sistema Biliar/epidemiologia , Neoplasias do Sistema Biliar/etiologia , Cisto do Colédoco/complicações , Cisto do Colédoco/diagnóstico , Cisto do Colédoco/cirurgia , Saúde Global , Humanos , Incidência , Fatores de Risco
7.
J Perinatol ; 37(1): 67-72, 2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27684417

RESUMO

OBJECTIVE: Investigation of bacterial invasion into the intestinal wall in necrotizing enterocolitis (NEC) specimens. STUDY DESIGN: We compared 43 surgical NEC specimens with 43 age-matched controls. We used fluorescent in situ hybridization (FISH), a universal bacterial probe together with species-specific probes for Clostridium spp., Enterobacteriaceae, bacteroides and enterococci/lactobacilli. We used a FISH scoring system to reveal invasion of the intestinal wall, in which 1 represented no colonies and 4 invasion of the intestinal wall. RESULTS: We observed invasion of the intestinal wall in 22/43 of the most affected NEC tissue samples as compared with 16/43 in the least affected NEC tissue samples (P=0.03). A FISH score of 4 was reached in 7/43 control cases. Enterobacteriaceae dominated the NEC specimens. Clostridium spp. were detected occasionally in NEC samples. CONCLUSION: Bacterial invasion of the intestinal wall is more present in most affected NEC tissue samples compared with least affected NEC tissue samples or controls. Enterobacteriaceae are prevalent in advanced NEC.


Assuntos
Enterobacteriaceae/isolamento & purificação , Enterocolite Necrosante/patologia , Intestinos/microbiologia , Intestinos/patologia , Estudos de Casos e Controles , Clostridium/isolamento & purificação , Enterocolite Necrosante/cirurgia , Feminino , Humanos , Hibridização in Situ Fluorescente , Recém-Nascido , Masculino , Países Baixos , Estudos Retrospectivos , Centros de Atenção Terciária
8.
J Pediatr Surg ; 51(7): 1126-30, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26472655

RESUMO

INTRODUCTION: Survivors of necrotizing enterocolitis (NEC) often develop a post-NEC intestinal stricture, causing severe and prolonged morbidity. OBJECTIVES: We first aimed to determine the incidence of post-NEC strictures. Second, we aimed to determine risk factors associated with intestinal post-NEC strictures. MATERIALS AND METHODS: A total of 441 patients diagnosed with NEC Bell's stage ≥2 were retrospectively included in three academic pediatric surgical centers between January 2005 and January 2013. Clinical data were related to the occurrence of intestinal post-NEC strictures. Post-NEC strictures were defined as clinically relevant strictures with a radiological and/or surgical confirmation of this post-NEC stricture. RESULTS: The median gestational age of the 337 survivors of the acute phase of NEC was 29weeks (range 24-41) and median birth weight was 1130g (range 410-4130). Of the survivors, 37 (17%) medically treated NEC patients developed a post-NEC strictures versus 27 surgically treated NEC patients (24%; p=0.001). Highest C-reactive protein (CRP) level measured during the NEC episode was associated with the development of post-NEC strictures (OR 1.20, 95% confidence interval 1.11-1.32; p=0.03). No post-NEC strictures were detected in patients with CRP levels <46mg/L. CONCLUSION: This multicenter retrospective cohort study demonstrates an overall incidence of clinical relevant post-NEC strictures of 19%, with a higher rate (24%) in NEC cases treated surgically. Increased CRP levels during the NEC episode were associated with the development of post-NEC strictures.


Assuntos
Enterocolite Necrosante/complicações , Obstrução Intestinal/etiologia , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Obstrução Intestinal/diagnóstico , Obstrução Intestinal/epidemiologia , Masculino , Estudos Retrospectivos , Fatores de Risco
9.
J Pediatr Surg ; 50(11): 1837-41, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26259559

RESUMO

INTRODUCTION: Bacterial involvement is believed to play a pivotal role in the development and disease outcome of NEC. However, whether a bloodstream infection (BSI) predisposes to NEC (e.g. by activating the pro-inflammatory response) or result from the loss of gut wall integrity during NEC development is a longstanding question. OBJECTIVE: We hypothesize that the occurrence of a BSI plays a complementary role in the pathogenesis of NEC. The first aim of the study was to correlate the occurrence of a BSI during the early phase of NEC with intestinal fatty acid-binding protein (I-FABP) levels, as a marker for loss of gut wall integrity owing to mucosal damage, and Interleukin (IL)-8 levels, as a biomarker for the pro-inflammatory cascade in NEC. The second aim of the study was to investigate the relation between the occurrence of a BSI and disease outcome. MATERIAL AND METHODS: We combined data from prospective trials from two large academic pediatric surgical centers. Thirty-eight neonates with NEC, 5 neonates with bacterial sepsis, and 14 controls were included. RESULTS: BSIs occurred in 10/38 (26%) neonates at NEC onset. No association between the occurrence of BSIs and I-FABP levels in plasma (cohort 1: median 11ng/mL (range 0.8-298), cohort 2: median 6.8ng/mL (range 1.3-15)) was found in NEC patients (cohort 1: p=0.41; cohort 2: p=0.90). In addition, the occurrence of BSIs did not correlate with IL-8 (median 1562pg/mL (range 150-7,500); p=0.99). While the occurrence of a BSI was not correlated with Bell's stage (p=0.85), mortality was higher in patients with a BSI (p=0.005). CONCLUSION: The low incidence of BSIs and the absent association of both the markers for loss of gut wall integrity and the pro-inflammatory response during the early phase of NEC, support the hypothesis that the presence of a BSI does not precede NEC.


Assuntos
Bacteriemia/complicações , Enterocolite Necrosante/etiologia , Proteínas de Ligação a Ácido Graxo/sangue , Interleucina-8/sangue , Bacteriemia/sangue , Bacteriemia/epidemiologia , Biomarcadores/sangue , Enterocolite Necrosante/sangue , Feminino , Humanos , Incidência , Recém-Nascido , Masculino , Estudos Prospectivos
10.
J Pediatr Surg ; 50(7): 1115-8, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25783297

RESUMO

BACKGROUND: Intestinal fatty acid-binding protein (I-FABP) is considered as a specific marker for enterocyte damage in necrotizing enterocolitis (NEC). OBJECTIVE: The purpose of this study was to evaluate the association of plasma and urinary I-FABP levels with the extent of macroscopic intestinal necrosis in surgical NEC. METHODS: We combined data from prospective trials from two large academic pediatric surgical centers. Nine and 10 infants with surgical NEC were included, respectively. Plasma and urinary of I-FABP at disease onset were correlated with the length of intestinal resection during laparotomy. RESULTS: Median length of bowel resection was 10cm (range 2.5-50) and 17cm (range 0-51), respectively. Median I-FABP levels were 53ng/mL (range 6.3-370) and 4.2ng/mL (range 1.1-15.4) in plasma in cohort 1 respectively cohort 2 and 611ng/mL (range 3-23,336) in urine. The length of bowel resection significantly correlated with I-FABP levels in plasma (Rho 0.68; p=0.04 and Rho 0.66;p=0.04) and in urine (Rho 0.92; p=0.001). CONCLUSION: This 'proof of concept' study demonstrates that plasma and urine I-FABP levels at disease onset was strongly associated with the length of intestinal resection in surgical NEC. This offers further evidence that I-FABP levels are a promising biomarker for assessing intestinal necrosis in infants with advanced NEC.


Assuntos
Enterocolite Necrosante/patologia , Proteínas de Ligação a Ácido Graxo/sangue , Proteínas de Ligação a Ácido Graxo/urina , Biomarcadores/sangue , Biomarcadores/urina , Estudos de Coortes , Enterocolite Necrosante/sangue , Enterocolite Necrosante/cirurgia , Enterocolite Necrosante/urina , Humanos , Lactente , Intestinos/patologia , Intestinos/cirurgia , Necrose/patologia , Necrose/cirurgia , Estudos Prospectivos
11.
J Pediatr Surg ; 50(8): 1304-9, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25783404

RESUMO

BACKGROUND: The murine model of biliary atresia (BA) is used for examining the pathogenesis of BA. The aim of the study was description of the morphological features and illustrating the detailed development of fibrosis using the Biliary Atresia Research Consortium (BARC) system. METHODS: Neonatal mice were injected intraperitoneally with rhesus rotavirus (RRV) strain (N=17). Healthy mice were the control group (N=29). All mice were sacrificed at 7 or 14days after birth. Two pathologists examined the morphological features using the BARC system; CK19, αSMA and collagen type I were assessed by immunohistochemistry. RESULTS: In RRV mice, portal fibrous expansion with focal bile duct proliferation and strong portal cellular infiltrate was found in contrast to healthy mice. In RRV mice, CK19 bile duct staining was significantly less or absent (p<0.01), with stronger portal staining of collagen type I (p=0.02). Expansion of staining for αSMA was more in RRV mice (p<0.01), but αSMA portal staining was stronger in healthy mice (p=0.02). CONCLUSIONS: The morphological features observed in the murine model of BA correspond with the BA characteristics according to the BARC criteria. Fibrosis is an important feature of the model. Therefore, this murine model is useful for investigating the pathogenesis of BA.


Assuntos
Atresia Biliar/patologia , Cirrose Hepática/etiologia , Fígado/patologia , Animais , Atresia Biliar/virologia , Modelos Animais de Doenças , Progressão da Doença , Cirrose Hepática/patologia , Cirrose Hepática/virologia , Camundongos , Camundongos Endogâmicos BALB C , Infecções por Rotavirus/complicações , Índice de Gravidade de Doença
12.
Transplant Proc ; 46(6): 2102-8, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25131117

RESUMO

AIM: In 2001, a multidisciplinary intestinal rehabilitation program, prompted by a nationwide collaboration on intestinal failure (Dutch Registry for Intestinal Failure and Intestinal Transplantation), was started for children who have short bowel syndrome (SBS). This study evaluates this program, focusing on children who have SBS after extensive bowel resection. DESIGN: This is a retrospective cohort study. METHOD: Demographic data, general information on disease status, and outcome of intestinal rehabilitation of patients treated between 2001 and 2009 were collected. Outcome measures were intestinal autonomy, intestinal and/or liver transplantation, and survival. RESULTS: Ten boys and 9 girls, median gestational age 36 weeks, were treated. Eight were referred, 3 times as many as in the period 1991-2000. Causes of SBS were intestinal atresia (3), gastroschisis (2), volvulus (9), necrotising enterocolitis (3), and strangulation (2). The median remaining small-intestinal length was 35 cm (range, 10 to 70 cm). In 14 patients the ileocecal valve was still present. In all patients at least 25% of colon was still present. The median follow-up was 25 months (range, 50 days to 9 years). After a median of 138 days (range, 41 days to 11 years) on total parenteral nutrition, 16 patients (84%) reached intestinal autonomy. Central venous catheter-related complications occurred in all; there were liver function disorders in 68%, and a failure to thrive in 26%. One patient underwent intestinal lengthening. No patient needed intestinal transplantation, but one underwent liver transplantation for intestinal failure-associated liver disease. Overall mortality was 11%: those 2 patients died of abdominal sepsis. CONCLUSION: This specialized intestinal rehabilitation program led to intestinal autonomy in 84% of the patients who had SBS. None of the patients underwent an intestinal transplantation.


Assuntos
Síndrome do Intestino Curto/reabilitação , Criança , Pré-Escolar , Nutrição Enteral , Feminino , Seguimentos , Humanos , Lactente , Absorção Intestinal/fisiologia , Masculino , Países Baixos , Nutrição Parenteral Total , Equipe de Assistência ao Paciente , Estudos Retrospectivos , Síndrome do Intestino Curto/etiologia , Síndrome do Intestino Curto/patologia , Resultado do Tratamento
13.
J Adolesc Health ; 50(6): 641-4, 2012 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-22626493

RESUMO

PURPOSE: To investigate the course of life of young adults diagnosed with biliary atresia (BA) in infancy by comparing patients who did and did not underwent transplantation with an age-matched Dutch reference group. METHODS: All patients from the Dutch BA registry, aged >18 years, were invited to complete the course of life questionnaire. RESULTS: Forty patients participated (response = 74%). Twenty-five had not undergone transplantation; 15 had undergone orthotopic liver transplantation. One significant between-group difference was found, namely in substance use and gambling. BA patients who underwent transplantation reported less use than the reference group (p = .01, moderate effect size). Additional moderate effect sizes were found for differences in psychosexual and social development and antisocial behavior. Patients who underwent transplantation had lower scores than one or both other groups. CONCLUSIONS: Development of BA survivors who did not undergo transplantation seems not delayed, whereas that of transplanted patients does seem somewhat delayed. However, patients who underwent transplantation display less risk behavior. Larger samples are necessary to confirm these findings.


Assuntos
Atresia Biliar/epidemiologia , Atresia Biliar/psicologia , Deficiências do Desenvolvimento/epidemiologia , Deficiências do Desenvolvimento/psicologia , Adulto , Transtorno da Personalidade Antissocial/epidemiologia , Transtorno da Personalidade Antissocial/psicologia , Atresia Biliar/cirurgia , Estudos de Coortes , Estudos Transversais , Feminino , Jogo de Azar/epidemiologia , Jogo de Azar/psicologia , Humanos , Vida Independente/psicologia , Transplante de Fígado , Masculino , Países Baixos , Portoenterostomia Hepática , Disfunções Sexuais Psicogênicas/epidemiologia , Disfunções Sexuais Psicogênicas/psicologia , Ajustamento Social , Transtornos Relacionados ao Uso de Substâncias/epidemiologia , Transtornos Relacionados ao Uso de Substâncias/psicologia , Inquéritos e Questionários , Adulto Jovem
14.
Am J Med Genet A ; 158A(3): 626-9, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22302579

RESUMO

A 9-year-old boy with the classical type of Ehlers-Danlos syndrome (EDS) developed a symptomatic aneurysm of the superior mesenteric artery. His EDS diagnosis had been confirmed biochemically and genetically. Vascular complications are known to be associated with the vascular type of EDS, but this is the first report of a child with classical EDS who developed a major vascular complication. Clinicians should be aware that severe vascular complications albeit rare, can also occur in classical EDS.


Assuntos
Aneurisma/diagnóstico por imagem , Síndrome de Ehlers-Danlos/complicações , Artéria Mesentérica Superior/diagnóstico por imagem , Aneurisma/complicações , Angiografia , Criança , Humanos , Masculino , Artéria Mesentérica Superior/patologia , Tomografia Computadorizada por Raios X
16.
Eur J Pediatr Surg ; 21(4): 234-7, 2011 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21404168

RESUMO

INTRODUCTION: There has been a shift from operative treatment (OT) to non-operative treatment (NOT) of splenic injury. We evaluated the outcomes of treatment of pediatric patients with blunt splenic trauma in our hospital, with special focus on the outcomes after NOT. PATIENTS AND METHODS: The data of all patients <18 years with radiologically proven blunt splenic injury admitted between 1988 and 2007 were retrospectively analyzed. Mechanism of injury, type of treatment, ICU stay, total hospital stay, morbidity and mortality were assessed. Patients suffering isolated splenic injuries were assessed separately from patients with multiple injuries. Patients were subsequently divided into those admitted before and after 2000. RESULTS: There were 64 patients: 49 males and 15 females with a mean age of 13 years (range 0-18). 3 patients died shortly after admission due to severe neurological injury and were excluded. In the remaining 61 patients concomitant injuries, present in 62%, included long bone fractures (36%), chest injuries (16%), abdominal injuries (33%) and head injuries (30%). Mechanisms of injury were: car accidents (26%), motorcycle (20%), bicycle (19%), fall from height (17%) and pedestrians struck by a moving vehicle (8%). A change in treatment strategy was evident for the pre- and post-2000 periods. Significantly more patients had NOT after 2000 in both the isolated splenic injury group and the multi-trauma group [4/11 (36%) before vs. 10/11 (91%) after (p=0.009); 15/19 (79%) before vs. 8/20 (40%) after 2000 (p=0.03)]. There was also a significant shift to spleen-preserving operations. All life-threatening complications occurred within <24 h after injury. Mortality for the entire cohort was 7%; all of these patients were treated operatively. When comparing the median ICU and hospital stay before and after 2000 it was found to be significantly higher in the isolated injury group and remained statistically the same in the multi-trauma group. CONCLUSION: Splenic injury in children is associated with substantial mortality. This is due to concomitant injuries and not to the splenic injury. Non-operative treatment is increasingly preferred to operative procedures when treating splenic injuries in hemodynamically, stable children. ICU and hospital stay have, despite the change from OT to NOT, remained the same. Complications after NOT are rare. We are still observing children in hospital for a longer period than is necessary.


Assuntos
Fidelidade a Diretrizes/estatística & dados numéricos , Baço/lesões , Ferimentos não Penetrantes/terapia , Acidentes/estatística & dados numéricos , Adolescente , Distribuição por Idade , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Tempo de Internação/estatística & dados numéricos , Masculino , Auditoria Médica , Traumatismo Múltiplo/etiologia , Traumatismo Múltiplo/mortalidade , Traumatismo Múltiplo/cirurgia , Traumatismo Múltiplo/terapia , Países Baixos , Estudos Retrospectivos , Baço/cirurgia , Esplenectomia/estatística & dados numéricos , Esplenectomia/tendências , Resultado do Tratamento , Ferimentos não Penetrantes/etiologia , Ferimentos não Penetrantes/mortalidade , Ferimentos não Penetrantes/cirurgia
17.
Eur J Pediatr Surg ; 21(1): 58-64, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21157690

RESUMO

BACKGROUND: Necrotizing enterocolitis (NEC) patients often have neurodevelopmental impairment, but the long-term follow-up data is limited. We determined whether surgical factors were of prognostic value for long-term neurodevelopment in children surviving surgery for NEC (SNEC). PATIENTS AND METHODS: SNEC patients born between 1996 and 2002 were tested for verbal (VIQ), performance (PIQ) and total (TIQ) intelligence using Wechsler's Intelligence Scale for Children, Third Edition, Dutch Version, and motor skills using the Movement Assessment Battery for Children (M-ABC). Neonatal and surgical data were obtained retrospectively to assess prognostic factors. RESULTS: 19 patients (12 boys), median age 9.9 years (range 6.2-13.1), gestational age 31.0 weeks (range 25.2-40), birth weight 1 250 g (range 780-3 175) were evaluated. Infants with an enterostomy (n=14) scored lower on intelligence than children with a primary anastomosis (n=5): VIQ 85 ± 12 vs. 101 ± 15, p=0.04; PIQ 79 ± 13 vs. 92 ± 11, p=0.06; TIQ 82 ± 11 vs. 97 ± 13, p=0.04. Motor skills were either suspect or clinically impaired in 74%. Clinical classification of results suggest more children in the enterostomy group had a performance rated as clinically impaired compared to the primary anastomosis group, although no statistical difference in M-ABC score was found. There were no differences between primary anastomosis and enterostomy patients with regard to gestational age, birth weight, comorbidities, preoperative Bell stage, residual small and large bowel lengths, inotropic medication, duration of ventilatory support, NICU and hospital stay, and physical exam data on follow-up. However, a selection bias could not be ruled out. CONCLUSIONS: The results suggest that an enterostomy in SNEC patients could be associated with worse neurodevelopmental outcomes by the age of 6-13 years compared to a primary anastomosis, although the severity of illness was comparable between both groups. Further studies are needed to prevent selection bias and to elucidate the impact of abdominal surgical factors on neurodevelopmental outcome and the underlying pathophysiology.


Assuntos
Transtornos Cognitivos/etiologia , Deficiências do Desenvolvimento/etiologia , Enterocolite Necrosante/cirurgia , Enterostomia , Doenças do Sistema Nervoso/etiologia , Adolescente , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento
19.
Eur J Pediatr Surg ; 19(6): 392-4, 2009 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-19899038

RESUMO

INTRODUCTION: The American Pediatric Surgical Association developed guidelines for the management of haemodynamically stable children with hepatic or splenic injury, based on grade of injury on CT scan. This study investigated the intra- and inter-observer agreement of radiologists, paediatric surgeons, trauma surgeons and hepatobiliary surgeons when scoring liver injury based on CT scan findings. PATIENTS AND METHODS: CT scans of patients with blunt abdominal trauma were independently assessed twice by a fellow and a consultant radiologist, paediatric surgeon, trauma surgeon and one consultant hepatobiliary surgeon. Reviewers were unaware of the clinical course. All scans were multislice CTs with a slice thickness of 3 mm, and both the arterial and venous phase were assessed. Injury was scored using the American Association for the Surgery of Trauma (AAST) liver injury scale. Intra-observer agreement was tested using Cohen's kappa coefficient. Inter-observer agreement was tested using Cohen's kappa for the second reading of individual observers and Spearman's rank correlation for the mean of both readings from each observer. RESULTS: CT scans of 27 patients (11 girls and 16 boys, median age 11.7+/-5.2 years) were reviewed. Mean AAST grade of liver injury was 3.3+/-1.1 for radiologists, 2.9+/-1.0 for paediatric surgeons, 3.0+/-0.9 for trauma surgeons and 3.2+/-0.8 for the hepatobiliary surgeon (p=0.30) Intra-observer agreement was moderate, with kappa below 0.7 for all observers except for one of the radiologists. Inter-observer correlation using Cohen's kappa coefficient was also moderate, with kappa below 0.5. In contrast, inter-observer correlation using Spearman's test was good, suggesting that there is agreement on the general severity of injury but not on the exact grading of injury using the AAST scoring system. CONCLUSION: Intra-observer agreement is only moderate when assessing liver injury using the AAST grading system. Only the most experienced radiologist demonstrated good intra-observer agreement which might indicate the necessity of the presence of a senior trauma radiologist at all times. However, this is not possible in most centres. Although there was agreement concerning the general severity of injury, inter-observer agreement is also moderate. These data cast doubt on the use of the AAST liver injury score alone as a decision-making tool when assessing haemodynamically stable children with blunt hepatic injury.


Assuntos
Traumatismos Abdominais/diagnóstico por imagem , Escala de Gravidade do Ferimento , Fígado/diagnóstico por imagem , Fígado/lesões , Médicos/estatística & dados numéricos , Tomografia Computadorizada por Raios X , Ferimentos não Penetrantes/diagnóstico por imagem , Traumatismos Abdominais/cirurgia , Adolescente , Criança , Feminino , Cirurgia Geral , Humanos , Fígado/cirurgia , Masculino , Variações Dependentes do Observador , Guias de Prática Clínica como Assunto , Valor Preditivo dos Testes , Prognóstico , Radiologia , Baço/diagnóstico por imagem , Baço/lesões , Ferimentos não Penetrantes/cirurgia
20.
Eur J Pediatr Surg ; 19(6): 358-61, 2009 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-19517354

RESUMO

INTRODUCTION: Paediatric blunt hepatic trauma treatment is changing from operative treatment (OT) to non-operative treatment (NOT). In 2000 the American Pediatric Surgical Association has published guidelines for NOT of these injuries. Little is known about the treatment of paediatric liver trauma in the Netherlands. PATIENTS AND METHODS: Data of all patients aged 18 years and younger admitted to our hospital for blunt hepatic trauma in the past 18 years were retrospectively analysed using a prospective trauma registry. The mechanism of injury, treatment, ICU admission time, total admission time, morbidity and mortality were assessed. Subsequently the group was divided into patients treated before and after 2000. RESULTS: Eighty patients were identified: 52M, 28F with a mean age of 12 years (range 2-18). Thirty patients sustained isolated liver injury. Concomitant injuries were fractures of long bones (28), abdominal (25), chest (24) and head injuries (18). Mean ISS score was 18 (range 4-57). Mortality was 8%. Mechanisms of injury consisted of bicycle (25%), car (20%), and motorcycle accidents (15%), pedestrian hit by vehicle (15%), fall from height (14%) and accidents associated with animals (11%). Haemodynamically stable patients underwent NOT (55). 25 patients (31%) underwent a laparotomy, which in 20 cases (80%) was related to hepatic injury. Although the groups treated before and after 2000 did not differ haemodynamically on admission to hospital, a shift to NOT is evident: 24/37 (63%) patients underwent NOT before 2000 versus 38/45 (84%) after 2000 (p=0.04). Complications following NOT were rare. Late onset bleeding did not occur. Two patients developed an infected biloma, requiring a laparotomy. Mean ICU stay before 2000 was 4.2 days (range 0-25 days) and 2.6 days (range 0-17 days) after 2000. Total hospital time did not decrease: 14 days (range 1-39 days) before 2000 and 14 days (range 1-60 days) after 2000. The overall mortality was 8%. All deaths occurred in the operative group and were spread evenly over both periods. CONCLUSION: In blunt paediatric liver trauma, the incidence and trauma mechanism seem age-related. A shift to NOT is found in the treatment of paediatric blunt hepatic trauma. NOT is the preferred treatment for the haemodynamically stable patient. Complications are rare and the success rate is 96%. The mean ICU stay has decreased but the total admission time could possibly be shortened.


Assuntos
Laparotomia , Fígado/lesões , Fígado/cirurgia , Traumatismo Múltiplo/cirurgia , Centros de Traumatologia , Ferimentos não Penetrantes/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Escala de Gravidade do Ferimento , Fígado/diagnóstico por imagem , Masculino , Traumatismo Múltiplo/complicações , Traumatismo Múltiplo/diagnóstico , Traumatismo Múltiplo/etiologia , Traumatismo Múltiplo/mortalidade , Países Baixos , Guias de Prática Clínica como Assunto , Radiografia , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento , Ultrassonografia , Ferimentos não Penetrantes/complicações , Ferimentos não Penetrantes/diagnóstico , Ferimentos não Penetrantes/mortalidade
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