Bone mass and vertebral fractures in South African children on prolonged oral glucocorticoids for chronic non-malignant illnesses.

INTRODUCTION: We hypothesized that the prevalence of vertebral fractures would be low and that bone mineral density (BMD) would be less severely affected in a black South African (SA) population treated with glucocorticoids (GCs) than that reported in mainly white populations. METHODS: All children aged 5-17.9 years with chronic non-malignant illnesses who were on GCs (intravenous or oral) for greater than 3 months duration were evaluated. DXA scans were performed using a Hologic Discovery machine (Software version Apex 4.0.2) and the Hologic paediatric reference database. Whole body less head (WBLH) and lumbar spine (LS) bone mineral content (BMC) and density (BMD) Z-scores unadjusted and adjusted for height were calculated using the Zemel equation calculator. RESULTS: Seventy-two patients (49% with renal, 24% with rheumatic, 14% with neurological, 11% with hepatic and 3% with respiratory conditions; mean age 11.6 ± 3.3 years, 57% boys, 92% SA black) were enrolled. The mean duration of GC treatment was 34.1 (±25.1) months. Mean WBLH and LS height adjusted BMD Z-scores were -1.2 ± 1.5 and -0.9 ± 1.0 respectively. Eleven percent of patients had a LS height adjusted BMD Z-score ≤ -2. The prevalence of vertebral fractures on lateral vertebral fracture assessment (VFA) was 15% (11 of 72 patients). CONCLUSION: The prevalence of vertebral fractures (15%) in predominantly black children on GCs with chronic non-malignant illnesses is similar to that reported from North America suggesting that routine yearly DXA scans including VFA are warranted in this highly at-risk population.

Review of children with Takayasu's arteritis at a Southern African tertiary care centre.

IntroductionTakayasu's arteritis is a rare idiopathic arteritis causing stenosis or aneurysms of the aorta, pulmonary arteries, and their branches. It usually occurs in women, but has been described in children. OBJECTIVE: The objective of this study was to determine the clinical presentation, demographic profile, vascular involvement, origins, management, and outcome of children diagnosed with Takayasu's arteritis at a Southern African tertiary care centre between 1993 and 2015. METHODS: This is a retrospective analysis of all children with Takayasu's arteritis captured on a computerised electronic database during the study period. RESULTS: A total of 55 children were identified. The female:male ratio was 3.2:1, and the mean age was 9.7±3.04 years. Most originated outside the provincial borders of the study centre. The majority presented with hypertension and heart failure. In all, 37 (67%) patients had a cardiomyopathy with a mean fractional shortening of 15±5%. A positive purified protein derivative test was documented in 73%. Abdominal aorta and renal artery stenosis were the predominant angiographic lesions. A total of 23 patients underwent 30 percutaneous interventions of the aorta, pulmonary, and renal arteries: eight stents, 22 balloon angioplasties, and seven had nephrectomies. All patients received empiric tuberculosis treatment, immunosuppressive therapy, and anti-hypertensive agents as required. Overall, there was a significant reduction in systolic blood pressure and improvement in fractional shortening (p<0.05) with all treatments. CONCLUSION: Takayasu's arteritis is more common in girls and frequently manifests with hypertension and heart failure. The abdominal aorta and renal arteries are mostly affected. Immunosuppressive, anti-hypertensive, and vascular intervention therapies improve blood pressure control and cardiac function.

Immunogenicity of 13-valent pneumococcal conjugate vaccine among children with underlying medical conditions.

BACKGROUND: Streptococcus pneumoniae is a leading cause of vaccine-preventable disease in children under 5years. Immunocompromised children and those with underlying diseases are at increased risk of severe complications from vaccine-preventable infections. We studied the humoral immune response to the 13-valent pneumococcal conjugate vaccine (PCV13) in children with HIV-infection, kidney or lung disease and compared this to the response in healthy control children. METHODS: Children aged 12-71months with underlying conditions including HIV-infection and those with kidney and lung diseases (at-risk children), and a healthy control group were vaccinated with PCV13. The at-risk children received two doses of PCV13 and the controls received one dose. Serotype-specific antibodies for all PCV13 serotypes were measured by a luminex-based enzyme immunoassay at baseline and post-vaccination. RESULTS: After the first PCV13 dose, the fold-increase in serotype-specific antibody geometric mean concentrations (GMCs) from baseline and the percentage of participants with ≥4-fold-increase in antibody concentrations was similar between the control and at-risk children. GMCs were, however, lower for three of the 13 serotypes in HIV-infected children, higher for serotype 6B in children with kidney disease and higher for serotypes 6B and 14 in children with lung disease. After second vaccine dose HIV-infected children had an increase in GMCs from post-first dose for nine serotypes but the percentage of participants with ≥4-fold-increase from baseline was similar post-second dose compared to post-first dose except for serotypes 6A and 19F. In children with kidney or lung diseases the immune responses after second vaccine dose were similar to post-first dose. Attenuated responses were observed for serotypes 3 and 19A in all study-groups, which was especially pronounced in the at-risk groups. CONCLUSION: All study-groups mounted an immune response to PCV13, with the at-risk groups having responses that were mostly similar to the control children.

Emphysematous Pyelonephritis in Children.

Emphysematous pyelonephritis is a severe necrotizing infection of the kidneys characterized by gas formation within the parenchyma, the collecting system or the perinephric tissue. To our knowledge, there have only been 4 cases of this condition reported in children. We report 2 additional cases in children managed at Chris Hani Baragwanath Academic Hospital, Johannesburg, South Africa.

Renal transplantation in human immunodeficiency virus (HIV)-positive children.

Renal transplantation is being performed in adult human immunodeficiency virus (HIV)-positive patients and increasingly in paediatric patients as well. A multidisciplinary team involving an infectious disease professional is required to assist with HIV viral-load monitoring and in choosing the most appropriate highly active antiretroviral therapy (HAART). Drug interactions complicate immunosuppressant therapy and require careful management. The acute rejection rates appear to be similar in adults to those in noninfective transplant recipients. Induction with basiliximab and calcineurin-based immunosuppression appears to be safe and effective in these recipients. Prophylaxis is advised for a variety of infections and may need life-long administration, especially in children. Organ shortage remains a significant problem, and kidneys from deceased HIV-positive donors have been used successfully in a small study population. Overall, with careful planning and close follow-up, successful renal transplantation for paediatric HIV-infected recipients is possible.

Kidney disease in children and adolescents with perinatal HIV-1 infection.

INTRODUCTION: Involvement of the kidney in children and adolescents with perinatal (HIV-1) infection can occur at any stage during the child's life with diverse diagnoses, ranging from acute kidney injury, childhood urinary tract infections (UTIs), electrolyte imbalances and drug-induced nephrotoxicity, to diseases of the glomerulus. The latter include various immune-mediated chronic kidney diseases (CKD) and HIV-associated nephropathy (HIVAN). DISCUSSION: The introduction of highly active anti-retroviral therapy (HAART) has dramatically reduced the incidence of HIVAN, once the commonest form of CKD in children of African descent living with HIV, and also altered its prognosis from eventual progression to end-stage kidney disease to one that is compatible with long-term survival. The impact of HAART on the outcome of other forms of kidney diseases seen in this population has not been as impressive. Increasingly important is nephrotoxicity secondary to the prolonged use of anti-retroviral agents, and the occurrence of co-morbid kidney disease unrelated to HIV infection or its treatment. Improved understanding of the molecular pathogenesis and genetics of kidney diseases associated with HIV will result in better screening, prevention and treatment efforts, as HIV specialists and nephrologists coordinate clinical care of these patients. Both haemodialysis (HD) and peritoneal dialysis (PD) are effective as renal replacement therapy in HIV-infected patients with end-stage kidney disease, with PD being preferred in resource-limited settings. Kidney transplantation, once contraindicated in this population, has now become the most effective renal replacement therapy, provided rigorous criteria are met. Given the attendant morbidity and mortality in HIV-infected children and adolescents with kidney disease, routine screening for kidney disease is recommended where resources permit. CONCLUSIONS: This review focuses on the pathogenesis and genetics, clinical presentation and management of kidney disease in children and adolescents with perinatal HIV-1 infection.

Palatial erosion caused by Mycobacterium Avium complex in a human immunodeficiency virus infected child.

Oral manifestations of Mycobacterium Avium complex (MAC) are rare and have only been described in adults. Here we report a 10-year-old female with advanced human immunodeficiency virus infection, who presented with disseminated MAC and an erosive left palate defect.

Polyarteritis nodosa presenting with a leg mass.

UNLABELLED: We report an unusual presentation of polyarteritis nodosa in a 2-y-old child. The child presented with a mass of the left leg adjacent to the calf, and the biopsy showed polyarteritis nodosa. Further investigations confirmed systemic features, and X-rays showed a periosteal reaction. CONCLUSION: Childhood polyarteritis nodosa may present with a lower limb inflammatory mass.