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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A method of automated red cell analysis suitable for the rapid classification of large numbers of red cells from individual blood specimens has been developed, and preliminarily tested on normal bloods and clinically proven cases of anemias and red cell disorders. According to this method digital image processing techniques provide several features relating to shape and internal central pallor configurations of red cells. These features are used with a fully automated decision logic to rapidly provide a quantitative "red cell differential" analysis, a report of the percentage subpopulations of recognized categories of red cells. For each subpopulation, measurements of mean cell area, mean cell hemoglobin content and mean cell hemoglobin density are provided. The nine types of red cell disorders studied with this method were: (a) iron deficiency anemia, (b) the anemia of chronic disease, (c) beta-thalassemia trait, (d) sickle cell anemia, (e) hemoglobin C disease, (f) intravascular hemolysis, (g) hereditary elliptocytosis, (h) hereditary spherocytosis, and (i) megaloblastic anemia due to folic acid deficiency. Preliminary indications are that the red cell differential is useful in distinguishing between these conditions.
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PMID:An automated method of differential red blood cell classification with application to the diagnosis of anemia. 33 Jul 16

An 11 year old Muslim boy with a 2 month history of fever, loss of appetite, pallor and abdominal distension, had hepato-splenomegaly. Haemoglobin electrophoresis showed the presence of haemoglobins S and F, with complete absence of haemoglobin A. The sickling test was positive. Marital consanguinity was present. In both parents, the sickling test was positive and haemoglobin electrophoresis showed the presence of haemoglobins A and S. This is the first report of homozygous sickle cell disease in Sri Lanka.
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PMID:A case of homozygous sickle cell disease in Sri Lanka. 172 6

The pattern of illness in 60 consecutive children with homozygous sickle cell disease who attended the Paediatric Emergency Room of a busy Lagos hospital with acute illness was studied prospectively. Their ages ranged from 3 months to 13 years with a peak in the 2nd year. There were twice as many boys as girls. The commonest symptoms were fever, limb or abdominal pain and cough, and the commonest signs were pallor and hepatomegaly. Painful crises occurred in 27, anaemic crises in 11, and a combination of these in 12 children. Infection was detected in 76% of subjects in crises. Infection was found in 82% of all the children and was mainly bacterial. The commonest infections were pneumonia (35%), bacteraemia (32%), tonsillitis/pharyngitis (17%) and osteomyelitis (8%). The predominant bacteria isolated were Klebsiella spp (38%), E. coli (23%), Staph. aureus (23%), Staph. albus (23%) and Pseudomonas spp (23%). Some children had multiple isolates. Bacterial infection was a major cause of morbidity in very young children and merits appropriate control and preventive measures in this age group. The spectrum of bacteria isolated makes it unlikely that the specific anti-pneumococcal measures widely advocated in Europe and America for young children with SCA would be appropriate in Nigeria.
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PMID:Acute illness in Nigerian children with sickle cell anaemia. 244 66

Between January 1976 and December 1978, the Microbiology Department of University College Hospital (UCH) Ibadan, isolated Salmonella typhi from the blood cultures of 93 children aged 0-14 years, who were admitted to the paediatric wards. Clinical case notes were retrieved and reviewed in 64 (68.8%) of them. Fifteen (23%) of the 64 children were less than one year of age while 22 (34%) were under the age of five years. The commonest presenting symptoms were fever, anorexia, diarrhoea and vomiting. A febrile convulsion was the presenting symptom in 13 (20%) of the patients, all of whom were under the age of five years. Hepatomegaly was almost twice as frequently observed as splenomegaly. Intestinal perforation was present in five of the patients. There was a high proportion of SS children who presented with fever, pallor, jaundice, generalized aches and pains and other clinical features of sickle cell disease and it is possible that such children are specially susceptible to typhoid fever. A clinical diagnosis of typhoid fever on admission was made in only 14 of the 64 children. Reasons are given for the low index of suspicion and it is suggested that any child with unremitting fever after adequate anti-malarial chemotherapy should be treated for enteric fever.
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PMID:Problems in the clinical diagnosis of typhoid fever in children in the tropics. 618 69

A sample of 354 individuals with sickle cell anemia ascertained in the city of Rio de Janeiro was studied to investigate the relationships between Hb F level, morbidity, and mortality. The mean Hb F level was 6.41 +/- 5.21%. The relationship between age and the proportion of Hb F can be described as a quadratic polynomial distribution, the level falling from approximately 11% in the first year to 4% at 25 years and then rising proportionally after 30 years. The correlations between Hb F level and 140 variables, including hematological values, signs and symptoms of the disease, as well as therapeutic requirements, showed that the patients with high Hb F levels are less anemic and have a more benign course. Several significant correlations (between amount of Hb F and the following clinical signs: pallor of mucous membranes, jaundice, cholelithiasis, leg ulcers, bronchial asthma, increased pulmonary vascularity, left ventricular hypertrophy, and osteomyelitis) are being reported here for the first time.
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PMID:Hb F levels, longevity of homozygotes and clinical course of sickle cell anemia in Brazil. 618 95

Seven hundred and three Nigerian village children in their first six years of life were subjected to anthropometric measurements and physical examination in early 1988. The heights of 66.9% and weights of 60.5% of them fell below the third percentile of a Nigerian equivalent for international reference population standard. Mid upper arm circumference values indicated moderate to severe malnutrition in over 25% of all 1-5 year old children surveyed. Fever, cough, headache and diarrhoea were the commonest symptoms encountered in the children. Mild pallor of the conjunctival mucosa and physical signs of protein energy malnutrition were commonly seen. Fungal and septic skin lesions were present in 11.45 and 11.1% of the children respectively, whilst rhinorrhoea was seen in 4.7%, otitis media in 6% and pharyngotonsillitis in 3.3%. Thirty four (4.8%) of the children had haemic whereas five had pathological murmurs. Dental calculi were present in 15.8%, umbilical herniae in 18.2%, hepatomegaly in 48.2% and splenomegaly in 23% of the children. Seven (1%) had cerebral palsy. The implication is that malnutrition, sickle cell disease, malaria and other infections are the prevailing causes of morbidity in the preschool aged children surveyed. Desirable improvements include upgrading socio-economic and living conditions and instituting appropriate control measures.
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PMID:Anthropometric measurement in children aged 0-6 years in a Nigerian village. 758 49

A 52-year-old black man with a history of sickle cell SS disease presented with sudden loss of vision in his right eye. Examination of the right eye revealed no light perception and an amaurotic pupil, but an otherwise normal neuro-ophthalmic examination. On initial presentation, there was no evidence of an acute vascular event on funduscopy. Computed tomography failed to demonstrate pathology of the brain or orbit. Repeat fundus examination, 2 weeks later, still failed to demonstrate retinal or optic nerve disease. However, optic nerve pallor and central cupping became evident after 2 months and continued to progress in the ensuing year. Magnetic resonance imaging performed at that time did not disclose the presence of demyelinating disease, or aneurysmal dilation of the carotid or ophthalmic artery. A diagnosis of retrobulbar ischemic optic neuropathy is considered likely in this patient. The association of retrobulbar ischemic optic neuropathy and sickle cell anemia has not been previously reported.
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PMID:Retrobulbar ischemic optic neuropathy associated with sickle cell disease. 803 80

Sickle cell disease is transmitted as an autosomal recessive trait. Symptoms of pallor, fever, abdominal and joint pain, and swelling of the liver, spleen, hands and feet first appear near the latter part of the first year of life. Intravascular sickling affects all organs. For clinical and therapeutic purposes, exacerbations may be classified as vasoocclusive or pain, aplastic, hemolytic or sequestration crisis. In addition to infection, complications include severe pain, cerebrovascular accidents, cholelithiasis, bone infarction, heart failure, hypotension and priapism. The most common cause of early childhood death is septicemia or meningitis due to Streptococcus pneumoniae. Complications may be reduced or prevented by early diagnosis through newborn screening, patient education, routine immunizations, administration of folic acid, pneumococcal and influenza vaccinations, penicillin prophylaxis, and early diagnosis and aggressive treatment of complications.
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PMID:Common problems in sickle cell disease. 781 Apr 78

Homozygous sickle cell anaemia (SCA) is an inherited red blood cell disorder in which haemoglobin A is replaced by haemoglobin S. The disease exhibits a wide spectrum of clinical behaviour which has been well described in neighbouring countries. In Jordan, however, the disease has never been characterized. We used reviews of patients' notes and clinic interviews at Princess Badi'a Teaching Hospital, Irbid, Jordan to describe the clinical presentation of the disease in our area. The total number of patients was 41 (28 boys and 13 girls) and the median age was 9 years (range 1.5-21). The median age at presentation was 2.5 years (range 0.5-11). The commonest presenting feature was pallor (62%). An unusual feature of the disease in this series is the presence of a palpable spleen in 44% of patients older than 8 years. Associated G6PD deficiency was present in 29% of boys. Different clinical patterns of SCA were observed. Haemoglobin F is unlikely to be an explanation for this variability since it has not been shown to correlate with a number of severity indices of the disease such as the frequency of blood transfusions, hospitalizations and painful crises. Although the disease haplotype is not known in Jordan, our geographical location between Asia and Africa may suggest the presence of more than one haplotype and consequently different clinical patterns.
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PMID:Sickle cell anaemia in Jordan and its clinical patterns. 889 56

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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PMID:Acute sickle cell syndromes in Nigerian adults. 1093 Nov 63


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