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SUMMARY A cord blood screening programme initiated in June 1973 had screened 68 000 normal deliveries by February 1979 with the detection of 216 cases of homozygous sickle cell disease. Regular review of these children in the Medical Research Council paediatric clinic has identified acute splenic sequestration as a major cause of morbidity and mortality in the first 5 years of life. In addition to classical episodes characterised by peripheral circulatory failure, minor episodes of increasing anaemia associated with an enlarging spleen and an active marrow were also common. These minor episodes appeared to have predictive value in children who later developed severe life-threatening episodes of acute splenic sequestration. Sustained hypersplenism was also appreciably more common in children developing minor or major episodes of acute splenic sequestration compared with those without such a history. It is proposed that the classification of acute splenic sequestration be expanded to include these minor episodes, and that consideration be given to prevention of recurrences by splenectomy particularly in patients who also develop sustained hypersplenism.

Serial liver function tests and percutaneous liver biopsies were performed on 21 children receiving treatment for acute lymphoblastic leukaemia (ALL). The patients received continuing chemotherapy either with daily 6-mercaptopurine and weekly methotrexate or with five-day pulses of these drugs every three weeks. Liver function tests were transiently abnormal in the majority of children, but the abnormalities bore no relationship to the histology of the liver biopsy. Mild inflammatory and fatty changes were commonly seen, and early portal fibrosis was found in three out of 16 patients biopsied at between 108-130 weeks on treatment. There was no correlation between treatment regime and results of biopsy. Three patients showed possible progression of abnormalities on repeat biopsy. The risk of development of portal fibrosis appears low after 2-3 years of continuing chemotherapy, but examination of liver histology may be indicated if more prolonged therapy is contemplated.

Over a period of one year from June 1993 to May 1994, 282 children under 6 years old who were household contacts of sputum positive adults with tuberculosis were evaluated in a screening clinic. Of these, 180 (63-8%) had evidence of tuber. culosis, a much higher transmission rate than reported elsewhere. HIV seropositivity was 77-40/o in the adult index cases and 18% in the contact children. No increased infectivity to household contacts was detected in HIV seropositive indq,x adults compared with those who were seronegative. Child tuberculosis contact tracing is essential in these families, where transmission of disease is higher than reported elsewhere, and attention to the health needs of the children may be diminished by the high morbidity and mortality among adult family members.

The clinical, laboratory and radiological features of 30 children with clinically diagnosed tuberculous meningitis (TBM) who were HIV-seronegative were compared with those of ten HIV-infected children with TBM. Such comparative data are not currently available in the literature and so are an important addition to our knowledge of the HIV-TB co-infection epidemic. In comparison with the HIV-negative children, those infected with HIV were younger, had a shorter duration of symptoms and were more often Mantoux-negative (HIV-positive 23% vs HIV-negative 70%; p = 0.01). On presentation, all children in both groups were in MRC TBM stages II or III. Clinical features were similar in both groups but computed tomography of the brain showed more ventricular enlargement (HIV-positive 80% vs HIV-negative 63%), gyral enhancement (HIV-positive 60% vs HIV-negative 17%; p = 0.01) and cerebral atrophy (HIV-positive 40% vs HIV-negative 17%). Outcome was considerably worse in the HIV-positive children, of whom 30% died (vs HIV-negative 0/30; p = 0.01) and the remainder were moderately (HIV-positive 30% vs HIV-negative 24%) or severely (HIV-positive 30% vs HIV-negative 19%) handicapped at the end of treatment. While clinical features were not markedly different in HIV-infected and uninfected children with TBM, abnormal radiological findings were more common in the HIV-infected group and outcome was considerably worse. Co-existing HIV encephalopathy and diminished immune competence undoubtedly contributed to the more severe clinical and neuro-radiological features.

SUMMARY A series of 100 Zimbabwean children aged between 5 months and 13 years with culture positive typhoid fever is presented. The disease was found to be fairly mild with a low prevalence of complications, and no patient in the series died. Possible explanations for the relative mildness of typhoid in this paediatric population are discussed.There are few reported studies of typhoid fever in children, and the available data suggests that its manifestations differ from those in adults.' -4 There is also considerable geographical variation in the prevalence of complications and mortality in reported series. -3 An impression of a rather better outcome than is suggested by other reports prompted this review.Patients and methods children was 7 years with a range of 5 months to 13 years. Figure 1 shows the distribution of patients throughout the age range, and while there was considerable variation between the different years there were no specific age peaks of incidence. There were 52 boys and 48 girls. The weight was recorded in 93 children, and in 53 (57%) it was below 80 per cent of the Boston 50th centile for weight. In six children (6%) the weight was below 60 per cent of the 50th centile.The 100 cases were selected by retrospective review of admissions to the Harare Infectious Diseases Hospitals, and criteria for selection were that patients were: (a) below the age of 14 years; (b) symptomatic; and (c) culture positive for Salmonella typhi.Patients were investigated in a standard manner. A single blood, three stool, and three urine specimens were cultured for S typhi. Stool and urine samples were also examined for ova and parasites. A full blood count, including platelet and reticulocyte counts, was performed on admission and repeated every seventh day during treatment. Other investigations were performed as dictated by the clinical findings and course. Antimicrobial treatment with chloramphenicol was routinely administered for 21 days by mouth unless vomiting precluded this, in which case it was given intravenously. Seventy two hours after completing treatment three stool and three urine specimens were cultured on consecutive days. If these were negative for S typhi the patient was discharged and was requested to attend for repeat cultures after one, three, six, and 12 months. Default from follow up was, however, the norm.

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The haematological changes in early years following neonatal diagnosis have been observed in representative groups of children with sickle cell-haemoglobin C (SC) disease, sickle cell-beta(+) thalassaemia, and in sickle cell-beta(0) thalassaemia. Most haematological indices in SC disease were intermediate between previously published values in SS disease and in AA controls, generally being closer to values in normal children. Exceptions were microcytosis which may be genetically determined and a striking elevation of mean cell haemoglobin concentration from age 2 months to 4 years. The combination of a raised MCHC and a lowered MCV is unusual and may be characteristic of SC disease. Features in sickle cell-beta thalassaemia generally differed according to the type of beta thalassaemia gene. Sickle cell-beta(0) thalassaemia had lower levels of haemoglobin, MCHC, red cell count, MCV, and higher reticulocytes, most differences being significant before 1 year. No differences between S beta(0) thalassaemia and S beta(+) thalassaemia were apparent in HbF levels (which resembled those in SS disease) or in HbA2 levels (which exceeded those in SS disease by 1 year of age).