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1.
Eye ; 7(5): 703-6, 1993.
Artigo em Inglês | MedCarib | ID: med-8043

RESUMO

The effect of sectoral, scatter laser photocoagulation on proliferative sickle retinopathy (PSR) was investigated by reviewing florescein angiograms of 88 sickle cell-haemoglobin C patients enrolled in a controlled, randomised trial. Follow-up was for a median period of 2.9 years. Complete infarction of all PSR in an eye occurred in 7 of 74 treated eyes and 2 0f 60 control eyes. Treatment resulted in significantly greater regression (decrease in number and size of PSR lesions) in eyes of patients aged <25 years at enrollment but not in eyes of patients> or = 25 years at enrollment. Infarction of the individual PSR lesions was significantly more common in treated eyes. Treated PSR was significantly more likely to infarct if small (< 15 degrees circumferential involvement) and if flat rather than elevated. New PSR was significantly less likely to develop in treated eyes.


Assuntos
Humanos , Adulto , Doenças Retinianas/cirurgia , Doença da Hemoglobina C/complicações , Lasers , Retina/cirurgia , Angiofluoresceinografia , Doenças Retinianas/etiologia
2.
Clin Lab Haematol ; 13: 229-37, 1991. tab, gra
Artigo em Inglês | MedCarib | ID: med-3596

RESUMO

The red cell distribution width (RDW) has been studied during the clinical steady state in 1121 patients with homozygous sickle cell (SS) disease, 344 with sickle cell-haemoglobin C (SC) disease, 68 with sickle cell-beta+ thalassaemia, 49 with cell beta§ thalassaemia and in 130 control subjects with a normal (AA) genotype. The mean RDW was moderately increased in Sbeta+ thalassaemia and SC disease and markedly increased in Sbeta§ thalassaemia and SS disease. In SS, SC and Sbeta§ thalassaemia genotypes, lower RDW values occurred in females and with alpha thalassaemia. The RDW correlated negatively with total haemoglobin, mean cell haemoglobin concentration, mean cell volume and fetal haemoglobin (HbF) and positively with reticulocyte count in SS disease. A low RDW was associated with higher weight and less frequent dactylitis, painful crisis, acute chest syndrome, acute splenic sequestration and hospital admissions. A low RDW in SS disease is consistent with a high total haemoglobin, high HbF, low reticulocyte count, alpha thalassaemia and a more mild clinical course. (AU)


Assuntos
Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Lactente , Anemia Falciforme/sangue , Índices de Eritrócitos , Eritrócitos Anormais/ultraestrutura , Traço Falciforme/sangue , Fatores Etários , Anemia Falciforme/patologia , Estudos de Coortes , Globinas/genética , Doença da Hemoglobina C/sangue , Doença da Hemoglobina C/complicações , Deficiência de Ferro , Índice de Gravidade de Doença , Fatores Sexuais , Traço Falciforme
3.
Trop Geogr Med ; 40(1): 7-12, Jan. 1988.
Artigo em Inglês | MedCarib | ID: med-10061

RESUMO

Out of about 200 patients with sickle cell disease (SCD) in the Netherlands, 6 percent are non-negroid patients from Turkey. 83 were assessed clinico-haematologically regarding the type of SCD, ethnic origin, concurrent O-thalassaemia (O-thal), and type of sickle cell gene (á-chromosome). 54 patients had homozygous sickle cell (SS),1 sickle cell haemoglobin D (SD) Punjab, 5 sickle cell á§-thalassaemia (Sá§-thal), 5 sickle cell á+-thalassaemia (Sá+-thal) and 18 sickle cell haemoglobin C (SC) disease. 14 percent of the 83 patients were from Turkey, the others were of West Indian and African origin, most (73 percent) of whom were from Surinam. The Netherlands may be the only country in the world where non-negroid SCD patients are present in such a proportion to negroid SCD patients. O-thal was detected in 16 patients and in 14 of their relative with sickle cell trait. Four main types of ás-chromosomes were identified: Benin, Central African Republic, Senegal and Saudi Arabia types. SS and Sá§-thal disease ran a more severe course than Sá+ +-thal and SC disease. No clinical difference was ascribable to ethnic origin, O-thal or HbF-level but in each ethnic group there were some patients with a remarkably mild course of SS disease, which was related to the type of ás-chromosome. These were the Senegal and Saudi Arabia ás-chromosomes. Proper differentiation between genotypes is of prognostic and therapeutic relevance,especially in SC disease as it is sometimes discovered too late. A proper screening program is encouraged not only for all negroid inhabitants or immigrants, but also for non-negroid immigrants especially from Turkey.(AU)


Assuntos
Humanos , Lactente , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Anemia Falciforme/epidemiologia , África/etnologia , Anemia Falciforme/complicações , Anemia Falciforme/etnologia , Grupos Étnicos , Globinas/genética , Doença da Hemoglobina C/complicações , Doença da Hemoglobina C/epidemiologia , Países Baixos , Talassemia/complicações , Talassemia/epidemiologia , Turquia/etnologia , Índias Ocidentais/etnologia
5.
Br J Ophthalmol ; 66(3): 149-54, Mar. 1982.
Artigo em Inglês | MedCarib | ID: med-9579

RESUMO

Ophthalmological examinations were performed on 59 of the 74 (80 percent) children with homozygous sickle cell (SS) disease and on 37 of the 54 (69 percent) children with sickle cell-haemoglobin C(SC) disease, aged 5-7.5 years, within the cohort study of sickle cell disease. Arteriolar sheathing was the commonest retinal vessel abnormality, occurring in 30/59 (51 percent) SS children and in 11/37 (30 percent) SC children. Peripheral arteriolar closure was observed in 14 (24 percent) SS children and in 6 (16 percent) SC children. Arteriovenous anastomoses were seen in 3 children, but proliferative retinopathy was not identified. Capillary changes often occurred in patients without confluent closure, suggesting that complex remodelling of the capillary bed may precede retinal nonperfusion. Discrete retinal patches similar to schisis cavities resulting from intraretinal haemorrhages were found in 22 (37 percent) SS children and in 9 (24 percent) SC children, but haemorrhages were observed in only 2 patients (1 SS, 1SC). Vitreous opacities were common and were generally associated with retinal vessel disease. Retinal changes were consistently more common in children with SS disease, though the differences failed to reach statistical significance. The prevalence of peripheral vascular closure and retinal patches showed a significant upward trend with age. These observations contrast with the greater prevalence of proliferative retinopathy characterising SC disease in adults.(AU)


Assuntos
Humanos , Pré-Escolar , Criança , Masculino , Anemia Falciforme/complicações , Doenças Retinianas/etiologia , Oftalmopatias/etiologia , Angiofluoresceinografia , Genótipo , Doença da Hemoglobina C/complicações , Doenças Retinianas/patologia , Hemorragia Retiniana , Vasos Retinianos/patologia , Corpo Vítreo/anormalidades
6.
Br J Ophthalmol ; 65(10): 712-7, Oct. 1981.
Artigo em Inglês | MedCarib | ID: med-14818

RESUMO

In a selected sample of patients with sickle cell-haemoglobin C (SC) disease, proliferative retinopathy (PSR) occurred in 90/243 (37 percent) patients, developed most frequently between the ages of 20 and 30 years, and affected 68 percent patients aged 45 years or over. Comparison of haematological indices in patients with and without PSR, after age-related effects were allowed for, indicated significant relationships with high mean cell volume in males and with low fetal haemoglobin in both sexes. A highly significant relationship with total haemoglobin level in males (as previously reported in SS disease) was shown in SC disease to be entirely secondary to a strong age-related trend in haemoglobin level (AU)


Assuntos
Humanos , Recém-Nascido , Lactente , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Masculino , Feminino , Anemia Falciforme/complicações , Doença da Hemoglobina C/complicações , Doenças Retinianas/etiologia , Traço Falciforme/complicações , Fatores Etários , Índices de Eritrócitos , Doença da Hemoglobina C/sangue , Hemoglobinas/análise , Doenças Retinianas/sangue , Traço Falciforme/sangue
7.
Trans R Soc Trop Med Hyg ; 75(2): 228-33, 1981.
Artigo em Inglês | MedCarib | ID: med-14740

RESUMO

The four common genotypes of sickle cell disease in Jamaica are homozygous sickle cell (SS) disease, sickle cell-haemoglobin C (SC) disease, sickle cell-á+ thalassaemia, and sickle cell-᧠thalassaemia with respective incidence at birth of 3.2, 2.0, 0.34, and 0.16 per 1000 live births. Haematological indices, clinical features, and over-all prognosis vary between these genotypes and also between patients within individual genotypes. Although symptomatic selection has tended to emphasize more severely affected patients, this wide variation of clinical and haematological severity is especially apparent in SS disease. Factors contributing to this variability in SS disease include the persistence of foetal haemoglobin, the association with alpha thalassaemia, and the interaction with environmental factors of which socioeconomic status is the most obvious. Further elucidation of factors determining the severity of SS disease will increase understanding of the pathogenetic mechanisms in the disease and may also indentify new possibilities for therapeutic intervention (AU)


Assuntos
Humanos , Recém-Nascido , Masculino , Feminino , Anemia Falciforme/epidemiologia , Anemia Falciforme/genética , Hemoglobina Fetal/análise , Genótipo , Doença da Hemoglobina C/epidemiologia , Doença da Hemoglobina C/genética , Doenças do Recém-Nascido/epidemiologia , Jamaica , Prognóstico , Fatores Sexuais , Fatores Socioeconômicos , Talassemia/epidemiologia , Talassemia/genética
8.
Trans Ophthalmol Soc U K ; 100(3): 434-9, Sept. 1980.
Artigo em Inglês | MedCarib | ID: med-9333

RESUMO

Small vessel obstruction characterizes sickle cell disease and when occurring in the peripheral retinal vessels, initiates a sequence of vascular events which may culminate in the development of proliferative sickle retinopathy (PSR). Repeated examinations of the retinal vasculature of patients with different genotypes of sickle cell disease over the last 10 years (Condon and Serjeant,1972a,b,c; 1975; 1980a) have allowed observations on the natural history of PSR and on factors related to its development. Choroidal neovascularization has been a common complication of a trial of xenon arc photo-coagulation in PSR. Observations on the aetiology and natural history of both retinal and choroidal neovascularization are presented in this report. (AU)


Assuntos
Humanos , Criança , Adolescente , Adulto , Masculino , Feminino , Anemia Falciforme/patologia , Corioide/irrigação sanguínea , Vasos Retinianos/patologia , Anemia Falciforme/sangue , Hemoglobina Fetal/análise , Angiofluoresceinografia , Doença da Hemoglobina C/sangue , Doença da Hemoglobina C/patologia , Hemoglobinas/análise , Infarto/patologia
9.
West Indian med. j ; 28(4): 219-21, Dec. 1979.
Artigo em Inglês | MedCarib | ID: med-11242

RESUMO

Anthropometric measurements were made in 52 adults with SC disease and compared with those on controls. Weight and interacromial diameter were significantly less in males but no other parameters differed from controls. In contrast to SS disease, SC disease does not appear to affect body habitus(AU)


Assuntos
Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Anemia Falciforme/epidemiologia , Antropometria , Doença da Hemoglobina C/epidemiologia , Jamaica
10.
Br J Haematol ; 43(1): 49-56, Sept. 1979.
Artigo em Inglês | MedCarib | ID: med-14423

RESUMO

The determinants of steady state haemoglobin levels in sickle cell-haemoglobin C (SC) disease were investigated by measuring routine haematological and biochemical indices, red cell survival, oxygen affinity, pitted erythrocytes, and red cell and plasma volumes in 31 adult patients (15 male; 16 female). Red cell survival was shortened in all subjects, and was positively correlated with haemoglobin level. However, many haemoglobin values were within the normal range, especially in male subjects. Palpable splenomegaly, which occurred in 53 percent of patients, did not appear to effect haemoglobin levels, red cell survival, plasma volume, but was associated with lower platelet counts and decreased pitted red cells. Sex related differences were found in total haemoglobin, packed cell volume, conductivity cell volume, red cell count, and in the blood volume measurements. Red cell, plasma and total blood volumes in patients varied with weight and cube of height in manner observed in normal subjects, although red cell volumes were lower and plasma volumes were greater than in normal subjects of given height and weight. Anaemia in SC disease is related to the haemolytic rate but the major determinant appears to be an inappropriate increase in plasma volume (Summary)


Assuntos
Humanos , Adolescente , Adulto , Masculino , Feminino , Anemia Falciforme/sangue , Doença da Hemoglobina C/sangue , Hemoglobinas/metabolismo , Volume Sanguíneo , Envelhecimento Eritrocítico , Hematócrito , Oxigênio/sangue , Pressão Parcial , Volume Plasmático , Fatores Sexuais , Esplenomegalia/sangue
11.
Br Med J ; 1(6126): 1515-6, June 10, 1978.
Artigo em Inglês | MedCarib | ID: med-12579

RESUMO

In Jamaican children with homozygous sickle cell (SS) disease diagnosed at birth two-year survival was 87 percent, compared with 95 percent in children with sickle cell-haemoglobin C (SC) disease, and 99 percent in normal controls. Death among those with SS disease occurred most often between the ages of 6 and 12 months. Principal causes were acute splenic sequestration and pneumococcal infection. Neonatal diagnosis of haemoglobinopathies must be followed by close observation if mortality is to be reduced by early diagnosis and treatment of these complications. (AU)


Assuntos
Humanos , Recém-Nascido , Lactente , Pré-Escolar , Criança , Anemia Falciforme/diagnóstico , Anemia Falciforme/mortalidade , Doença da Hemoglobina C/mortalidade , Fatores Etários , Seguimentos , Doenças do Recém-Nascido/diagnóstico , Infecções Pneumocócicas/mortalidade , Jamaica
12.
Thorax ; 33(1): 85-8, Feb. 1978.
Artigo em Inglês | MedCarib | ID: med-10201

RESUMO

Prediction equations have been evolved for the assessment of vital capacity, total lung capacity, and the single breath carbon monoxide transfer factor in haemoglobin SS and haemoglobin SC disease. These relationships take account of the growth disorder and anaemia in the sickle-cell states. The results suggest that, in the clinically stable state, and effects of alveolar capillary sickling and haemoconcentration and any altered reactivity of haemoglobins S and C with the test gas are of no significance for clinical respiratory physiology. Sex differences in lung function appear independent of haemoglobin type. (AU)


Assuntos
Humanos , Adulto , Masculino , Feminino , Anemia Falciforme/fisiopatologia , Pulmão/fisiopatologia , Fatores Etários , Monóxido de Carbono , Hemoglobina C/análise , Doença da Hemoglobina C/fisiopatologia , Hemoglobina Falciforme/análise , Medidas de Volume Pulmonar , Fatores Sexuais
14.
Br J Ophthalmol ; 57(7): 644-9, July 1974.
Artigo em Inglês | MedCarib | ID: med-9374

RESUMO

The ophthalmological findings in 54 Jamaican children with SC disease are reported. Evidence of peripheral retinal vessel disease was present in 94 percent and retinitis proliferans in 11 percent. Retinitis proliferans was noted as early as 7 years of age and was more common in patients with high haemoglobin levels. There was an unequivocal progression in severity of retinopathy in eight out of eleven children examined 2 years previously. The pathological processes leading to sickle cell proliferative retinopathy are well established in childhood and attempts at prophylactic therapy should be instituted at an early age. (Summary)


Assuntos
Humanos , Pré-Escolar , Criança , Adolescente , Masculino , Feminino , Anemia Falciforme/complicações , Doença da Hemoglobina C/complicações , Vasos Retinianos , Retinite/etiologia , Fístula Arteriovenosa/etiologia , Angiofluoresceinografia , Isquemia/etiologia , Jamaica , Doenças Retinianas/etiologia , Doenças Vasculares/etiologia
15.
Br J Radiol ; 46(551): 935-42, Nov. 1973.
Artigo em Inglês | MedCarib | ID: med-13323

RESUMO

A skeletal survey was carried out on 84 cases of haemoglobin SC disease. The radiological features noted were increased translucency of bone, abnormal trabecular pattern, medullary expansion, wide vascular channels in phalanges, and radiological evidence of infarction, both medullary and cortical. The frequency of bone lesions thus demonstrated increased with age. The most characteristic lesions in this region were those of vascular necrosis; contributing factors such as the high haemoglobin level characteristic of the condition are discussed. An interesting feature was the lack of correlation betweeen clinical severity and degree of radiological change. (AU)


Assuntos
Humanos , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Anemia Falciforme/diagnóstico por imagem , Doença da Hemoglobina C/diagnóstico por imagem , Anemia Falciforme/complicações , Osso e Ossos/irrigação sanguínea , Osso e Ossos/diagnóstico por imagem , Doenças Ósseas/diagnóstico por imagem , Reabsorção Óssea , Fêmur/diagnóstico por imagem , Necrose da Cabeça do Fêmur/complicações , Dedos/irrigação sanguínea , Dedos/diagnóstico por imagem , Doença da Hemoglobina C/complicações , Quadril/diagnóstico por imagem , Articulação do Quadril , Úmero/diagnóstico por imagem , Infarto , Jamaica , Vértebras Lombares/diagnóstico por imagem , Necrose/sangue , Osteoartrite/complicações , Periósteo/diagnóstico por imagem , Rádio (Anatomia)/diagnóstico por imagem , Vértebras Torácicas/diagnóstico por imagem , Tíbia/diagnóstico por imagem
16.
Br J Haematol ; 25(4): 437-44, Oct. 1973.
Artigo em Inglês | MedCarib | ID: med-13321

RESUMO

The prevalence of several haemoglobin defects, including the traits for á-thalassaemia (0.8 percent), hereditary persistence of foetal haemoglobin (0.2 percent) and the abnormal delta change haemoglobin, Hb B2 (2.4 percent), were determined from the combined results of surveys conducted on adults in a suburban and a rural community. Mean Hb A2 levels of 2.6 ñ0.4 percent, 5.3 ñ0.5 percent and 2.0 ñ0.2 percent were found in 639 Hb A homozygotes, seven á-thalassaemia traits and three traits for hereditary persistence of of foetal haemoglobin (HPFH), respectively. Levels of alkali resistence haemoglobin (A.R.Hb) ranged from 0.6 to 7.3 percent in the thalassaemia traits and were 21.0, 19.0 and 16.0 percent in the three HPFH traits; the remaining 770 subjects in whom A.R.Hb was measured had a mean value of 0.6 ñ0.6 percent. (AU)


Assuntos
Humanos , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Masculino , Feminino , Hemoglobinopatias/epidemiologia , Fatores Etários , Doença da Hemoglobina C/epidemiologia , Hemoglobinometria , Hemoglobinas Anormais/análise , Ferro/sangue , Afro-Americanos , Ligação Proteica , Saúde da População Rural , Talassemia/epidemiologia , Jamaica
17.
Br J Haematol ; 24(4): 491-501, Apr. 1973.
Artigo em Inglês | MedCarib | ID: med-13317

RESUMO

The clinical and haematological features of 90 Jamaican patients with haemoglobin SC disease are reviewed. Mean haemoglobin levels indicated mild anaemia although individual haemoglobin levels were often within the normal range. The clinical features were qualitatively similar to those of homozygous sickle cell disease(SS disease) although they were generlly less frequent and of lesser severity. Ocular pathology was an exception, occuring more frequently in SS disease even in age-sex-matched groups. There is some evidence that the higher haemoglobin level in SC disease may be aetiologically related to retinal vascular disease. (AU)


Assuntos
Humanos , Criança , Adolescente , Adulto , Masculino , Feminino , Anemia Falciforme/complicações , Doença da Hemoglobina C/complicações , Fatores Etários , Bilirrubina/sangue , Doenças Cardiovasculares/complicações , Eletroforese em Gel de Amido , Contagem de Eritrócitos , Oftalmopatias/complicações , Hematócrito , Hemoglobinas/análise , Hepatomegalia/complicações , Jamaica , Artropatias/complicações , Pneumonia/complicações , Reticulócitos , Fatores Sexuais , Esplenomegalia/complicações
18.
Clin Orthop ; 90: 22-8, Jan.-Feb. 1973.
Artigo em Inglês | MedCarib | ID: med-8732

RESUMO

In the Western hemisphere where malaria and parasitic infection is rare, the complications of the abnormal hemoglobins are those essentially of infarction. Hyperplastic changes never reach the extent seen in West Africa, infection is a less serious problem, patients survive into adult life and the hip joint produces a large proportion of the orthopedically crippled. Treatment follows the same lines which would be adopted in a patient with avascular necrosis of the femoral head with normal hemoglobin. The prognosis is worse because the age of onset is usually later resulting in slower and less complete restoration to normalcy. Special care in the general management of patients during operations is essential, particularly the avoidance of excessive blood loss or anoxia which may precipitate a crisis (summary)


Assuntos
Humanos , Criança , Adolescente , Adulto , Masculino , Feminino , Doenças Ósseas/etiologia , Necrose da Cabeça do Fêmur/etiologia , Quadril , Hemoglobinopatias/complicações , Anemia Falciforme/complicações , Doenças do Desenvolvimento Ósseo/etiologia , Doenças do Desenvolvimento Ósseo/diagnóstico por imagem , Diagnóstico Diferencial , Cabeça do Fêmur/diagnóstico por imagem , Doença da Hemoglobina C/complicações , Infarto/diagnóstico , Infarto/etiologia , Osteocondrite/etiologia , Osteocondrite/diagnóstico por imagem , Osteomielite/diagnóstico , Talassemia/complicações , Trombose/etiologia
19.
Br J Haematol ; 23(2): 205-13, 1972.
Artigo em Inglês | MedCarib | ID: med-14406

RESUMO

Red-cell characteristics were studied in the steady state in 3 sickle-cell syndromes, homozygous sickle-cell disease (SS), sickle-cell/heamoglobin-C disease (SC), and sickle-cell/á-thalassaemia (S/thal). Hb-SC disease had the highest haemoglobin levels, red cells counts, and mean corpuscular haemoglobin concentrations, all of which may contribute to the high thrombotic tendency noted in this disease. The two types of S/thal (with and without Hb A) generally had different haematological features. The non-Hb-A type of S/thal, which may resemble SS disease on electrophoretic techniques and present a diagnostic problem, was distinguishable on many red cell characteristics reported here (Summary)


Assuntos
Humanos , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Masculino , Feminino , Anemia Falciforme/sangue , Eritrócitos Anormais , Contagem de Eritrócitos , Hematócrito , Doença da Hemoglobina C/sangue , Hemoglobinometria , Ferro/sangue , Jamaica , Reticulócitos , Talassemia/sangue
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