Sickle cell anemia is an inherited abnormality of the globin chain with very high prevalence in the Indian subcontinent. A significant proportion of these patients present late in life and are at a risk of complications like acute chest syndrome and painful episodes till a definitive diagnosis is reached and appropriate treatment is started . We report a novel triad of abdominal imaging finding...

BACKGROUND We report the results of our experience on laparoscopic cholecystectomy in sickle cell disease patients in Niger, which is included in the sickle cell belt. METHODS A prospective study covering a period of 45 months, from July 2004 to March 2008. We included all sickle cell disease patients that underwent laparoscopic cholecystectomy. Blood transfusion was done for patients with ha...

The osteoarticular involvement in sickle cell disease has been poorly studied and it is mainly characterized by osteonecrosis, osteomyelitis and arthritis. The most frequent complications and those that require hospital care in sickle cell disease patients are painful vaso-occlusive crises and osteomyelitis. The deoxygenation and polymerization of hemoglobin S, which results in sickling and vas...

BACKGROUND Spinal cord stimulation (SCS) has recently been reported to be effective for truncal postural abnormalities such as camptocormia and Pisa syndrome in Parkinson's disease. In this case report, we describe a case of a woman with Parkinson's disease in whom SCS was effective for painful camptocormia with Pisa syndrome. CASE PRESENTATION A 65-year-old woman was admitted to our hospital...

INTRODUCTION We present a case involving a patient with sickle cell and hyposplenism, in which refractory septic shock quickly responded after the infusion of intravenous gammaglobulin (IV-GG) given as an adjuvant-rescue therapy CASE DESCRIPTION A 30-year-old African-American female with history of Sickle Cell disease was admitted for acute chest syndrome, septic shock and respiratory failure...

BACKGROUND Perioperative blood transfusion is usually given to sickle cell disease patients to reduce or prevent perioperative morbidity. Assessment of such a practice was the subject of our study. METHODS A retrospective one year survey of sickle cell disease patients undergoing surgery at Salmaniya Medical Complex, Bahrain was conducted. The medical records were reviewed to characterize the...

The acute chest syndrome (ACS), a pneumonia-like illness in sickle cell patients, is one of the most frequent causes of their morbidity and hospitalizations. Repeated ACS events may predict the development of chronic lung disease. ACS is reported as a frequent cause of death in these patients. We examine here the incidence and risk factors of ACS in 3,751 patients with sickle cell disease who w...

Sickle cell disease (SCD) is a group of disorders that affects hemoglobin, the red pigment in blood erythrocytes responsible for delivering oxygen throughout the body. This is the most commonly inherited blood disorder. One of the most widespread manifestations of SCD is painful vaso-occlusive crises and osteomyelitis. Dactylitis or hand-foot syndrome is the first clinical sign of SCD among chi...

Seventy cases of sickle-cell disease were identified in the London Borough of Brent from records dating back to 1962. All but three were still alive and, with one exception, were recalled for confirmation of the diagnosis and to provide personal and family histories. The group consisted of 22 individuals with homozygous sickle-cell anaemia (Hb SS), 12 with sickle-cell/beta-thalassaemia double h...

Pulmonary hypertension is a life-threatening complication of sickle cell disease. L-Arginine is the nitrogen donor for synthesis of nitric oxide, a potent vasodilator that is deficient during times of sickle cell crisis. This deficiency may play a role in pulmonary hypertension. The enzyme arginase hydrolyzes arginine to ornithine and urea, and thus, it may compete with nitric oxide synthase, l...