Faculty Bibliography

Random fecal alpha 1-antitrypsin levels were determined in 34 patients, 24 with Crohn's disease, and 10 'controls' having diarrheal illnesses not associated with intestinal protein loss, in an effort to evaluate its usefulness as a measure of Crohn's disease intestinal activity. In the control group, all alpha 1-antitrypsin levels were less than 2 mg/g dry wt of stool. The mean fecal level among those with Crohn's disease was 52.9 mg/g (range less than 2 to greater than or equal to 200). There was a strong correlation between disease activity, as measured by a clinical score, and the alpha 1-antitrypsin levels (Spearman r = 0.65, p = 0.001). This correlation was similarly strong among those with colitis or ileitis. A fecal value greater than 20 mg/g may provide a rough guideline to separate patients with clinically active disease from those with inactive Crohn's disease, despite a considerable range of fecal levels among patients with a particular clinical score. Fecal alpha 1-antitrypsin levels correlated with several other laboratory measures that have been proposed as indicators of Crohn's disease activity. The serum orosomucoid, C-reactive protein, and albumin correlated with the clinical activity score among some of our patient groups. Both clinical scores and laboratory parameters, however, may have limited usefulness in a variety of circumstances. Random fecal alpha 1-antitrypsin determinations seem to provide a reliable, although not directly quantitative, measure of the intestinal activity among patients with Crohn's disease, especially when other methods may be inconclusive

Intestinal obstruction of the duodenum by entrapment between the aorta and the superior mesenteric artery (SMA) is an uncommon cause of megaduodenum. Despite many case reports, acceptance of the SMA syndrome as a clinical entity has been controversial on account of its confusion with other causes of megaduodenum. We therefore report a case of SMA syndrome which sharply exemplifies its clinical and anatomic features. The clinical findings are proximal duodenal obstruction with an abrupt cutoff and active peristalsis. The anatomic features of this entity are a narrow angle between the aorta and the SMA, together with high fixation of the duodenum by the ligament of Treitz and/or an anomalous SMA crossing directly over the aorta at its intersection with the duodenum. The SMA syndrome may occur as an acute self-limited event due to a reversible precipitating factor, or as a chronic recurring disorder. The acute form subsides with correction of the specific initiating factor; the chronic form responds favorably to simple surgical mobilization of the duodenum

Five patients with primary biliary cirrhosis and prolonged cholestasis underwent intensive plasmapheresis. The indications for plasmapheresis included intractable pruritus or hypercholesterolemia and xanthomatous neuropathy. Patients noted a rapid improvement of pruritus and fatigue which was sustained as long as plasmapheresis was continued. Cholesterol levels were lowered an average of 10.3 mmol/l and xanthomata were reduced in three of four patients. Two patients with painful neuropathy caused by xanthomata experienced relief of this symptom. The liver and spleen size were not affected by plasmapheresis, and activities of aminotransferases, alkaline phosphatase and titres of mitochondrial antibody remained unchanged. We conclude that plasmapheresis has a role in the therapeutic management of patients with advanced primary biliary cirrhosis who are disabled by the complications of pruritus, xanthomatous neuropathy, or hypercholesterolemia with xanthoma formation

Primary biliary cirrhosis (PBC) is a chronic nonsuppurative, destructive cholangitis, whose etiology is unknown. Morbidity arises early from pruritus and later from hypercholesterolemia with xanthoma formation. Therapy is supportive and directed at the complications of cholestasis. Plasmapheresis has been reported to benefit patients with hyperlipidemia and PBC; thus a pilot study of plasmapheresis utilizing the Haemonetics Model 30 with replacement by albumin and saline was conducted. Five patients (four female and one male) with a mean age of 43 (range 29-58) and a mean duration of illness of 9.5 years (range 6-21) with marked jaundice, xanthomas, xanthelasma, hepatomegaly, fatigability, anorexia, and pruritus, as well as mild nausea were studied. Peripheral neuropathy was present in two patients. Two patients had splenomegaly. Two patients had an associated Sjogren syndrome. All patients had high serum bilirubin, alkaline phosphatase, and cholesterol levels and mild elevations in aspartate amino transferase and alanine amino transferase activities. Immune complexes measured in four patients were present. Antimitochondrial antibody titers were significant in all patients. Patients underwent a mean of 63 plasmapheresis procedures over a mean of 112 weeks removing a mean of 94.7 liters of plasma. No serious toxicity was seen. All patients showed a reduction in pruritus, xanthomas, xanthelasmas, and serum cholesterol values. The two patients who had evidence of Sjogren syndrome noted subjective improvement. All patients who had fatigue, anorexia and nausea also noted moderate improvement. There was no change in hepatomegaly or splenomegaly in patients demonstrating such organomegaly. Liver function did not change significantly. Overall, four patients had improvement in their condition and one patient achieved stability.(ABSTRACT TRUNCATED AT 250 WORDS)