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BackgroundPediatric patients with chronic kidney disease (CKD) are at increased risk of early cardiovascular disease and premature death. Abnormalities in microvascular structure and function may presage end-organ damage including vascular calcification and myocardial ischemia associated with disordered mineral metabolism. Early detection of microvascular rarefaction (reduced density of capillaries) may identify at-risk patients and prompt timely therapeutic interventions. Our objective was to study capillary rarefaction in pediatric hemodialysis (HD) patients and to determine possible associations with mineral metabolism and cardiac risk biomarkers.MethodsCapillary density (CD) was measured by nailfold capillaroscopy in 19 pediatric HD patients and 20 healthy controls. Demographic and biochemical markers were collected at entry and 6-month follow-up.ResultsCD was significantly decreased in HD patients compared with controls with a deficit of 24 and 31% at baseline and subsequent follow-up. Maximal CD correlated significantly with intact parathyroid hormone (iPTH) (r = −0.45; P = 0.005), serum calcium (r = −0.38; P = 0.02) and 25(OH) vitamin D levels (r = +0.36; P = 0.03) in HD patients. Capillary functional measures were similar to controls. By multivariate analysis, the primary negative determinants of CD were African American race and hyperparathyroidism; whereas, glomerular disease had a positive influence on capillary rarefaction (R2 = 64.2% variance; P = 0.001).ConclusionPediatric HD patients demonstrate a ‘structural deficit’ in CD but show preserved ‘functional integrity’. Capillary rarefaction, an early risk factor of incipient vascular calcification, was strongly associated with biomarkers of altered mineral metabolism. Further studies are warranted to determine the impact of optimizing blood pressure and metabolic control on changes in capillary rarefaction in young CKD patients.

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nancy loss in the presence of antiphospholipid antibodies (aPL) including lupus anticoagulant (LAC), anticardiolipin (aCL) or anti-β2-glycoprotein-1 (anti-β2GP-1) [1]. Primary APS occurs spontane- AbstractBackground: Catastrophic antiphospholipid syndrome (CAPS) is a rare, potentially fatal variant of antiphospholipid syndrome associated with small vessel thromboses and multi-organ failure. Methods:A case summary with review of the literature since 1992 was performed. Inclusion criteria were patients <18 years of age who met at least 3 of 4 diagnostic criteria for primary or secondary CAPS.Results: Twenty one patients were identified from case reports or series, ages ranged from. 3 months to 18 years (mean 10.5 ± 4.8 years). Fourteen (66%) were female. The majority (76%) had primary onset; whereas, 5 (24%) were secondary to systemic lupus erythematosus (SLE). There was an infectious trigger in the majority of cases (13=62%). There were 5 (24%) deaths. Most patients were treated with immunosuppressive regimens including pulse corticosteroids, plasma exchange, cyclophosphamide and/or rituximab. Although not statistically significant, the patients who received immune suppression were 4 times more likely to survive than those who did not. None of the patients who received rituximab died suggesting a survival advantage when used as an adjunctive medication.Conclusions: CAPS should be included in the differential diagnosis of children who present with sudden onset multi-organ failure. Multimodal therapy including plasma exchange and rituximab is potentially life-saving. Appropriate long term treatment regimens need to be developed and rheumatology follow-up is essential.

Familial hemophagocytic lymphohistiocytosis is a rare, life-threatening disorder characterized by impaired cytotoxicity, hypercytokinemia and immune-mediated organ injury. We report a 7-week-old male of consanguineous parents who presented with fever, pancytopenia and multi-organ failure. Elevated inflammatory markers and hypercytokinemia led to the diagnosis of familial hemophagocytic lymphohistiocytosis, which was confirmed with genetic testing. With the fulminant multiorgan failure, therapeutic plasma exchange was instituted, using the Prismaflex? platform, followed by standard chemo-immunotherapy. There was dramatic reversal of the multi-organ failure and stabilization of the coagulopathy with this neo-adjuvant therapy. Thereafter, he was maintained in clinical remission with chemo-immunotherapy for 3 mo while awaiting stem cell transplantation.