Cases reported "Pyloric Stenosis"

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1/17. 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.
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keywords = atresia
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2/17. 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.
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keywords = atresia
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3/17. Radial aplasia, poikiloderma and auto-immune enterocolitis--new syndrome or severe form of rothmund-thomson syndrome?

    A syndrome is described in three isolated patients in whom the main features are bilateral radial aplasia, short stature, an inflammatory based 'elastic' pyloric stenosis, a pan-enteric inflammatory gut disorder that appears to be due to an autoimmune process, and poikiloderma. Other features in individual cases include cleft palate, micrognathia, anal atresia, patellar aplasia/hypoplasia and sensorineural deafness. This combination may represent a severe form of rothmund-thomson syndrome or possibly a previously unrecognized condition.
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keywords = atresia
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4/17. Congenital focal segmental glomerulosclerosis associated with beta4 integrin mutation and epidermolysis bullosa.

    We report the occurrence of congenital nephrotic-range proteinuria secondary to focal segmental glomerulosclerosis in an infant with epidermolysis bullosa and pyloric atresia. A homozygous missense mutation, R1281W, in exon 31 of the beta4 integrin gene, ITGB4, was identified. By immunofluorescence, beta4 integrin expression was reduced in both dermal keratinocytes and glomerular podocytes. This is the first demonstration of beta4 integrin expression in human glomeruli. We postulate a role for altered beta4 integrin function in the mediation of the glomerular permeability defect.
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keywords = atresia
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5/17. Alpha 6 beta 4 integrin abnormalities in junctional epidermolysis bullosa with pyloric atresia.

    Junctional epidermolysis bullosa with pyloric atresia (JEB-PA) (MIM 226730) is an autosomal recessive disorder resulting from mutations in the genes encoding alpha 6 beta 4 integrin (ITGA6 and ITGB4). Clinically, it is characterized by mucocutaneous fragility and gastrointestinal atresia, which most commonly affects the pylorus. Additional features of JEB-PA include involvement of the urogenital tract, aplasia cutis and failure to thrive. While most affected individuals have a poor prognosis resulting in death in infancy, others have milder clinical features and a better prognosis. We report two previously undescribed homozygous ITGB4 mutations in two unrelated families, which resulted in severe skin blistering, pyloric atresia and lethality in infancy. Delineation of the mutations was used to undertake dna-based prenatal diagnosis in subsequent pregnancies at risk for recurrence in both families. We review all previously published ITGA6 and ITGB4 mutation reports to help define genotype--phenotype correlation in this rare genodermatosis.
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ranking = 7
keywords = atresia
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6/17. cystic fibrosis and infantile hypertrophic pyloric stenosis: is there an association?

    cystic fibrosis (CF) is the most common lethal genetic disease among Caucasian populations. The generally accepted incidence of CF in the united states is 1 in 3,200 in the Caucasian population. Intestinal obstructions and atresias have been described among patients with CF. An association of CF with infantile hypertrophic pyloric stenosis (IHPS) has not been previously documented. A review in our clinic of 72 patients with CF revealed IHPS in two. The incidence of 2.7% is greater than the 0.3% incidence expected in the general population. This ninefold increase in IHPS in patients with CF suggests an association between the two and warrants further investigation.
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keywords = atresia
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7/17. Intermittent 'double bubble' sign in a case of congenital pyloric atresia.

    We report a case of congenital pyloric atresia in which two cystic masses in the fetal upper abdomen and polyhydramnios were observed from 24 weeks' gestation to term. The two cystic masses were thought to indicate the 'double bubble' sign in utero, leading to a misdiagnosis of congenital duodenal obstruction. At 33 weeks' gestation, continuous ultrasound observation of the two cystic masses was made for 60 min. During the periods when gastric peristalsis was absent, the 'double bubble' sign was observed. However, during the periods when gastric peristalsis was present, the 'double bubble' sign disappeared. In retrospect, both cystic masses were stomach and the 'double bubble' sign disappeared with peristalsis of the antrum. In order to differentiate from congenital duodenal obstruction, whole stomach configuration should be delineated by continuous observation covering periods when gastric peristalsis is active as well as quiet.
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ranking = 5
keywords = atresia
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8/17. Patient with partial trisomy 9q and learning disability but no pyloric stenosis.

    Partial trisomy of the long arm of chromosome 9 represents a very rare and heterogeneous group of chromosomal aberrations. Associated clinical features include learning disability and pyloric stenosis. We present the first patient to be reported with a duplication of the chromosome region 9q22.1-->q33. The patient (female, age 17 years) presented with growth retardation, microcephaly, facial dysmorphia, oesophageal atresia, aortic stenosis, ventricular septal defect, atrial septal defect II, hypothyroidism, and learning disability, but no pyloric stenosis. A review of all cases of partial trisomy 9q reported in the literature demonstrates that learning disability is a characteristic feature of this group of chromosomal aberrations. However, there are cases of duplications of the same chromosome 9 material, with and without pyloric stenosis. This study provides new information for future genetic counselling, especially in cases of prenatal diagnosis of partial trisomy 9q.
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ranking = 1
keywords = atresia
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9/17. Atretic, obstructive proximal duodenal mass associated with annular pancreas and malrotation in a newborn male.

    A case is described of duodenal atresia involving the first portion of the duodenum associated with a partial annular pancreas, complete nonrotation and nonfixation of the intestines, and a congenitally small glottic region in a newborn male. The atretic segment was a 1.3 cm mass adjacent to the pylorus unlike previous reports of duodenal atresia. The diagnosis was delayed for seven days until the child was fed and had bowel movements. Pyloroduodenostomy was performed resulting in cure. An older sibling had a duodenal web requiring previous duodenoduodenostomy.
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ranking = 2
keywords = atresia
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10/17. Congenital gastric outlet obstruction.

    Two additional cases of congenital gastric outlet obstruction are presented. A comprehensive review of the literature was undertaken and as a result a classification for congenital gastric outlet obstruction is suggested. The management of the cases reported in the literature has also been reviewed together with the genetics of pyloric atresia and associated dermatologic lesions. Guidelines are given for the management of congenital gastric outlet obstruction with and without associated, inherited dermatologic conditions.
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ranking = 1
keywords = atresia
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