Porphyria cutanea tarda associated with autoimmune hypothyroidism, vitiligo and alopecia universalis.

The aetiology of porphyria cutanea tarda (PCT) has not been elucidated, but the possibility of an autoimmune mechanism has been proposed. We report a case of an unknown clinical combination of PCT with autoimmune hypothyroidism, alopecia universalis and vitiligo with thyroid and parietal cell circulating antibodies. This is highly suggestive of underlying autoimmune damage in this patient.     

In vivo 23Na NMR studies of myotonic dystrophy

Myotonic dystrophy is an inherited multi-system disease. Its pathophysiology leading to muscle malfunction and damage is not well understood. ²³Na NMR spectroscopy was applied here for an in vivo comparative study of the calf muscles of 7 myotonic dystrophy patients at various stages of the disease and 11 healthy volunteers. Both the total sodium content, expressed as the ratio of the ²³Na and ¹H water signals, and the fast transverse relaxation time, T₂₁, determined from the triple quantum-filtered spectra, increased in correlation with the severity of the disease. The results demonstrate that ²³Na NMR enables the quantitation of myotonic dystrophy progression.     

Hepatotoxicity in HIV-infected children and adolescents on antiretroviral therapy.

CONTEXT AND OBJECTIVE: Adverse drug reactions are a significant problem in patients on antiretroviral therapy (ART). We determined liver enzyme elevation frequencies in HIV-infected children and adolescents receiving ART, and their association with risk factors. DESIGN AND SETTING: Cross-sectional study, at the Pediatrics Immunodeficiency Division, University Hospital, Universidade Estadual de Campinas. METHODS: Medical records of 152 children and adolescents (54.6% male; median age 7.48 years) were analyzed, with a mean of 2.6 liver enzyme determinations per patient. Clinically, patients were classified in categories N (6), A (29), B (78) and C (39). Serum levels of aspartate aminotransferase and alanine aminotransferase were evaluated. Hepatotoxicity was scored as grade 1 (1.1-4.9 times upper limit of normality, ULN), grade 2 (5.0-9.9 times ULN), grade 3 (10.0-15.0 times ULN) and grade 4 (> 15.0 times ULN). To assess hepatotoxicity risk factors, odds ratios (OR) and adjusted odds ratios (aOR) for age, gender, TCD4+ cell count, viral load and medication usage were calculated. RESULTS: We observed grade 1 hepatotoxicity in 19.7 % (30/152) patients. No cases of grade 2, 3 or 4 were detected. There was a significant association between hepatotoxicity and use of sulfonamides (OR, 3.61; 95% confidence interval (CI), 1.50-8.70; aOR, 3.58; 95% CI, 1.44-8.85) and antituberculous agents (OR, 9.23; 95% CI, 1.60-53.08; aOR, 9.05; 95% CI, 1.48-55.25). No toxicity was associated with ART. CONCLUSIONS: One fifth of patients experienced mild hepatotoxicity, attributed to antituberculous agents and sulfonamides. Our results suggest that ART was well tolerated.     

The efficacy of propafenone in preventing recurrent auricular fibrillation and flutter]

In order to investigate the efficacy of propafenone in the prevention of paroxysmal flutter or fibrillation, we treated 21 patients without left ventricular disfunction. Age was 60 +/- 14 (mean +/- sd) years, left atrial diameter by echocardiography 37 +/- 7 mm, cardiothoracic index 0.48 +/- 0.05 (0.41-0.57) and P wave duration 100 +/- 17 ms. The frequency of recurrences before treatment was: daily in five (23%), weekly or more in eight (38%), monthly-weekly in seven (33%) and quarterly-monthly in one (5%). Propafenone (671 +/- 187 mg/24 h) was given after recurrences were demonstrated under treatment with 1-3 antiarrhythmic drugs per patient. During 8.9 +/- 3.5 months of follow-up (range 6-19) 5 patients (23%) were completely free of recurrences; in seven (33%) the incidence decreased by greater than 50% with a marked decrease in duration. Side effects appeared in 12 cases (57%), leading to its discontinuation in four (19%). Arrhythmogenic effects were observed in 2 cases (9%). Propafenone is effective in greater than 50% of patients with paroxysmal atrial flutter or fibrillation, resistant to other antiarrhythmic agents. The incidence of side effects is high, but they are usually not severe and reversible.     

An epidural abscess due to resistant Staphylococcus aureus following epidural catheterisation

We report a case of abscess formation after epidural analgesia, a rare complication that developed in our patient 13 days after placement of a thoracic epidural catheter for patient controlled analgesia. Culture of the pus grew methicillin-resistant Staphylococcus aureus . Although early diagnosis and rapid management have been reported to yield a satisfactory outcome, the case we describe ended in severe sequelae.     

Comparative study of oral conditions in schoolchildren of Strasbourg, France, 1974–85

A representative sample of 1650 children randomly selected in the 6-15-yr-old schoolchild population of Strasbourg was examined by well-calibrated examiners. The prevalence of caries was determined with the DMFT, DMFS and dft indices using bitewing radiographs. Plaque, calculus and gingival indices were also determined. The results obtained were compared with the initial study of 1974 performed in Strasbourg using the same epidemiologic methods. Whereas no important variations were observed in caries prevalence of primary teeth, a significant reduction of caries activity was observed in DMFT and DMFS indices in all age groups. There was a reduction of these two indices of respectively 32% and 33% in the 12-yr-old children. The reduction was the most significant on approximal surface lesions. A statistically significant decrease of the calculus and gingival indices was also observed between 1974 and 1984. A less significant decrease was observed for the plaque index.     

Degradation of extracellular matrix proteins (fibronectin, vitronectin and laminin) by serine-proteinases isolated from Lonomia achelous caterpillar hemolymph.

Lonomia achelous is a caterpillar distributed in southern Venezuela and in northern Brazil that causes an acute hemorrhagic syndrome in people who have contact with its bristles. The effect of the crude hemolymph and its chromatographic fractions (FDII, Lonomin V and Lonomin V-2) on extracellular matrix proteins was studied. The chromatographic fractions show activities similar to plasmin and urokinase. In sodium dodecyl sulfate-polyacrylamide gel electrophoresis, both lonomins appear as a protein band of 25 kDa under reduced conditions. By exclusion chromatography, the molecular weights of Lonomin V and Lonomin V-2 were 26.5 and 24.5 kDa, respectively. Fibronectin, laminin and vitronectin were degraded by all venom components. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis, under reduced conditions, shows that lonomins degrade fibronectin in four main fragments of 116, 60, 50 and 30 kDa. Molecular exclusion chromatography in native conditions shows that the molecular masses of these fragments are > or = 300, 62 and 27 kDa. The proteolytic effect of lonomins was abolished by benzamidine/HCl, iodoacetic acid and aprotinin. The extracellular matrix protein degradation together with the fibrino(geno)lytic activity of hemolymph and its fractions could explain, in part, the hemorrhagic syndrome, and the wound dehiscence in persons who have had contact with the L. achelous caterpillar.     

与长寿有关的特殊脂蛋白表型和基因型

背景：长寿个体年龄相关疾病的发病率较低且发病时凤明显后延，其家族成员可能遗传有某些调节衰老过程或疾病敏感性的因子。