Assuntos
Humanos , Lactente , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Masculino , Feminino , Anemia Falciforme/complicações , Manifestações Oculares , Talassemia/complicações , Anemia Falciforme/epidemiologia , Malformações Arteriovenosas/etiologia , Hemoglobinas Anormais , Jamaica , Artéria Retiniana , Doenças Retinianas/etiologia , Hemorragia Retiniana/etiologia , Talassemia/epidemiologiaRESUMO
Nine patients had a syndrome characterized by the presence of a sea-blue histiocyte and splenomegaly. Lipid analysis of liver from the most recent patient revealed accumulations of specific glycosphingolipids and phospholipids. Urinary mucopolysaccharide excretion was increased, and there was a suggestion of mucopolysaccharide storage in the hepatic parenchymal cells. Clinically, patients with the disease may have a relatively benign course, with mild purpura secondary to thrombocytopenia, or may have progressive hepatic cirrhosis, hepatic failure and death. The name "syndrome of the sea-blue histiocyte," the striking morphologic feature of the disease, is suggested for these cases (AU)
Assuntos
Humanos , Adulto , Masculino , Feminino , Histiócitos , Doenças Linfáticas , Grânulos Citoplasmáticos/análise , Manifestações Oculares , Fundo de Olho , Glicolipídeos/análise , Histiócitos/análise , Fígado/patologia , Linfonodos/patologia , Glicosaminoglicanos/urina , Fosfolipídeos/análise , Doenças Linfáticas/diagnóstico , Doenças Linfáticas/genética , Baço/patologiaRESUMO
Retinal detachment, brown mottled areas and microcapillary abnormalities at the posterior pole are previous undescribed retinal features of sickle cell anemia that were found in a series of 76 Jamaicans with homozygous sickle cell anemia. These subjects were consecutive patients in a sickle cell clinic during a one-month period; thus, the series is unique in that bias in selective inclusion of cases with ocular pathology was avoided. Tortuosity of the major retinal vessels, previously reported as a major feature of the retinopathy was found in unaffected relatives of SS patients in the same family with normal hemoglobin genotype. Angioid streaks were not seen in this series. (AU)
Assuntos
Humanos , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Masculino , Feminino , Anemia Falciforme/complicações , Manifestações Oculares , Doenças Retinianas/etiologia , Fístula Arteriovenosa/etiologia , Genótipo , Hemoglobinas , Transtornos da Pigmentação/etiologia , Descolamento Retiniano/etiologia , Hemorragia Retiniana/etiologia , Pigmentos da Retina , Vasos Retinianos , Retinite/etiologia , Doenças Vasculares/etiologia , JamaicaRESUMO
Patients with sickle cell disease were divided into 5 groups according to the extent of abnormal vessels in the conjunctival vasculature. There was a significant relationship between the degree of abnormality and the number of irreversibly sickled cells, suggesting that these cells must be related to the genesis of these vessel anomalies. Evidence is accumulating that irreversibly sickled cells are related to several pathological processes in sickle cell anaemia, and we believe the sign may be useful not only in suggesting the diagnosis but also in gauging the severity of the condition (AU)
Assuntos
Humanos , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Masculino , Feminino , Anemia Falciforme/diagnóstico , Capilares , Túnica Conjuntiva/irrigação sanguínea , Eritrócitos , Manifestações Oculares , Anemia Falciforme/complicações , Dilatação , Genótipo , Jamaica , Doenças Vasculares/etiologiaRESUMO
A study in Jamaica of 60 patients with sickle-cell anaemia over the age of 30 years showed that most of them were in full-time employment. Pains in the bones or joints, leg ulceration, and jaundice were the most frequent types of presentation, but only two patients had a haemoglobin level consistently below 6 g./100 ml. Most of the patients were well developed and of average height, and, though the development of secondary sexual characteristics was delayed, there was an average of 2.6 pregnancies per patient. These findings suggest that the course is more benign than has been realized. (AU)
