[High-dose intravenous gammaglobulin in the treatment of immune thrombocytopenic purpura].
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Biomedical subjects
Publications and source records attributed to D Branski.
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A 12-month-old infant developed bilateral facial paresis 4 weeks following a febrile illness associated with tonsillitis, bronchopneumonia and hepatosplenomegaly. Complement fixing antibody titer to Mycoplasma pneumoniae was 1:128, which subsequently dropped to 1:16. The clinical presentation in our patient was unusual in that he developed bilateral facial palsy at a very young age, and there was also a possible association of the palsy with Mycoplasma infection.
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A 69-year old woman suffered from severe dysphagia, abdominal pain, and weight loss. The dysphagia was accompanied by nasal speech, nasal regurgitation of food, weakness, and wasting of the proximal muscles of the upper and lower girdles. Laboratory data revealed T3 sephadex uptake 65.2%; T4 15.1 mcg%; and T3 250 ng%. Treatment with antithyroid medication reversed the manifestation of all the symptoms, including dysphagia. Cine-studies revealed esophageal motor dysfunction as the cause of the dysphagia. Hyperthyroidism is a rare, but treatable cause of unexplained dysphagia.
Diarrhea is one of the important clinical symptoms in patients suffering from celiac disease and is attributed mainly to malabsorption. We determined prostanoid content in small intestinal mucosa of five patients with active celiac disease and in a control group consisting of six patients. Prostaglandin E2 and thromboxane B2 content in duodenal mucosa of patients with active celiac disease was 1,581 +/- 161 and 118 +/- 40 pg/mg wet wt, respectively, significantly higher than their content in duodenal mucosa of the control group, 378 +/- 86 and 8 +/- 8, p less than 0.001 and p less than 0.02, respectively. 6-Ketoprostaglandin F1 alpha content in celiac patients was not significantly different from its content in the control group: 908 +/- 437 and 124 +/- 53 pg/mg wet wt, respectively. It is possible that, in celiac disease, increased mucosal prostanoid content may contribute, at least in part, to intestinal electrolyte and fluid secretion and consequent diarrhea.
In 13 patients with endoscopically proven duodenal ulcer, biopsies were obtained from the stomach body and antrum and from the duodenal bulb before and, in 10, after 4 weeks of cimetidine treatment (1 g/day). The specimens were organ-cultured for 90 min, and prostanoid accumulation in the medium was determined by radio-immunoassay. After 4 weeks of cimetidine treatment, prostaglandin E2 and 6-keto-prostaglandin F1 alpha synthesis by cultured specimens obtained from the body of the stomach (1304 +/- 197 and 497 +/- 124, no. +/- 10) was significantly higher than their respective synthesis before therapy (734 +/- 90 and 222 +/- 26; no. = 13) (X +/- SE, pg/mg wet wt/90 min). Prostanoid synthesis by cultured specimens from the antrum and duodenum was not significantly different before and after cimetidine treatment. It is therefore suggested that cimetidine, in addition to its antisecretory effects, accelerates ulcer healing also by induction of endogenous gastric prostanoid synthesis.
A 15-month-old girl initially suspected of having Kawasaki disease is presented. The diagnosis was based on the combination of prolonged fever, conjunctivitis, edema of hands and feet, exanthem and lymphadenopathy. A workup for infectious etiologies was negative. She was subsequently found to have acetaminophen hypersensitivity. To our knowledge this clinical presentation of acetaminophen hypersensitivity has not previously been described in medical literature in English.
A responsive, iterating program is described (available on diskette from first author), which enables the physician to formulate a balanced, total parenteral nutrition (TPN) for low-birth-weight and sick newborns. The program allows for the possibility of TPN or simultaneous intravenous, intraarterial, and oral feeding. It calculates the overall balance of fluids, nutrients, calories, electrolytes, minerals, trace elements, and vitamins. It features the integration of algorithms and limit tests of nutritional balance, to produce a feeding program that can be modified by clinical considerations of the physician, specific for each patient. The entire procedure can be accomplished, and a record of the entries and orders to the pharmacy and to the ward staff printed, within about 4-5 min. The program, which is written in BASIC, can be accommodated within and operated with a 16K byte microcomputer, equipped with a monitor, a printer, and a diskette or other program storage device.
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A 5-year-old girl suffering from Henoch--Schonlein purpura developed severe abdominal pain accompanied by vomiting and fever. Concomitantly, the serum amylase level became elevated and leukocytosis developed, with a shift to the left. A diagnosis of pancreatitis complicating Henoch--Schonlein purpura was made. This rare complication is presented, along with a review of the literature.
Campylobacter has recently been recognized as a common pathogen of the intestinal tract in pediatric practice. We report on 21 patients who were diagnosed as having enteritis due to Campylobacter jejuni infection. The most common symptom was diarrhea, accompanied by fever, vomiting and abdominal pain. The in vitro sensitivity test demonstrated the efficacy of aminoglycosides, chloramphenicol and erythromycin in the treatment of this disease. All the patients were symptom-free when discharged from the hospital.
A 2 1/2-month-old infant suffering from pyrexia, purpura, hepatosplenomegaly, pancytopenia and hyperlipidemia is reported. Liver and spleen biopsies revealed mononuclear histiocytic infiltration with marked erythrophagocytosis. The girl died at 7 1/2 months of age. Her brother died in infancy with an analogous clinical picture. The parents were first cousins. The clinical presentation and laboratory findings are consistent with the diagnosis of familial erythrophagocytic lymphohistiocytosis.
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A leukemic mouse model was employed to elucidate the separate effect of leukemia and cytotoxic drugs on the jejunal mucosa and its associated digestive enzymes. The mitotic activity, depth of the crypt and villus-crypt quotient were not significantly changed in leukemic mice in comparison to normal mice. The mitotic activity and the depth of the crypt 48 h after 20 mg methotrexate (MTX)/kg were significantly reduced (p less than 0.01) in leukemic mice. Sucrase (p less than 0.001) and maltase (p less than 0.025) activities in the jejunum from leukemic mice were significantly elevated in comparison with non-leukemic controls. In both non-leukemic and leukemic mice, the dose-response curves for MTX administration revealed a significant decrease and a nadir in sucrase (p less than 0.001) and maltase (p less than 0.0025) activities at the dosage of 20 mg/kg. Thus, in the mouse model, leukemia per se does not contribute to significant diminution in small intestinal function. In the small intestine, MTX appears to be responsible for a decrease in the mitotic activity of crypt cells, depth of the crypt and diminished sucrase and maltase activities.
The association of lactase deficiency with recurrent abdominal pain was investigated. One hundred three white children between the ages of 6 to 14 years with recurrent abdominal pain were evaluated. Sixty-nine underwent lactose tolerance tests and 26 had intestinal biopsies with lactase determinations; 21 of 69 (30.4%) had abnormal lactose tolerance tests and eight of 26 (31%) were lactase deficient. However, 16 of 61 (26.4%) control subjects matched for age and ethnic background exhibited lactase deficiency. Thus, a similar prevalence of lactase deficiency was found in the control and the recurrent abdominal pain groups. Thirty-eight patients with recurrent abdominal pain completed three successive six-week diet trials conducted in a double-blind fashion. An increase above base line value in pain frequency was seen in ten of 21 (48%) lactose malabsorbers and four of 17 (24%) lactose absorbers. After a 12-month milk elimination diet, six of 15 (40%) malabsorbers and five of 13 (38%) absorbers had elimination of their pain. This result compared with improvement occurring in five of 12 (42%) absorbers with recurrent abdominal pain who received a regular diet for one year and suggests that the elimination of lactose will not affect the overall frequency of improvement in recurrent abdominal pain. In addition, the recovery rate from recurrent abdominal pain is similar in both lactose absorbers and nonabsorbers independent of dietary restrictions.
Whether prolonged cholestasis is followed by hepatic cirrhosis is still controversial. We have studied two unrelated children who have had cholestasis for 15 years, but neither of whom have developed cirrhosis. Both have severe growth retardation, peculiar facies, pulmonic stenosis, transitory renal tubular acidosis, and vitamin D-resistant rickets. The patients presented in infancy with hepatomegaly and direct hyperbilirubinemia; liver biopsy at that time revealed cholestasis and paucity of bile ducts. Subsequent serial liver biopsies have continued to demonstrate cholestasis, but there has been no evidence of cirrhosis. Electron microscopy has revealed swollen and blunted microvilli of the canalicular membrane of the hepatocyte. The patients have had elevated bile acids in the serum as well as a reversed ration of tri- to dihydroxy bile acids. Treatment with cholestyramine and phenobarbital has brought about symptomatic relief from severe pruritus and excoriation and has lowered the level of serum bile acids, although they are still above the normal range. These findings suggest that cholestasis accompanied by an elevated and reversed bile acid ratio does not universally cause hepatic cirrhosis.
Alterations in pancreatic function and structure were examined in suckling mice infected intraperitoneally with reovirus type 3. The results were compared to pancreatic zymogen enzyme activities and histology in adult mice infected with the same virus. No effect of the rovirus type 3 on the adult mice could be elicited. In contrast, the suckling mice infected by the reovirus type 3 revealed a definite change in pancreatic zymogen enzymes. However, the zymogen enzymes were affected in a nonparallel fashion and three groups of enzymes with different responses were noted. Amylase and lipase activities were significantly diminished (P less than 0.001) at 6 days of viral infection. The endopeptidases, trypsin (P less than 0.025) and cymotrypsin (P less than 0.001) activities were increased significantly in the infected group. The exopeptidases, carboxypeptidase A and B in the infected animals were not changed significantly compared to the control. It seems reasonable that the reovirus type 3 infection in the suckling mouse causes diminished lipase and amylase activities that might contribute to the pathogenesis of viral enteritis.
Suckling mice infected with reovirus type 3 were examined for changes in the epithelial brush border of the small intestine. After 3 days of infection with reovirus type 3, no significant changes were found in intestinal morphology or activity of any enzymes tested. After 6 days, villi were shortened and blunted with lymphangiectatic lesions and mild mononuclear infiltration in the lamina propria. In addition, there was a significant decrease in lactase (P < 0.001) and enterokinase activity (P < 0.05). However, there were no significant changes in the activities of alkaline phosphatase. In contrast, maltase (P < 0.001) and leucine aminopeptidase (P < 0.05) activities in the infected mice were significantly increased. These data suggest that brush border enzymes are affected differently by reovirus infection.