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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 9 (1994), S. 366-367 
    ISSN: 1437-9813
    Keywords: Hirschsprung's disease ; Trisomy 21 ; Duodenal obstruction ; Child
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The association of trisomy 21 and Hirschsprung's disease (HD) is well known. In a neonate, HD characteristically presents with delayed passage of meconium and/or intestinal obstruction. However, the presence of duodenal atreasia (DA), which may present similarly, can mask the associated HD. Over an 18-year period, 17 of 135 patients with HD had trisomy 21. Three of these 17 patients presented with duodenal obstruction in the newborn period — 2 had DA and 1 a complete duodenal web. One patient developed a perforation of the jejunum 7 days after laparotomy and duodenoduodenostomy and was found to have HD while the other 2 were later investigated because of persistent unresponsive constipation and confirmed to have HD at 1 and 2 years of age. In spite of associated Down's syndrome and DA, the possibility of HD should be considered in patients who have a complicated postoperative course or persisting abnormal stooling patterns.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 16 (2000), S. 473-477 
    ISSN: 1437-9813
    Keywords: Key words Congenital diaphragmatic hernia (CDH) ; Surfactant-associated protein ; Hypoplastic lung ; Ventilation ; Oxygen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The hypoplastic lung in congenital diaphragmatic hernia (CDH) has both a quantitative and qualitative reduction in surfactant. Recently, the role of oxygen (O2) as a regulator of pulmonary surfactant-associated protein (SP) gene expression has been reported. The mRNA level of SP has been demonstrated to be increased in the lungs of animals exposed to hyperoxia. The aim of this study was to investigate SP mRNA expression in hypoplastic CDH lung in rats during mechanical ventilation in order to determine the effect of O2 on SP synthesis in CDH. A CDH model was induced in pregnant rats following administration of nitrofen. The newborn rats with CDH and controls were intubated and ventilated. Ventilation was continued for 6 h under 100% oxygen. Reverse-transcription polymerase chain reaction (RT-PCR) was performed to evaluate the relative amounts of mRNA expression of SP-A, SP-B, SP-C, and SP-D. Relative amounts of SP-A, SP-B, and SP-D mRNA expression in CDH lung were significantly decreased compared to controls at birth and 6 h after ventilation. There was no significant difference in SP-C mRNA expression between CDH animals and controls. Upregulated mRNA expression of SP-A, SP-B, and SP-D in lungs of control animals at 6 h after ventilation suggests that oxygenation accelerates postnatal SP synthesis in normal lungs. The inability of O2 to increase SP mRNA expression in hypoplastic CDH lung suggests that the hypoplastic lung is not responsive to increased oxygenation for the synthesis of SP.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1437-9813
    Keywords: Key words Atrial natriuretic peptide ; Congenital diaphragmatic hernia ; Hypoplastic lung ; Reverse transcription polymerase chain reaction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Atrial natriuretic peptide (ANP) plays a major role in electrolyte and volume homeostasis through potent biological effects including vasorelaxation, bronchorelaxation, lung permeability, and clearance. There are two distinct biochemical and functional classes of ANP receptors, guanylate cyclase receptor (GC-R) and clearance receptors (clearance-R). Two subtypes of GC-R have been described, GCA-R and GCB-R. Antenatal glucocorticoid therapy (AGT) has been demonstrated to improve pulmonary immaturity and abnormal structure of pulmonary arteries in animal models of congenital diaphragmatic hernia (CDH). The aim of this study was to investigate the effect of antenatal glucocorticoid administration on the ANP system in nitrofen-induced CDH hypoplastic lung in rats. A CDH model was induced in pregnant rats following administration of nitrofen on day 9.5 of gestation. Dexamethasone (Dex) was given intraperitoneally on days 18.5 and 19.5; cesarean section was performed on day 21. Reverse transcription polymerase chain reaction was performed to evaluate the relative amounts of GCA-R, GCB-R and clearance-R mRNA expression. The mRNA expression of GCA-R, GCB-R, and clearance-R was significantly increased in CDH compared to control lung. ANP receptor mRNA expression was significantly decreased in CDH lung with compared to without Dex treatment. Our finding of increased ANP receptor mRNA expression in CDH lung suggests that the hypoplastic lung has high sensitivity for ANP. Decreased mRNA expression of ANP receptors in CDH lung after Dex treatment suggests that AGT may improve pulmonary physiological function of ANP in hypoplastic CDH lung.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 9 (1994), S. 234-236 
    ISSN: 1437-9813
    Keywords: Hirschsprung's disease ; Enterocolitis ; Child
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Enterocolitis remains a major cause of morbidity and mortality in patients with Hirschsprung's disease (HD). Forty-one (30%) of 135 patients treated for HD from 1975 to 1992 developed enterocolitis; enterocolitis occured preoperatively in 25. It was a presenting feature in 17 infants, including 11 neonates. The proportion of patients presenting with enterocolitis in the neonatal period increased with advancing age, 6 (11%) out of 56 presenting within the 1st week and 5 (24%) out of 21 after 1 week. Episodes of enterocolitis continued in 7 of these 25 patients after a pull-through procedure. Sixteen patients had a first episode of enterocolitis after surgery: 3 after a colostomy, and 13 after a pull-through procedure. The incidence of enterocolitis was 28% in patients with rectosigmoid involvement and 38% in patients with long-segment or total colonic aganglionosis (P= 0.1). Enterocolitis occured in 8 (47%) of 17 patients with trisomy 21 as compared to 33 of 118 (28%) other patients (P= 0.1). Four of the 41 patients died as a result of enterocolitis, 3 in the neonatal period after a colostomy performed at a mean age of 22 days, although the symptoms suggestive of HD were present since birth. Prompt diagnosis and expeditious management are necessary in patients with HD.
    Type of Medium: Electronic Resource
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