Hormone-responsive alkaline proteinase in rat skeletal muscle is not a mast cell-derived enzyme.

Proteinase activity was determined in myofibrils from intact rat skeletal muscle and from skeletal muscle myocytes grown in culture. In vivo administration of the mast cell degranulator compound 48/80 abolished the alkaline proteinase activity in myofibrils obtained from normal or streptozotocin-diabetic rats. Exposure of myocytes to compound 48/80 in cell cultures had no effect on their myofibrillar proteinase activity, nor did it affect the rate of overall protein degradation in these cells. Co-incubation of cultured mast cells (line P815Y) with myocytes followed by sonication of the cell mixture resulted in a marked reduction of the proteinase activity in the pellet fraction, suggesting that the mast cells contain inhibitor(s) of myofibrillar proteinase activity. It is suggested that the myofibril-bound alkaline proteinase activity is not a mast cell-derived enzyme but a genuine component of muscle cells. The in vivo 48/80-induced reduction of muscle myofibrillar proteinase activity appears to be due to release of a soluble inhibitory activity rather than removal of mast cell proteinase from the tissue by degranulation.

Hormone-responsive myofibrillar protease activity in cultured rat myoblasts.

The effect of exposure to dexamethasone and serum-deprivation on myofibrillar protease activity was determined by following cleavage of [14C]globin by isolated myofibrils obtained from rat skeletal muscle in culture. Dexamethasone [10(-7) M] produced a 46% increase in protease activity, and serum-deprivation caused a 50% increase in activity over that of the enzyme in control cultures. The increases in proteolysis occurred concurrently with increased rate of overall protein degradation in these cells and were not associated with changes in cell viability. In cultured rat cardiac muscle cells dexamethasone failed to enhance myofibrillar protease activity, while serum-deprivation produced a 52% increase in the enzyme activity. Addition of insulin (50 mU/ml) to the cultures did not affect proteolysis or myofibrillar protease activity, but completely prevented the dexamethasone-induced increase of these activities. This effect of insulin suggests that the increase of muscle proteolysis in insulin-deficient diabetic animals reflects an enhanced response of the muscle to circulating glucocorticoids rather than a direct effect of insulin-deprivation on muscle proteolysis. Taken together, the present observations indicate that muscle cells in culture retain the ability to respond to catabolic stimuli by adaptive changes in the myofibrillar protease activity in a manner analogous to that of their parent tissue in the intact animal.

Torsion of the spleen with incomplete infarction: case report.

Torsion and infarction of a "wandering spleen" is a rare disease which is often confused with other acute abdominal crises. A correct preoperative diagnosis, when made, has usually been determined by arteriographic studies. A child is described in whom changes in the TcSC scan made a correct diagnosis possible by non-invasive methods.

Rectal mucosal biopsy in aganglionosis and allied conditions.

Rectal mucosal biopsies were performed in 146 patients suspected of having Hirschsprung's disease. The biopsies were serially sectioned and screened for the presence of ganglion cells. In the 101 cases in which ganglion cells were found, the diagnosis of Hirschsprung's disease was ruled out. In 45 patients, histologic examination revealed the typical features of Hirschsprung's disease, i.e., the absence of ganglion cells and the presence of numerous hypertrophied nerve bundles. The varied morphologic features of Meissner's plexus in patients of different ages and the diagnostic pitfalls are described in detail. Serial frozen sections stained with hematoxylin-eosin were examined in eight cases of neonatal intestinal obstruction and enterocolitis. This method was found to be useful and reliable in emergencies. In three of these cases ganglion cells were found and major surgical procedures were avoided. In 73 cases, staining for acetylcholinesterase activity was performed. The results matched the microscopic findings in all but three false-positive cases.

Corrosive injury to the stomach due to acid ingestion.

Ingestion of acid often causes severe damage to the stomach. Ingestion of alkali, however, usually injures the esophagus and spares the stomach. Early complications of acid ingestion, e.g., massive gastric hemorrhage or perforation, are unusual. The absence of severe symptoms in most patients soon after ingestion of acid is often misleading. Gastric outlet obstruction is a common late result and may develop following an asymptomatic interval. The authors' experience with three patients with severe complications following ingestion of acid is presented. The initial treatment, as well as the surgical management, are discussed.

Low anterior resection with a long posterior anorectal myectomy and sphincterectomy for Hirschsprung's disease.

Nine patients with the severe form of Hirschhsprung's Disease (HD) underwent low anterior resection with posterior anal myectomy and sphincterectomy. Good results were achieved in six patients. Three patients had bouts of enterocolitis following the surgical treatment which was attributed to residual spasm of the aganglionic rectum and the internal anal sphincter. Myectomy had to be redone in two patients. One patient developed constipation which responded to toilet training. One patient died at home of an unknown cause, and one patient was lost to follow-up. The different methods of treatment of HD are discussed with emphasis on the role of the internal anal sphincter. The relatively high complication rate in this small group of patients does not justify its use in the severe form of HD.

Demonstration of rectourinary fistulas by pressure gastrografin enema.

Rectourethral and vesical fistulas in patients with high anorectal atresia can be demonstrated by using a pressure Gastrografin enema through the distal colostomy opening. A technique is described where contrast medium is forced by gentle pressure, filling the fistula and the urethra. This picture is of great value during the repair of these anomalies.

Nerve mediated responses to drugs and electrical stimulation in aganglionic muscle segments in Hirschsprung's disease.

The activity of isolated muscle strips from normal and aganglionic human large bowel was studied in vitro. The intrinsic nerves were stimulated electrically and by nicotinic agonists. The ganglionic preparations displayed a strong inhibitory response due to the release of both norepinephrine and a noncholinergic, nonadrenergic inhibitory neurotransmitter. In the aganglionic strips (obtained from patients with Hirschsprung's disease), nerve activation tended to evoke contraction, apparently due to enhancement in the release of acetylcholine. At the same time, the release of norepinephrine appeared to be less than normal. A particularly interesting finding in the aganglionic muscle strips was the presence of a substantial inhibitory response due to the release of a noncholinergic, nonadrenergic substance. These results provide further evidence for the importance of the innervation of the aganglionic segment in Hirschsprung's disease.

Congenital cricopharyngeal achalasia treatment by dilatations.

An infant with cricopharyngeal achalasia, recurrent aspiration pneumonia, and severe failure to thrive was fed for 5 months through a nasogastric tube. She responded promptly to dilatations of the spastic cricopharyngeal muscle. Normal oral feedings followed the treatment with progressive weight gain and no further pulmonary complications over a follow-up period of 1 1/2 years. Dilatations were used in the infant described here, in analogy to the well-known success of treatment by dilatations in infants with achalasia of the lower esophagus.

Endesophageal resection of short benign strictures of the esophagus.

A relatively simple operation for the treatment of short esophageal strictures that do not respond to dilatations has been described. This operation was successfully carried out at all levels of the esophagus. The strictures treated were caused by lye burns, peptic esophagitis and previous anastomoses.

Hypoganglionosis of the myenteric plexus with normal Meissner's plexus: a new variant of colonic ganglion cell disorders.

A 16-year-old girl with a history of achalasia of the lower esophagus was investigated for progressive constipation since the age of five years. Normal ganglion cells of the Meissner's plexus were found in a mucosal rectal biopsy. Manometric studies showed absence of the normal rectosphincteric relaxation reflex. A long posterior rectal myectomy was performed in which no ganglion cells were found between the muscular layers. The patient underwent a rectosigmoid resection with a low anastomosis according to Duhamel. In the resected bowel, a normal Meissner's plexus was again found while marked hypoganglionosis of the myenteric plexus was evident. Intranuclear inclusion bodies in Schwann cells and scattered inflammatory cells were noteworthy. This case may be an example of an acquired hypoganglionosis localized to the myenteric plexus.

Gastric devascularization--an emergency treatment for hemorrhagic gastritis in the neonate.

A newborn baby suffering from massive hemorrhage due to erosive gastritis, was treated successfully by devascularization of the stomach which is a rather simple procedure. Experience with adults as well as with this patient justifies the use of this operation when other measures fail to stop massive bleeding in hemorrhagic gastritis.

Strangulated congenital diaphragmatic hernia in infants.

Two previously healthy children, 7 and 12 months old, respectively, presented with respiratory distress and severe sepsis. Chest x-rays were suggestive of inflammatory disease of the left lung. At operation, necrotic bowel was found to be herniated through a left congenital posterolateral diaphragmatic hernia. These patients illustrate the difficulty in establishing the diagnosis of strangulated congenital diaphragmatic hernia and the grave consequences when operation is delayed.