Haemophilia patients aged 0 - 18 years in the Western Cape

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, physicalexamination 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

Kaposi’s sarcoma: Good outcome with doxorubicin, bleomycin and vincristine sulphate (ABV) chemotherapy and highly active antiretroviral therapy

There is little published information on effective treatment of Kaposi’s sarcoma (KS) in children in low-income countries. We prospectively treated 12 patients with an institutional review board-approved protocol consisting of four monthly courses of doxorubicin (Adriamycin), bleomycin and vincristine sulphate (ABV), with highly active antiretroviral therapy (HAART) plus co-trimoxazole prophylaxis for those who were HIV-positive, with additional vincristine if remission was not achieved after 4 months. Maintenance HAART plus co-trimoxazole was given to all HIV-positive patients. A fine-needle aspirate and CD4+ count were done if possible, and staging was performed according to Mitsuyasu. Eight of ten HIV-positive patients with stage III - IVB disease, and both HIV-negative patients with stage I disease, were in remission after 473 - 1 490 (mean 939) days. One patient died after absconding during treatment, and one died from neutropenia-related pulmonary infection. ABV with or without HAART is an effective treatment option for children with KS

Onyalai at Rundu, Namibia 1981-1988: age, sex, morbidity, mortality and seasonal variation of 612 hospitalized patients

Of 51263 admissions to Rundu State Hospitala in Namibia between 1981 and 1988, 612 (1.19%) were diagnosed as onylai. The annual incidence varied between 0.96% and 1.66% of all admissions. The female to male ration was 3.2. The mean age at presentation was 24.8 years (range 6 months to 80 years) and the mean hospital stay (and duration of clinical bleeding) for the years 1981 and 1982 and 1985 to 1988 was 7.68 d (range 1-38 d). Although the highest number of cases occurred during the months March, April and May a statistically significant monthly variation was not found. The treatment policy of commencing intravenous fluid on admission and a blood transfusion whenever the haemoglobin dropped below 10 g/dl in patients with active bleeding was associated with a mortality rate of 2.78% compared to 9.8% in cases recorded up to 1981.Articl

Onyalai in pregnancy. Effects on the mother and the newborn

ArticleThe original publication is available at http://www.samj.org.zaEight pregnant Kavango women with documented active onyalai during pregnancy or a history of previous attacks of onyalai gave birth to 8 healthy infants who had no clinical signs of a tendency to haemorrhage and had normal platelet counts. Postpartum haemorrhage occurred in 2 patients. A splenectomy was successfully performed on the 7th day after delivery in 1 patient to control postpartum haemorrhage, and 3 months after confinement in another patient to control haemorrhage from another clinical attack of onyalai. Corticosteroids are not indicated in the management of onyalai. Infants of mothers with onyalai, unlike infants of mothers with idiopathic thrombocytopenic purpura, do not appear to be at risk of thrombocytopenia and haemorrhage.Publisher’s versio

The SIOP burkitt lymphoma pilot study in Malawi, Africa

[No abstract available]Conference Pape

Inactivated Proplex for the hemophiliac with inhibitors: A case report

ArticleThe original publication is available at http://www.samj.org.zaInactivated prothrombin complex (Proplex) with a Kingdon time between 100 and 138 seconds effected functional recovery and adequate haemostasis in a haemophiliac with inhibitors of the high-responder type at comparatively low cost, and with no adverse effects. Home treatment was successful. Liver function remained normal during treatment. Statistically significant changes occurred in the prothrombin time (PT), fibrinogen levels and the thrombin time after each infusion. No evidence of diffuse intravascular coagulation (DIC) was ever detected. The inhibitor level decreased from 10 to 2 Bethesda units, and the radio-immunoassay (RIA) for Australia antigen remained negative.Publishers' versio

High-dose immunoglobulin therapy in four patients with onyalai

Onyalai, a form of immune thrombocytopenia in Africa, has a recorded death rate of 9.8% in the acute phase due to haemorrhagic shock or central nervous system bleeding. Four patients with active bleeding and a mean platelet count of 6 x 109/litre were each treated with 0.67 g/kg intravenous globulin (Sandoglobulin®) daily on 3 successive days. Clinical bleeding ceased within 3 d and all patients responded with a rise in the platelet count, which peaked at 19-21 d. No side effect was recorded. Intravenous globulin therapy may reduce the morbidity of the acute phase of onyalai.Articl

Onyalai in Namibia. Clinical manifestations, haematological findings, course and management of 103 patients in the Kavango territory

A series of 103 patients with onyalai, from the Kavango territory in Namibia, is recorded. In addition to haemorrhagic bullae in every patient, epistaxis was present in 53, petechiae and ecchymoses in 23, subconjunctival or scleral bleeds in 17, melaena and haematemesis in 16, haematuria in 12 and menorrhagia in 9 patients. The male:female ratio was 43:60. The ages varied from 6 months to 70 years with a peak incidence between 11 and 20 years. The mean platelet count was 22 × 109/l and the mean haemoglobin 10·3g/dl on admission. The management consisted of the correction of blood loss. A splenectomy in 2 patients with uncontrollable haemorrhage caused a rise in the platelet count. Prednisolone and intravenous gammaglobulin had the same effect on the platelet count as ascorbic acid (placebo). Vincristine sulphate appeared to benefit some patients. One year's observation of 21 patients demonstrated that 80% of cases will have chronic thrombocytopenia with a risk of intermittent attacks of acute haemorrhage. Six patients died, 4 of cerebral haemorrhage and 2 of haemorrhagic shock. Four infants born to mothers with onyalai were normal at birth. Onyalai should be clearly distinguished from classical idiopathic thrombocytopenic purpura, a disease also encountered in Africa. © 1987.Articl

Granulocyte macrophage colony stimulating factor (GM-CSF) after cyclophosphamide treatment in the 1983 BFM acute lymphoblastic leukaemia protocol improves delivery of scheduled chemotherapy

Of 44 children treated before 1992 with the ALL-BFM 1983 medium risk schedule, 24 experienced a delay in or reduction of scheduled chemotherapy in the two weeks after cyclophosphamide (CP) administrations. Infections occurred in 24.5% and a blood transfusion was administrated in 50.9% of these periods. E. coli recombinant human GM-CSF was given for 7 days, starting on day 4 after CP, to 11 consecutive children treated with the ALL- BFM 83 schedule after 1992. Serial full blood counts and liver function tests were performed. A delay in scheduled treatment occurred in one patient. Infections occurred in 42.4% and a blood transfusion was administered in 48.5% of observation periods. Transient elevations of SGPT and SGOT were observed in both controls and patients, especially when L. asparaginase had preceded CP. No side effects of note were associated with GM-CSF therapy. The addition of GM-CSF significantly improved the ability to deliver scheduled chemotherapy. The infections did not delay planned therapy. Nine of 11 patients are in continued remission after 18- 35 months (mean 27) follow-up. One child is in second remission after a CNS relapse, which was treated with chemotherapy followed by a marrow transplant.Articl