Sickle cell anemia ??? clinical manifestations in 100 patients and review of the literature

Karayalcin, Gungor; Rosner, Fred; Kim, Kir Y.; Chandra, Pradeep; Aballí, Arturo J.

doi:10.1097/00000441-197501000-00007

Cited by 38 publications

(19 citation statements)

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“…Although there is no sex difference in the incidence of sickle cell anaemia, the male preponderance seen in this study had been previously reported ( Karayalcin et al 1975 ; Akinyanju & Johnson 1987). Whether it is a result of higher morbidity rate in males is however, not established.…”

Section: Discussioncontrasting

confidence: 47%

Acute sickle cell syndromes in Nigerian adults

Ibidapo¹,

Akinyanju²

2000

Clinical &Amp; Laboratory Haematology

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The pattern of acute illness was determined in 102 adolescents and adults with sickle cell anaemia who presented to the emergency unit of a Lagos hospital. The patients had a mean age of 20.5 years (SD 13.1) and a male-female ratio of 1.5. The symptoms included fever (72%), fatigue and weakness (59%), anorexia (59%) and pain (57.5%) while major clinical signs were pallor (100%), jaundice (71%) and hepatomegaly (68%). Sixty-eight per cent of patients had sickle cell crises, including one with hemiplegic stroke, 10% with combined anaemia and pain crises, 33% with anaemia crises only and 23.5% with pain crises only. Sixty-three per cent had infection which was malaria in 24.5%, bacterial in 17% and viral in 6%. Of 16 patients with pyrexia of unknown origin, seven responded to treatment with chloroquine and eight to antibiotics. Infection was detected in 50% of the patients with sickle cell crises. The association between anaemia crises and malaria was significant (P < 0.05). Of the eight deaths, seven (88%) had anaemia crises. In contrast to studies conducted two decades ago in the same hospital, the prevalence of anaemia crises now exceeds that of pain crises and malaria now exceeds that of bacterial infection. Severe symptomatic anaemia (anaemia crisis) was more frequently associated with infection (mostly malaria) than was bone pain crisis. The Girdle pain crisis more frequently resulted in a fatal outcome than the uncomplicated bone pain crisis.

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Section: Discussioncontrasting

confidence: 47%

Acute sickle cell syndromes in Nigerian adults

Ibidapo¹,

Akinyanju²

2000

Clinical &Amp; Laboratory Haematology

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“…The incidence of avascular necrosis of the vertebral bodies varies in different papers from 0.6% [20], to 1% [10] and 4.3% [4]. In our study 5.2% of patients suffered from avascular necrosis.…”

Section: Discussionsupporting

confidence: 48%

The spine in sickle cell disease

Sadat-Ali

Ammar

Corea

et al. 1994

International Orthopaedics

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Bone changes in sickle cell disease occur due to marrow hyperplasia, tissue ischaemia and infarction due to vaso-occlusion. Between 1982 and 1991 thirty four patients were treated in the Orthopaedic and Neurosurgery Departments of the Kind Fahd University Hospital, Al-Khobar, with spinal complications due to sickle cell disease. There were 21 males and 13 females aged between 4 and 28 years (mean 17.4 years). Structural changes in the vertebral bodies due to marrow hyperplasia occurred in 44% of the patients. Avascular necrosis leading to collapse of the vertebral bodies was seen in 9 (27%) patients. Infective spondylitis was the most serious complication seen in 8 (24%) patients; the majority needed anterolateral decompression and bone grafting. The spine is often affected in sickle cell disease and aggressive treatment with close follow-up is required to avoid disabling complications.

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“…Acute pain in the right upper quadrant is common in sickle cell patients [65–67]. The symptom of hepatobiliary disease often must be separated from the more common symptoms of sickle cell disease.…”

Section: Gastrointestinal/hepatobiliary Complicationsmentioning

confidence: 99%

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Ballas

Kesen

Goldberg

et al. 2012

The Scientific World Journal

151

144

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The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position 6 of the β globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.

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Sickle cell anemia ??? clinical manifestations in 100 patients and review of the literature

Cited by 38 publications

References 0 publications

Acute sickle cell syndromes in Nigerian adults

Acute sickle cell syndromes in Nigerian adults

The spine in sickle cell disease

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

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