Epidemiology of stroke in sickle cell disease

Sickle cell disease is the most common cause of stroke in childhood, both ischaemic and haemorrhagic, and it also affects adults with the condition. Without any screening or preventative treatment, the incidence appears to fall within the range 0.5 to 0.9 per 100 patient years of observation. Newborn screening with Penicillin prophylaxis and vaccination leading to reduced bacterial infection may have reduced the incidence, alongside increasing hydroxyurea prescription. Transcranial Doppler screening and prophylactic chronic transfusion for at least an initial year has reduced the incidence of stroke by up to 10-fold in children with time averaged mean of the maximum velocity >200 cm/s. Hydroxyurea also appears to reduce the incidence of first stroke to a similar extent in the same group but the optimal dose remains controversial. The prevention of haemorrhagic stroke at all ages and ischaemic stroke in adults has not yet received the same degree of attention. Although there are fewer studies, silent cerebral infarction on magnetic resonance imaging (MRI), and other neurological conditions, including headache, epilepsy and cognitive dysfunction, are also more prevalent in sickle cell disease compared with age matched controls. Clinical, neuropsychological and quantitative MRI screening may prove useful for understanding epidemiology and aetiology

Awareness and use of Gross Motor Function Classification System (GMFCS) by health professionals in a developing country

Introduction The degree of disability in children with Cerebral Palsy (CP) can be evaluated with the Gross Motor Function Classification System (GMFCS), a valid tool which was designed for such purposes. However, there appears to be paucity of data on the awareness and use of the GMFCS particularly in the Sub-Saharan continent where the condition is still prevalent. Thus, this study aims to describe awareness, use, merits and demerits of the GMFCS system as perceived by health professionals who take care of children with CP.Methods: This was a cross sectional study of health professionals from three Nigerian teaching hospitals. Information obtained via structured questionnaire were demographic data, years of qualification, years of experience of working with children with CP, awareness, use, merits and demerits of the GMFCS. Factors associated with use of the GMFCS were also evaluated with the Chi-square analysis.Results: 78 health professionals participated in the study and majority (58.9%) were doctors. 55 (70.5%) of the study participants were aware of the GMFCS but only 33(42.3%) were using it. The nurses were neither aware of the system nor using it. A higher proportion of therapists (65.4%) were familiar with the system compared to the doctors (54.3%) . The location of practice was significantly associated with the use of the GMFCS (p=0.013). More doctors noted the merits and demerits of the system compared to the therapists.Conclusion: Though health professionals were aware of the GMFCS only a small proportion was using this vital tool in the clinical setting. Thus, there is the need to create more awareness on GMFCS and its clinical utility, and the training of nurses should be of paramount importance in the developing country.Key words: Awareness, cerebral palsy, gross motor function classification system, developing countr

Morbidity and mortality pattern in hospitalized children with sickle cell disorders at the University College Hospital, Ibadan, Nigeria

Objectives: To determine the causes of hospitalization and outcome of children with sickle cell disorders at the University CollegeHospital, Ibadan.Methods: Case files of patients with sickle cell disease who were admittedbetween March 2009 and February 2012 were analysed. Data extractedinclude demographic variables, diagnoses, types of crises, associated infections, complications and outcome of treatment.Results: There were 174 admissions of 161 children with a male femaleratio of 1.3:1. Their ages ranged from nine months to 18 years witha mean of 7.3(4.0) years. Vasoocclusive crisis was present in 107(61.5%), hyper haemolytic crisis in 29 (16.7%) and acute splenic  sequestration in 12 (6.9 %) of all admissions. Associated infectionswere septicaemia in 56 (32.2 %), malaria in 49 (28.2 %), acute osteomyelitis in 24 (13.8%), pneumonia in 23 (13.2%), urinary tract infection in 12 (6.9%) and septic arthritis in 10 (5.7%). Haematocrit was less than 15 % in 36 (20.7%) and blood transfusion administered in 68(39.1%) of admissions. There were three (1.7%) deaths from  cerebrovascular accident, adverse reaction to blood transfusion and meningitis.Conclusion: Prevention and prompt management of crises and infectionsin sickle cell disease is recommended to reduce morbidity andmortality

Subacute combined degeneration of the spinal cord in a Nigerian child: a need for a high index of suspicion

Background: Vitamin B12 deficiency has been reported to be associated with a spectrum of neurological disorders among which is subacute combined degeneration of the spinal cord.Method: We report a case of subacute combined degeneration of the spinal cord secondary to vitamin B12 deficiency and discussed the clinical presentation and management challenge.Result: The diagnosis was made through a high index of suspicion when the clinical presentation ranked highly with the mode of presentation of Vitamin B12 deficiency. Patient responded well to parentheral vitamin B12 preparation but subsequently defaulted from follow up for 8 years after which he represented with paraparesis and urinary incontinence. He was managed again with parentheral vitamin B12 preparations with good outcome and total resolution of symptoms at discharge.Conclusion: A high index of suspicion is needed in identifying vitamin B12 deficiency related paraparesis in paediatric practice while the need for indefinite treatment and follow up is hereby emphasized.Keywords: Vitamin B12 deficiency; myelinopathy; paraparesis; subacute combined degeneratio

Transcranial Doppler and Magnetic Resonance in Tanzanian Children With Sickle Cell Disease

Background and Purpose: We determined prevalences of neurological complications, vascular abnormality, and infarction in Tanzanian children with sickle cell disease. // Methods: Children with sickle cell disease were consecutively enrolled for transcranial Doppler; those with slightly elevated (>150 cm/s), low (150 cm/s was associated with frequent painful crises and low hemoglobin level. Absent/low CBFv was associated with low hemoglobin level and history of unilateral weakness. In 49 out of 67 children with low/absent/elevated transcranial Doppler undergoing magnetic resonance imaging, 43% had infarction, whereas 24 out of 48 (50%) magnetic resonance angiographies were abnormal. One had hemorrhagic infarction; none had microbleeds. Posterior circulation infarcts occurred in 14%. Of 11 children with previous seizure undergoing magnetic resonance imaging, 10 (91%) had infarction (5 silent) compared with 11 out of 38 (29%) of the remainder ( P=0.003). Of 7 children with clinical stroke, 2 had recurrent stroke and 3 died; 4 out of 5 had absent CBFv. Of 193 without stroke, 1 died and 1 had a stroke; both had absent CBFv. // Conclusions: In one-third of Tanzanian children with sickle cell disease, CBFv is outside the normal range, associated with frequent painful crises and low hemoglobin level, but not hemolysis. Half have abnormal magnetic resonance angiography. African children with sickle cell disease should be evaluated with transcranial Doppler; those with low/absent/elevated CBFv should undergo magnetic resonance imaging/magnetic resonance angiography

Case Report: Camptomelic Dysplasia in One of Twins

Camptomelic dysplasia, a very rare and lethal form of skeletal dysplasia is reported in one of a set of twins. He presented on the second day of life with short, deformed lower limbs and respiratory distress from birth and died on the fifth day of life. This report highlights the features of camptomelic dysplasia and draws attention to its occurrence in a Nigerian child. With advances in neonatal life support, there is need to proffer solutions to the problems that presently make the prognosis of camptomelic dysplasia, poor. Nigerian Journal of Paediatrics 2002; 29:122-124