Search

The increase in the incidence of tuberculosis infection in the last few years has caused a recurrence of atypical clinical forms, as well as the development of associations and uncommon complications during the clinical course, which include the adult respiratory distress syndrome (ARDS) and septic shock. Three patients with ARDS are here reported; two patients had findings of septic shock and negative serology to human immunodeficiency virus and the only etiological agent documented was M. tuberculosis. The three patients required hemodynamic support and two of them mechanical ventilation. None of the patients survived the episode. Tuberculosis, particularly the disseminated forms, should be considered as possible etiology in high risk patients with septic shock, ARDS or both.

Although several clinical and morphological changes observed in overt systemic lupus erythematosus have been associated with the presence of antiphospholipid antibodies (aPL), the relation between these antibodies and lupus nephropathy (LN) is not clear. Twenty-three patients with biopsyproved LN were retrospectively studied (average age 28.5 +/- 12.3 years, all women) in order to investigate the relationship between the presence of aPL and clinical and immunobiological data. The average follow-up period was 55 +/- 42 months. The presence of aPL (IgG and IgM) was detected at least once in all patients by ELISA and/or lupus anticoagulant (kaolin time). Seven patients (30.4%) were aPL+, and the remainder aPL-. We did not find differences related to age, period of follow-up, blood pressure and livedo reticularis. However the prevalence of thrombosis, strokes and hemolysis was slightly higher in the aPL+ patients. The levels of antinuclear antibodies or anti-DNA antibodies, immunoglobulins and complement serum levels (C3, C4) were also similar in both groups. In the aPL+ group, proteinuria was significantly higher than in aPL- cases (2.21 +/- 1.5 and 0.91 +/- 1.07 g/24 h, respectively; p = 0.029). The renal histological pattern in both series was similar. However, microthrombosis in the glomerular capillary lumens was more frequent in the aPL+ group. The evolution of renal function was less favorable in aPL+ patients when compared with aPL- patients. We conclude that the presence of aPL in patients with LN is associated with several characteristics of renal impairment which may contribute to its evolution.

The lack of activity of the xanthine-oxidase conversion produces hypouricemia and hypocuria, with high urine elimination of xanthine and hypoxanthine. Due to the low solubility, it can result in urinary lithiasis. Two rare cases of xanthinuria caused by total lack of xanthine-oxidase are presented. A differential diagnosis of several congenital metabolic purine defects was carried out.

A 5-year-old patient developed pseudotumor cerebri, spinal and radicular pain, and hyporeflexia. Ataxia and multiple cranial nerve involvement also were evident. With the exception of residual hyporeflexia and the abnormalities present in the electrophysiologic studies, the patient's prompt recovery suggested the diagnosis of Guillain-Barré polyradiculoneuritis.

A previously healthy five years old boy, following a mild nonspecific upper respiratory infection developed, fever (39 degrees C), vomiting, clouding of consciousness and focal seizures. The CSF showed a mononuclear cell reaction with negative bacterial and viral cultures. A cranial CT scan on the 4th day of admission showed bilateral low density lesions on the basal ganglia region. After 30 days of severe involvement of muscle tone (rigidity) which kept the patient immobilized in bed and without a meaningful communication with his surroundings, improvement was noticed. A repeated CT scan 40 days after admission, was considered normal. Two months after the beginning of disease, patient's physical examination was normal. This case shows striking clinical and radiological similarities to the ones described by Aicardi and Goutieres in 1982 and most likely is explained by bilateral basal ganglia edema complicating viral encephalitis. Mumps virus, being so far, the most commonly implicated.

A child is described with accelerated bone maturation, large dysplastic ears, and hypertelorism. These are features of both the Marshall-Smith and Weaver's syndrome. The child was mentally retarded and had enlargement of the middle phalanges, features only seen in Marshall-Smith syndrome. However, there was also enlargement of the metaphyses, a characteristic of Weaver's syndrome. The possiblity that these syndromes may represent different manifestations of the same entity is discussed.

Sixty children, aged between 2 and 10 years, had boutonneuse fever during the summer months of 1979 and 1980. They presented with fever and a generalised maculopapular rash. The tàche noire could be seen at the site of the tick bite in 38 (63%) of them. The antibody response, assayed nonspecifically, by the Weil-Felix reaction was positive in 52. A singe titre of more than 1:80 or a 4-fold increase between two paired specimens separated by a 7-day interval was considered diagnostic. Maximum titres were reached at the end of the second week of convalescence in 81% of patients. Treatment with oral oxytetracycline was effective in all cases.