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A possible role of the plasmalemmal cytoskeleton, nitric oxide synthase, and innervation in infantile hypertrophic pyloric stenosis. A confocal laser scanning microscopic study.

In reference to a possible neuropathy in the pathogenesis of infantile hypertrophic pyloric stenosis (IHPS), previous studies have described alterations in peptidergic transmission while others have recently attributed an important role to nitrinergic activity. Little attention has been given to the organization of the extracellular matrix (ECM) and the constituent cytoskeleton and subsarcolemma of the pyloric smooth-muscle cell. To study a possible relationship between neuronal and muscular elements in IHPS, 9 biopsies from patients with IHPS and 5 biopsies of normal pylorus were examined using immunohistochemical techniques with regard to the distribution of nerve cells and fibers (bNOS and PGP 9.5) and the ECM (laminin) and cytoskeleton (talin, vinculin, dystrophin, alpha-smooth iso-actin, desmin) components of the pyloric muscle. Our results showed anti-protein gene product 9.5 and b-nitric oxide synthase immunoreaction respectively reduced or absent in nerve fibers with a positive reaction inside the ganglion cells. An uneven distribution of the ECM component laminin was evident, together with a negative immunoreaction to talin and dystrophin. The imunolocalization of vinculin, alpha-smooth iso-actin, and desmin was similar to the controls. Our findings suggest that there is a close relationship between the nerve and muscle elements in the pathophysiology of IHPS and that non-alteration of some elements of cytoskeleton organization can play an important role in regaining pyloric function after pyloromyotomy.

Cytoskeletal Proteins↗

Gallstone, causing bleeding and pyloric stenosis syndrome, diagnosed by urgent gastroscopy.

By means of urgent gastroscopy the authors, right before surgery, recognized a pyloric stenosis syndrome and a gallstone that had caused massive melena in a female patient of 73. Cholecystectomy and a Billroth II gastric resection were performed. Following surgery, a transitory duodenal fistula developed. After a month of clinical treatment the patient was sent home cured. This case draws attention to the importance of removing a gallbladder filled with stones as soon as possible in order to prevent complications, as well as to the necessity for gastroscopy in cases of unexplained abdominal complaints.

Aged↗

The management of pyloric stenosis in a district hospital.

Over a 10-year period, 80 infants were admitted with a diagnosis of pyloric stenosis. Seventy-nine underwent surgical pyloromyotomy (63 male, 16 female; mean age 5.6 weeks). Fifty-nine infants (75%) were operated upon by one of four consultant surgeons and 20 (25%) operations were undertaken by a registrar. Seventy operations (89%) were performed during normal working hours and thirty of these (43%) were placed first on the operating list. The mean length of stay was 6.9 days. Three infants required re-operation: there were two instances of wound dehiscence and one episode of bleeding from the edge of the pyloromyotomy. There were no deaths. The morbidity and mortality in this series was comparable with published results from specialist units.

Female↗

Renal abnormalities in children with hypertrophic pyloric stenosis--fact or fallacy?

Retrospective review of the abdominal ultrasound (US) examination of 274 children studied for hypertrophic pyloric stenosis (HPS) was undertaken to determine if there is an increased incidence of renal disease as previously reported. Five major abnormalities were detected in the 126 children with HPS. Three lesions were newly diagnosed and two had been diagnosed previously. Five children had abnormalities classified as minor or normal variants. Renal abnormalities were found in six of the 148 children who did not HPS. Only three of these were newly diagnosed and medically important. Eight children without HPS had minor abnormalities or normal variants of the kidneys. Newly diagnosed medically important renal lesions were present in 2.4% of children screened for HPS. The incidence of the finding was the similar in children with and without HPS.

Abnormalities, Multiple↗

Infantile hypertrophic pyloric stenosis: the changing trend in treatment in a Danish county.

From 1950 to 1984, 679 patients were treated for infantile hypertrophic pyloric stenosis (IHPS) in the County of Funen, Denmark. Medical treatment was performed in 114 patients and pyloromyotomy in 565. A pyloric tumor was palpated in 80% and a barium meal examination was performed in 24% of the patients. A shift toward intravenous fluid and general anesthesia is seen during the study period. Complications occurred in 17% of medically treated patients and in 8% of surgically treated patients. Six deaths occurred among the medically treated and four among the surgically treated patients. During the study period, a shift toward centralization and surgical treatment took place.

Anesthesia, General↗

Parallel incidences of sudden infant death syndrome and infantile hypertrophic pyloric stenosis: a common cause?

OBJECTIVE: To determine whether there was a correlation between the incidence of infantile hypertrophic pyloric stenosis (IHPS) and the incidence of sudden infant death syndrome (SIDS) during the period 1970 to 1997 and to discuss different causative factors that could be influencing the changing trend in incidence. METHODS: We compared the incidence of IHPS in the Stockholm Health Care Region with the incidence of SIDS in Sweden each year between 1970 and 1997. First, the relation was assessed by calculation of a correlation coefficient; second, the relative linear decrease was estimated for the time period 1990 to 1997. RESULTS: The incidence of IHPS increased steadily during the 1970s, from 0.5 per 1000 live births in 1970 to 2.7 in 1979. During the 1980s, the average incidence was 2.8. During the 1990s, there was a significant decrease in the number of IHPS cases in Stockholm. The incidence rate of IHPS parallels the incidence of SIDS during the study period (r = 0.58). The incidence of SIDS dropped after the risk-reduction campaign in the beginning of the 1990s, which recommended that infants sleep on their back. We could not identify any other changes of behavioral risk factors in early exposures that could explain the temporal trends. CONCLUSIONS: The statistical findings suggest that IHPS and SIDS have causative factors in common. We suggest that prone sleeping is one of those factors.

Female↗

Gastrin, somatostatin and infantile hypertrophic pyloric stenosis.

UNLABELLED: Despite multiple and often contradictory research, no firm conclusions regarding the role of hypergastrinaemia in infantile hypertrophic pyloric stenosis (IHPS) have been established. Evaluation of somatostatin, the main physiological antagonist of gastrin, has not been assessed in previous studies. Long-term evaluation following pyloromyotomy suggests persistent abnormalities in gastrin and somatostatin in IHPS. The objective of this case-controlled study was to compare fasting serum gastrin and somatostatin levels in IHPS. Serum sample were collected from 39 children with IHPS at the time of pyloromyotomy and 20 age-matched controls with no evidence of gastrointestinal disease. Standard radioimmunoassay techniques were used to detect circulating levels of the hormones. A two-tailed t-test was used for statistical analysis. The levels of the two hormones (mean +/- SEM) revealed that there was no evidence of hypergastrinaemia in IHPS compared with controls (75.6 +/- 16.1 and 68.1 +/- 7.8 ng l(-1), respectively), but that the level of somatostatin was significantly elevated (38.9 +/- 6.4 and 30.5 +/- 5.8 ng l(-1), p = 0.016). An inverse trend in the gastrin/somatostatin levels could not be identified in IHPS. CONCLUSION: Somatostatin but not gastrin is raised in IHPS. Somatostatin is known to inhibit the actions of inhibitory neurotransmitters in the pylorus and may explain the development of pylorospasm, which is believed to be important in the development of pyloric tumours. These results do not agree with a previous long-term follow-up study, but reflect the hormonal imbalance at the time of pyloric hypertrophy.

Biomarkers↗

Increased prevalence of urinary tract infections and anomalies in infants with pyloric stenosis.

We performed a retrospective analysis of all patients admitted to our institution with a diagnosis of infantile hypertrophic pyloric stenosis (IHPS) during a 10-year period from 1985-95 in order to assess the possible association between IHPS and urinary tract infections (UTIs). All 285 patients with IHPS had radiological or ultrasonographic confirmation of that diagnosis and underwent the Ramstedt procedure. Those who continued to be symptomatic were evaluated for UTI by urine analysis and culture. Positive cases were further evaluated for urinary system anomalies. The male:female ratio of IHPS was 3.4:1. Concomitant UTI was diagnosed in 8 patients by suprapubic aspiration or bladder catheterization. The prevalence of UTI in this series was 2.8%, 20-fold higher than the expected prevalence. Three of the 8 patients with UTI (37.5%) had urinary tract anomalies. These findings suggest an association between IHPS and UTI. We recommend that all IHPS patients be evaluated for UTI and positive cases undergo further evaluation for urinary anomalies.

Female↗

Infantile hypertrophic pyloric stenosis in a regional centre.

BACKGROUND: It has been suggested that only specialist paediatric surgeons should manage infantile hypertrophic pyloric stenosis (IHPS). AIM: The aim of this retrospective study was to ascertain whether the majority of these infants might be managed in a well-equipped regional centre. METHODS: Using the Hospital Inpatient Enquiry database, all cases of IHPS within a single administrative health region were identified over a six-year period. A chart review was performed to obtain demographic and clinical information for each patient. Reports from the Central Statistics Office were used to obtain data on population and live births for the region. RESULTS: Seventy patients with IHPS were identified from this region from 1991 to 1996, 63 (90%) of which were treated in the region. Of the remaining seven, four were referred directly to specialised paediatric surgical hospitals because of prematurity (n = 1), low birth weight (n = 1), capillary haemangioma (n = 1) and severe metabolic derangement (n = 1) while three were assessed and treated in general hospitals outside the region. Of the 63 infants undergoing pyloromyotomy in this region, the duodenal mucosa was breached in four (6%); there were wound complications in three (5%); and one required a re-pyloromyotomy. The mean postoperative stay was eight days (range 2-42 days). CONCLUSION: The majority of infants with IHPS may be safely managed in regional centres with transfer to specialist paediatric centres reserved for 'high risk' cases. The management of IHPS at a regional level has important implications and presents opportunities for surgical training.

Female↗

Evolution in the recognition of infantile hypertrophic pyloric stenosis.

PURPOSE: To analyze changes in the clinical condition at presentation and methods of establishing the diagnosis of infantile hypertrophic pyloric stenosis (IHPS). METHODS: Retrospective review of patients who underwent pyloromyotomy (PM) for suspected IHPS at two institutions from 1969 through 1994 was performed. For the purposes of comparison, the population was divided into five equal time periods. RESULTS: Over the 25-year period, 901 infants underwent PM. Patients presented at a younger age, weighed more, and had a shorter length of illness in the most recent time period. Hypochloremic alkalosis was found half as frequently in the most recent time period compared to the earliest group. A palpable pyloric tumor was present in 79% of patients in the earliest time period compared with 23% in the most recent time period. Sixty-one percent of patients in the earliest group and 96% in the latest group underwent an imaging study, reflecting the referring physician's evaluation before referral to the surgeon. CONCLUSIONS: Currently, patients with IHPS less frequently present with the clinical hallmarks of the disease. The use of imaging studies to establish the diagnosis has become common practice. The result has been the diagnosis of IHPS before alkalosis has developed, a shorter clinical course, less morbidity, and a shorter postoperative hospital stay.

Alkalosis↗

Congenital mesoblastic nephroma, nephrocalcinosis, and hypertrophic pyloric stenosis.

A cellular variant of congenital mesoblastic nephroma (CMN) occurring in a newborn is presented. Coincidental findings were congenital hypertrophic pyloric stenosis (CHPS) and nephrocalcinotic hypercalcemia that reverted after nephrectomy. As of the day of writing, this multiple association has not been reported. The authors believe that both CMN and CHPS could be interpreted as fibroblastic proliferative-related entities. Transient hypercalcemia seems to be a paraneoplastic phenomenon.

Humans↗