Sickle cell anemia

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Symptom/sign Organ system Percent affected Pubmed id Added on(yyyy-mm-dd) Edit/add reference
Cor pulmonale respiratory 100 % 7148875 2012-05-09
Acute chest syndrome multi 67 % 10861320 2011-10-27
Anemia circulatory 55 % 19500105 2011-10-27
Asplenia circulatory 45 % 7718896 2011-10-27
Priapism urinary 36 % 12460353 2011-10-27
Pulmonary hypertension circulatory 32 % 14985486 2011-10-27
Cholelithiasis digestive 31 % 10636979 2011-10-27
Stroke nervous 28 % 19500105 2011-10-27
Cor pulmonale respiratory 14 % 13410950 2012-05-09
Osteonecrosis skeletal 9 % 1944426 2011-10-27



List of references:


Pulmonary hypertension and cor pulmonale in the sickle hemoglobinopathies.
F S Collins, E P Orringer,

Although pulmonary hypertension is frequently mentioned as a complication of the sicklemic state, careful review of the medical literature revealed only a single subject in whom cardiac catheterization data substantiated this diagnosis. In two additional patients, both clinical and autopsy findings of pulmonary vascular disease and cor pulmonale were described, although no hemodynamic studies had been performed. We have therefore detailed the clinical history, cardiac catheterization results, and autopsy findings in three previously undescribed patients. These three patients, along with the three case reports culled from the medical literature, from the substance of this review. Pulmonary hypertension should be suspected in patients with sickle hemoglobinopathy in whom either fixed dyspnea or unexplained syncope develops. Early in the course of the disease, right heart catheterization remains the only way to establish the diagnosis with certainty. Noninvasive studies such as chest x-ray, electrocardiography, and echocardiography tend to be nondiagnostic until late in the course of right ventricular failure. Although specific therapy has yet to be defined, the ominous prognosis of this complication of sickle hemoglobinopathy supports the application of experimental modalities such as continuous oxygen therapy, partial exchange transfusion, or even limited phlebotomy.

The American journal of medicine - Dec 1982



Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group.
E P Vichinsky, L D Neumayr, A N Earles, R Williams, E T Lennette, D Dean, B Nickerson, E Orringer, V McKie, R Bellevue, C Daeschner, E A Manci,

The acute chest syndrome is the leading cause of death among patients with sickle cell disease. Since its cause is largely unknown, therapy is supportive. Pilot studies with improved diagnostic techniques suggest that infection and fat embolism are underdiagnosed in patients with the syndrome.

The New England journal of medicine - Jun 2000



Silent infarcts in young children with sickle cell disease.
Janet L Kwiatkowski, Robert A Zimmerman, Avrum N Pollock, Wendy Seto, Kim Smith-Whitley, Justine Shults, Anne Blackwood-Chirchir, Kwaku Ohene-Frempong,

Silent infarcts have been reported most commonly in school-aged children with homozygous sickle cell disease (SCD-SS) and are associated with neurocognitive deficits. However, the prevalence of silent infarcts in younger children with SCD-SS is not well defined. In this retrospective study, brain magnetic resonance imaging and angiography (MRI/A) studies performed before 6 years of age in a cohort of children with SCD-SS were analysed and the prevalence of abnormalities was calculated. Clinical and laboratory parameters were compared between the groups with and without silent infarcts. Sixty-eight of 96 children in the cohort had brain MRI/A performed prior to age 6 years. Of the 65 who were neurologically asymptomatic, 18 (27.7%, 95% CI 17.3-40.2%) had silent infarcts (mean age 3.7 +/- 1.1 years, range 1.3-5.9 years). Factors associated with silent infarcts included cerebral vessel stensosis by magnetic resonance angiography, lower rates of vaso-occlusive pain and acute chest syndrome and lower haemoglobin levels. The prevalence of silent infarcts in young children with SCD-SS is similar to that of older children and anaemia and severe vasculopathy may be risk factors.

British journal of haematology - Aug 2009



Functional asplenia in hemoglobin SC disease.
P A Lane, J L O'Connell, J L Lear, Z R Rogers, G M Woods, K L Hassell, D L Wethers, D W Luckey, G R Buchanan,

The incidence of functional asplenia in sickle-hemoglobin C (SC) disease has not been defined, and the use of prophylactic penicillin to prevent life-threatening septicemia in this disorder is controversial. The percentage of red blood cells with pits (pit count) is a reliable assay of splenic function in other disorders but has not been validated in hemoglobin SC disease. To address these issues, we conducted a prospective, multicenter study of splenic function in persons with hemoglobin SC disease. Baseline clinical data were recorded, and red blood cell pit counts were performed on 201 subjects, aged 6 months to 90 years, with hemoglobin SC; 43 subjects underwent radionuclide liver-spleen scanning. Pit counts greater than 20% were associated with functional asplenia as assessed by liver-spleen scan, whereas pit counts less than 20% were found in subjects with preserved splenic function. Pit counts greater than 20% were present in 0 of 59 subjects (0%) less than 4 years of age, in 19 of 86 subjects (22%) 4 to 12 years of age, and in 25 of 56 subjects (45%) greater than 12 years of age. Other subjects with hemoglobin SC, who had previously undergone surgical splenectomy, had higher pit counts (59.7% +/- 9.5%) than splenectomized subjects without hemoglobinopathy (38.5% +/- 8.8%) or with sickle cell anemia (20.5% +/- 1.9%; P < .001). Two subjects with hemoglobin SC disease (not splenectomized), ages 14 and 15 years, with pit counts of 40.3% and 41.7% died from pneumococcal septicemia. These data indicate that functional asplenia occurs in many patients with hemoglobin SC disease, but its development is usually delayed until after 4 years of age. The pit count is a reliable measure of splenic function in hemoglobin SC disease, but values indicative of functional asplenia (> 20% in our laboratory) are higher than in other disorders. The routine administration of prophylactic penicillin to infants and young children with hemoglobin SC disease may not be necessary.

Blood - Apr 1995



Priapism in sickle-cell disease; incidence, risk factors and complications - an international multicentre study.
A B Adeyoju, A B K Olujohungbe, J Morris, A Yardumian, D Bareford, A Akenova, O Akinyanju, K Cinkotai, P H O'Reilly,

To define the incidence, risk factors and complications of priapism in a large population of patients with sickle-cell anaemia in five centres in the UK and Nigeria, as priapism is common among these patients, but the precise characteristics of the condition in this population are poorly documented.

BJU international - Dec 2002



Pulmonary hypertension as a risk factor for death in patients with sickle cell disease.
Mark T Gladwin, Vandana Sachdev, Maria L Jison, Yukitaka Shizukuda, Jonathan F Plehn, Karin Minter, Bernice Brown, Wynona A Coles, James S Nichols, Inez Ernst, Lori A Hunter, William C Blackwelder, Alan N Schechter, Griffin P Rodgers, Oswaldo Castro, Frederick P Ognibene,

The prevalence of pulmonary hypertension in adults with sickle cell disease, the mechanism of its development, and its prospective prognostic significance are unknown.

The New England journal of medicine - Feb 2004



Gallstones in sickle cell disease: observations from The Jamaican Cohort study.
T M Walker, I R Hambleton, G R Serjeant,

The prevalence, incidence, risk factors, clinical associations, and morbidity of gallstones were studied in 311 patients with homozygous sickle cell disease and 167 patients with sickle cell-hemoglobin C disease in a cohort study from birth. Gallstones developed in 96 patients with homozygous sickle cell disease and 18 patients with sickle cell-hemoglobin C disease; specific symptoms necessitating cholecystectomy occurred in only 7 patients with homozygous sickle cell disease.

The Journal of pediatrics - Jan 2000



The relationship between pulmonary infarction, cor pulmonale and the sickle states.
K M MOSER, J G SHEA,



The American journal of medicine - Apr 1957



Sickle cell disease as a cause of osteonecrosis of the femoral head.
P F Milner, A P Kraus, J I Sebes, L A Sleeper, K A Dukes, S H Embury, R Bellevue, M Koshy, J W Moohr, J Smith,

Osteonecrosis of the femoral head is an important complication of sickle cell disease. We studied 2590 patients who were over 5 years of age at entry and followed them for an average of 5.6 years. Patients were examined twice a year, and radiographs of the hips were taken at least twice: at study entry and approximately three years later.

The New England journal of medicine - Nov 1991