Mignon McCulloch

Angiographic features of 26 children with Takayasu's arteritis

Abstract Background. Takayasus arteritis (TA) is a chronic idiopathic inflammatory disease affecting primarily the aorta, its proximal branches and the pulmonary arteries. Objectives. To retrospectively review the angiograms of children with TA so as to describe the patterns of vascular involvement. Patients and methods. Twenty-six children with TA who differed from most other studies in that almost all of them presented with hypertension, reflecting the incidence of abdominal aortic and renal artery involvement. Results. The most consistent finding was stenosis of the aorta. Marginal irregularity/undulation of the aorta was also a useful angiographic diagnostic feature in subtle disease. The incidence of aneurysms was high compared to other studies and both fusiform and saccular aneurysms were encountered. Percutaneous transluminal angioplasty (PTA) was successful in all eight patients in whom it was performed. MRI, CT angiography and US are discussed as less invasive imaging alternatives. TA is a significant cause of renovascular hypertension in children in South Africa where there is a high incidence of tuberculous infection. Knowledge of the angiographic features and pattern of aortic involvement is essential for diagnosis and initiation of early and appropriate treatment, including PTA.

Peritoneal Dialysis for Acute Kidney Injury

Renal Unit,1 Greys Hospital, Pietermaritzburg, South Africa; Renal and Intensive Care Units,2 Royal Devon and Exeter Hospital, Exeter, United Kingdom; Pediatric Nephrology Unit,3 Soba University Hospital, University of Khartoum, Sudan; Pondicherry Institute of Medical Sciences and Madras Medical Mission,4 Chennai, India; Department of Medicine,5 Botucatu School of Medicine, Sao Paulo, Brazil; Division of Nephrology-Hypertension,6 University of California, San Diego, USA; Division of Pediatric Nephrology,7 Shaare Zedek Medical Center, Jerusalem, Israel; Pediatric Nephrology Unit,8 Instituto da Criança of the Hospital das Clinicas of the University of Sao Paulo Medical School, Sao Paulo, Brazil; Pediatric Nephrology Department,9 Red Cross War Memorial Children’s Hospital, University of Cape Town, Cape Town, South Africa; Department of Surgery,10 Red Cross War Memorial Children’s Hospital, University of Cape Town, Cape Town, South Africa; School of Medicine,11 Pontificia Universidade Catolica do Parana, Curitiba, Brazil; Division of Pediatric Nephrology,12 University of Missouri-Kansas City School of Medicine, Kansas City, USA; Division of Nephrology,13 Queen’s University, Kingston, Canada; and Yale University,14 New Haven, USA ispd guidelines/ReCOMMendATiOns

Kidney Disease in HIV-Positive Children

Before the era of highly active antiretroviral therapy, more than 40% of human immunodeficiency virus (HIV)-infected children experienced renal complications. In sub-Saharan Africa, approximately 2.1 million children are infected with HIV-1. In the absence of antiretroviral therapy, young African children frequently died of AIDS-related complications before renal diseases could be manifested or diagnosed. As antiretroviral therapy has become more available, and their survival has increased, our experience in treating kidney disease in HIV-infected children has improved. This article discusses relevant clinical and pathologic findings related to kidney disease in HIV-infected children.

Liver transplantation for children – the Red Cross Children's Hospital experience

Abstract:  Liver transplantation for infants and children has been available in South Africa at a single centre, the only established service in Sub‐Saharan Africa, for more than a decade. Current concerns have shifted from an initial target of early post‐transplant survival to quality of life in the long‐term. Materials and methods: Since 1985, 225 infants and children have been assessed, with 146 accepted for transplantation. Sixty‐nine have had 71 orthotopic liver transplants (OLTx). Biliary atresia was the most frequent diagnosis (54%) followed by acute liver failure (ALF) (15%). Waiting list mortality has remained high (23%), particularly for the ALF group (50%). Forty‐three were reduced size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1. Twenty‐seven were <10 kg. Results: Fifty (74%) survive 1 month–12 years post‐transplant. Actuarial survival after 1996 since HBV core antibody positive donor livers were refused and prophylactic IV ganciclovir used has been >82%. Early post‐OLTx mortality was low (5%), one primary non‐function, one IVC thrombosis, one PV thrombosis, but late morbidity and mortality (20%) was mainly due to viral infection: de novo hepatitis B (five patients, three deaths), EBV‐related post‐transplantation lymphoproliferative disease (PTLPD) (eight patients, six deaths) and CMV disease (11 patients, five deaths). Tuberculosis prophylaxis, required in six cases, resulted in major morbidity in two and mortality in one. Poor compliance played a significant role in seven deaths. Hypertension requiring medication along with some compromise of renal function has been present in all but two patients. However, all those of school‐going age (25) attend school normally and remain in good health and only three of the survivors have abnormal liver function tests. Conclusions: Successful liver transplantation is possible in a developing country with limited resources. Scarcity of virus‐free donors (HBV and HIV) leading to waiting list mortality and infrequent re‐transplantation along with long‐term consequences of immunosuppression (infection, lymphoma and renal toxicity) remain problems. Intense education of the caregiver and close follow‐up, particularly of those living at long distances has partly addressed the compliance problem.

Corticosteroid-Free Kidney Transplantation Improves Growth: 2-Year Follow-up of the TWIST Randomized Controlled Trial.

Background Corticosteroid withdrawal (CW) after pediatric kidney transplantation potentially improves growth while avoiding metabolic and other adverse events. We have recently reported the results of a 196 subject randomized controlled trial comparing early CW (tacrolimus, mycophenolate mofetil (MMF), daclizumab, and corticosteroids until day 4) with tacrolimus, MMF, and corticosteroid continuation (CC). At 6 months, CW subjects showed better growth with no adverse impact on acute rejection or graft survival (Am J Transplant 2010; 10: 828–836). This 2-year investigator-driven follow-up study aimed to determine whether improved growth persisted in the longer term. Methods Data regarding growth, graft outcomes and adverse events were collected at 1 year (113 patients) and 2 years (106 patients) after transplantation. The primary endpoint, longitudinal growth calculated as delta height standard deviation score, was analyzed using a mixed model repeated measures model. Results Corticosteroid withdrawal subjects grew better at 1 year (difference in adjusted mean change, 0.25; 95% confidence interval, 0.10, 0.40; P = 0.001). At 2 years, growth remained numerically better in CW subjects (0.20 (−0.01, 0.41); P = 0.06), and significantly better in prepubertal subjects (0.50 (0.16, 0.84); P = 0.004). Bacterial and viral infection was significantly more common in CW subjects at 1 year only. Corticosteroid withdrawal and CC subjects received similar exposure to both tacrolimus and MMF at 1 and 2 years. No significant difference in patient or graft survival, rejection, estimated glomerular filtration rate, or other adverse events was detected. Conclusion Early CW effectively and safely improves growth up to 2 years after transplantation, particularly in prepubertal children.

Laparoscopic insertion with tip suturing, omentectomy, and ovariopexy improves lifespan of peritoneal dialysis catheters in children.

UNLABELLED Over the past two decades, chronic peritoneal dialysis (PD) has emerged as the first choice pediatric dialysis modality. A recent study visually identified the cause of malfunction of PD catheters at the Red Cross Childrens Hospital in Cape Town. The reasons that could be found, lead to changed Tenckhoff insertion-techniques from open to laparoscopic. This included suturing of the tip, omentectomy and ovarian-pexy by laparoscopy. In the present paper we prospectively analyzed, if changed insertion technique lead to an improved outcome. PATIENTS AND METHODS 26 Patients required 36 laparoscopic Tenckhoff insertions during the period August of 2003 and July of 2006. Overall a total number of 222.5 catheter-months have been observed. Laparoscopic insertion technique required 3 port placements. The tip of the catheter was sutured to pelvic peritoneum, omentectomy performed through a port site and ovariopexy done when required. RESULTS The mean lifespan of all Tenckhoffs was 6.4 +/- 6.3 months. The tip of the catheter was sutured 20 times, omentectomy done in 9 cases and 6 patients underwent ovarian pexy. In the group where the tip was sutured to the pelvic peritoneum catheter life was 8.4 months compared to the non-sutured group which was only 4.1. Omentectomy lead to an overall catheter survival of 8.0 months compared to the no omentectomy group, which had a survival of 5.8 months. The complication-rate concerning early problems and malfunctions in the sutured and omentectomy groups was also lower. Patients who underwent both, suturing of the tip and omentectomy had no malfunctions at all. CONCLUSION Omentectomy and suturing the tip can lower the complication-rate and prolong catheter survival. Using these procedures could decrease costs and morbidity and prevent patients from having further operations.

A renal registry for Africa: first steps

There is a dearth of data on end-stage renal disease (ESRD) in Africa. Several national renal registries have been established but have not been sustainable because of resource limitations. The African Association of Nephrology (AFRAN) and the African Paediatric Nephrology Association (AFPNA) recognize the importance of good registry data and plan to establish an African Renal Registry. This article reviews the elements needed for a successful renal registry and gives an overview of renal registries in developed and developing countries, with the emphasis on Africa. It then discusses the proposed African Renal Registry and the first steps towards its implementation. A registry requires a clear purpose, and agreement on inclusion and exclusion criteria, the dataset and the data dictionary. Ethical issues, data ownership and access, the dissemination of findings and funding must all be considered. Well-documented processes should guide data collection and ensure data quality. The ERA-EDTA Registry is the worlds oldest renal registry. In Africa, registry data have been published mainly by North African countries, starting with Egypt and Tunisia in 1975. However, in recent years no African country has regularly reported national registry data. A shared renal registry would provide participating countries with a reliable technology platform and a common data dictionary to facilitate joint analyses and comparisons. In March 2015, AFRAN organized a registry workshop for African nephrologists and then took the decision to establish, for the first time, an African Renal Registry. In conclusion, African nephrologists have decided to establish a continental renal registry. This initiative could make a substantial impact on the practice of nephrology and the provision of services for adults and children with ESRD in many African countries.

The clinical spectrum of hemolytic uremic syndrome secondary to complement factor H autoantibodies.

BACKGROUND Atypical hemolytic uremic syndrome (aHUS) is associated with significant mortality, progression to end-stage renal disease and recurrence post transplantation. The deficiency of CFHR plasma proteins and autoantibody-positive hemolytic uremic syndrome (DEAP-HUS) has a more favorable outcome. Guidelines suggest plasma therapy be initiated within 24 hours of presentation of aHUS. Presentation of aHUS, particularly, DEAP-HUS is associated with a diarrheal prodrome in up to 53% of patients and initiation of appropriate therapies is frequently delayed. CASES We report on 3 patients with DEAP-HUS, who presented with a diarrheal prodrome that delayed diagnosis and initiation of plasma therapy past the 24-hour window recommended. C3 was low in 2 cases at presentation. All patients had positive complement factor H (CFH) autoantibodies. Despite delay in initiating plasma therapy, all 3 cases remitted with restoration of normal renal function following initial presentation. One patient had a relapse but responded to further plasma exchange and immunosuppression. The remaining 2 patients were relapse-free without maintenance immunosuppression. CONCLUSION Our cases highlight the complexity of diagnosing DEAP-HUS due to the common occurrence of diarrhea in the prodromal phase and the subsequent delay in initiating of plasma therapy. We therefore advocate a low threshold for testing CFH autoantibodies in ambiguous cases where there is no history of bloody diarrhea or Shiga-toxin exposure.

Fluid Overload in a South African Pediatric Intensive Care Unit

OBJECTIVE Fluid resuscitation is integral to resuscitation guidelines and critical care. However, fluid overload (FO) yields increased morbidity. METHODS Prospective observational study of Red Cross War Memorial Childrens Hospital pediatric intensive care unit admissions (February to March 2013). FO % = (fluid in minus fluid out) [liters]/weight [kg] × 100%. PRIMARY OUTCOMES FO ≥ 10%, 28 day mortality. RESULTS Median [interquartile range (IQR)] age: 9.5 (2.0-39.0) months, median (IQR) admission weight: 7.9 (3.6-13.7) kg. Median (IQR) FO with admission weight: 3.5 (2.1-4.9)%; three patients had FO ≥ 10%. The 28 day mortality was 10% (n = 10). Patients who died had higher mean (IQR) FO using admission weight [4.9 (2.9-9.3)% vs. 3.4 (1.9-4.8)%; p = 0.04]. CONCLUSIONS Low FO ≥ 10% prevalence with 28 day mortality 10%. Higher FO% with admission weight associated with mortality (p = 0.04). We advocate further investigation of FO% as a simple bedside tool.

Kidney disease in the setting of HIV infection: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference

HIV-positive individuals are at increased risk for kidney disease, including HIV-associated nephropathy, noncollapsing focal segmental glomerulosclerosis, immune-complex kidney disease, and comorbid kidney disease, as well as kidney injury resulting from prolonged exposure to antiretroviral therapy or from opportunistic infections. Clinical guidelines for kidney disease prevention and treatment in HIV-positive individuals are largely extrapolated from studies in the general population, and do not fully incorporate existing knowledge of the unique HIV-related pathways and genetic factors that contribute to the risk of kidney disease in this population. We convened an international panel of experts in nephrology, renal pathology, and infectious diseases to define the pathology of kidney disease in the setting of HIV infection; describe the role of genetics in the natural history, diagnosis, and treatment of kidney disease in HIV-positive individuals; characterize the renal risk-benefit of antiretroviral therapy for HIV treatment and prevention; and define best practices for the prevention and management of kidney disease in HIV-positive individuals.