A cluster of choanal atresia.

During a 5-month period in 1991 four infants with choanal atresia or stenosis were born at one hospital. We therefore reviewed our experience of this condition during the past 6 years and compared the patients who presented before with the four clustered cases. No definite explanation has been ascertained for this cluster of cases of choanal obstruction but a viral or other environmental teratogen could not be excluded.

Larsen syndrome in siblings with consanguineous parents.

Two siblings with Larsen syndrome and consanguineous parents are described, both with multiple joint dislocations and the typical facial appearance. Short stature is marked in both children and one had a diaphragmatic hernia which has not been previously described in this condition.

Neonatal tetanus--a simplified treatment and preventive measures.

PIP: The conservative management of neonatal tetanus usually involves use of intravenous Iv sedative drugs and is hazardous unless monitored with a high level of nursing observation. This is often not available in hospitals where this condition is most commonly seen and many iatrogenic deaths occur. A simplified oral regime for the treatment of neonatal tetanus in 22 patients admitted to Mutare General Hospital in Zimbabwe is described and a mortality of 36.4% was recorded. The traditional birth attendant training scheme which commenced in Mainicaland in 1982 is described. The oral treatment regime involves the passing of a naso-gastric tube after sedation if necessary, and administration of tetanus antiserum (1 intramuscular dose of 10,000 units), procaine penicillin for 7 days and kanamycin it necessary. Expressed breast milk was given by naso-gastric tube until the baby was able to nurse. Cooperation with traditional birth attendants has been demonstrated elsewhere to dramatically reduce neonatal mortality rates, and to a lesser degree, neonatal tetanus deaths. In combination with antenatal tetanus prophylaxis it would be expected that neonatal tetanus could be eradicated even in a population where a large proportion of birth take place at home.^ieng

HIV infection in Zimbabwe.

A total of 188 children with positive serology for HIV were identified during an 18 month period. Two seronegative children with clinical features of AIDS had seropositive mothers. Ten children were asymptomatic on initial testing; one has since died with infection. The commonest presenting features were generalised lymphadenopathy, failure to thrive, chronic diarrhoea, and pneumonia. Thirty four children are known to have died.

Mild typhoid fever.

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.

Paediatric intensive care in Harare.

A review of 1 year's experience in the paediatric intensive care unit in Harare is presented. Two hundred and eighty-six children were admitted and 66.4% survived. The major conditions for which the children were admitted were respiratory tract infections, surgical conditions, sepsis, tetanus, the Guillain Barré syndrome and acute renal failure. Sixty-nine per cent of the children lived within 50 km of the city of Harare and 31% came from more distant parts of the country. The problems of paediatric intensive care in Third World countries are discussed and we conclude that there is justification for such a facility in Zimbabwe.

Transmission of tuberculosis to contacts of sputum positive adults in Malawi.

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 tuberculosis, a much higher transmission rate than reported elsewhere. HIV seropositivity was 77.4% in the adult index cases and 18% in the contact children. No increased infectivity to household contacts was detected in HIV seropositive index 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.

Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease.

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. 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.

Hepatotoxicity in the treatment of acute lymphoblastic leukaemia.

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.

Pneumococcal and other infections in children with sickle-cell hemoglobin C (SC) disease.

The pattern of infection was compared in 139 children with sickle cell-hemoglobin C (SC) disease and in 250 control children with a normal hemoglobin (AA) genotype ascertained at birth and followed prospectively for periods of zero to six years. Both infection incidence rates and survival curve analysis indicated highly significant increases in serious infection among children with SC disease. Respiratory infection and gastroenteritis were the most common infections, but only respiratory infections were significantly more frequent in SC disease. Pneumococcal bacteremia was confined to the SC group. No hematologic differences were apparent between SC patients with and without a history of serious infection, but infection was significantly more common in patients manifesting early splenomegaly.

Tuberculous meningitis and co-infection with HIV.

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.

Haematological change in sickle cell-haemoglobin C disease and in sickle cell-beta thalassaemia: a cohort study from birth.

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).

Outbreak of aplastic crises in sickle cell anaemia associated with parvovirus-like agent.

Since 1952, 112 children with sickle cell anaemia (SCA) in Jamaica have had an aplastic crisis. Outbreaks occurred in 1956, 1960, 1065-67, 1971-73, and 1979-80. Most cases occurred in children under 10 years of age, and an aplastic crisis in a patient over the age of 15 years is rare. There were 38 cases in 1979-80 and stored serum specimens from 28 of these were available for virus studies. Evidence for infection with a parvovirus-like agent was found in 24 of these 28 cases. Viral antigen was detected in 2 patients, both of whom demonstrated seroconversion. Seroconversion during 1980 was detected in a further 7, increasing amounts of antibody during the convalescent period were found in 5, antibody was found in 2 of 4 patients from whom only an acute phase specimen was available and the remaining 10 were antibody positive in the only convalescent phase sample available for testing. Antibody was found in 4 of 94 controls with the SS genotype (in retrospect 2 of these may have had an aplastic crisis) and in 17% of 48 controls with a normal haemoglobin (AA) genotype. The results accord with the possibility that the parvovirus-like agent is the principal cause of aplastic crisis in SCA.