RESUMO
Mollaret's meningitis is a recurrent meningitis, usually caused by Herpes simplex virus type 2. Symptoms are typical for meningitis, but other causes for recurrent and chronic meningitis have to be excluded in the cerebrospinal fluid. Usually Mollaret's meningitis is a benign form of meningitis without sequelae. There are no evidence-based data about therapy because of few reported cases.
Assuntos
Transtornos da Cefaleia/etiologia , Herpes Simples/diagnóstico , Herpesvirus Humano 2 , Meningite Asséptica/diagnóstico , Meningite Viral/tratamento farmacológico , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Recidiva , Reação em Cadeia da Polimerase Via Transcriptase ReversaRESUMO
At routine follow-up a 23-year-old female presented a high erythrocyte sedimentation rate and handicapping lipodystrophy of the lower limbs. Protein electrophoresis showed absence of an albumin peak and the diagnosis of analbuminaemia was therefore proposed. Investigation of family members disclosed that one brother out of a total of four siblings also had analbuminaemia. The hereditary pathway often appears to be autosomalrecessive. Subsequent review of the literature revealed only 28 other observed cases, although today electrophoresis has become a worldwide routine examination. Surprisingly, the almost complete absence of so important a protein as albumin does not trigger disease. The body is able to compensate for the lack of albumins with other proteins. The absence of abumin is typically associated with hyperlipidaemia and lipodystrophy in the female sex.
Assuntos
Lipodistrofia/sangue , Albumina Sérica/deficiência , Adulto , Sedimentação Sanguínea , Feminino , Genes Recessivos , Humanos , Lipodistrofia/genética , Masculino , Núcleo Familiar , Linhagem , Albumina Sérica/genéticaRESUMO
A patient with familial analpha-lipoproteinaemia (Tangier disease) was found to have stomatocytosis and haemolytic anaemia. The analysis of the red cell membrane constituents revealed a low cholesterol content (90 nmol/ml red cells, control 130 nmol/ml red cells), a decreased cholesterol/phospholipid ratio (0.54, control 0.78), high phosphatidylcholine (41.5%, control 30.6%) and low sphingomyelin (18.8%, control 27.6%). The electrophoretic membrane protein pattern was normal. Osmotic gradient ektacytometry and osmotic resistance showed a decreased surface/volume ratio, which caused an increased filtration resistance in 3 microns pores. The elasticity of the membrane was unchanged. Functional membrane properties were altered: the anion exchange rate was increased, whereas alkali cation fluxes were normal. The capacity to release vesicles was reduced. This case represented a new type of stomatocytosis. It contributes to the understanding of the role of cholesterol and phospholipids in the red cell membrane and biomembranes in general.
Assuntos
Anemia Hemolítica Congênita/etiologia , Eritrócitos Anormais/ultraestrutura , Hipolipoproteinemias/complicações , Doença de Tangier/complicações , Anemia Hemolítica Congênita/sangue , Colesterol/sangue , Membrana Eritrocítica/análise , Feminino , Humanos , Microscopia Eletrônica de Varredura , Pessoa de Meia-Idade , Fosfolipídeos/sangue , Doença de Tangier/sangueAssuntos
Anormalidades Múltiplas/diagnóstico por imagem , Colo/anormalidades , Diafragma/anormalidades , Intestino Delgado/anormalidades , Anormalidades Múltiplas/etiologia , Adolescente , Colo/diagnóstico por imagem , Diafragma/diagnóstico por imagem , Feminino , Humanos , Intestino Delgado/diagnóstico por imagem , Radiografia , SíndromeRESUMO
A 50-year-old severely immunodeficient woman with malignant non-Hodgkin lymphoma died from graft-versus-host disease due to transfusion of a single unit of packed red cells. Three days after this transfusion a maculo-papular rash appeared, followed by generalized erythroderma refractory to therapy and eventually progressing into generalized ulcero-squamous dermatitis. This case, and a review of other similar cases published elsewhere, prompt the authors to recommend prophylactic irradiation of blood products prior to their administration to patients with cellular immunodeficiency, particularly in cases of acute leukaemia or malignant lymphoma where patients receive intensive radio- and/or chemotherapy regimens. To appreciate the degree of cellular immunodeficiency in such risk patients, simple criteria should be developed to assess the efficiency of the cellular immune system.