An 8-year-old-girl with juvenile dermatomyositis and autoimmune thyroiditis in Tanzania: a case report.

BACKGROUND: Juvenile dermatomyositis is an inflammatory disease of muscles, skin, and blood vessels of unknown cause affecting all age and ethnic groups, with a reported incidence of 1.9-4.1 per million. It manifests with weakness in axial and proximal muscles and typical skin lesions. Historically, the Bohan and Peter classification schema has been used to diagnose juvenile dermatomyositis. CASE PRESENTATION: We report an 8-year-old African female child, who presented with features of juvenile dermatomyositis and a rare association with subclinical autoimmune thyroiditis. This case illustrates the typical presentation, diagnosis, and treatment outcomes of this highly misdiagnosed condition. CONCLUSION: Due to the limited resources and knowledge about this under-reported disease in resource-constrained settings, the characteristic manifestations of juvenile dermatomyositis can be easily missed and thus requires a high index of suspicion for earlier diagnosis and management.

Juvenile arthritis management in less resourced countries (JAMLess): consensus recommendations from the Cradle of Humankind.

Juvenile idiopathic arthritis (JIA) is the most prevalent chronic rheumatic disease in children and young people (CYP) and a major cause of pain and disability. The vast majority of the world's children and their families live in less resourced countries (LRCs) and face significant socioeconomic and healthcare challenges. Current recommendations for standards of care and treatment for children with JIA do not consider children living in less resourced countries. In order to develop appropriate recommendations for the care of CYP with JIA in less resourced countries a meeting of experienced pediatric rheumatologists from less resourced countries was convened with additional input from a steering group of international pediatric rheumatologists with experience in developing recommendations and standards of care for JIA. Following a needs assessment survey of healthcare workers caring for CYP with JIA in LRC, a literature review was carried out and management recommendations formulated using Delphi technique and a final consensus conference. Responses from the needs assessment were received from 121/483 (25%) practitioners from 25/49 (51%) less resourced countries. From these responses, the initial 84 recommendations were refined and expanded through a series of 3 online Delphi rounds. A final list of 90 recommendations was proposed for evaluation. Evidence for each statement was reviewed, graded, and presented to the consensus group. The degree of consensus, level of agreement, and level of evidence for these recommendations are reported. Recommendations arrived at by consensus for CYP with JIA in less resourced countries cover 5 themes: (1) diagnosis, (2) referral and monitoring, (3) education and training, (4) advocacy and networks, and (5) research. Thirty-five statements were drafted. All but one statement achieved 100% consensus. The body of published evidence was small and the quality of evidence available for critical appraisal was low. Our recommendations offer novel insights and present consensus-based strategies for the management of JIA in less resourced countries. The emphasis on communicable and endemic diseases influencing the diagnosis and treatment of JIA serves as a valuable addition to existing JIA guidelines. With increasing globalization, these recommendations as a whole provide educational and clinical utility for clinicians worldwide. The low evidence base for our recommendations reflects a shortage of research specific to less resourced countries and serves as an impetus for further inquiry towards optimizing care for children with JIA around the world.

Prevalence of CKD, Diabetes, and Hypertension in Rural Tanzania.

INTRODUCTION: Chronic kidney disease (CKD), diabetes, and hypertension play a disproportionate role in the growing public health challenge posed by noncommunicable diseases (NCDs) in East Africa. The impact of these NCDs may pose the greatest challenge in rural areas with limited screening and treatment facilities, although precise prevalence estimates of these conditions in rural Tanzania are lacking. METHODS: The prevalence of CKD, diabetes, and hypertension, were estimated from a probability sample of adults (n = 739) residing in 2 communities within Kisarawe, a rural district of Tanzania. Following consent, participants were studied in their homes. Random point-of-care (POC) measures of glycosylated hemoglobin and blood pressure, were obtained. Serum creatinine, drawn at the POC and measured at Muhimbili National University, was used to calculate estimated glomerular filtration rate with the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation. RESULTS: The median age was 35 years (interquartile range 25-45 years). Overall the pooled prevalence for CKD stages III, IV, and V was 12.4% (95% confidence interval [CI] = 10.2-14.8). Surprisingly, the prevalence of CKD stage V (3.0%; 95% CI = 2.1-4.4) was high among the youngest age group (18-36 years). The prevalence estimates for prehypertension and hypertension were 38.0% (95% CI = 34.6-41.5) and 19.9% (95% CI = 17.1-22.9), respectively. The prevalence estimates for prediabetes and diabetes were 25.7% (95% CI = 22.6-29.1) and 14.8% (95% CI = 12.4-17.6), respectively. CONCLUSION: Although this pilot study had a relatively small sample size, the prevalence estimates for CKD, diabetes, and hypertension were higher than we expected based on previous estimates from Tanzania. CKD was not significantly associated with diabetes or hypertension, suggesting the possibility of an alternative causality.

Cardiorenal Anemia Syndrome and Survival among Heart Failure Patients in Tanzania: A Prospective Cohort Study.

BACKGROUND: Cardiorenal anemia syndrome (CRAS) is an evolving global epidemic associated with increased morbimortality and cost of care. The management of patients with CRAS remains a challenging undertaking worldwide and the lack of evidence-based clinical guidelines adds to the challenge. We aimed to explore the prevalence and survival rates of heart failure patients with CRAS in Tanzania. METHODS: We screened 789 patients and consecutively recruited 463 who met the inclusion criteria. Each participant underwent an interview, physical examination, anthropometric measurements, anemia, renal functions and echocardiographic assessment. All participants were followed until death or for up-to 180 days, whichever came first. Bivariate comparison and subsequent Cox proportional-hazards regression model were used to compare the CRAS and non-CRAS groups with respect to the primary end point. RESULTS: The mean age of participants was 46.4 ± 18.9 years, and 56.5% were women. Overall, 51.9% of participants had renal insufficiency, 72.8% were anemic and 44.4% had CRAS. During a mean follow-up of 103 ± 75 days, 57.8% of participants died. Patients with CRAS displayed a higher mortality rate (73.5%) compared to those free of CRAS (45.8%), (p < 0.001). During multivariate analysis in a cox regression model of 21 potential predictors of mortality; renal dysfunction (HR 1.9; 95% CI 1.0-3.5; p = 0.03), severe anemia (HR 1.8; 95% CI 1.0-3.1; p = 0.04), hyponatremia (HR 2.2; 95% CI 1.3-3.7; p = 0.004) and rehospitalization (HR 4.3; 95% CI 2.2-8.4; p < 0.001) proved to be the strongest factors. CONCLUSION: Cardiorenal anemia syndrome is considerably prevalent and is associated with an increase in mortality amongst patients with heart failure. In view of this, timely, aggressive and collaborative measures to improve renal functions and/or correct anemia are crucial in the management of CRAS patients. Furthermore, these findings call for guideline committees to revise and/or develop evidence-based recommendations for management of patients with CRAS.

Developing a Screening Algorithm for Type II Diabetes Mellitus in the Resource-Limited Setting of Rural Tanzania.

BACKGROUND: Noncommunicable diseases are on pace to outnumber infectious disease as the leading cause of death in sub-Saharan Africa, yet many questions remain unanswered with concern toward effective methods of screening for type II diabetes mellitus (DM) in this resource-limited setting. We aim to design a screening algorithm for type II DM that optimizes sensitivity and specificity of identifying individuals with undiagnosed DM, as well as affordability to health systems and individuals. METHODS: Baseline demographic and clinical data, including hemoglobin A1c (HbA1c), were collected from 713 participants using probability sampling of the general population. We used these data, along with model parameters obtained from the literature, to mathematically model 8 purposed DM screening algorithms, while optimizing the sensitivity and specificity using Monte Carlo and Latin Hypercube simulation. RESULTS: An algorithm that combines risk assessment and measurement of fasting blood glucose was found to be superior for the most resource-limited settings (sensitivity 68%, sensitivity 99% and cost per patient having DM identified as $2.94). Incorporating HbA1c testing improves the sensitivity to 75.62%, but raises the cost per DM case identified to $6.04. The preferred algorithms are heavily biased to diagnose those with more severe cases of DM. CONCLUSIONS: Using basic risk assessment tools and fasting blood sugar testing in lieu of HbA1c testing in resource-limited settings could allow for significantly more feasible DM screening programs with reasonable sensitivity and specificity.

A pediatric epilepsy diagnostic tool for use in resource-limited settings: A pilot study.

OBJECTIVE: It is estimated that nearly 80% of the 50 million people affected with epilepsy globally live in regions where specialist care and diagnostic tests are scarce and care is often delivered through a primary health provider with limited training. To improve diagnostic accuracy of the history and physical examination, we developed and piloted a questionnaire to discriminate between focal versus generalized epilepsy, with the future goal to guide medication choices. METHODS: Through literature review and retrospective chart review of 75 children with epilepsy at Boston Children's Hospital, a 15-item questionnaire was developed. Simple motor seizures were excluded for the purposes of this questionnaire. The questionnaire was then translated in local dialects and prospectively validated at Muhimbili National Hospital in Dar Es Salaam, Tanzania, and University Teaching Hospital in Lusaka, Zambia. Children 6months-18years of age with suspected or active epilepsy were identified, and a nonphysician administered the questionnaire to the patient's caregiver. Next, each patient was evaluated by a pediatric neurologist blinded to the questionnaire results, and together with locally obtained but remotely interpreted EEG, an electroclinical diagnosis was made. The questionnaire data were compared with this clinical gold standard. RESULTS: A total of 59 children participated: 28 from Tanzania and 31 from Zambia. Sixteen patients were excluded: 5 were excluded because of incomplete data, and 11 did not meet criteria for epilepsy based on initial screening questions. Of the remaining 43 patients, 28 had focal or multifocal epilepsy (65%), and 15 (35%) had generalized epilepsy. The questionnaire had a sensitivity of 78% and positive predictive value of 81.5%. Data were analyzed using a Rasch model, testing the questionnaire's internal consistency, reliability, and its discriminative validity in classifying focal versus generalized epilepsy against an electroclinical diagnosis. The mean epilepsy score for focal epilepsy was 0.084 logits compared with -1.147 logits for generalized epilepsy, demonstrating a large effect size [F (1, 41)=13.490, p<0.001]. CONCLUSIONS: Our questionnaire provides a straightforward method to improve diagnostic accuracy, and could assist in bridging the diagnostic gap in pediatric epilepsy in resource-limited settings. This tool was specifically designed to be easily implemented by any healthcare provider. This pilot study prompts broader prospective validation in additional settings for further refinement, and for performance assessment of impact on provider's practice, ability to guide medication choices, and ultimately improve treatment outcomes in resource-limited regions.

Renal abnormalities among HIV infected children at Muhimbili National Hospital (MNH)-Dar es Salaam, Tanzania.

BACKGROUND: Human Immunodeficiency Virus infection is a multisystem disease that contributes to significant morbidity. Renal involvement is reported to be common among patients with HIV. This study was carried out to determine renal involvement using simple bedside tests combined with ultrasonography examination. METHODS: We recruited 240 children from the HIV clinic at Muhimbili National Hospital. Data were collected using structured questionnaires and included demographic, clinical information, radiological tests; renal ultrasound and laboratory tests; serum creatinine, white blood cells, CD4+ counts and percent, urine for microalbuminuria and proteinuria. RESULTS: Microalbuminuria and proteinuria were present in 20.4 % and 7.1 % respectively. Significantly higher prevalence of microalbuminuria (p < 0.01) and proteinuria p < 0.01) were noted with low CD4 percent (<25 %). Lower mean CD4+ count were noted among children with microalbuminuria [937.4 ± 595.3 cells/µL vs 1164.7 ± 664.3 cell/µL, (p < 0.05)] and proteinuria [675.5 ± 352.3 cells/µL vs 1152 ± 662 cells/µL (p < 0.001)]. Fourteen (5.8 %) HIV infected children had estimated glomerular filtration rate (eGFR of 30-59) consistent with severe renal impairment. Increased cortical echogenicity was noted in 69/153 (39.2 %) of participants who had ultrasound examination. CONCLUSION: Microalbuminuria, proteinuria and renal dysfunction were noted to be prevalent among HIV infected children indicating the need to consider routine screening of renal complications in these children.

A seven weeks old baby with diabetic ketoacidosis: a case report.

Diabetes mellitus is rare during infancy, however, it should be suspected in infants presenting with features consistent with sepsis and hyperglycemia. This is crucial in initiating the treatment of diabetes ketoacidosis which if delayed may result in significant morbidity and death.