Upper airways obstruction and cerebrovascular accident in children with sickle cell anaemia.
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ABSTRACT: Sickle cell anaemia (SCA) is the most common cause of childhood stroke, occurring with the highest frequency before the age of 6 years. Despite the relative frequency of stroke in SCA, few predictors of risk exist. Anaemia, leucocytosis, hypertension, silent infarction, and history of acute chest syndrome are well-documented risk factors for ischaemic stroke in SCA. Recent data suggest that other environmental and genetic factors, many unrelated to SCA, influence the development of cerebrovascular disease. Non-invasive assessment of individual stroke risk using transcranial Doppler ultrasonography has provided a means of selecting and prophylactically treating SCA children at highest risk. With the ultimate goal of preventing stroke, the information gained from the studies reviewed here may lead to improved prediction of stroke so that clinical trials to assess risk-based therapy may be carried out on selected children with SCA.British Journal of Haematology 04/2005; 128(6):751-66. DOI:10.1111/j.1365-2141.2004.05310.x
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ABSTRACT: 61 snoring children selected for adenotonsillectomy, mainly for recurrent tonsillitis, were compared with a matched group of 31 healthy children for symptoms of sleep apnoea, extent of sleep hypoxaemia, and amount of sleep disturbance. The studies were repeated six months postoperatively, and after six months in the healthy children. Preoperatively, 61% of the children had degrees of sleep hypoxaemia above normal and 65% had abnormally disturbed sleep. A questionnaire administered to the parents about their children showed abnormal patterns of answers about sleep problems daytime sleepiness, hyperactivity, aggression, learning difficulties, restless sleep, and odd sleeping positions. After adenotonsillectomy, the abnormal hypoxaemia, excessive sleep disturbance, and multiple symptoms almost resolved; a growth spurt also occurred.The Lancet 03/1990; 335(8684):249-53. DOI:10.1016/0140-6736(90)90068-G
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ABSTRACT: A cohort of 53 patients (age range 1.9-16.5 years) with sickle cell disease (49 homozygous SS and four S beta zero-thalassaemia) was studied for evidence of sleep related upper airway obstruction (UAO). This involved (i) a clinical assessment based on a history of snoring, a score of tonsillar size, and (for 50 patients) overnight multichannel respiratory recordings, and (ii) a blinded analysis of arterial oxygen saturation (SaO2) from the above recordings, and comparison with results from 50 healthy age matched controls of both white (n = 25) and Afro-Caribbean race. There was no difference in the baseline SaO2 values of the white and Afro-Caribbean controls. Eighteen patients with sickle cell disease (36%) were found to have sleep related UAO. The blinded analysis showed that eight patients (16%) had episodic hypoxaemia (SaO2 less than or equal to 80%, a value not observed in controls) and/or low baseline SaO2 values (less than 95.8%, the lowest value seen in the controls). Postoperative assessment was undertaken in 15 patients who underwent adenotonsillectomy. All demonstrated an improvement in symptoms and a reduction or abolition of episodic hypoxaemia. Of the 47 patients assessed when free of UAO (not demonstrated on screening, n = 32, or resolved following surgery, n = 15), seven continued to show baseline hypoxaemia. Sleep related UAO and baseline hypoxaemia are common complications of sickle cell disease in children.Archives of Disease in Childhood 08/1992; 67(7):925-9. DOI:10.1136/adc.67.7.925