Atypical Hemolytic Uremic Syndrome (aHUS) and Adenosine Deaminase (ADA)-Deficient Severe Combined Immunodeficiency (SCID)-Two Diseases That Exacerbate Each Other: Case Report.

Hemolytic uremic syndrome (HUS) is defined by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). Atypical HUS (aHUS), distinguished by its etiology, is caused by uncontrolled overactivation of the alternative complement pathway. The correct diagnosis of aHUS is complex and involves various gene mutations. Severe combined immunodeficiency (SCID), characterized by severe T-cell lymphocytopenia and a lack of antigen-specific T-cell and B-cell immune responses, is of seldom occurrence. In 10-15% of pediatric patients, SCID is caused by adenosine deaminase (ADA) deficiency. The authors describe the case of a boy who suffered from both aHUS and ADA-deficient SCID. At the age of 9 months, the patient presented acute kidney injury with anuria and coagulopathy. The diagnosis of aHUS was established on the basis of alternative complement pathway deregulation and disease-associated gene mutations. Further examination revealed immune system failure and, at the age of 13 months, the ADA deficiency was confirmed by genetic tests and the boy was diagnosed with ADA-SCID. ADA SCID has recently been described as a possible triggering factor of aHUS development and progression. However, more research is required in this field. Nevertheless, it is crucial in clinical practice to be aware of these two co-existing life-threatening diseases.

Galectin-3-A New Player of Kidney Damage or an Innocent Bystander in Children with a Single Kidney?

The aim of this study was to evaluate the galectin-3 (Gal-3) level in children with a congenital solitary functioning kidney (cSFK) and determine its association with common renal function parameters. The study consisted of 68 children (49 males) with cSFK. We demonstrated that children with cSFK had a lower level of galectin-3 than that of healthy subjects (p < 0.001). No significant differences in serum cystatin C (Cys C) levels between the cSFK children and the reference group were found. The subjects with cSFK and reduced estimated glomerular filtration rate (eGFR) had significantly higher levels of Gal-3 and Cys C compared to those with normal eGFR (p < 0.05). Children with eGFR <60 mL/min/1.73 m2 showed significant statistical differences between the values of area under ROC curve (AUC) for Gal-3 (AUC 0.91) and Cys C (AUC 0.96) compared to that for creatinine level (AUC 0.76). Similar analyses carried out among cSFK children with eGFR <90 mL/min/1.73 m2 revealed an AUC value of 0.69 for Gal-3, 0.74 for Cys C, and 0.64 for creatinine; however, no significant superiority was shown for any of them. The receiver operating characteristic (ROC) analyses for identifying the SFK children among all participants based on the serum levels of Gal-3 and Cys C did not show any diagnostic profile (AUCs for Gal-3 and Cys C were 0.22 and 0.59, respectively). A positive correlation between the Gal-3 and Cys C concentrations was found (r = 0.39, p = 0.001). We demonstrated for the first time that Gal-3 might play an important role in the subtle kidney damage in children with cSFK. However, further prospective studies are required to confirm the potential applicability of Gal-3 as an early biomarker for kidney injury and possible progression to CKD.

Evaluation of adipocytokines in children with chronic kidney disease.

INTRODUCTION: Adipose tissue through the many secreted adipocytokines creates a highly active metabolic and endocrine organ. The evaluation of serum adipocytokine concentration in children with chronic kidney disease (CKD) could serve as a marker of cardio-vascular complication progression and an index of outcome in adulthood and after kidney transplantation. MATERIAL AND METHODS: The aim of the study was to evaluate simultaneously the serum concentrations of six different adipocytokines: adiponectin, apelin, chemerin, omentin, resistin, and vaspin, in 28 children with CKD stage 5 on haemodialysis and peritoneal dialysis. RESULTS: The concentration of apelin, omentin, and resistin in children with CKD was significantly higher and the concentration of vaspin, adiponectin, and chemerin was significantly lower than in the control group. After adjusting to body mass index (BMI), the same results were obtained. After adjusting to body surface area (BSA), the concentration of vaspin, adiponectin, and chemerin did not differ between children with CKD and the control group. In analysis of the correlation between serum total adipocytokine levels in children with CKD we found a negative relationship in pairs: omentin-apelin and omentin-vaspin, and positive in pairs: adiponectin-chemerin and adiponectin-resistin. CONCLUSIONS: Our results show that changes in serum adipocytokines concentration are associated with the kidney dysfunction in CKD in children. Longitudinal studies on larger groups of paediatric cohorts would be helpful in investigating whether adipocytokines play a harmful role in the development of CKD and would enable further understanding of the risk factors for CKD progression.

[Arterial hypertension in children with end-stage renal failure treated with hemodialysis]. / Nadcisnienie tetnicze u dzieci w okresie schylkowej niewydolnosci nerek leczonych hemodializami.

UNLABELLED: Cardiovascular disorders contribute in increased morbidity and mortality of patients with chronic renal failure. Uncontrolled, long-term arterial hypertension is noted as a major risk factor. The study aimed at evaluation of arterial hypertension incidence and efficiency of its treatment among patients on maintenance hemodialysis (HD). MATERIAL AND METHODS: The study included 24 children and young adults with chronic renal failure on hemodialysis treatment, for the period longer than 6 months, in the 1997-2002 years. Patients data were retrospectively analyzed in 3-month's intervals. At the beginning of follow-up systolic and diastolic arterial hypertension (values exceeding 95 percentile value for age and height) before dialysis session was revealed respectively in 58% and 46% of children, and after dialysis session the occurrence of both systolic and diastolic hypertension was 42%. RESULTS: After 33 months of treatment the incidence of predialysis systolic and diastolic hypertension was respectively 15.5% and 8% and postdialysis both systolic and diastolic hypertension - 23%. Ejection fraction (EF) increased almost significantly after 18 months of treatment with hemodialysis, but after 30 months EF reached baseline value. Cardiac contractility was nonsignificantly improved after 18 months of treatment, and after 30 months declined to baseline value. 62.5% of children in examined group were treated with more than one antihypertensive drug. CONCLUSIONS: We found that the amount of antihypertensive drugs correlated with values of systolic and diastolic blood pressure till 18 month of follow-up as well as in 30 month of observation.