Osteonecrosis of the femoral head among adult patients with sickle-cell disease in Guadeloupe: prevalence, clinical features and risk factors

Osteonecrosis of the femoral head (ONFH) is a frequent complication of sickle-cell disease (SCD). We conducted systematic screening of ONFH by using the association of radiography, bone scintigraphy and tomodensitometry. This paper presents our first results concerning 113 adult patients (67 SS, 46 SC), prevalence, clinical features, risk factors, and association with other SCD complications. Forty-two patients (37.2 percent) were found to have osteonecrosis of one or both hips (67 hips, 29.6 percent) without association to a particular genotype, although bilateral involvement was considerably more frequent among SS patients. ONFH affected patients of all ages and in particular SC young adult patients were found to be affected frequently. ONFH was diagnosed at pre-radiological stages (Stage 1) for 31.3 percent of hips and was frequently aysmptomatic (60 percent of all cases, 95 and 90 percent of Stages I and II, respectively), indicating need for systematic screening when early diagnosis is desired. For SS patients, ONFH was more frequent when follow-up began later. ONFH was also associated with higher parity and history of leg ulcer for both genotypes. No correlation was found with biological data, in particular haematological variables, except for high glucose levels for SS patients. We have initiated a prospective study of ONFH among patients followed at the SCD Centre of Guadeloupe, with special concern in diagnosis at pre-radiological stages, and outcome of patients. Further data will be provided by longitudinal follow-up of adult patients as well as inclusion of children followed from birth after neonatal screening (AU)

Two different forms of homozygous sickle-cell disease - abstract

Haematological, clinical and some molecular genetic features have been compared in two groups of patients with homozygous sickle-cell (SS) disease in Saudi Arabia, 33 patients from the Eastern Province (eastern) and 30 from the South Western Province (Western). Eastern patients all had the Asian haplotype of DNA polymorphisms within the beta globin gene cluster whereas Western patients were more variable but predominantly of the Benin haplotype. Eastern patients had significantly more deletional alpha thalassaemia, higher levels of total haemoglobin and foetal haemoglobin, and lower of HBA, mean volume reticulocytes, and platelets. Clinically, Eastern patients had a greater persistence of splenomegaly, less dactylitis, less acute chest syndrome, a more normal body build and greater subscapular skin fold thickness. Painful crises occurred with equal frequency in both groups. Avascular necrosis of the femoral head was common in both groups. The disease in the Eastern province has many mild features consistent with the higher HbF levels and more frequent alpha thalassaemia but bone pathology (painful crises, avascular necrosis of the femoral head, osteomyelitis) remains common. The disease in the West is more severe, consistent with the Benin haplotype suggesting an African origin (AU)

Haematological factors associated with avascular necrosis of the femoral head in homozygous sickle cell disease

The haematological indices in 50 patients with homozygous sickle cell (SS) disease and avascular necrosis of the femoral head and in 180 patients with SS disease without this complication have been compared. Avascular necrosis of the femoral head was significantly associated with high total levels and with low fetal haemoglobin levels. Low fetal haemoglobin levels are associated with increased intravascular sickling. These results suggest that both intravascular sickling and the increased viscosity associated with high haemoglobin levels are related to the development of avascular necrosis of the femoral head in SS disease. (AU)

Haemoglobin SC disease in Jamaica

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)

Conditions of the hip associated with hemoglobinopathies

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)