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1.
BMC Pediatr ; 23(1): 336, 2023 07 01.
Artículo en Inglés | MEDLINE | ID: mdl-37393233

RESUMEN

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.


Asunto(s)
Enterocolitis Necrotizante , Enfermedades Fetales , Enfermedades del Recién Nacido , Lactante , Femenino , Recién Nacido , Humanos , Recien Nacido Extremadamente Prematuro , Enterocolitis Necrotizante/diagnóstico , Estudios de Cohortes , Edad Gestacional
2.
Br J Surg ; 105(5): 482-490, 2018 04.
Artículo en Inglés | MEDLINE | ID: mdl-29480528

RESUMEN

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.


Asunto(s)
Neoplasias del Sistema Biliar , Quiste del Colédoco , Conducto Colédoco/anomalías , Neoplasias del Sistema Biliar/diagnóstico , Neoplasias del Sistema Biliar/epidemiología , Neoplasias del Sistema Biliar/etiología , Quiste del Colédoco/complicaciones , Quiste del Colédoco/diagnóstico , Quiste del Colédoco/cirugía , Salud Global , Humanos , Incidencia , Factores de Riesgo
3.
Am J Med Genet A ; 158A(3): 626-9, 2012 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-22302579

RESUMEN

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.


Asunto(s)
Aneurisma/diagnóstico por imagen , Síndrome de Ehlers-Danlos/complicaciones , Arteria Mesentérica Superior/diagnóstico por imagen , Aneurisma/complicaciones , Angiografía , Niño , Humanos , Masculino , Arteria Mesentérica Superior/patología , Tomografía Computarizada por Rayos X
4.
J Pediatr Surg ; 56(2): 239-244, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-32829881

RESUMEN

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.


Asunto(s)
Atresia Esofágica , Esofagoplastia , Adolescente , Anastomosis Quirúrgica , Niño , Atresia Esofágica/cirugía , Femenino , Humanos , Masculino , Calidad de Vida , Encuestas y Cuestionarios , Adulto Joven
5.
Semin Pediatr Surg ; 29(4): 150942, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32861446

RESUMEN

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.


Asunto(s)
Procedimientos Quirúrgicos del Sistema Biliar , Colangiocarcinoma , Colangitis , Quiste del Colédoco/cirugía , Evaluación de Resultado en la Atención de Salud , Calidad de Vida , Procedimientos Quirúrgicos del Sistema Biliar/efectos adversos , Colangiocarcinoma/etiología , Colangitis/etiología , Humanos
6.
Early Hum Dev ; 147: 105091, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32492527

RESUMEN

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.


Asunto(s)
Discapacidades del Desarrollo/epidemiología , Nutrición Enteral , Enterocolitis Necrotizante/complicaciones , Recien Nacido Prematuro/fisiología , Desarrollo Infantil , Preescolar , Enterocolitis Necrotizante/epidemiología , Femenino , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro/crecimiento & desarrollo , Masculino
7.
J Pediatr Surg ; 54(9): 1755-1760, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-30635129

RESUMEN

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.


Asunto(s)
Enterocolitis Necrotizante , Cardiopatías Congénitas , Enfermedades del Recién Nacido/epidemiología , Estudios de Casos y Controles , Enterocolitis Necrotizante/complicaciones , Enterocolitis Necrotizante/epidemiología , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/epidemiología , Humanos , Recién Nacido , Recien Nacido Prematuro , Estudios Retrospectivos
9.
J Perinatol ; 37(1): 67-72, 2017 01.
Artículo en Inglés | MEDLINE | ID: mdl-27684417

RESUMEN

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.


Asunto(s)
Enterobacteriaceae/aislamiento & purificación , Enterocolitis Necrotizante/patología , Intestinos/microbiología , Intestinos/patología , Estudios de Casos y Controles , Clostridium/aislamiento & purificación , Enterocolitis Necrotizante/cirugía , Femenino , Humanos , Hibridación Fluorescente in Situ , Recién Nacido , Masculino , Países Bajos , Estudios Retrospectivos , Centros de Atención Terciaria
10.
J Pediatr Surg ; 51(7): 1126-30, 2016 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-26472655

RESUMEN

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.


Asunto(s)
Enterocolitis Necrotizante/complicaciones , Obstrucción Intestinal/etiología , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Obstrucción Intestinal/diagnóstico , Obstrucción Intestinal/epidemiología , Masculino , Estudios Retrospectivos , Factores de Riesgo
11.
J Clin Oncol ; 22(20): 4202-8, 2004 Oct 15.
Artículo en Inglés | MEDLINE | ID: mdl-15483031

RESUMEN

PURPOSE: To assess 3 years of quality of life in patients with esophageal cancer in a randomized trial comparing limited transhiatal resection with extended transthoracic resection. PATIENTS AND METHODS: Quality-of-life questionnaires were sent at baseline and at 5 weeks; 3, 6, 9, and 12 months; and 1.5, 2, 2.5, and 3 years after surgery. Physical and psychological symptoms, activity level, and global quality of life were assessed with the disease-specific Rotterdam Symptom Checklist. Generic quality of life was measured with the Medical Outcomes Study Short Form-20. RESULTS: A total of 199 patients participated. Physical symptoms and activity level declined after the operation and gradually returned toward baseline within the first year (P < .01). Psychological well-being consistently improved after baseline (P < .01), whereas global quality of life showed a small initial decline followed by continuous gradual improvement (P < .01). Quality of life stabilized in the second and third year. Three months after the operation, patients in the transhiatal esophagectomy group (n = 96) reported fewer physical symptoms (P = .01) and better activity levels (P < .01) than patients in the transthoracic group (n = 103), but no differences were found at any other measurement point. For psychological symptoms and global quality of life, no differences were found at any follow-up measurement. A similar pattern was found for generic quality of life. CONCLUSION: No lasting differences in quality of life of patients who underwent either transhiatal or transthoracic resection were found. Compared with baseline, quality of life declined after the operation but was restored within a year in both groups.


Asunto(s)
Adenocarcinoma/fisiopatología , Adenocarcinoma/psicología , Neoplasias Esofágicas/fisiopatología , Neoplasias Esofágicas/psicología , Esofagectomía/métodos , Calidad de Vida , Adulto , Anciano , Femenino , Estado de Salud , Humanos , Masculino , Persona de Mediana Edad , Factores de Tiempo
12.
Ned Tijdschr Geneeskd ; 149(49): 2705-11, 2005 Dec 03.
Artículo en Holandés | MEDLINE | ID: mdl-16375012

RESUMEN

In 3 children, 2 boys aged 13 years and an 8-year-old girl, blunt trauma to the liver was diagnosed after a fall. The first patient underwent drainage of abdominal fluid collection on multiple occasions and a stent was introduced in the biliary duct to ensure normal bile flow; the second patient was treated by arterial catheter embolisation of a segmental branch of the right hepatic artery; in the third patient, acute laparotomy was performed in view ofhaemodynamic instability and a large hepatic rupture was treated by packing. All patients recovered successfully. In the paediatric population, blunt trauma to the liver requires a multidisciplinary approach. For haemodynamically stable children, a conservative course can be taken. Arterial embolisation is the treatment of choice if there is a persistent need for volume resuscitation or blood transfusion. In case of massive haemorrhagic shock, the child should undergo laparotomy, and if the bleeding cannot be stopped, the liver should be packed according to damage-control principles.


Asunto(s)
Hígado/lesiones , Heridas no Penetrantes/terapia , Accidentes por Caídas , Adolescente , Niño , Drenaje , Embolización Terapéutica , Femenino , Hemodinámica/fisiología , Humanos , Laparotomía , Masculino , Rotura/cirugía , Stents , Resultado del Tratamiento , Heridas no Penetrantes/diagnóstico , Heridas no Penetrantes/cirugía
13.
J Pediatr Surg ; 50(11): 1837-41, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-26259559

RESUMEN

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.


Asunto(s)
Bacteriemia/complicaciones , Enterocolitis Necrotizante/etiología , Proteínas de Unión a Ácidos Grasos/sangre , Interleucina-8/sangre , Bacteriemia/sangre , Bacteriemia/epidemiología , Biomarcadores/sangre , Enterocolitis Necrotizante/sangre , Femenino , Humanos , Incidencia , Recién Nacido , Masculino , Estudios Prospectivos
14.
J Pediatr Surg ; 50(7): 1115-8, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25783297

RESUMEN

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.


Asunto(s)
Enterocolitis Necrotizante/patología , Proteínas de Unión a Ácidos Grasos/sangre , Proteínas de Unión a Ácidos Grasos/orina , Biomarcadores/sangre , Biomarcadores/orina , Estudios de Cohortes , Enterocolitis Necrotizante/sangre , Enterocolitis Necrotizante/cirugía , Enterocolitis Necrotizante/orina , Humanos , Lactante , Intestinos/patología , Intestinos/cirugía , Necrosis/patología , Necrosis/cirugía , Estudios Prospectivos
15.
J Pediatr Surg ; 50(8): 1304-9, 2015 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-25783404

RESUMEN

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.


Asunto(s)
Atresia Biliar/patología , Cirrosis Hepática/etiología , Hígado/patología , Animales , Atresia Biliar/virología , Modelos Animales de Enfermedad , Progresión de la Enfermedad , Cirrosis Hepática/patología , Cirrosis Hepática/virología , Ratones , Ratones Endogámicos BALB C , Infecciones por Rotavirus/complicaciones , Índice de Severidad de la Enfermedad
16.
Ned Tijdschr Geneeskd ; 146(42): 1993-5, 2002 Oct 19.
Artículo en Holandés | MEDLINE | ID: mdl-12420426

RESUMEN

A 22-year-old homeless male psychiatric patient of Ghanaian origin was diagnosed with severe frostbite in both lower legs. The lesion consisted of haemorrhagic blistering with already visible demarcation. When sepsis developed both lower legs had to be amputated as a matter of urgency.


Asunto(s)
Congelación de Extremidades/cirugía , Personas con Mala Vivienda/psicología , Trastornos Mentales/complicaciones , Adulto , Amputación Quirúrgica , Frío/efectos adversos , Congelación de Extremidades/etnología , Ghana/etnología , Humanos , Masculino , Trastornos Mentales/etnología , Trastornos Mentales/psicología , Sepsis/complicaciones , Tiempo (Meteorología)
17.
Ned Tijdschr Geneeskd ; 146(15): 719-22, 2002 Apr 13.
Artículo en Holandés | MEDLINE | ID: mdl-11980373

RESUMEN

OBJECTIVE: To gain insight into the shortage in health care capacity for patients who require immediate admission to hospital. DESIGN: Prospective, descriptive. METHOD: During the period 1 March-30 November 2001, data were collected on all patients presenting at the casualty department at the Sint Lucas Andreas Hospital in Amsterdam, the Netherlands, who had a surgical, internal medicine or neurological condition which required immediate admission and who could not be admitted due to a shortage in health care capacity. The following data were registered: date of transfer, age, gender, diagnosis, referring specialty, time of telephone call, accepting hospital and time of acceptance. During the same period, the surgery department also noted details of patients requiring immediate admission or transfer whom they turned away after presentation via the telephone by either the general practitioner or a different hospital. RESULTS: 131 patients could not be admitted, 68 men and 63 women with a mean age of 69 years. The distribution across the specialties was as follows: surgery: 63 patients (48%; mean age: 68 years); internal medicine: 48 patients (37%; 65 years); neurology: 20 patients (15%; 74 years). The most common reasons for admission were proximal femur fractures (24; 18%) and gastrointestinal disorders (27; 21%). Mean duration from making the telephone call until acceptance elsewhere was 70 min (range: 1-330) for surgery, 42 min (5-180) for internal medicine and 116 min (10-870) for neurology. The transfer of patients with proximal femur fractures (97 min) and cerebrovascular accident (129 min) took the longest. During the same period, 170 patients were refused by the department of surgery for first-time admission or transfer.


Asunto(s)
Servicio de Urgencia en Hospital/organización & administración , Admisión del Paciente , Personal de Hospital/provisión & distribución , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Países Bajos , Administración de Personal en Hospitales , Estudios Prospectivos
18.
Transplant Proc ; 46(6): 2102-8, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-25131117

RESUMEN

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.


Asunto(s)
Síndrome del Intestino Corto/rehabilitación , Niño , Preescolar , Nutrición Enteral , Femenino , Estudios de Seguimiento , Humanos , Lactante , Absorción Intestinal/fisiología , Masculino , Países Bajos , Nutrición Parenteral Total , Grupo de Atención al Paciente , Estudios Retrospectivos , Síndrome del Intestino Corto/etiología , Síndrome del Intestino Corto/patología , Resultado del Tratamiento
19.
J Adolesc Health ; 50(6): 641-4, 2012 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-22626493

RESUMEN

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.


Asunto(s)
Atresia Biliar/epidemiología , Atresia Biliar/psicología , Discapacidades del Desarrollo/epidemiología , Discapacidades del Desarrollo/psicología , Adulto , Trastorno de Personalidad Antisocial/epidemiología , Trastorno de Personalidad Antisocial/psicología , Atresia Biliar/cirugía , Estudios de Cohortes , Estudios Transversales , Femenino , Juego de Azar/epidemiología , Juego de Azar/psicología , Humanos , Vida Independiente/psicología , Trasplante de Hígado , Masculino , Países Bajos , Portoenterostomía Hepática , Disfunciones Sexuales Psicológicas/epidemiología , Disfunciones Sexuales Psicológicas/psicología , Ajuste Social , Trastornos Relacionados con Sustancias/epidemiología , Trastornos Relacionados con Sustancias/psicología , Encuestas y Cuestionarios , Adulto Joven
20.
Eur J Pediatr Surg ; 21(4): 234-7, 2011 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-21404168

RESUMEN

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.


Asunto(s)
Adhesión a Directriz/estadística & datos numéricos , Bazo/lesiones , Heridas no Penetrantes/terapia , Accidentes/estadística & datos numéricos , Adolescente , Distribución por Edad , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Tiempo de Internación/estadística & datos numéricos , Masculino , Auditoría Médica , Traumatismo Múltiple/etiología , Traumatismo Múltiple/mortalidad , Traumatismo Múltiple/cirugía , Traumatismo Múltiple/terapia , Países Bajos , Estudios Retrospectivos , Bazo/cirugía , Esplenectomía/estadística & datos numéricos , Esplenectomía/tendencias , Resultado del Tratamiento , Heridas no Penetrantes/etiología , Heridas no Penetrantes/mortalidad , Heridas no Penetrantes/cirugía
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