1/173. Functional intestinal obstruction due to deficiency of argyrophil neurones in the myenteric plexus. Familial syndrome presenting with short small bowel, malrotation, and pyloric hypertrophy.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.- - - - - - - - - - ranking = 1keywords = pyloric (Clic here for more details about this article) |
2/173. Excessive nodular hyperplasia of brunner glands associated with gastric hypersecretion and lipomatous atrophy of the pancreas.The case of a 34-year-old woman complaining of diarrhoea and abdominal pain is presented. Contrast radiography and endoscopy showed multiple polypoid tumours in the second part of the duodenum. Moreover, a severe fatty infiltration of the pancreas was shown by magnetic resonance and computed tomography scans. Due to pain, pancreatoduodenectomy (Whipple operation) was performed, and subsequent histopathologic examinations showed excessive Brunner gland hyperplasia of the duodenum and severe lipomatous atrophy of the pancreas. The occurrence of these two rare conditions in one patient has not been described previously, and it is conceivable that the lipomatous atrophy and exocrine insufficiency of the pancreas may have caused a compensatory stimulation of the submucosal structures of the duodenum.- - - - - - - - - - ranking = 0.0053399810640006keywords = gland, mucosa (Clic here for more details about this article) |
3/173. Novel gastrointestinal tract manifestations in juvenile dermatomyositis.We report a case of juvenile dermatomyositis in which a dilated atonic esophagus was associated with delayed gastric emptying and intestinal mucosal thickening, resulting in a radiographic "stacked coin" appearance. These findings, which can also occur in infectious, neoplastic, or other immune-mediated diseases, broaden the spectrum of gastrointestinal tract manifestations in juvenile dermatomyositis. physicians should be alert for these treatable manifestations in children with myositis who present with unexplained gastrointestinal symptoms, which are reversible with immunosuppressive therapy.- - - - - - - - - - ranking = 5.2636932948654E-5keywords = mucosa (Clic here for more details about this article) |
4/173. The development of hypertrophic pyloric stenosis in a patient with prostaglandin-induced foveolar hyperplasia.BACKGROUND: Hypertrophic pyloric stenosis (HPS) has been described in association with several obstructive antropyloric lesions including idiopathic foveolar hyperplasia (gastric mucosal hypertrophy), feeding tubes, eosinophilic gastroenteritis, and hypertrophic antral polyps. Non obstructive antral webs have also been described with HPS. PATIENT AND methods: We present a case of gastric-outlet obstruction in association with HPS, namely, prostaglandin-induced foveolar hyperplasia. This entity has been previously described, but rarely in association with HPS. We report a female infant requiring prostaglandin therapy for pulmonary atresia who developed dose-related prostaglandin-induced foveolar hyperplasia and symptoms of progressive non-bilious vomiting. RESULTS: Initially, ultrasonography demonstrated evidence of antral mucosal hypertrophy as the cause for gastric-outlet obstruction. The patient subsequently developed progressive thickening of the antropyloric muscle, resulting in sonographic appearances of hypertrophic pyloric stenosis. Pyloromyotomy was eventually required for treatment of HPS. CONCLUSION: A common denominator of most of the above-described entities is thickening and/or hypertrophy of the antral mucosa. We suggest that the antropyloric musculature may hypertrophy in an effort to overcome the gastric-outlet obstruction caused by the adjacent thickened antral mucosa. In other words, these entities may represent examples of "secondary" hypertrophic pyloric stenosis.- - - - - - - - - - ranking = 2.0076128295153keywords = pyloric, gland, mucosa (Clic here for more details about this article) |
5/173. Hypertrophic pyloric stenosis in infants following pertussis prophylaxis with erythromycin--Knoxville, tennessee, 1999.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.- - - - - - - - - - ranking = 1keywords = pyloric (Clic here for more details about this article) |
6/173. An uncommon association of H-type tracheoesophageal fistula with infantile hypertrophic pyloric stenosis.Although infantile hypertrophic pyloric stenosis following esophageal atresia repair is known, infantile hypertrophic pyloric stenosis following H-type tracheoesophageal fistula has not been encountered previously. A case of H-type tracheoesophageal fistula and infantile hypertrophic pyloric stenosis is presented. The patient, operated on for H-type fistula, a rare congenital anomaly of the esophagus, on the tenth day of life was readmitted 19 days later because of continuous vomiting after every feeding. The clinical findings and physical and radiological examinations revealed infantile hypertrophic pyloric stenosis which required surgical treatment. It is suggested that the association of H-type tracheoesophageal fistula with infantile hypertrophic pyloric stenosis is coincidental, given the estimated incidence of one in every 84,375,000 males and 337,500,000 females.- - - - - - - - - - ranking = 1.8keywords = pyloric (Clic here for more details about this article) |
7/173. Proportion of cells with paternal 11p15 uniparental disomy correlates with organ enlargement in Wiedemann-beckwith syndrome."Genetic mosaicism" describes the presence of two or more populations of cells within a single individual that differ in their genomic constitution. Although the occurrence of asymmetric overgrowth in Wiedemann-Beckwith syndrome (WBS) suggests that mosaicism has some role in the WBS phenotype, no direct evidence for this has been published. WBS is a congenital overgrowth syndrome with variable phenotype linked to the imprinted gene cluster on chromosome region 11p15. We have performed a molecular survey of multiple organs and tissues in a case of WBS with a high degree of mosaic paternal 11p15 uniparental disomy (UPD). The organs most severely affected were those with the highest percentage of cells with UPD. In particular there was a striking difference in the degree of mosaicism for 11p15 UPD between the extremely enlarged left adrenal and non-enlarged right adrenal gland. This result indicates that the proportion of paternal 11p15 UPD cells correlates with the tissue phenotype of WBS. Our results suggest that high proportions of abnormal cells result from a combination of stochastic events and cell selection. mosaicism may explain the variable phenotypes including hemihyperplasia and predisposition to childhood cancers in WBS patients.- - - - - - - - - - ranking = 0.0010574688262104keywords = gland (Clic here for more details about this article) |
8/173. Laparoscopic pyloroplasty in idiopathic hypertrophic pyloric stenosis in an adult.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.- - - - - - - - - - ranking = 1.6keywords = pyloric (Clic here for more details about this article) |
9/173. Primary hypertrophic colonopathy.We report a 45-year-old man and a 60-year-old woman who presented with features of intermittent intestinal obstruction. barium enema revealed narrowing at the pelvic-rectal junction in the man, and from the pelvic colon to the anal verge in the woman. histology of the resected sections showed marked hypertrophy of the muscularis propria in both cases, with normal mucosa, submucosa and myenteric plexus. Both patients are asymptomatic at 4 years' and 2 years' follow up. This entity of primary hypertrophic colonopathy may be a variant of primary visceral myopathy.- - - - - - - - - - ranking = 0.00010527386589731keywords = mucosa (Clic here for more details about this article) |
10/173. Infantile hypertrophic pyloric stenosis in a 5-month-old baby: case report.Hypertrophic pyloric stenosis is commonly seen in infants 2 to 4 weeks old. We report a case of pyloric stenosis diagnosed in a boy 5 months and 11 days old suffering from the sudden onset of vomiting. Gastric volvulus was initially diagnosed at another hospital. Abdominal ultrasonography at first using an Acuson 5-MHz transducer revealed a negative diagnosis. However, a tubular pyloric mass measuring 5.5 mm in thickness, 15 mm in the transverse diameter, and 2.0 cm in length was detected by a 7-MHz transducer immediately after the infant vomited. On physical examination, no abdominal mass was palpable. This suggested that this might have been a case of hypertrophic pyloric stenosis which was missed until the infant was older than 5 months. We believe this is the oldest reported case of infantile hypertrophic pyloric stenosis in taiwan.- - - - - - - - - - ranking = 1.8keywords = pyloric (Clic here for more details about this article) |
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