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Changes in gastric myoelectrical activity in hypertrophic pyloric stenosis and after surgical correction.

The changes of gastric myoelectrical activity were investigated in 20 infants by cutaneous electrogastrography (EGG) before and after the surgical correction of infantile hypertrophic pyloric stenosis (IHPS). The dominance of 2-4 cycles per minute (CPM) "slow waves" is typical of the healthy gastric function. The shift of the dominant frequencies towards the slower frequency (0-2 CPM) is defined as bradygastria, whereas a shift towards the more frequent waves (4-10 CPM) is called tachygastria. Unlike with healthy infants, the electrogastrogram showed pathologic patterns in 85% (18 out of 20) of IHPS patients. In all except two of these infants with pathologic electrical patterns, the frequency of the waves significantly shifted towards tachygastria. The effect of feeding on the gastric myoelectrical activity could only be studied in limited (9/20) cases because of recurring vomiting during the preoperative period. In IHPS infants, a significant increase in the bradygastria group was observed in the postprandial period compared with healthy infants. Three to 5 days after surgical repair (pyloromyotomy) and the reintroduction of feeding in gradually increasing amounts, the gastric myoelectrical activity showed physiologic patterns again, showing that the pyloric function was back to normal. Cutaneous EGG is a useful, noninvasive method to obtain indirect information about the motor function of the stomach and might be further applicable to pediatric gastric motility disorders.

Electrodiagnosis↗

The extent of muscle hypertrophy in infantile hypertrophic pyloric stenosis does not depend on age and duration of symptoms.

There are conflicting views in the literature about the relationship between the extent of muscle hypertrophy in infantile hypertrophic pyloric stenosis (IHPS) and the age of the patient and duration of symptoms. This study was undertaken to elucidate the exact nature of these relationships. The degree of muscle hypertrophy was assessed by measurement of the DNA concentration in biopsy specimens, a technique that has been used to quantify organ hypertrophy objectively and accurately. Forty-one patients were studied prospectively over 12 months. The results show that the degree of hypertrophy is not age-related, and that all the patients tend to progress to about the same degree of hypertrophy, which then remains constant regardless of the duration of symptoms.

Age Factors↗

Selective reduction in intramuscular nerve supporting cells in infantile hypertrophic pyloric stenosis.

Nerve supporting cells were examined immunohistochemically for the expression of S-100 protein and D7 antigen (markers of peripheral Schwann cells) and GFAP (a marker of central-type nerve supporting cells) in pyloric tissue from 18 patients with infantile hypertrophic pyloric stenosis (IHPS) and 10 age-matched controls. In both the IHPS and control groups there was strong staining of the myenteric plexus by all three antibodies. Within the normal pyloric muscle, there was strong staining of nerve fibers by S-100 and D7, but fibers positive for GFAP were considerably less frequent. In the IHPS cases, S-100, D7, and GFAP immunoreactive fibers were either absent or markedly reduced within the hypertrophied circular and longitudinal muscles. Because supporting nerve cells are essential in the maintenance of basic physiological functions of neurons, their absence within the hypertrophied pyloric muscle may be an important factor in the pathogenesis of IHPS.

Biomarkers↗

The association of erythromycin and infantile hypertrophic pyloric stenosis: causal or coincidental?

The safety profile of erythromycin is notable for the frequent occurrence of intolerable gastrointestinal effects. One of the more serious of these is infantile hypertrophic pyloric stenosis (IHPS). A recent cluster of IHPS cases prompted an epidemiological investigation which identified oral erythromycin chemoprophylaxis of pertussis as the major risk factor. Evidence suggests an association between early postnatal erythromycin exposure and IHPS. There is no substantive evidence of a risk associated with prenatal exposure, with the single published case-control study to date producing negative findings. The epidemiological investigations of the association with early postnatal exposure have reported significantly elevated odds ratios but have a variety of methodological limitations that prevent definitive conclusions being made. Nevertheless, the concordance of findings across studies increases the strength of evidence favouring an association. The prominent gastrokinetic properties of erythromycin have been postulated as the mechanism behind this phenomenon. A comprehensive assessment of this potential adverse effect should consider its biological plausibility in light of known gastrointestinal physiology, its modulation by erythromycin, and the known pathophysiology of IHPS. Gastrointestinal motor activity in the fasted mammal consists of three phases, phase III being large amplitude contractions called migrating motor complexes (MMC) that can be initiated by motilin and erythromycin. The gastrokinetic effects of erythromycin are variable and complex and include effects on the timing, duration, amplitude and distribution of MMCs. It has been speculated that the motilinomimetic effects of erythromycin on antral smooth muscle function, such as the MMC, may mediate the effect via work hypertrophy. Although intuitively plausible and consistent with hypertrophic obstructive changes similar to IHPS observed in hyperplastic rat ileum after artificially induced mechanical obstruction, there is no direct evidence of this phenomenon. Further complicating the association is the limitations of our knowledge about the pathophysiology of IHPS, including numerous genetic abnormalities, increased parietal cell mass, and gastric hyperacidity. The implications of the reported findings with erythromycin on the benefit-risk profiles of newer macrolides and azalides must be considered. The available data on the comparative gastrokinetic properties of macrolides are significant for the potent gastrokinetic properties and its acid degradation products, the marked variation in gastrokinetic properties associated with macrolide ring size, and the requirement for specific glycosidic linkages at the C-3 and C-5 carbons of the macrolide ring. The variation in gastrokinetic properties associated with variations in molecular structure suggests that if the association between erythromycin and IHPS is causal it may not be a class effect.

Adverse Drug Reaction Reporting Systems↗

[Infantile hypertrophic pyloric stenosis. 22 years' data].

The report is a retrospective study of the journals of 123 patients operated from 1967-89. In all patients, the Fredet-Ramstedt pyloromyotomy was used. The incidence of pyloric stenosis is stable in Tromsø, 1.5 +/- 1 pr. 1,000 newborn. The incidence among boys and girls is 4:1. Serious complications after surgical treatment are infrequent; with no mortality and 4% reoperations. The duration of symptoms significantly increased the incidence of peroperative pyloric perforations, with a more tedious postoperative course. Early diagnosis and surgical treatment are therefore recommended.

Female↗

The development of pyloric stenosis during transpyloric feedings.

Three infants, ages 3 to 4 months, had nasojejunal feeding tubes placed for recurrent aspiration and/or feeding intolerance after upper gastrointestinal cineradiographs (ugi) had documented gastroesophageal reflux (GER) with normal pyloric channels and prompt gastric emptying. The tubes had been in place for 3 and 4 weeks, respectively, in the first two infants when classic hypertrophic pyloric stenosis (HPS) was found during fundoplication and gastrostomy tube placement. The last child had a failed attempt at nasogastric tube feedings following 3 months of nasojejunal tube feedings. A repeat ugi suggested HPS, which was confirmed by pyloric ultrasound. This infant underwent pyloromyotomy alone. The late presentation of HPS in these infants suggests that transpyloric tubes might cause the development of HPS and exacerbate the symptoms of preexisting GER. In infants who are expected to eat by mouth, pyloromyotomy alone might allow the reinstitution of orogastric feedings without the perioperative morbidity of fundoplication and gastrostomy tube placement.

Enteral Nutrition↗

Cost-effectiveness in diagnosing infantile hypertrophic pyloric stenosis.

PURPOSE: The purpose of this study was to determine which imaging study, upper gastrointestinal series (UGI) or abdominal ultrasonography (US), is more cost-effective in diagnosing infantile hypertrophic pyloric stenosis (IHPS) using a decision analysis model. METHODS: Probabilities were calculated from a review of the records of all infants less than 6 months of age referred for UGI or US to rule out IHPS over a 3-year period from January 1992 to December 1995. Cost-effectiveness was determined from hospital charges for each imaging study and its possible outcomes. RESULTS: The positive predictive value of UGI was 1.0 and US was 0.98 in the 246 infants evaluated for possible IHPS. In patients who had an initially normal study finding (UGI or US), 25% of patients undergoing US first required a second study for persistent symptoms, whereas only 6% of patients who had a negative initial UGI finding required a second study. CONCLUSIONS: Cost analysis found UGI to be more cost-effective than US because fewer secondary studies were required. UGI provides information regarding other pathological conditions as compared with US.

Age of Onset↗

An evaluation of applied potential tomography in the diagnosis of infantile hypertrophic pyloric stenosis.

In a study of gastric emptying on 28 children using applied potential tomography, we have shown that a group of children with delayed gastric emptying can be identified, including those with proven hypertrophic pyloric stenosis. The place of APT in the investigation of gastric emptying in children is discussed, and in particular its usefulness in early confirmation of HPS in equivocal cases.

Diagnosis, Differential↗

Selective neurotrophin deficiency in infantile hypertrophic pyloric stenosis.

BACKGROUND/PURPOSE: Increasing evidence suggests that the enteric nervous system is under the control of neurotrophins. Nerve growth factor (NGF), brain-derived neurotrophic factor (BDNF), neurotrophin-3 (NT-3), and neurotrophin-4/5 (NT-4/5), promote differentiation, growth, and survival of various central and peripheral nervous system neurons. The biological effects of neurotrophins are mediated by the interactions with high-affinity tyrosine kinase receptors (TrkA, TrkB, TrkC). Recently, abnormalities of intramuscular innervation have been reported in infantile hypertrophic pyloric stenosis (IHPS). To further understand the reported abnormalities in pyloric innervation in IHPS, the authors analyzed the expression of Trk receptors and the neurotrophins content in IHPS. METHODS: Full-thickness muscle biopsy specimens were obtained from 8 IHPS patients (age range, 23 to 41 days) at pyloromyotomy and from 8 age-matched controls without gastrointestinal disease at autopsy performed within 12 hours after death. Indirect immunohistochemistry was performed using ABC (Avidin Biotin peroxidase Complex) method with anti-Trk specific antibodies (A,B,C). Quantitative analysis was performed using sandwich-type ELISA for NGF, BDNF, NT-3, and NT-4/5. RESULTS: The intensity of staining of the myenteric plexus for TrkA, TrkB, and TrkC was similar among IHPS and controls. There was a lack of TrkA-positive nerve fibers in IHPS compared with controls. The quantity of total NGF, NT-3, and BDNF in IHPS was significantly lower than in controls. CONCLUSIONS: The reduced production of neurotrophins in IHPS may be responsible for the delay in the functional and structural maturation of pyloric innervation in IHPS. The lack of TrkA-positive nerve fibers in pyloric muscle may explain the abnormal intramuscular innervation in IHPS.

Analysis of Variance↗

Gastric emptying 16 to 26 years after treatment of infantile hypertrophic pyloric stenosis.

Long-term follow-up was performed 16 to 26 years after conservative (group I, n = 18) and operative (group II, n = 38) treatment of 56 patients who had infantile hypertrophic pyloric stenosis (IHPS). The study encompassed the scintigraphic determination of gastric emptying rates for solids and liquids, an interview to obtain medical history and ascertain whether a current disorder of the upper gastrointestinal tract was present, and a clinical examination. Gastric emptying rates were measured on two different days for solids and liquids. The standard solid meal consisted of two scrambled eggs, two slices of toast, and 20 g of margarine. The gastric emptying rate for liquids was measured using 300 mL of apple juice. The scrambled eggs and apple juice were each marked with 2.2 MBq technetium 99m-sulphur-colloid. Two control collectives were used in this study; one group (physicians) served to create a reference curve for gastric emptying, and the other group, with the same age and gender distributions as those of the patients, served to evaluate the frequency of gastrointestinal complaints, by means of a questionnaire. There was no significant rate difference for gastric emptying between the patients treated conservatively or surgically and the controls. No association could be construed between the frequency of gastrointestinal symptoms or disorders and the gastric emptying rates for solids and liquids. The results presented here substantiate that clinically relevant disturbances of stomach motility after IHPS appear to be rare.

Adolescent↗

[Pyloric stenosis - a complication of cholelithiasis. (Case of gallstone perforation into the duodenal bulb)].

Pressure-necrosis followed by stone perforation is ralatively rare among the complications of cholelithiasis. There is always a danger of a stenosis in the gastro-intestinal tract. Report of a case of perforation of a gallstone into the duodenal bulb producing few signs but the clinical picture of pyloric stenosis. Symptoms, signs and differential diagnosis are discussed on the basis of the literature.

Aged↗