Combined surgical treatment of achalasia of the esophagus and hypertrophic pyloric stenosis; report of a case.
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We report on a 9-month-old boy who had duplication of the long arm of chromosome 9 [46,XY, -12, +der(12) inv ins (12;9)(p13;q32q13)mat.]. The clinical manifestations of the patient were different from those seen in distal 9q duplication. Pyloric stenosis appears to be common in cases with proximal 9q duplications.
Endogenous prostaglandins (PGs) have been isolated in large amounts from the gastrointestinal tract of man. These substances are intimately related to muscle contraction and cytoprotection. The gastric juice and serum levels of PGE2 and F2 alpha have been assayed in nine infants with infantile hypertrophic pyloric stenosis (IHPS) and seven age-matched controls. The gastric juice levels were significantly raised in the IHPS group for both PGE2 (P = 0.001) and PGF2 alpha (P = 0.02) as compared to controls. The possible role of PGs in the etiology of IHPS is discussed.
Nitric oxide (NO) has been described as a mediator of smooth muscle relaxation in the mammalian gastrointestinal tract. The enzyme neuronal nitric oxide synthase (NOS) catalyzes the formation of NO. We examined the expression of the neuronal NOS gene at the messenger RNA (mRNA) level in pyloric smooth-muscle biopsy specimens from six patients with infantile hypertrophic pyloric stenosis (IHPS) using a reverse transcription-polymerase chain reaction (RT-PCR) technique. For controls, smooth-muscle layer specimens of pylorus (n=3), ileum (n=2), and colon (n=2) were used. With 31 cycles of PCR reaction, control specimens revealed detectable signals for neuronal NOS mRNA. In contrast, signals of IHPS specimens were undetectable in five cases and very weak in one. By increasing the PCR to 37 cycles, detectable signals for neuronal NOS mRNA were observed in all IHPS specimens, but they were significantly weaker than those of controls. Since a low level of neuronal NOS mRNA may lead to impaired production of NO, our observations indicate that the excessively contracted, hypertrophied pyloric muscle in IHPS is a result of reduced expression of the neuronal NOS gene at the mRNA level.
This study was aimed at evaluating haemodynamic changes during an anaesthetic sequence for full stomach, using propofol as induction agent and volatile anaesthetics for maintenance of anaesthesia in infants scheduled for surgical cure of hypertrophic pyloric stenosis. After correction of preoperative blood electrolyte and metabolic disturbances with appropriate i.v. hydrating solutions, anaesthesia was induced with propofol and suxamethonium. Infants were divided in two groups according to the volatile anaesthetic agent used for maintenance of anaesthesia after tracheal intubation: halothane (n = 16) or isoflurane (n = 15). The two groups were identical regarding weight (4.28 +/- 0.6 vs 4.14 +/- 0.76 kg), age (1.6 +/- 0.9 vs 1.5 +/- 0.6 months), preinduction heart rate (155 +/- 22 vs 151 +/- 22 b.min-1) and systolic-diastolic arterial pressure (96 +/- 18/58 +/- 12 vs 105 +/- 16/67 +/- 15 mmHg). Propofol and suxamethonium doses were identical, 3.9 +/- 1 mg.kg-1 and 1.3 +/- 0.6 mg.kg-1 respectively in halothane group, vs 4.3 +/- 0.8 mg.kg-1 and 1.3 +/- 0.4 mg.kg-1 in isoflurane group. Heart rate did not change after induction of anaesthesia, while arterial blood pressure decreased significantly (p < 0.001). However, blood pressure remained within the normal range for age throughout the procedure. Mean duration of surgery was shorter in halothane group (64 +/- 16 vs 79 +/- 17 min, p < 0.05), however time-interval from the end of surgery to tracheal extubation (12 +/- 6 vs 15 +/- 8 min) was short and identical in the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)
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Because somatostatin (SS) inhibits basal and stimulated gastric acid secretion and gastrin release, it is conceivable that decreased gastric SS concentration may be one of the factors responsible for hypergastrinemia found in patients formerly operated on for hypertrophic pyloric stenosis (HPS). To investigate this issue, the SS-like immunoreactivity (SLI) concentration was estimated in antral and fundic mucosal samples from control and HPS children. In addition, SS binding to cytosol from gastric mucosa (fundus and antrum), fasting serum gastrin levels, and serum gastrin response to a standard breakfast were studied. The mean fundic and antral SLI concentrations were significantly lower in HPS children than in controls. The depletion of fundic and antral SLI content was associated with an increase in the number of gastric SS binding sites. The fasting serum gastrin levels and serum gastrin responses to the standard breakfast (after 60 minutes) of HPS children were significantly higher than those of controls. Since, together with the increase of SS binding to gastric mucosa, there is an increase in the gastrin serum levels, despite the inhibitor effect of SS on gastrin release, the binding capacity cannot be the main factor determining the response to SS in children with HPS. The present results suggest that both SS and gastrin have a role in the pathogenesis of HPS.
A two month old Thoroughbred filly was presented with signs of depression, grinding of the teeth, frothing of the mouth and abdominal pain. These signs had persisted for two weeks despite treatment with mineral oil, dioctyl sodium sulfosuccinate, meperidine and antibiotics. A variety of diagnostic tests were done, the only abnormal finding was a stress leukon. On exploratory laparotomy the stomach was dilated with fluid and gas and the pyloric canal was constricted. Pyloroplasty resulted in correction of the condition. The etiological possibilities are discussed. This is believed to be the first report of pyloric stenosis in the horse.
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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