Sinn Anuras

Jejunal Manometry Patterns in Health, Partial Intestinal Obstruction, and Pseudoobstruction

The purpose of this study was to determine the utility of jejunal manometry in evaluating patients having partial small intestinal obstruction or the pseudoobstruction syndrome. We studied 10 healthy volunteers, 9 patients with documented mechanical obstruction, and 3 patients with chronic intestinal pseudoobstruction during fasting and for 2 h after a 650-kcal meal. Contractile activity, especially during phase 2, was extremely variable in all individuals studied. After the meal, the most striking finding in mechanical obstruction was the regular occurrence of clustered contractions. These were defined as 3-10 regular contractions, occurring 1 per 5 s, preceded and followed by at least 1 min of absent motor activity. The associated periods of quiescent motor activity may account for the unexpectedly reduced frequency and motility index in obstructed patients compared with normal subjects after the meal. Two different contractile patterns occurred in patients with pseudoobstruction. Before and after the meal, infrequent and low-amplitude contractions occurred in 2 patients, and multiple, aborally migrating, clustered contractions occurred in the other patient with pseudoobstruction. Manometric abnormalities occur in both mechanical obstruction and pseudoobstruction. However, the technique is probably not more sensitive than radiologic procedures, and the abnormalities that are detected by manometry are not specific. Some of the same motility changes occurred in both disorders.

A familial visceral myopathy.

A kindred contained at least 18 members with visceral myopathy. Sixteen had symptoms of chronic obstruction of the gastrointestinal or urinary tracts. Of six patients with megaduodenum on contrast roentgenograms, two were asymptomatic. Four patients had redundant colon on barium enema, and four had megacystis. Specimens from duodenum, jejunum, ileum, colon, or urinary bladder from five patients showed thinning and extensive collagen replacement of the longitudinal muscle layer; ganglion cells were normal by light and electron microscopy. Esophageal manometry in three patients showed decreased gastroesophageal sphincter pressures and no contractions in the smooth muscle segment of the esophagus; duodenal manometry showed a low frequency and amplitude of contractions. Three patients developed fever and signs of peritonitis after operations to bypass dilated segments. This seems to be a generalized smooth muscle disease with variable clinical manifestations and with an autosomal dominant or sex-linked dominant mode of inheritance.

Migrating Spike Bursts and Electrical Slow Waves in the Cat Colon: Effect of Sectioning

Abstract The circular muscle electromyogram was recorded with up to 16 electrodes spaced uniformly along the whole cat colon in vitro before and after complete division of the colon between the electrodes. Before division, the electromyogram of the proximal colon contained slow waves with superimposed short spike bursts, while the distal threefourths of the colon exhibited both slow waves and a periodic prolonged migrating spike burst. Seventy per cent of migrating spike bursts started about the hepatic flexure of the colon and spread aborad; 30% started distally and spread orad. The period of the bursts was 74.3 to 83.0 sec, their duration 26.6 to 35.8 sec, and their velocity, 5.15 ± 2.86 mm per sec aborad and 4.77 ± 2.23 mm per sec orad. The migrating spike bursts were abolished by sectioning of the colon. Before partition, slow wave frequency was 4.5 cycles per min in the most proximal 10% of the colon, and 6.0 cycles per min in the most distal 10%, with intermediate values at intermediate points. After partition, slow wave frequency declined to an average of 1.9 cycles per min, 44% of the control value, in the most proximal 10% of the colon, and rose to an average of 7.3 cycles per min, 121% of the control value, in the most distal 10%.

A familial visceral myopathy with external ophthalmoplegia and autosomal recessive transmission

A new visceral myopathy family was identified. The disease in this family is transmitted by an autosomal recessive gene. Only 3 patients were identified from approximately 1500 family members. All 3 patients are the products of intermarriage. The patients had gastric atony, dilatation of the entire small bowel, and multiple diverticula throughout. Pathology of the jejunum showed fibrosis and degeneration, mainly of the longitudinal muscle layer, indistinguishable from that of previously reported families. Two of the patients also had ptosis and external ophthalmoplegia. Jejunal manometric studies were performed on the probands asymptomatic mother and five siblings. All had normal esophageal manometric studies and upper gastrointestinal x-rays. The mother and three siblings had abnormal jejunal manometric studies characterized by the absence of phase 1 in some of the migrating motor complexes and increased motility indices in phase 2. We conclude that familial visceral myopathy can be transmitted by an autosomal recessive gene, and that jejunal manometry is a sensitive technique to identify asymptomatic heterozygotes.

Carcinoma of the papilla of vater: A review of fifty-seven cases

Fifty-seven cases of carcinoma of the papilla of Vater are reviewed. Clinical and pathologic features as well as results of various surgical treatments are summarized. The Whipple operation may be the operation of choice for cancer of the papilla of Vater, offering a chance for cure and obviating further surgery.

Pathologic features of familial visceral myopathy

Familial visceral myopathy is the most common cause of chronic primary (idiopathic) intestinal pseudo-obstruction. We studied four family groups with this disease and found that it has a characteristic morphologic appearance. Grossly, there is segmental dilatation of the alimentary tract, often involving multiple sites and most commonly producing a megaduodenum. Microscopically, the involved areas show a characteristic change consisting of degenerating muscle cells and fibrosis, which may involve the full thickness of the muscularis propria but is often more prominent in or limited to the external layer. Degenerating muscle cells appear pale, poorly defined, and fragmented. As residual thread-like remnants become surrounded by collagen or as muscle cells are destroyed, leaving apparent spaces surrounded by collagen, the longitudinal and circular muscles take on a vacuolated appearance easily recognized at low magnifications. Recognition of this change is greatly facilitated by use of a trichrome stain, and mild lesions may be recognized only with such stains. The nondilated segments of intestine show similar changes but of a less severe degree. Neural and vascular structures are apparently normal. Although the lesion most closely resembles progressive systemic sclerosis, the degenerating muscle cells and vacuolated appearance of the muscle serve to distinguish familial visceral myopathy from the latter entity.

An Inhibitory Innervation at the Gastroduodenal Junction

Transverse muscle strips, 2-mm wide, were cut serially from the gastroduodenal junction in opossums, cats, dogs, and man. Electrical field stimulation with trains of rectangular current pulses of 0.5 ms in all opossums, all cats, some dogs, and the one human specimen induced relaxation in strips from the thickened circular muscle proximal to the mucosal junction. In some opossums weak relaxations also occurred in the first few strips below the mucosal junction. All other strips contracted or showed no response. This relaxation in opossums was abolished by tetrodotoxin but was not affected by antagonists to adrenergic and cholinergic transmission, nor by tripelennamine, methysergide, pentagastrin, secretin, cerulein, or cholecystokinin. Optimal frequency for stimulus-relaxation was 12 Hz. Chronaxie was 0.85 ms. The junctional strips also showed greater resistances to stretch than those remote from the junction. With apparent species variations, the junctional muscle possesses a nonadrenergic inhibitory innervation which is either absent or unexpressed in adjacent muscle of stomach and duodenum. This suggests the existence of a distinctive inhibitory neural control mechanism for pyloric muscle.

Fulminant Herpes Simplex Hepatitis in an Adult: Report of a Case in Renal Transplant Recipient

A case of disseminated herpes simplex infection is reported in a 31-year-old renal transplant recipient. The patient presented with a unique clinical syndrome: high fever, severe sore throat with buccal and pharyngeal ulcerations, fulminant hepatitis, thrombocytopenia, and leukopenia. The patient died from hepatic failure, disseminated intravascular coagulopathy, and upper gastrointestinal bleeding. The diagnosis was made by positive herpes simplex virus culture from the throat, and was confirmed at autopsy by typical Cowdrys type A intranuclear inclusions in hepatocytes with positive herpes simplex virus culture from the liver. Review of the literature reveals that other reported cases have had very similar clinical findings, making disseminated herpes simplex infection with fulminant hepatitis a recognizable syndrome.

A familial visceral myopathy with dilatation of the entire gastrointestinal tract

We are reporting a new kindred in which familial visceral myopathy was identified in four siblings. There was no other symptomatic case identified in this 75-member, four-generation family, suggesting an autosomal recessive inheritance. All patients had recurrent symptoms of intestinal obstruction, and 3 died from malnutrition. Gastrointestinal lesions showed marked dilatation of the entire digestive tract from the esophagus to the rectum, which is different from other types of familial visceral myopathies. Microscopic examination of the gastrointestinal smooth muscle showed degeneration and increased fibrosis indistinguishable from other types of familial visceral myopathy.

Urological Manifestations of Patients with Visceral Myopathy

Of 10 patients with familial or nonfamilial visceral myopathy studied for urological manifestations only 3 had symptoms of urinary retention. Excretory urography revealed megacystis in all 3 symptomatic patients, while the 7 asymptomatic patients had normal-sized bladders. Cystometrograms performed in 4 asymptomatic patients also were normal. Bladder tissue from 5 patients was studied. Of 3 autopsy cystectomy specimens 2 were dilated severely and thin-walled without trabeculations or diverticula suggestive of mechanical obstruction. Microscopic sections showed segmental intrafascicular fibrosis in 4 patients, which was associated with elastosis in 3. Electron microscopy of leiomyocytes in abnormal fascicles revealed myofilament disorganization, lipid accumulation and increased numbers of cytoplasmic organelles. We conclude that pathological changes in the bladder occur commonly but urinary symptoms occur in less than half of the patients with visceral myopathy available for study. An abnormal excretory urogram was seen in only patients who had symptoms suggestive of lower urinary tract dysfunction.