Assessment of the amino acid substitution in HLA-B*5719 for allorecognition.

The novel allele human leukocyte antigen (HLA)-B*5719 differs from HLA-B*5701 by a synonymous nucleotide exchange at position 539 in exon 3 (C-->T), replacing Leucine by Arginine at amino acid position 156 in the alpha2 domain.

Knowledge gaps, attitude and beliefs of the communities about sickle cell disease in Eastern and Western Uganda.

BACKGROUND: The management of sickle cell disease (SCD) has remained insurmountable in developing countries such as Uganda, because most communities are not aware of it. OBJECTIVE: To determine knowledge gaps, attitudes and beliefs of the communities about sickle cell disease in Eastern and Western Uganda. DESIGN: Cross sectional descriptive study. SETTING: The districts of Sironko and Mbale in Eastern Uganda and Mbarara and Ntungamo in Western Uganda. SUBJECTS: Households, students and health workers. RESULTS: Household respondents from Eastern Uganda were more aware of SCD than those from Western (p < 0.001), with the majority reporting that they had seen more people with SCD in their communities than those from the West (p < 0.001). Fewer (< 1.9%) believed SCD was due to witch craft. Eight per cent of household respondents in Eastern believed it was a curse from God compared to 2% in the West. Less than 18% of the household respondents knew they could have children with SCD and (< 52%) of health workers knew SCD screening methods. Fewer (< 14%) of the health workers had participated in screening. Less than 20% of the respondents knew their sickle cell status. CONCLUSION: Respondents from Eastern Uganda were more aware of SCD than those from Western. Minority of the respondents knew their SCD status and few health staff knew how to screen it. There is need to sensitise communities and policy makers about prevention, screening and treatment of SCD.

Sickle haemoglobin and haemoglobin Stanleyville II: possible confusion with sickle cell-haemoglobin C disease.

OBJECTIVES: To bring to the attention of East African practitioners, the characteristics of Hb Stanleyville II, its interaction with HbS, and the resemblance of the double heterozygote to sickle cell-haemoglobin C (SC) disease. DATA SOURCES: A prospective study of 100 patients with Sickle Cell (SS) disease in the steady state attending the sickle cell Clinic at Mulago Hospital, Kampala, Uganda. STUDY SELECTION: Out of 100 patients with SS disease, two were also heterozygous for an alpha chain variant identified as Hb Stanleyville II. CONCLUSIONS: In association with HbS, Hb Stanleyville II produces a hybrid haemoglobin band which on alkaline haemoglobin electrophoresis, travels in the position of HbC. Such cases may cause confusion with sickle cell-haemoglobin C (SC) disease. The index cases in both families had associated alpha thalassaemia but from this small group, no conclusions may be drawn on the haematological or clinical significance of the interaction of Hb Stanleyville II with SS disease.

Morbidity and mortality patterns in HIV-1 seropositive/ seronegative women in Kampala and Harare during pregnancy and in the subsequent two years.

OBJECTIVE: To compare birth outcomes, hospital admissions and mortality amongst HIV-1 seropositive and HIV-1 seronegative pregnant women in Kampala, Uganda and Harare, Zimbabwe. DESIGN: In Kampala and Harare about 400 HIV-1 seropositive and 400 HIV-1 seronegative pregnant women were recruited at initial visit for antenatal care into a prospective study and followed for two years after delivery. The women were classified as HIV-1 seropositive at recruitment if initial and second ELISA tests were positive and confirmed by Western Blot assay. Data on demographic, reproductive, contraceptive and medical histories were obtained using a comprehensive questionnaire at entry, 32 and 36 weeks gestation, at delivery and at six, 12, and 24 months post delivery. In addition, a physical examination and various blood tests were performed at each antenatal and post natal visit. RESULTS: During the two years after delivery, HIV-1 seropositive women had higher hospital admission and death rates than HIV-1 seronegative women. HIV-1 seropositive mothers had a two-fold increase in risk of being admitted to hospital (Kampala: RR = 2.09; 95% CI = 0.95 to 4.59; Harare: RR = 1.98; 95% CI = 1.13 to 3.45). In the six weeks after delivery eight deaths occurred, six of which were among HIV-1 seropositive women and in the period from six weeks to two years after delivery, 53 deaths occurred, 51 of which were among HIV-1 seropositive women (Kampala: RR = 17.7; 95% CI = 4.3 to 73.2; Harare: RR = 10.0; 95% CI = 2.3 to 43.1). However, there was no difference in hospital admission rates between HIV-1 seropositive and seronegative women during pregnancy itself and there was only one death during that period (in a HIV-1 seronegative woman). There was no difference in the frequency of complications of delivery between HIV-1 seropositive and HIV-1 seronegative women and the outcome of births were also similar. CONCLUSIONS: A significant number of HIV-1 positive pregnant women presented at both Harare and Kampala although there was no difference in the number of hospital admissions or mortality between HIV-1 seropositive and HIV-1 seronegative women during pregnancy. Although there were no differences in complications during pregnancy or outcome at delivery, in the two years after delivery, HIV-1 seropositive women in both centres were at increased risk of being admitted to hospital and of dying.

Evidence for the occurrence of Schistosoma intercalatum at Albert Nile in northern Uganda.

During a field investigation in Rhino Camp at Albert Nile in northern Uganda, 77.8% of 636 persons excreted Schistosoma mansoni ova that were detected by the Kato-Katz method. Six patients, 8-17 years of age, had terminally spined schistosome eggs in their stools. These findings were confirmed when preserved specimens were examined at the Institute of Medical Parasitology in Bonn using a concentration technique. The mean +/- SD length of 36 terminally spined eggs was 156 +/- 6 microns and the mean +/- SD width was 59 +/- 3.6 microns. Urine filtration in the study patients revealed no schistosome eggs in the urine. It is concluded that these findings provide evidence for the occurrence of S. intercalatum at Albert Nile in northern Uganda.

Evidence for a long-term effect of a single dose of praziquantel on Schistosoma mansoni-induced hepatosplenic lesions in northern Uganda.

Treatment with praziquantel reduces the prevalence and intensity of Schistosoma mansoni infection. However, reversibility of periportal fibrosis of the liver, which potentially leads to fatal complications, is not unequivocally substantiated. In the Nile District of Uganda, 460 patients were parasitologically (Kato-Katz method) and ultrasonographically examined during October 1991, October 1992, and May 1994. Treatment with praziquantel at a dosage of 40 mg per kilogram of body weight was given in October 1991 and October 1992 to 460 individuals (group A). Another 192 patients were seen during the baseline study in October 1991 and missed the follow-up in October 1992 but took part in the second follow-up in May 1994. Thus, they received praziquantel only once in October 1991 (group B) and had an interval of 2.7 years until the next investigation in May 1994. Periportal thickening (PT) of the liver was assessed by ultrasound at each time point. Praziquantel therapy reduced the prevalence of S. mansoni in group A from 84% in 1991 to 31% in 1992 and 30% in 1994. The respective intensities of infection (geometric means of egg output) were 81 eggs per gram (epg) of stool in 1991, 31 epg in 1992, and 30 epg in 1994. Periportal thickening was found in 46% of patients in 1991, 32% of patients in 1992, and 35% of patients in 1994. Reversibility of PT was influenced by age (markedly lower reversibility in individuals older than 30 years) and sex (women and girls responded less favorably than did men and boys). Surprisingly, no significant difference was detected between group A and group B with respect to reversibility of PT The outcome between the 2 groups did not differ significantly. This may indicate that a single dose of praziquantel (as given to group B) may have a longer lasting effect than previously thought, that is, more than 2.5 years.

Aseptic necrosis of the head of the femur among sickle cell anaemia patients in Uganda.

A number of HbSS patients presenting with aseptic necrosis of the femoral head have been seen at Mulago Hospital in Kampala, Uganda. A record review of the hospital's Sickle Cell Anaemia Clinic for the period 1985-1989 revealed that aseptic necrosis of the femoral head has been diagnosed in 2.9% (47/1611) of patients attending regularly. The largest number of diagnosed patients were in the 15-24 year old age group; peak incidence occurred in 20-24 year old women. Diagnosis in this series of patients was generally delayed and management was accordingly difficult. It is suggested that clinicians ask directly about pain in the hip of sickle cell patients rather than waiting spontaneous reporting. Such an approach may improve early detection of this potentially crippling problem.

Sickle cell disease in Uganda: a time for action.

OBJECTIVES: To draw attention to the extent of homozygous sickle cell (SS) disease as a public health problem in Uganda where a mean 20% frequency of the sickle cell trait implies that 25,000 babies with SS disease are born each year. To highlight the dangers of applying interventions developed in non-malarial areas to regions where malaria may change the natural history and outcome of sickle cell disease. DATA SOURCES: The published literature from Africa and from the US and Caribbean in populations of African ancestry. STUDY SELECTION: The world literature especially, that derived from the US, Caribbean, and equatorial Africa. DATA EXTRACTION AND SYNTHESIS: In non-malarial areas, simple interventions applied early in life have significantly improved survival and the quality of life. Two well documented interventions are pneumococcal prophylaxis and the early parental diagnosis of acute splenic sequestration. The available literature from Africa suggests that neither of these may be appropriate in malarial areas. CONCLUSIONS: Manifestations of SS disease differ in malarial areas and it is questionable whether interventions developed in non-malarial areas apply. There is an urgent need to document the causes of death so that locally appropriate interventions may be developed to improve survival. Equally urgent is the need to define the pattern of clinical problems so that models of care may be evolved for use in malarial areas. Without this knowledge, health care planners will not have the information necessary to develop strategies and limited resources may be inappropriately deployed.

Uganda: paediatric AIDS.

PIP: The occurrence of reported cases of AIDS in children in Uganda, and the most common symptoms are discussed. By May 1988, 359 cases of AIDS in children has been reported. All but 12 were in babies less than 2 years of age, suggesting that maternal transmission, rather than casual contact, had caused the infection. Information was available on HIV status of 224 mothers. 42% of these had AIDS or ARC (AIDS related complex). 85 of 87 mothers whose sera had been tested were positive for HIV. Blood testing is not accurate in children until about 15 months of age, since maternal antibodies persist after birth. The most common symptoms seen in childhood AIDS in Uganda are weight loss, failure to thrive, chronic diarrhea, and repeated, chronic oral thrush (candidiasis). Other indicators are otitis media, generalized dermatitis, tuberculosis, septicemia and meningitis. Less common signs are shingles, Kaposi's sarcoma and Cryptospor meningitis. Some of these clinical findings are common in this area, so it is important to define a working clinical case definition of pediatric AIDS.^ieng

Epidemiologic and clinical features of HIV-infected and HIV-uninfected Ugandan children younger than 18 months.

METHODS: Groups of HIV-infected and HIV-uninfected infants younger than 18 months (mainly younger than 6 months) were compared to identify clinical features that could differentiate the two groups. The HIV-infected group also was compared with HIV-infected children older than 18 months. Recruitment was as follows for the group younger than 18 months: 708 children admitted with sepsis and clinical features suggestive of HIV infection were screened for HIV1 and HIV2 by HIV enzyme-linked immunosorbent assay (ELISA), and polymerase chain reaction (PCR) was undertaken on all ELISA-seropositive blood samples (270). HIV infection was confirmed in 136 (19.2%), 438 (61.9%) were HIV-seronegative, 27 (3.8%) were HIV seroreverters, 36 (5.1%) were HIV-seropositive but PCR negative (uninfected), and 71 (10.0%) were indeterminate. One hundred thirty-six HIV-infected children were compared with 501 uninfected children. Confirmed HIV-infected children older than 18 months attending the pediatric HIV clinic were compared with the 136 HIV-infected children younger than 18 months. RESULTS: Under 18 months, the median age of HIV-infected children (n = 136) was 4.0 months (range, 3 d -18 mo ) and the median age of the uninfected children (n = 501) was 1.0 month (range, 3 d -18 mo ). HIV-infected children were more likely to have had injections, chloroquine, and nystatin, and to have attended a health center or hospital (p <.001). In the HIV-infected group, the Z score for weight-for-age was -1.75, length-for-age -0.78, and weight-for-length 1.86, significantly lower scores than those of the uninfected group, which were -0.60, -0.23, and 3.05, respectively (p <.05). The mean head circumference was below the third percentile in 40% of HIV-infected compared with 22% of uninfected children (p <.001). Overall, 56 (8%) children had marasmus, 6 (0.8%) kwashiorkor, and 3 (0.4%) marasmic kwashiorkor. Sixteen percent of the HIV-infected and 7% of uninfected children had marasmus (p <.05). The 1989 revised World Health Organization clinical criteria for diagnosis of AIDS had sensitivity, specificity, and positive predictive values of 28%, 98%, and 93%, respectively. Older than 18 months (n = 109), the median age was 24 months (range, 18-60 mo ). The following were significantly more common in HIV-infected children older than 18 months than in those younger than 18 months: bacille Calmette-Guérin vaccination scar, parotid enlargement, nonspecific generalized dermatitis, and chronic diarrhea ( p <.001). Oral candidiasis was more common in the group younger than 18 months (p <.001). In infants examined in the hospital for infective conditions, oropharyngeal candidiasis, ear discharge, dermatologic disorders, generalized lymphadenopathy, lobar consolidation, hepatosplenomegaly, and failure to thrive, especially marasmus, were important indicators of HIV infection.

[Why compare hepatosplenic bilharziasis in six African countries with the help of ultrasonography? Presentation of a WHO project including Madagascar]. / Pourquoi comparer la morbidité de la bilharziose hépatosplénique dans six pays africains à l'aide de l'échographie? Présentation d'un projet OMS incluant Madagascar.

During a morbidity survey in two endemic foci of hepatosplenic schistosomiasis in the malagasy Hauts Plateaux (Tetikanana, 80 km East of Ambositra and Lohanosy, in the vicinity of Antananarivo), the objectives of a comparative pathology WHO project are presented. The survey in Madagascar was concurrently organized by the Ministry of Health (Bilharziasis central laboratory, DLMT), the Parasitology Unit of the local Pasteur Institute and the Medical Parasitology Institute of Bonn University, in Germany. This was the fourth part of this investigation of the morbidity variation due to Schistosoma mansoni infection in six african countries, a project sponsored by the WHO within its TDR programme. This comparison was based on the standardized description of morbidity as visualized by echography, following the "Cairo-1990" and the "Hannover-Managil-1989" classifications, and on coprology. One of the main objectives of this study was to demonstrate a morbidity variation at the scale of the African region (due to different S. mansoni strains or to a variable genetic background within the studied human populations), while participating to an international standardization effort for field echography. The project was conducted since July 1991 by a constant medical staff from Bonn and Hannover german universities. Eight foci in four african countries (Senegal, Mali, Uganda and Madagascar) are still analyzed and we are presenting here the first results. The data of an independent ultrasonographic investigation, organized by the Blair Institute and the Danish Bilharziasis Laboratory in Zimbabwe, was kindly proposed for this comparison. This project will end with the study of two supplementary foci in Tanzania and Burundi.