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[Results of periumbilical approach to hypertrophic pyloric stenosis treatment. Personal experience].

BACKGROUND: Ramstedt's pyloromyotomy is the universally accepted treatment for hypertrophic pyloric stenosis. It could be performed through different surgical approaches: Robertson muscle splitting incision, transverse right transrectal incision, median longitudinal incision laparoscopic approach and circumumbilical incision. METHODS: This latter technique was successfully personally utilized for the treatment of 33 consecutive patients; 29 patient were male and 4 female, age ranged from 15 to 52 days (median value 32.6 days), previously full term, normal weighing newborns. In all patients but one, the large pyloric mass bulged through the incision and the Ramstedt's pyloromyotomy was performed as usual. In one case, the pyloromyotomy has been done through the incision by holding the large pylorus firmly beneath the abdominal wall. The operative time has been quite similar to that obtained with the transverse right incision. The mean hospitalization period has been of four days. RESULTS: No operative or postoperative complications were observed. Particularly, despite no antibiotics were used, no wounds infections occurred. CONCLUSIONS: The esthetic result was satisfying in all cases after one month follow-up.

Female↗

[Anatomo-clinical and echocardiographic correlations in hypertrophic pyloric stenosis. Apropos of 32 cases].

The authors present diagnosis particularities in 32 cases of hypertrophic pyloric stenosis, from 1980 to 1984, whom 21 cases since 1982 (25 boys, 7 girls). The diagnosis of HPS was confirmed by sonographic and roentgen explorations, isolated or associated: ultrasound alone 3 cases, ultrasound and roentgen combined 29 cases either roentgen first (22) or ultrasound first (7). Fiability was made for 22 cases; two times, error came from false interpretation; eight times were "false negative" results, corrected by roentgenogram, and corresponding with a voluminous pylorus (3) or little, sclerosis pyloric lesions (5). Amelioration of results give, from the last year, right diagnosis in 85 per cent, about 13 cases.

Echocardiography↗

[Echography of pyloric stenosis].

In order to assess the accuracy of ultrasonographic (US) criteria for the diagnosis of pyloric stenosis (PS), the record of 78 infants admitted to Hôpital Sainte-Justine with the clinical diagnosis of PS during the past year was reviewed. Fifty-eight patients had PS at laparatomy. Thirty-four of these had US with 30 true positives and four "doubtful" findings. Twenty-two had normal US. Two of these had PS, nine gastroesophageal reflux and five normal barium studies of the upper gastrointestinal tract (the last six were followed clinically and remained well). The following were criteria for US diagnosis of PS: pyloric anteroposterior diameter greater than or equal to 1.5 cm, length greater than or equal to 2 cm or a muscle thickness greater than or equal to 4 mm. This series contains no false positive US studies and two false negatives, for a positive predictive accuracy of 94% and a negative predictive accuracy of 100% when we measure the length greater than or equal to 2 cm or combine a length of less than or equal to 2 cm with a muscle thickness greater than or equal to 4 mm.

Female↗

[Jaundice associated with congenital hypertrophic pyloric stenosis (author's transl)].

The clinical and biological findings in six infants with congenital hypertrophic pyloric stenosis with associated jaundice are described. Pathogenesis of this association is not known but current hypothesis are based on either a decrease in the activity of hepatic glucuronyl transferase or an increase in the activity of intestinal glucuronidase. Correction of dehydration and alkalosis does not modify the hyperbilirubinemia which only disappears after pyloromiotomy.

Glucuronidase↗

Genetic evidence for the neuronal nitric oxide synthase gene (NOS1) as a susceptibility locus for infantile pyloric stenosis.

The etiological role of the gene for neuronal nitric oxide synthase (NOS1) in infantile pyloric stenosis (PS) was investigated by analysis of two intragenic polymorphisms (NOS1a and NOS1b) in 27 families. There was significant overall transmission disequilibrium between PS and NOS1a (P = .006). Consideration of each allele independently revealed a highly significant tendency for allele 7 (210 bp) to be preferentially transmitted to the affected offspring (P = .0006). These observations suggest that NOS1 is a susceptibility locus for PS.

Alleles↗

Factors associated with infantile hypertrophic pyloric stenosis.

We examined perinatal factors in relation to the rise in incidence of infantile hypertrophic pyloric stenosis among children in Olmsted County, Minnesota, during the period from 1950 through 1984. Primogeniture was associated with male infants but not female infants; some factor related to primogeniture, such as breast-feeding, may be etiologically important. Our data did not support a role for maternal disease, use of doxylamine succinate-pyridoxide hydrochloride (Bendectin), or an infectious process. Further study should be directed toward environmental factors associated with primogeniture.

Birth Order↗

Increasing infantile hypertrophic pyloric stenosis? Experience in an overseas military hospital.

Fifty-four children with proven infantile hypertrophic pyloric stenosis (IHPS) were treated by Ramstedt's pyloromyotomy during a 14-year period. Between the years 1986 and 1989 the mean incidence of the disease was noted to have risen by 2.72 per 1000 live births over the preceding 10 years. Statistical analysis confirmed a significant upward trend between the years 1976 and 1989. Analysis of the majority of the cases revealed comparable perioperative morbidity rates with other UK district general hospitals. There was no operative mortality. The character of IHPS in military personnel overseas has more in common with UK than international trends. The incidence in this subgroup may be rising.

Germany↗

Development of infantile hypertrophic pyloric stenosis in patients treated for oesophageal atresia. A case report.

Two cases of infantile hypertrophic pyloric stenosis (IHPS) developed in 74 patients treated for oesophageal atresia. Treatment of oesophageal atresia is frequently followed by vomiting and failure to thrive due to gastrooesophageal reflux or anastomotic stricture. The diagnose of IHPS must be considered in such patients under the age of 8 weeks as symptoms of IHPS are similar to those of gastrooesophageal reflux. The diagnose of IHPS is strongly supported by the presence of a non-carbonic alkalosis. Contrast studies including oesophagus, stomach and duodenum are to be made early in the course, as clinical signs of IHPS are often masked.

Esophageal Atresia↗

The epidemiology of infantile hypertrophic pyloric stenosis in Sweden 1987-96.

AIMS: To find out whether the incidence of infantile hypertrophic pyloric stenosis (IHPS) has changed over the past decade, and if so, to investigate possible contributory factors. METHODS: All infants undergoing pyloromyotomy for IHPS in Sweden between 1987 and 1996 were studied. Using the national patient registers the yearly incidence was determined and evaluated in relation to sex, latitude, urbanisation, and type of surroundings by use of a Poisson model. RESULTS: There was a substantial decline from 2.7/1000 to 0.85/1000 over the time period. The incidence in the south was almost three times greater than in the north. CONCLUSION: The declining incidence and geographical difference suggest that environmental factors are of importance in this disorder.

Female↗

Recessive X-linked ichthyosis associated with hypertrophic pyloric stenosis: a chance occurrence?

The association of recessive X-linked ichthyosis (RXLI) and hypertrophic pyloric stenosis (HPS) has been considered to be due to a probable contiguous gene defect. However, there are several reports of patients with large deletions on both sides of the steroid sulphatase gene (responsible for RXL1) that show no signs of HPS. We report the third pedigree wherein RXL1 was associated with HPS. Apart from the proband, both diseases showed themselves as independent events in the family tree with ichthyosis present in two other individuals and HPS in three other relatives. We calculated the probability that both diseases occurred simultaneously in the index case as a chance occurrence as 1 : 40 (using the Independence principle of probability). We conclude that in our pedigree it is likely that these two rare diseases show an accidental and not a true genetic association.

Child↗

Changing patterns of diagnosis and treatment of infantile hypertrophic pyloric stenosis: a clinical audit of 303 patients.

This review of 303 patients with infantile hypertrophic pyloric stenosis (IHPS) concentrates on the influence of clinical audit on diagnosis, complications, and factors contributing to hospital stay. Although the audit has enabled improvement in care by pediatric surgeons, there has been less change in areas controlled by other specialities. During a 12-year period, the number of patients diagnosed solely by clinical examination decreased from 74% to 28%, and the use of diagnostic tests increased (ultrasonography from 16% to 65% and barium meal from 12% to 28%). This trend continued throughout the series despite a review after 8.5 years, which recommended fewer tests. Although there may be some benefit from earlier confirmation of IHPS (the percentage of patients with a serum chloride value of less than 85 mmol/L decreased from 26% to 15%), the need for diagnostic tests could be reduced by expectant management. Better improvement occurred with surgical complications; the incidence of mucosal perforation decreased from 7 of 151 (4.6%) in the first 6 years to 0 of 152 in the last 6 years, and wound dehiscence was reduced from 3 to 0. The wound infection rate decreased from 9% to 4%, but had fluctuations. The average length of stay was reduced from 3.7 to 3.2 days. Further reductions in hospital stay will depend on earlier operation for patients with normal electrolyte values at the time of admission (61%) and a preparedness to confidently discharge patients even if there is vomiting. From the data available, early operation on the day of admission and discharge the next day would be a reasonable strategy for the majority of patients whose admission electrolyte values are normal. Complications such as mucosal perforation and wound dehiscence should be rare. This is not to suggest that IHPS is a condition of minor consequence; the surgeon must be skilled and care meticulous. If electrolytes are disturbed at the time of operation or if unrecognized mucosal perforation occurs, what should be an uneventful illness can result in disaster.

Age Factors↗