RESUMO
This paper presents 2 cases of hemophagocytic lymphohistiocytosis (HLH) in whom recombinant factor VIIa (rFVIIa) was used for the management of hemorrhage. Both patients were diagnosed as HLH and were bleeding from the gut, which could not be controlled. Patients received rFVIIa at total doses of between 90 and 180 microg/kg body weight. Hemostatic affect was achieved in both of the patients but lasted only a short time. The response was achieved after 1 h of administration of rFVIIa, lasting for 24 h. The use of rFVIIa was well tolerated. These 2 patients suggest that rFVIIa is a beneficial agent in the management of hemorrhage in patients with HLH, although for a permanent homeostasis the control of primary disease is essential.
Assuntos
Fator VIIa/uso terapêutico , Hemorragia/prevenção & controle , Linfo-Histiocitose Hemofagocítica/complicações , Proteínas Recombinantes/uso terapêutico , Pré-Escolar , Feminino , Humanos , Lactente , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , MasculinoRESUMO
Familial chylomicronemia syndrome is a group of rare genetic disorders characterized by deficient activity of an enzyme lipoprotein lipase or apo-protein C-II deficiency. In this paper we present an infant with massive hyperchylomicronemia and severe pancreatitis. Exchange transfusion for controlling hypertriglyceridemia and pancreatitis led to an increase in hyperviscosity which resulted in encephalopathy.
Assuntos
Apolipoproteína C-II/deficiência , Encefalopatias Metabólicas/diagnóstico , Hiperlipoproteinemia Tipo I/complicações , Hiperlipoproteinemia Tipo I/diagnóstico , Hipertrigliceridemia/complicações , Pancreatite/diagnóstico , Troca Plasmática , Viscosidade Sanguínea , Encefalopatias Metabólicas/etiologia , Humanos , Hiperlipoproteinemia Tipo I/etiologia , Lactente , Lipase Lipoproteica/deficiência , Lipoproteínas , Masculino , Pancreatite/etiologiaAssuntos
Edema/complicações , Edema/patologia , Face/patologia , Caxumba/complicações , Caxumba/patologia , Criança , Humanos , MasculinoRESUMO
Systemic capillary leak syndrome (SCLS) is a rare disorder of unknown pathophysiology, characterised by episodic life-threatening hypotension, haemoconcentration and hypo-albuminaemia. A 5-month-old child presented with episodes of relapsing diarrhoea, vomiting and hyponatraemia and developed generalised oedema during treatment. Clinical and laboratory findings were consistent with acute SCLS. Treatment with careful fluid replacement, fresh frozen plasma and aminophylline in the acute phase and teophylline and gingko biloba in the chronic phase led to sustained remission during follow-up for over 1 year. To our knowledge, this is the youngest case of SCLS in the literature and is unusual in that it presented with diarrhoea.