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At least 649 records · Page 36Linked to original sources
PubMed1987

A 26-month-old child with Marden-Walker syndrome and pyloric stenosis.

We recently examined a 26-month-old boy with abnormal face, blepharophimosis, hypertelorism, apparently low-set ears, micrognathia, arachnodactyly, talipes equinovarus, and joint contractures. Subsequently he manifested failure to thrive, respiratory infections, and developmental delay. These congenital anomalies and associated findings are consistent with a diagnosis of the Marden-Walker syndrome. He also had mild pyloric stenosis and duodenal bands, not previously reported in this syndrome. This syndrome appears to be an autosomal recessive trait in some families. A summary of findings of the 16 previous published patients is presented.

Abnormalities, Multiple↗
PubMed2005

A study of 11,003 patients with hypertrophic pyloric stenosis and the association between surgeon and hospital volume and outcomes.

AIM: The availability of large clinical databases allows for careful evaluation of surgical practices, indicators of quality improvement, and cost. We used a large clinical database to compare the effect of surgeon and hospital volume for the care of children with hypertrophic pyloric stenosis (HPS). METHODS: Patients with International Classification of Diseases-9 codes for HPS and pyloromyotomy were selected from the 1994 to 2000 National Inpatient Samples database. Multiple and logistic regression models were used to evaluate the risk-adjusted association between provider volume and outcomes. RESULTS: Postoperative complications occurred in 2.71% of patients. Patients operated on by low- and intermediate-volume surgeons were more likely to have complications compared with those operated on by high-volume surgeons (95% confidence interval [CI], 1.25-3.78 and 95% CI, 1.25-2.69, respectively). Patients operated at low-volume hospitals were 1.6 times more likely to have complications compared with those operated at intermediate- or high-volume hospitals (95% CI, 1.19-2.20). Procedures performed at high-volume hospitals were less expensive than those at intermediate-volume hospitals by a margin of 910 dollars (95% CI, 443-1377 dollars). CONCLUSIONS: These data represent the largest study to date on the epidemiology, complication rate, and cost for care for HPS. Patients treated by both high-volume surgeons and at high-volume hospitals have improved outcomes at less cost.

Clinical Competence↗
PubMed1987

Membranous atresia of esophagus associated with pyloric stenosis.

Membranous atresia of the esophagus without tracheoesophageal fistula (TEF) is very rare, only four cases have been reported since 1928. We present a case in whom a thick membrane was present 2 cm proximal to the diaphragm. The membrane was resected and the longitudinal esophagotomy wound was closed transversely. The postoperative course was complicated with hyperbilirubinemia and hypertrophic pyloric stenosis (HPS). The problems of the feeding tube used for diagnostic evaluation of this rare esophageal anomaly are discussed. In spite of its low incidence, the potentiality of HPS as a cause of postoperative vomiting in esophageal atresia should be borne in mind in order to avoid delay in diagnosis.

Esophageal Atresia↗
PubMedDate not supplied

[Role of nitric oxide synthase in the etiopathogenesis of hypertrophic pyloric stenosis in infants]

OBJECTIVE: To experimentally reproduce, in rats, the findings corresponding to the histopathology of infantile hypertrophic pyloric stenosis (IHPS), using nitric oxide synthase (NOS) inhibitor (L-NAME). METHODS: L-NAME was administered to pregnant rats (L-NAME group), from the 14th gestational day on in order to reproduce the model of NOS inhibition in the production of IHPS. This group was then compared to control animals. After birth, all the animals in the L-NAME group were maintained under NOS inhibition until the 42nd day of life, when they were sacrificed. The control animals, which did not receive any kind of drug, were also sacrificed on the 42nd day of life. The animals and their internal organs were analyzed and weighed. The pyloric region was technically prepared and observed through light microscopy. RESULTS: The L-NAME group presented lower body and intestinal weight and higher gastric weight than the control group. Light microscopy revealed hypertrophy of the circular smooth muscle layer of the pyloric muscle in L-NAME animals. CONCLUSIONS: This work reproduced an experimental model of an IHPS study, confirming the effect of NOS blockade on the pyloric musculature.

Journal Article↗
PubMed1979

Extraordinary hyperbilirubinemia in a neonate with idiopathic hypertrophic pyloric stenosis.

This case describes an extraordinarily elevated total bilirubin level that reverted to normal 9 1/2 wk after Fredet-Ramstedt pyloromyotomy. Although the etiology of jaundice occurring in patients with IHPS remains uncertain, theories implicating inhibition of the glucuronyl transferase system have been proposed. Infants with IHPS have a documented hypergastrinemia. An hypothesis is offered, illustrated by this case, to explain the inhibition of the glucuronyl transferase system with resultant hyperbilirubinemia by the hypergastrinemia of idiopathic hypertrophic pyloric stenosis.

Exchange Transfusion, Whole Blood↗
PubMed1997

[Pyloric stenosis complicated by Wernicke-Korsakoff syndrome].

The Wernicke-Korsakoff syndrome (WKS) is a picture of oculomotor alterations, ataxia and confusion presented in chronic alcoholics. It has more rarely been described in non alcoholic patients with malnutrition. The case of a patient with ulcerous peptic disease of long evolution who consulted for a picture compatible with WKS following clinical manifestations of repeated vomiting secondary to complete pyloric stenosis is presented. The peculiarity of the picture and the convenience of prevention in malnourished patients receiving intravenous glucose sera is discussed.

Alcohol Amnestic Disorder↗
PubMed1978

Electron microscopic observations in infantile hypertrophic pyloric stenosis (IHPS).

Electron microscopic observations of the pyloric parasympathetic ganglia in IHPS have demonstrated, contrary to previous reports, that no abnormalities exist in the neurons and interstitial cells of the plexuses or the cells of the pyloric smooth muscles. The significance of finding a small number of altered large axons is unclear. In view of finding essentially normal morphology, a "functional" etiology of this disease is suggested.

Female↗
PubMed1983

Early gastric cancer and its complications: bleeding, perforation and pyloric stenosis.

Some cases of early gastric cancer are accompanied with complications of the upper gastro-intestinal tract. The characteristics of these complications were investigated, and the problems of diagnosis and treatment were discussed. Out of 297 cases of early gastric cancer, 18 cases were accompanied with complications of the upper gastro-intestinal tract, including 11 cases of bleeding, a case of perforation and 6 cases of pyloric stenosis. All 18 cases were of the macroscopically depressed type, and about 85 percent of the 297 early gastric cancer cases were of the depressed type. The depressed lesions were often accompanied by ulceration which was an important factor causing the complications, and the mechanism of which appeared to be the same as that of a benign ulcer. There are some cases of early gastric cancer which are discovered by their complications, and it would be more difficult to find an early gastric cancer lesion if there were a benign lesion at the same time. Therefore, it is necessary to take much care when diagnosing and treating cases which have such complications. An endoscopic examination before the operation is especially important, and a biopsy is indispensable.

Adult↗
PubMed1980

[Propionic acidemia associated with hypertrophic pyloric stenosis and bouts of severe hyperglycemia (author's transl)].

A newborn is presented with hyperexcitability, drowsiness and later-on with frequent vomiting and muscular hypotonia. Examination of the urine by gas chromatography-mass spectrometry lead to the diagnosis of propionic acidemia which was confirmed enzymatically in fibroblasts. Two unusual features were encountered in this case: There were severe bouts of hyperglycemia with blood glucose values up to 396 and 747 mg/100 ml; furthermore x-ray studies and autopsy revealed a hypertrophic pyloric stenosis.

Autopsy↗
PubMedDate not supplied

Infantile hypertrophic pyloric stenosis after pertussis prophylaxis with erythromcyin: a case review and cohort study.

BACKGROUND: In February, 1999, a local US health department identified a cluster of pertussis cases among neonates born at a community hospital and recommended oral erythromycin for post-exposure prophylaxis for about 200 neonates born at that hospital between Feb 1 and Feb 24, 1999. We investigated a cluster of seven cases of infantile hypertrophic pyloric stenosis (IHPS) that occurred the following month among the neonates who had received erythromycin. METHODS: We obtained a masked, independent review of the IHPS ultrasonography diagnoses, calculated the monthly IHPS incidence, and compared index and historical (1998-99) IHPS cases with respect to several characteristics including erythromycin exposure. We used a retrospective cohort of infants born in January and February, 1999, to investigate further erythromycin exposure and development of IHPS. FINDINGS: An independent review confirmed the ultrasonographic diagnoses of all seven index IHPS cases. All index cases versus none of the historical IHPS cases had been given erythromycin for pertussis prophylaxis. The IHPS rate for infants born in the hospital in February, 1999, was 32.3 per 1000 liveborn infants, representing nearly a seven-fold increase over 1997-98 (relative risk 6.8 [95% CI 3.0-15.7]). Among infants born in January and February, 1999, erythromycin was associated with IHPS (absolute risk 4.5%, relative risk infinity [1.7-infinity]). INTERPRETATION: Neonates receiving oral erythromycin may have an increased risk of IHPS. The risks and benefits of erythromycin for neonatal pertussis prophylaxis should be re-evaluated, and caution should be used in defining risk groups for prophylaxis.

Administration, Oral↗
PubMed2000

[Infantile hypertrophic pyloric stenosis. Decreasing incidence].

The aim of this study was to confirm a clinical suspicion that the frequency of this disease is in decline. A retrospective review of case notes of all children admitted and operated in all hospitals in Viborg County during the period of 1.1.1973 to 31.12.1997 was performed. The local survey was supplemented with a national survey. One hundred and forty-seven patients from Viborg County were operated for infantile hypertrophic pyloric stenosis during the period described. Divided into five year periods the number per 1000 living births was consecutively 1.6, 2.4, 2.0, 2.8 and 0.9. The same tendency was found nationally, Table 1. The decrease started in 1993 and has since strengthened. The reason is unknown, but there is a relation regarding to the timing of a change in recommendations concerning positioning of infants during sleep. However, a causal relation is unknown.

Denmark↗
PubMed2003

Distribution of heme oxygenase-2 in nerves and interstitial cells of Cajal in the normal pylorus and in infantile hypertrophic pyloric stenosis.

CONTEXT: Interstitial cells of Cajal (ICCs) are pacemaker cells, which are of fundamental importance in regulating gastrointestinal motility. Recent evidence suggests that carbon monoxide is a neurotransmitter involved in neurotransmission between ICC and smooth muscle cells. Heme oxygenase-2 (HO-2) is the major physiological mechanism for the generation of carbon monoxide in the enteric nervous system. OBJECTIVE: To investigate the immunocolocalization of HO-2 and ICCs in the normal pylorus and in infantile hypertrophic pyloric stenosis (IHPS). DESIGN: Specimens from 18 infants with IHPS and 8 control specimens were examined using double-immunostaining with c-Kit and HO-2 antibodies. The immunolocalization was detected with the help of confocal laser scanning microscopy. RESULTS: Abundant HO-2 immunoreactivity was found in ICCs in the smooth muscle layer of normal pylorus. There was a decrease in the number of ICCs and lack of HO-2 immunoreactivity in ICCs in IHPS. CONCLUSIONS: The results of the present study provide the first evidence for the presence of HO-2 in ICCs in the normal human pylorus. The lack of ICCs and HO-2 in IHPS suggests impaired intracellular communication between ICCs and smooth muscle cells, contributing to motility dysfunction in IHPS.

Enteric Nervous System↗
PubMed1999

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.

Anti-Bacterial Agents↗
PubMed1978

A goodness-of-fit test for the polygenic threshold model: application to pyloric stenosis.

A new test of goodness of fit for the polygenic threshold model is proposed. This test, when applied to disorders showing different incidence rates in males and females, is designed to account for ascertainment in more detail than previously done by other investigators. This is accomplished by computing the expected distribution of nuclear families with more than one affected sib conditioned on several family-dependent variables, including whether each family was ascertained via only affected boys or via at least one affected girl. A direct measure of the probability of observing a data set is thereby derived. The test, when applied to data on pyloric stenosis, exposes the critical nature of the ascertainment procedures. Different levels of statistical significance are obtained when mode of ascertainment is taken into account than when the mode of ascertainment is ignored.

Female↗
PubMed1995

Tuberculous mesenteric lymphadenitis presenting as pyloric stenosis.

A 17-year-old Filipina with a three-year history of intermittent, projectile vomiting and weight loss was admitted. A diagnosis of peptic ulcer disease was made, but she was unresponsive to antiulcer therapy. Fever, anorexia, cough, and exposure to tuberculosis were denied. Chest x-ray was normal. On barium swallow, the stomach and duodenal bulb were dilated. Endoscopic antral biopsy showed chronic inflammation. Computed tomography revealed enlarged periportal and peripancreatic lymph nodes and an intrahepatic mass. Liver biopsy failed to show any acid-fast bacilli. On laparotomy, the pyloroduodenal area was extrinsically compressed by surrounding lymph nodes, which, on biopsy, contained granulomatous inflammation with caseation necrosis and Langhan's giant cells. Gastrojejunostomy was done and antituberculous drugs were given. Pyloric stenosis due to tuberculosis is rare, but it should be considered in patients who come from areas where the disease is endemic. Medical management for such cases may suffice.

Adolescent↗
PubMed2000

Glial-derived growth factor signaling pathway in infantile hypertrophic pyloric stenosis.

BACKGROUND/PURPOSE: Glial-derived growth factor (GDNF), which is the ligand of RET is reported to be essential for the development of enteric nervous system. A GDNF knockout mouse model has shown that the gastric region is a critical passing site between GDNF-RET-independent neuroblasts (colonizing the esophagus) and GDNF-RET-dependent neuroblasts (colonizing the small and large bowel). The earliest GDNF site of production is the mesenchyme and the outer smooth muscle cell (SMC) layer of the developing bowel. In the mature gastrointestinal tract the presence of GDNF is restricted to enteric glial cells. The aim of this study was to investigate the expression of GDNF and RET in infantile hypertrophic pyloric stenosis (IHPS). METHODS: Full-thickness muscle biopsy specimens were obtained from 8 IHPS patients at pyloromyotomy and from 8 age-matched controls without gastrointestinal disease. Indirect immunohistochemistry was performed using avidin-biotin-peroxidase complex method with anti-GDNF and anti-RET antibodies. Quantitative analysis was performed using sandwich-type enzyme-linked immunosorbent assay (ELISA) for GDNF. RESULTS: GDNF- and RET-positive nerve fibers were absent or markedly reduced in IHPS compared with controls. GDNF was expressed strongly by smooth muscle cells of both muscular layers in IHPS, whereas no GDNF expression was detected in pyloric muscle of controls. The quantity of total GDNF in IHPS was significantly higher than in controls (P < .01). CONCLUSIONS: The lack or markedly decreased number of GDNF-positive nerve fibers in IHPS supports the hypothesis of a selective immaturity of the enteric glia in the muscular layers in IHPS. The strong expression of GDNF in smooth muscle cells in IHPS and the increased levels of GDNF in IHPS suggest a compensatory mechanism by which the smooth muscle cells continue to produce GDNF until maturation of the enteric glial cells occurs.

Drosophila Proteins↗