Browsing by Author "Hartley, P."

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    Haemophilia patients aged 0-18 years in the Western Cape
    (Health & Medical Publishing Group, 2003) Hazewinkel, M. H.; Hoogerwerf, J. J.; Hesseling, P. B.; Hartley, P.; MacLean, P. E.; Peters, M.; Wessels, G.
    Objectives. To record the number of haemophiliacs aged 0 - 18 years in the Western Cape (WC), what event led to the diagnosis, the level of clotting factor, treatment, functional status of their joints and impact of the disease on the family. Design. A prospective study of patients registered with the South African National Haemophilia Registry and new patients, utilising the patients' paediatricians, hospital records, patient and guardian interviews, physical examination and provincial nurse haemophilia co-ordinators. Setting. Haemophilia care centres, at the three WC academic hospitals, regional hospitals and homes of patients. Two elective medical students, MHH and JJH, collected the information. Subjects. All boys with confirmed haemophilia A or B in the WC. Outcome measures. Events that led to diagnosis, degree of haemophilia, use of clotting factor, functional status, and effect on family. Results. Of 78 patients (59 haemophilia A, 19 haemophilia B) identified, 49 could be studied. Forty-three per cent had severe, 29% moderate and 22% mild disease (6% unknown). Family history was present in 49%, but led to diagnosis in only 12%. The most common first symptoms were subcutaneous and mucosal bleeding. Delay in diagnosis varied from 0 to 9 months. Twenty-nine per cent of guardians were suspected of child abuse. RSA produced clotting factor was used 'on demand' in 73% of patients, for periodic prophylaxis in 20% and as continuous prophylaxis in 7%. Joints were functionally restricted in 43% of patients. The majority of guardians (59%) said the disease had a major impact on the family. Conclusions. The diagnosis of haemophilia in children with a positive family history was often delayed. Haemophilia causes significant morbidity in our patients and their families.
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    Incidence of acute lymphoblastic leukaemia in white and coloured children in the Western Cape
    (Health & Medical Publishing Group, 2004) Hesseling, P. B.; Hartley, P.; Zietsman, L.; Van Lill, S.; Preston-Martin, S.; Wessels, G.
    Objectives. To record the age-specific incidence rate (ASIR) for diagnosed acute lymphoblastic leukaemia (ALL) in coloured and white children aged 0-12 years in the Western Cape (WC). Design. A retrospective population-based study using the 1991 population census to calculate the mean annual childhood population and the ASIR for ALL in the 0-4, 5-9 and 10-12-year age groups in rural and Cape Town metropolitan areas for the period 1983-1999. Odds ratios were calculated using EpiInfo 2000. Setting. Registry records of the paediatric cancer units at Tygerberg and Red Cross War Memorial Children's hospitals where all children with ALL in the WC were initially treated. Subjects. All white and coloured children aged 0-12 years diagnosed as having ALL from 1983-1999. Outcome measures. The ASIR by age and ethnic group in rural and metropolitan patients in the WC. Results. The estimated annual childhood population in 1991 was 709 151 with 80.4% coloured and 19.6% white children, of whom 60% were resident in the Cape Town metropolitan area and 40% in the rural area of the WC. Of 246 children with ALL diagnosed in the period 1983 - 1999, 144 were male and 102 female. The ASIR in coloured children aged 0 - 4 years was 17.1/106 in the rural and 30.5/106 in the metropolitan area, compared with 55.7/106 and 56.2/106 respectively in white children. In the 5-9-year age group the ASIR in coloured children was 10.0/106 in the rural and 16.6/106 in the metropolitan area compared with 27.6/106 and 26.7/106 respectively in white children. The 10-12-year age group had comparable incidence rates in both populations and geographical areas. Only one case occurred within a 20 km radius of the Koeberg nuclear reactor. Conclusions. White children have an ASIR for ALL comparable to rates of diagnosis in the USA, while only half as many coloured children aged 0-9 years were diagnosed in both the rural and metropolitan areas. This contrast may indicate significant underdiagnosis of ALL in coloured children over the period in question. The change in health policy since 1994, which has improved access to primary health care, may improve the rate of diagnosis among coloured and black children.