Chronic intestinal pseudo-obstruction.

Intestinal pseudo-obstruction (IP) is an uncommon disorder of gut motility which must be differentiated from mechanical intestinal obstruction. We have seen 11 such patients over the last 5 years. Characteristic symptoms, shared by mechanical obstruction, include abdominal distention and pain, nausea, and vomiting. Radiologic studies reveal dilated loops of bowel with air fluid levels. In most patients a major differentiating feature from obstruction may be the presence of diarrhea rather than obstipation. Steatorrhea is secondary to an overgrowth of anaerobic bacteria in the motionless dilated loops of bowel. IP has been associated with various disorders: in our series two patients had scleroderma, one multiple small bowel diverticula, one systemic amyloidosis, one celiac disease, and one spinal cord injury; in only two patients was the disorder considered "idiopathic." Three patients had previously undergone a jejuno--ileal bypass for morbid obesity. During the acute episode, the patients were treated symptomatically with decompression by nasogastric or rectal tube with fluid and electrolyte replacement. Malabsorption treated with broad spectrum antibiotics reversing the steatorrhea but not episodes of pseudo-obstruction. Magnesium deficiency was present in seven patients and its correction resulted in amelioration of the symptom complex. In two patients episodes of pseudo-obstruction were markedly reduced by metoclopramide which was not effective in two others.

Anorectal manometry in the diagnosis of Hirschsprung's disease in adults.

Anorectal manometry is a useful tool in evaluating the cause of chronic constipation and is reliable in the diagnosis of Hirschsprung's disease. Two adult patients ages 35 and 69 with constipation since birth underwent anorectal manometry. Balloons positioned at the internal and external anal sphincters were connected to pressure transducers and their responses to a distention of rectal balloon were recorded. Both patients demonstrated failure of relaxation of the internal anal sphincter which is characteristic of Hirschsprung's disease. This diagnosis was confirmed at surgery by histologic examination failing to show ganglia. Anorectal manometry is a useful adjunct in the differential diagnosis of adult patients with megacolon.

Acquired hypomegakaryocytic thrombocytopenic purpura. Occurrence in a patient with absent thrombopoietic stimulating factor.

A 49-year-old woman had purpura and thrombocytopenia not associated with drugs or identifiable underlying disease. The platelet survival was normal and the marrow showed a sharp reduction in megakaryocytes with preservation of other cell lines. There was no response to steroids or infusion of fresh frozen plasma. Lithium carbonate therapy similarly had no effect. Thrombopoietic activity was absent in serum and urine samples. Erythropoietin activity was normal. In vitro formation of granulocyte-macrophage colonies in soft agar was normal. The case represents a unique incidence of selective megakaryocytic hypoplasia, though to result from a failure in stem cell differentiation.

Hepatic infarction in ulcerative colitis during pregnancy.

Acute hepatic failure developed at the time of parturition in a 27-yr-old woman. Liver biopsy demonstrated disseminated hepatic infarctions which were confined to the distal segments of the hepatic microcirculatory units. This episode of hepatic infarction was followed by persistent fever and cutaneous vasculitis, which resolved on prednisone therapy. The diagnosis of ulcerative colitis was made 5 mo after recovery from severe hepatic failure.