Udai Kala

Management of severe hypertension in childhood Takayasu's arteritis

Six children presented with severe hypertension caused by Takayasus arteritis (TA), of whom four had bilateral renal artery narrowing and two coarctation syndrome. Two presented with hypertensive encephalopathy and four with congestive cardiac failure. All had a strongly positive skin reactions to purified protein derivative of mycobacterium tuberculosis. Bilateral renal arterial bypass grafts performed in two children resulted in prolonged normalization of their blood pressures, but the grafts clotted 12–18 months later. Primary renal autotransplantation was unsuccessful in two children, one with bilateral renal arterial narrowing and iliac vessel involvement and one with a long coarctation. Secondary renal autotransplantation was successful in a third child with localized aortitis. A successful aortic patch graft was performed in one child with coarctation of the aorta. Angiotensin-converting-enzyme inhibitors should be used with caution in treating the hypertension caused by TA, since bilateral renal arterial narrowing is common and their administration may result in renal insufficiency. The long-term prognosis is guarded in severely hypertensive children with extensive vascular disease due to TA.

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

Involvement of the kidney in children and adolescents with perinatal (HIV‐1) infection can occur at any stage during the childs 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).

Evaluation of urinary tract infection in malnourished black children.

Urinary tract infection (UTI) is a well recognized complication in malnourished children. The need to investigate these patients for underlying renal pathology has not been clearly defined. Seventy-five children with malnutrition were evaluated for UTI by culture of urine obtained suprapubically prior to antibiotic therapy. All patients with UTI were investigated with renal ultrasonography, intravenous pyelography (IVP) and voiding cystourethrography (VCU). Haemoglobin, white cell count, serum urea, creatinine and electrolytes were determined in all the children. The mean age of the children was 15.5 months (range 3-60 months). UTI was diagnosed in 26 (34.7%), of whom 21 (81%) were boys. The overall prevalence of UTI in those with kwashiorkor/marasmic kwashiorkor was 42%. Escherichia coli was the organism most commonly cultured (84.6%). Renal sonography, IVP and VCU were normal in all infected cases and vesicoureteric reflux was not detected in any. This study confirms the high prevalence of UTI in malnourished children. As no anatomical abnormalities were demonstrated in the patients with UTI, imaging of the renal tract other than real sonography does not appear to be indicated in the malnourished child in a first episode of UTI with normal renal function.

Impact of tuberculosis in children with idiopathic nephrotic syndrome

Forty black South African children (mean age 4.7±2.6 years) with idiopathic nephrotic syndrome due to focal glomerulosclerosis (FSGS) were evaluated. Tuberculosis (TB) was found in 37.5% of children with FSGS (FSGS-TB) compared with 6% of a comparable group with minimal lesion nephrotic syndrome. No significant differences were found in the initial mean serum albumin, cholesterol, triglyceride and creatinine levels in FSGS-TB compared with children with glomerulosclerosis but without TB (FSGS-nonTB). The mean serum levels of C4, IgA and IgM were increased by 30%, 25% and 23%, respectively in children with FSGS-TB compared with FSGS-nonTB. Initial estimated creatinine clearance was similar in the two groups, but after a mean follow-up of 2.4 years, the mean estimated creatinine clearance of children with FSGS-TB was significantly reduced by 46% from the initial value, but remained stable in the FSGS-nonTB group. FSGS-TB children also had significantly increased requirements for renal replacement therapy compared with children with FSGS-nonTB. We conclude that TB infection is commonly associated with FSGS in black South African children; this may have deleterious effects on renal function.

Biochemical and serological characteristics of children with membranous nephropathy due to hepatitis B virus infection: correlation with hepatitis B e antigen, hepatitis B DNA and hepatitis D

Fourteen children with biopsy-proven membranous nephropathy associated with hepatitis B virus (HBV-MN) were evaluated biochemically and serologically and compared to 45 children with idiopathic nephrotic syndrome (INS). The mean ages of the two groups were similar (4.9 +/- 1.6 vs. 4.6 +/- 2.6 years). Serum albumin levels were similar in both groups, but serum cholesterol was significantly reduced in children with HBV-MN compared to INS. Serum C3 was also significantly depressed in children with HBV-MN compared to INS, but no differences in C4 levels were noted. Serum alanine transaminase as well as aspartate transaminase concentrations were significantly elevated in children with HBV-MN compared to those with INS, suggesting the presence of chronic hepatitis in children with HBV-MN. Hepatitis B surface and e antigens were present in serum of all children with HBV-MN, but only 54% had circulating HBV-DNA particles demonstrable in their serum. Serum C3 levels were higher in children with HBV-MN and circulating HBV-DNA, compared to those without circulating HBV-DNA. No other serological or biochemical differences occurred between these two groups. Glomerular deposition of IgG and C3 occurred in 91% of children with HBV-MN; but IgM deposition appeared to occur more frequently and with greater intensity in those children positive for circulating HBV-DNA. Antibody to delta antigen was negative in all children with HBV-MN. We conclude that biochemical and serological differences can be identified between HBV-MN and INS.(ABSTRACT TRUNCATED AT 250 WORDS)

Fulminant hepatitis in children: report of 12 cases

Twelve cases of childhood fulminant hepatitis seen over a 4-year period are described. Six had hepatitis A, five hepatitis B and one non-A non-B hepatitis. Encephalopathy, the cardinal feature of fulminant hepatitis, was usually evident within 2 weeks of onset of illness, and the median duration of illness in fatal cases was 19 days. Deep jaundice, prolongation of the prothrombin time and raised serum ammonia were invariable. Eight children died and the four survivors were critically ill before recovering. Acute viral hepatitis is generally a benign illness in childhood. Although infrequently recorded, fulminant hepatitis may, however, ensue and is associated with a high mortality.

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.

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.

Hypertension as the trigger for posterior reversible encephalopathy syndrome in paediatric renal patients: An important diagnosis that should not be missed

BACKGROUND Posterior reversible encephalopathy syndrome (PRES) is a reversible neurological condition presenting with seizures and visual disturbances and diagnosed on magnetic resonance imaging (MRI). Little is understood about its pathogenesis, particularly in children, but it is thought to be related to hypertension. OBJECTIVES To review the presentation, diagnosis and outcome of PRES in paediatric renal patients at Chris Hani Baragwanath Academic Hospital, Johannesburg, South Africa, between 1 January 2000 and 31 January 2017 and compare these with published case reports to date. METHODS This was a retrospective analysis of five new cases and a review of the existing literature. RESULTS The five reported patients were all hypertensive at the time of diagnosis and presented with seizures. Most (91%) of the 64 reviewed patients were also hypertensive at initial presentation. All five of the reported and 91% of the reviewed patients presented with seizures. The most common pattern of change on MRI occurred in the parietal and occipital regions. Complete neurological recovery occurred in four of the five reported and 87.5% of the reviewed patients. CONCLUSION All patients presented clinically with hypertensive crises and radiological evidence of PRES. Seizures were the most common presenting symptom. The prognosis for paediatric patients with PRES is favourable, so it is important to confirm the diagnosis in low-resource settings where intensive care is limited.

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.