Biomedical subjects
Publications and source records attributed to J Goldenberg.
Reports of dermatomyositis (DM) and polymyositis complicating pregnancy are rare. Only nineteen cases have been published. We describe the first case in which juvenile DM appeared during pregnancy. An emergency cesarean section had to be performed due to fetal distress at 37 weeks of gestation. Patient and infant are doing well after 8 months of followup.
Two cases of systemic juvenile chronic arthritis, complicated by cardiac tamponade and eight previously reported cases are described. The clinical features and radiological, electrocardiographic, and echocardiographic findings were similar for all cases. One patient in this series was treated with corticosteroids and underwent pericardiectomy, dying of sepsis 30 days after surgery. The other patient was also treated with corticosteroids, underwent pericardiocentesis and developed fatal ventricular tachycardia. Of the 10 cases, only six were successfully treated. Early diagnosis and appropriate treatment are essential for a favourable outcome.
1. Twenty-eight patients with active definite primary ankylosing spondylitis and fifty-four healthy control subjects were studied. 2. The HLA-B27 antigen was found in 75% of patients and 3.7% of controls. 3. Fecal samples from these subjects were cultured for gram-negative enteric bacteria on two occasions within one month. Positive cultures for Klebsiella sp were found in 32.1% of patients and in 22.2% of healthy controls, but this difference was not statistically significant. All other microorganisms detected were qualitatively and quantitatively similar in both groups. 4. Significantly increased mean values of serum IgA levels were found in the patient group when compared with the control group (P less than 0.01). The mean serum IgG and IgM levels did not differ statistically between the two groups. There was no correlation between any laboratory or clinical parameter and presence of Klebsiella sp carriage in ankylosing spondylitis patients. 5. These data are consistent with the view that a long time elapses between exposure to a trigger factor and clinical manifestations of the disease.
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One hundred thirty five patients with carpal tunnel syndrome were studied trough electrophysiological techniques. Nerve sensory conductions, motor conductions and electromyography were performed in all patients and the results discussed. The authors call the attention for the technic of sensory conduction stimulating palma and picking up the potentials on the wrist as the most sensitive technic for the early detection of carpal tunnel syndrome.
A flavokinase preparation from Bacillus subtilis is described which catalyzes the phosphorylation of reduced, but not oxidized, riboflavin. The enzyme is distinguished from other known flavokinases also in having an unusually low Km for the flavin substrate, 50 to 100 nM. ATP is the obligatory phosphate donor; one ATP is utilized for each FMNH2 formed. Mg2+ or Zn2+ is required for the reaction; Co2+ and Mn2+ will substitute, but less effectively. The same enzyme preparation catalyzes the synthesis of FADH2 from FMNH2 and ATP, but not the synthesis of FAD from FMN and ATP. FADH2 is also formed from reduced riboflavin, presumably by sequential flavokinase and FAD synthetase action. Zn2+ cannot replace Mg2+ in FADH2 formation. The reverse reaction, formation of FMN from FAD, occurs only with reduced FAD, giving rise to FMNH2, and is dependent on the presence of inorganic pyrophosphate. The enzyme thus appears to be an FADH2 pyrophosphorylase. The two enzymatic activities, flavokinase and FADH2 pyrophosphorylase, although not separated during the purification procedure, are distinguished by differences in metal ion specificity, in concentration dependence for ATP (apparent Km for ATP = 300 microM for FADH2 synthesis and 6.5 microM for flavokinase), and in the inhibitory effects of riboflavin analogues.
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No abnormality of exocrine function of the pancreas was found in 10 patients with amyotrophic lateral sclerosis. Eight patients showed abnormal glucose metabolism, attributed to the effects of age, malnutrition, diminished physical activity, and decreased muscle mass.
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