Clinical profile of neonates with hypernatremic dehydration in a nephrology clinic.

AIM: The aim of the study was to assess clinical profile of neonates with hypernatremic dehydration (HD) and identify risk factors associated with acute kidney injury (AKI). MATERIALS AND METHODS: A retrospective study included 18 neonates with HD (serum Na ≥150 mmol/L) hospitalized in the Department of Pediatrics and Nephrology between the years 2009-2019. RESULTS: The age at presentation was 7.5±4.7 days (range 2-18), weight loss was 15.9±8.3% (range 7.1-32.6) and serum Na range was 151- 192 mmol/L (median 155.5 mmol/L). In 12 (67%) neonates, breast or mixed fed, HD occurred due to inadequate milk intake, in 6 (33%) neonates feeding difficulties were secondary to an acute infection. There was positive correlation between serum Na level and percentage weight loss at presentation (r=0.88; p<0.001). In 6 (33%) patients serious complications of HD were found: AKI in 5 patients, convulsions in one. Percentage weight loss was significantly higher in neonates with HD-associated AKI than in neonates with HD without AKI (p<0.01). Serum Na level was marginally higher in neonates with AKI than in those without AKI (p=0.08). In univariate logistic regression analysis, higher percentage of weight loss and higher serum Na level at presentation were important diagnostic factors of AKI in neonates with HD (both p<0.05). ROC analysis determined good diagnostic profile only for percentage weight loss, with a best cut-off value of 24.8%, for predicting AKI in neonates with HD (AUC 0.862, sensitivity 80%, specificity 100%). CONCLUSIONS: Neonatal HD mostly occurs due to inadequate milk intake in breast or mixed fed babies, and rarely due to feeding difficulties in babies affected by an acute infection. Percentage weight loss at presentation has strong association with neonatal HD and is the most important factor of AKI in neonates with HD.

[Evaluation of renal lesions in children with tuberous sclerosis - summary of the first year of follow-up program]. / Ocena zmian w nerkach u dzieci chorych na stwardnienie guzowate ­ podsumowanie pierwszego roku programu opieki.

Tuberous sclerosis complex (TSC) is a genetic disease that leads to formation of tumors i.e. in brain kidneys, heart, lungs, and skin. AIM: The aim of the study was to summarize center's experience in the first year of program of nephrologic follow-up in patients with TSC. MATERIALS AND METHODS: During 12 months 30 children with TSC (14 boys and 16 girls aged from 3 months to 17 years 11 months, mean 7.57±5.02 years) were hospitalized. Following parameters were evaluated: genetic and biochemical tests, blood pressure in ambulatory blood pressure monitoring (ABPM), kidney lesions in ultrasonography (30 patients) and in magnetic resonance (14 patients). RESULTS: Genetic tests were performed in 6 children - in 5 TSC2 mutation was found, in one boy with TSC and numerous renal cysts only PKD1 mutation was revealed. Mean GFR was 130.81±23.23 mL/ min/1.73 m2. Four children (13.3%) had arterial hypertension. Renal lesions were found in 28 (93.3%) children: 18 patients had angiomyolipomas (AML) (mean diameter 15.4±12.5, max 38 mm), 23 patients had renal cysts (mean diameter 7.6±7.0, max 30 mm); 13 patients had AMLs and cysts. A dysplastic lesion (39x26x15 mm) in right kidney was found in one girl. Children with AML were older than remaining patients (10.08±4.55 vs. 4.25±3.50 [years], p<0.001). Children with cysts were characterized by higher systolic (p=0.017), diastolic (p=0.027) and mean (p=0.014) arterial pressure, and mean arterial pressure Z-score (p=0.025) in ABPM. Maximal kidney cyst diameter correlated positively with systolic, diastolic, mean arterial pressure, mean arterial pressure Z-score, and diastolic blood pressure load in ABPM (r = 0.61-0.75, p = 0.033-0.005). Two children with numerous AML with diameter >30 mm were treated with sirolimus. CONCLUSIONS: Because of common focal lesions in kidneys children with TSC should be kept under regular nephrologic follow-up. Presence of large renal cysts may predispose children with TSC to arterial hypertension.