Familial dysequilibrium-diplegia with T-lymphocyte deficiency.

A second family is described with a combination of defective thymus-dependent immunity and cerebral palsy. The cerebral palsy comprised nonprogressive dysequilibrium and mild spastic diplegia without limb ataxia. This genetic entity of presumed autosomal recessive inheritance is clearly distinguished from ataxia-telangiectasia. Immunological abnormalities should be sought in other familial or unexplained cerebral palsy syndromes.     

Effects of Eleutherococcus senticosus extracts on hexobarbital metabolism in vivo and in vitro

Eleutherococcus senticosus (Siberian Ginseng) is widely exported from China as a health food. Pharmacologically it has been classified as an adaptogen and enzyme induction has been proposed as its mechanism of action. To evaluate this hypothesis E. senticosus was administered to mice on an acute (40–320 mg/kg i.p., × 1 day) or chronic (80–320 mg/kg i.p., × 4–5 days) basis. Sleep latency and duration, in response to hexobarbital sodium (100 mg/kg i.p.), were determined either 1 h (acute and chronic) or 24 h (chronic) following the last E. senticosus injection. E. senticosus produced a sedative effect which decreased the sleep latency (47%) and increased sleep duration (45–228%) following acute administration. A similar effect was seen following chronic administration (125–202% increase in sleep duration). E. senticosus was also shown to produce an inhibition (66%) of hexobarbital metabolism, in vitro, as compared to controls. These data support enzyme inhibition rather than enzyme induction as a mechanism for the actions of Siberian Ginseng.     

Duodenal Crohn''s Disease Complicated by Common Bile Duct Obstruction: Report of a Case and Review of the Literature

A 28-year-old woman with Crohn's disease involving the duodenum and terminal ileum presented with biochemical evidence of incomplete, extrahepatic biliary obstruction. Percutaneous transhepatic cholangiography showed partial obstruction of the intramural duodenal portion of the common bile duct. Cholangiography was complicated by cholangitis. Surgical decompression of the biliary system and bypass of the affected duodenal segment were performed after intraoperative confirmation of Crohn's disease involving the ileum, duodenum, and ampulla of Vater. The patient made a complete recovery and all previously abnormal cholestatic liver function test results returned to normal.     

Benign duodenocolic fistula

Three cases of benign duodenocolic fistula are presented, and the diagnosis and treatment reviewed. Patients with benign duodenocolic fistulas usually complain of diarrhea, and occasionally nausea and feculent vomiting. Physical examinations are nonspecific, revealing wasting from the chronic diarrhea. Barium enemas are usually diagnostic. Therapy consists of excision of the fistula and repair of the duodenal and colonic defects.     

Cancellous bone resorption in the proximal ilium of the ovariectomized rat

Summary Bone histomorphometry was performed on the proximal ilium of mature Sprague-Dawley rats following ovariectomy, and these rats were compared with sham-operated controls. Bone volume per unit tissue volume (BV/TV), osteoid surface, and the depth and extent of eroded cavities were measured in animals killed at intervals after operation. The rate of bone loss and the mean osteoid surface in the proximal ilium of the ovariectomized rats was significantly greater than that of the control rats over a 210-day postoperative period. The eroded surface and mean trabecular thickness in the proximal ilium of the ovariectomized rats were not significantly different from that of the control rats, and therefore failed to explain the difference in the rate of bone loss. The distribution of the depths of trabecular eroded cavities in the ilium of ovariectomized rats was different from that in the control rats. The loss of trabecular bone mass in the proximal ilium of ovariectomized, mature rats appeared due to increased activity of individual osteoclasts, rather than to increased osteoclast numbers and thinning of trabeculae.     

Perioperative blood transfusion adversely affects prognosis of patients with stage I non-small-cell lung cancer

It has been speculated that blood transfusion might adversely affect prognosis in cancer patients by immunosuppression. To avoid the confounding affect of advanced disease, we tested this hypothesis in 117 patients with stage I non-small-cell lung cancer. Mean and median follow-up were 49.7 months and 47 months, respectively. Patients who died during the postoperative period were not included. Perioperative transfusion was defined as administration of whole blood or packed cells within 30 days of operation. The overall cumulative 5-year disease-free survival rate was 67%. In patients with transfusion, it was 53% and in patients without transfusion it was 81% (p = 0.0055). A multivariate analysis was performed that included patient age, race, sex, cell type, extent of operation (pneumonectomy versus lobectomy/segmentectomy), operative blood loss, admission hematocrit, discharge hematocrit, and the presence or absence of perioperative transfusion. The only variable that significantly correlated with 5-year disease-free survival was the presence or absence of perioperative transfusion (p = 0.0278), and this effect was not related to the number of transfusions. Retrospective analysis of long-term results of patients surviving curative operation for stage I lung cancer shows that any perioperative transfusion significantly worsens the patient's prognosis and suggests very strongly that this association is due to an adverse effect of the transfusion rather than the transfusion serving as a marker for another risk factor.     

Cyclosporine-associated renal arteriopathy resulting in loss of allograft function

Cyclosporine-associated arteriopathy was the cause of graft loss in 40 percent of all allografts that failed in a series of 200 consecutive cadaveric renal transplants. Arteriopathy was diagnosed by biopsy and renal uptake of indium 111m labeled platelets in the face of acute renal deterioration. A moderate thrombocytopenia and microangiopathic picture of hemolytic uremia was also present on peripheral blood smear. Immunofluorescence and histologic characteristics of the allograft biopsy specimens failed to show evidence for acute rejection: immunoglobulin M, immunoglobulin A, immunoglobulin G, C1q, C3, and C4 were not present, and there was no evidence of an interstitial or vascular mononuclear cellular infiltrate. Two clinical presentations have been described. In Group I (seven patients), anuria occurred rapidly within the first 2 weeks after transplantation. In Group II (nine patients) renal function gradually diminished 1 to 5 months after starting cyclosporine therapy. Fifteen of the 16 recipients had progressive and irreversible loss of renal function which was pathologically associated with fibrin deposition, intimal proliferation, and thrombotic occlusion of the cortical interlobular and arcuate arteries, with subsequent focal glomerular ischemia and cortical infarction. One recipient with rapid loss of renal function received an intraarterial allograft infusion of streptokinase and subsequent systemic heparinization, which resulted in return of normal allograft function. The syndrome of cyclosporine-associated arteriopathy has been linked to a lack of or reduced amounts of prostacyclin-stimulating factor or prostacyclin.     

Quipazine and light have similar effects on c-fos induction in the rat suprachiasmatic nucleus

The effects of the serotonin agonist, quipazine, on the induction of c-fos in the suprachiasmatic nucleus of the rat was examined at different times of the 24 h cycle. Quipazine administered at night induced Fos production in a dose dependent manner (1, 3, 10, 30 μmol/kg) in the ventrolateral portion of the suprachiasmatic nucleus at ZT18. Administration of the highest dose at other times resulted in c-fos induction at ZT15 but not at other times of the day or subjective day examined (CT6 and ZT12). When compared to the effects of light pulses (2 lux/1 min), quipazine only caused c-fos induction at times when light caused induction. Our results support a role of serotonergic pathways in the transmission or modulation of photic information from the retina to the suprachiasmatic nucleus of the rat.