Lepra leucomelanodérmica / Leukomelanodermic leprosy

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Leukomelanodermic leprosy

A 39-year-old woman presented with a 6-year history of asymptomatic macules on her back. The patient had no other complaints, and she did not report any case of infectious disease in her family. Examination revealed hyperpigmented macules, presenting irregular edges but with a sharp demarcation, on the midline of the back from the cervical to the lumbar region. Within these hyperpigmented areas, islands of normal appearing skin were observed. There were also some hypopigmented macules on the lateral and posterior aspects of the trunk (Fig. 1). The patient presented thickening of the left ulnar nerve and sensory loss to temperature on the lateral aspect of the left arm. Biopsy specimens were obtained from the hyper- and hypopigmented areas. In the hyperpigmented macule, the biopsy revealed focal areas of hypomelanosis in the epidermis and the presence of melanophages in the superficial dermis, but no acid-fast bacilli were found (Fig. 2a). The Masson-Fontana stain showed an evident pigmentary incontinence (Fig. 2b). The biopsy obtained from the hypopigmented lesion also revealed focal areas of hypomelanosis, but in the superficial dermis an infiltrate of foamy macrophages was observed, as typically found in lepromatous leprosy (Fig. 3a); acid-fast bacilli were found by Fite-Faraco stain. The focal hypomelanosis was confirmed by Masson-Fontana stain (Fig. 3b). Mitsuda's reaction in this patient was negative and slit-skin smears (cutaneous lesion, earlobes, elbows, and knees) were negative for acid-fast bacilli. The patient was treated with multidrug therapy for multibacillary leprosy: rifampin, 600 mg once monthly (supervised), clofazimine, 300 mg once monthly (supervised), dapsone, 100 mg daily, and clofazimine, 50 mg daily, all given for 2 years. After 7 months of treatment, the hypopigmented lesions diminished and the hyperpigmented lesions improved after 2 years of treatment and the sensory loss to temperature was restored.

Síndrome de CHILD (hemidisplasia congênita com eritrodermia ictiosiforme e defeito de membros): relato de caso / Child's syndrome (congenital hemidysplasia with icththyosiform erythroderma and limb defects): a case report

Relato de caso de uma criança apresentando síndrome de CHILD (hemidisplasia congênita com eritrodermia ictiosiforme e defeito nos membros). Aparentemente a presença de um gene mutante no cromossomo X levaria a distúrbio no metabolismo de fibroblastos da pele, tendo como consequência displasias e anomalias cutâneas, esqueléticas e viscerais

Metabolismo da cafeína e apnéia neonatal / Metabolism of caffeine and apnea newborn infant

Os autores realizaram uma revisäo da literatura sobre o metabolismo da cafeina e seu uso na apnéia neonatal. A cafeina é uma droga eficaz no tratamento da apnéia neonatal estimulando o centro respiratório por bloqueio dos receptores de adenosina. Possui boa absorçäo gastrointestinal, sendo esta a via preferencial de administraçäo. A cafeina é metabolizada no fígado pelo citrocromo P-450; porém, no neonato esta via metabólica é deficiente, sendo que 85% da droga ingerida é eliminada intacta na urina. Em doses terapêuticas (10 - 15mg/Kg/24 horas dose inicial e 2mg/Kg/dia dse de manutençäo) näo se observaram efeitos de toxicidade aguda como vômitos, distensäo abdominal, tremores ou desidrataçäo. Porém, os níveis séricos de bilirrubinas e glicose, frequência cardíaca e presäo arterial, devem ser acompanhados durante o tratamento. Efeitos da toxicidade tardia, como alteraçöes no comprimento, peso, perímetro cefálico e seqüelas neurológicas näo foram observadas aso 6 e 12 meses de idade em crianças tratadas com cafeína no período neonatal