Functional intestinal obstruction due to deficiency of argyrophil neurones in the myenteric plexus. Familial syndrome presenting with short small bowel, malrotation, and pyloric hypertrophy.
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In 3 infants functional intestinal obstruction, associated with a short small intestine, malrotation, and pyloric hypertrophy, was shown to be due to failure of development of the argyrophil myenteric plexus, with the absence of ongoing peristalsis. 4 infants with similar clinical features have been described previously, and there is evidence for an autosomal recessive mode of inheritance of this syndrome. (+info)
Pyloric stenosis in the Oxford Record Linkage Study area.
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The files of the Oxford Record Linkage Study were employed to identify 220 infants presenting with infantile hypertrophic pyloric stenosis (IHPS) in the 6-year period 1966 to 1971. Information on these infants was obtained from birth certificates and maternity notes. The overall incidence was 2.5 per 1000 livebirths. There was a distinct seasonal variation, with highest incidence to infants born in the third quarter of the year as well as variation in incidence with area: the cities had much lower rates of IHPS than the adjacent rural or small urban areas. It was shown that the rates in the south and east of the area studied were far greater than in the north and west. In the present study there was no excess of primiparae, the peak maternal age group was 20 to 24; there was a slight excess of parents of social classes I and II; and a significant association with mothers who were Rhesus negative. The rate of IHPS among sibs was 85 per 1000. Though there was the usual correlation with the male sex (M:F ratio = 5.5:1), there was no variation with birthweight and only among the females was an association found with prolonged gestation. There appeared to be an inverse relation between gestation and age on admission to hospital. (+info)
Simultaneous pyloric and colonic obstruction associated with hiatus hernia in a weightlifter: a case report.
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Hiatus hernia is usually attributed to conditions that cause a chronic increase in intra-abdominal pressure such as multiple pregnancies and obesity. A 30-year-old man, a weightlifter, had a massive hiatus hernia causing both high and low gastrointestinal obstruction but no involvement of the gastroesophageal junction or fundus. The onset of the obstruction is attributed to an extreme increase in intra-abdominal pressure caused by the action of lifting weights. (+info)
Jaundice with hypertrophic pyloric stenosis as an early manifestation of Gilbert syndrome.
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Jaundice associated with hypertrophic pyloric stenosis was recognised in three patients; previous reports have suggested that this is a possible early manifestation of Gilbert syndrome. Most patients with Gilbert syndrome are homozygous for a (TA)(7)TAA polymorphism in the gene promoter coding for bilirubin glucuronosyltransferase. Two of the reported patients were homozygous for the (TA)(7)TAA polymorphism whereas the third was heterozygous for the same polymorphism. Furthermore, no other factors contributing to jaundice in the three patients were found. These results suggest that jaundice associated with hypertrophic pyloric stenosis is due to molecular defects within the gene promoter. (+info)
Pyloric ratio: efficacy in the diagnosis of hypertrophic pyloric stenosis.
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We sought to define a weight independent, highly sensitive and specific measurement to diagnose hypertrophic pyloric stenosis. A retrospective review of 87 children was performed. We determined the pyloric ratio (wall thickness/pyloric diameter) and its relationship to weight and compared it to standard criteria. The average pyloric ratios in normal children and in those with hypertrophic pyloric stenosis were 0.205 and 0.325, respectively (P < 0.001). A pyloric ratio of 0.27 yielded a sensitivity and specificity of 96% and 94%, respectively. The pyloric ratio maintained a linear relationship to weight in normal patients and those with hypertrophic pyloric stenosis. We conclude the pyloric ratio can be a highly sensitive, specific, and weight independent indicator of hypertrophic pyloric stenosis. (+info)
Hypertrophic pyloric stenosis in infants following pertussis prophylaxis with erythromycin--Knoxville, Tennessee, 1999.
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In February 1999, pertussis was diagnosed in six neonates born at hospital A in Knoxville, Tennessee. Because a health-care worker at hospital A was most likely the source of exposure, the local health department recommended on February 25, 1999, that erythromycin be prescribed as postexposure prophylaxis for the approximately 200 infants born at hospital A during February 1-24, 1999. In March 1999, local pediatric surgeons noticed an increased number of cases of infantile hypertrophic pyloric stenosis (IHPS) in the area, with seven cases occurring during a 2-week period. All seven IHPS cases were in infants born in hospital A during February who were given erythromycin orally for prophylaxis following possible exposure to pertussis, although none had pertussis diagnosed. The Tennessee Department of Health and CDC investigated the cluster of IHPS cases and its possible association with use of erythromycin. This report summarizes the results of the investigation, which suggest a causal role of erythromycin in this cluster of IHPS cases. (+info)
Molecular cytogenetic characterisation of partial trisomy 9q in a case with pyloric stenosis and a review.
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Partial trisomy 9q represents a rare and heterogeneous group of chromosomal aberrations characterised by various clinical features including pyloric stenosis. Here, we describe the case of a 1 year old female patient with different dysmorphic features including pyloric stenosis and prenatally detected partial trisomy 9q. This partial trisomy 9q has been analysed in detail to determine the size of the duplication and to characterise the chromosomal breakpoints. According to the data gained by different molecular cytogenetic techniques, such as fluorescence in situ hybridisation (FISH) with whole and partial chromosome painting probes, yeast artificial chromosome (YAC) probes, and comparative genomic hybridisation (CGH), the derivative chromosome 9 can be described as dup(9)(pter-->q22. 1::q31.1-->q22.1::q31.1--> q22.1::q31.1-->qter). Four breakpoint spanning YACs have been identified (y806f02, y906g6, y945f5, and y747b3) for the proximal breakpoint. According to this new case and previously published data, the recently postulated putative critical region for pyloric stenosis can be narrowed down to the subbands 9q22.1-q31.1 and is the result of either partial trisomy of gene(s) located in this region or a gene disrupted in 9q31. (+info)
Laparoscopic pyloroplasty in idiopathic hypertrophic pyloric stenosis in an adult.
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BACKGROUND AND OBJECTIVES: Idiopathic hypertrophic pyloric stenosis, in adults, is a rare disease. Partial gastrectomy, gastroenterostomy, pyloromyotomy, pyloroplasty and endoscopic dilatation have all been recommended with variable results. A 54-year-old white female is presented with the onset of symptoms of idiopathic hypertrophic pyloric stenosis one year prior to operation. Two endoscopic pyloric sphincter balloon dilatations provided only temporary relief. METHOD: A laparoscopic pyloroplasty was performed. RESULT: The patient tolerated a solid diet on postoperative day three. The patient was symptom-free at a 13 month follow-up. CONCLUSIONS: Idiopathic hypertrophic pyloric stenosis in adults can be treated with laparoscopic pyloroplasty, offering a minimally invasive alternative to open repair. (+info)