Favorable Outcome After Single-kidney Transplantation From Small Donors in Children: A Match-controlled CERTAIN Registry Study.

BACKGROUND: Kidney transplantation (KTx) from small donors is associated with inferior graft survival in registry studies, whereas single-center studies show favorable results. METHODS: We compared 175 pediatric KTx from small donors ≤20 kg (SDKTx) with 170 age-matched recipients from adult donors (ADKTx) from 20 centers within the Cooperative European Paediatric Renal Transplant Initiative registry. Graft survival and estimated glomerular filtration rate (eGFR) were analyzed by Cox regression and mixed models. Detailed data on surgical and medical management were tested for association with graft survival. RESULTS: One-year graft survival was lower after SDKTx compared with ADKTx (90.9% versus 96.5%; odds ratio of graft loss, 2.92; 95% confidence interval [CI], 1.10-7.80; P = 0.032), but 5-y graft survival was comparable (90.9% versus 92.7%; adjusted hazard ratio of graft loss 1.9; 95% CI, 0.85-4.25; P = 0.119). SDKTx recipients had an annual eGFR increase of 8.7 ±â€…6.2 mL/min/1.73 m² compared with a decrease of 6.9 ±â€…5.7 mL/min/1.73 m² in ADKTx recipients resulting in a superior 5-y eGFR (80.5 ±â€…25.5 in SDKTx versus 65.7 ±â€…23.1 mL/min/1.73 m² in ADKTx; P = 0.008). At 3 y posttransplant, eGFR after single SDKTx was lower than after en bloc SDKTx (86.6 ±â€…20.4 versus 104.6 ±â€…35.9; P = 0.043) but superior to ADKTx (68.1 ±â€…23.9 mL/min/1.73 m²). Single-kidney SDKTx recipients had a lower rate of hypertension at 3 y than ADKTx recipients (40.0% versus 64.7%; P = 0.008). CONCLUSIONS: Compared with ADKTx, 5-y graft function is superior in SDKTx and graft survival is similar, even when performed as single KTx. Utilizing small donor organs, preferably as single kidneys in experienced centers, is a viable option to increase the donor pool for pediatric recipients.

Children With Type 1 Spinal Muscular Atrophy Are at Increased Risk for Nephrolithiasis.

BACKGROUND: Nephrolithiasis is not a well-documented condition in children with spinal muscular atrophy (SMA). It is possible that this condition was underestimated before the era of nusinersen because of a much shorter life expectancy. We present our observational data on nephrolithiasis and its possible risk factors in children with type 1 SMA. METHODS: We retrospectively reviewed the charts of 20 children with genetically confirmed type 1 SMA. Thirteen patients (aged 9 to 55 months) who underwent urinary tract ultrasonography were included in the study. Medical records were retrospectively reviewed for demographic and clinical characteristics, ultrasound results, and metabolic abnormalities. RESULTS: Seven children (54%) had nephrolithiasis; 5 had multiple stones and two had a single stone. Two patients had microlithiasis (<3 mm), three had a stone in the size of 3 to 5 mm, and one had a stone in the size of more than 8 mm. Two patients with nephrolithiasis had urinary tract abnormalities. Patients with nephrolithiasis were more likely to have a history of urinary tract infections (UTIs) (P = 0.048) and higher urine specific gravity (P = 0.014) than patients without nephrolithiasis. Five of seven children with nephrolithiasis had a urine metabolic evaluation; all had hypercalciuria, three had hyperuricosuria, but none had hyperoxaluria, hypocitraturia, or hypomagnesemia. CONCLUSION: Children with SMA type 1 are at an increased risk for nephrolithiasis. Hypercalciuria and high urine specific gravity appear to be the most common risk factors for the occurrence of nephrolithiasis. In addition, UTI is more common in patients with type 1 SMA with nephrolithiasis.

Cardiometabolic Risk Factors in Pediatric Kidney Transplant Recipients.

OBJECTIVE: There is an increased risk of obesity and metabolic syndrome among kidney transplant recipients, which adversely affects cardiovascular and renal outcomes in these patients. The present study aims to investigate the prevalence of metabolic syndrome in pediatric kidney transplant recipients and the associations of metabolic syndrome with cardiovascular disease and graft function. MATERIALS AND METHODS: This cross-sectional, single-center study included 52 kidney transplant recipients (27 males) transplanted before 18 years of age. All subjects underwent a comprehensive assessment that included anthropometric and blood pressure measurements and laboratory tests. Metabolic syndrome was defined based on the recent recommendations of the Pediatric Renal Nutrition Taskforce. Left ventricular hypertrophy was assessed as a risk factor for cardiovascular disease, and estimated glomerular filtration rate was assessed to determine graft function. RESULTS: The median age of patients was 15.9 (13.8;18.4) years, and the median follow-up time was 35.5 (20.0;62;0) months after transplantation. Nineteen patients (36.5%) were obese or overweight, 43 (83%) had hypertension or controlled hypertension, 23 (44%) had dyslipidemia, and 9 (17%) had hyperglycemia. Ten patients (19.2%) were diagnosed with metabolic syndrome. Twenty-eight patients (54%) had left ventricular hypertrophy. The prevalence of left ventricular hypertrophy was higher in patients with metabolic syndrome than in those without metabolic syndrome (90% vs. 45%, P = .014), whereas estimated glomerular filtration rate did not differ between the 2 groups. CONCLUSION: Cardiometabolic risk factors are common in pediatric kidney transplant recipients. Approximately one-fifth of patients have metabolic syndrome, and left ventricular hypertrophy is much more common among patients with metabolic syndrome. However, there is no relationship between metabolic syndrome and graft dysfunction.

Elevated Urinary VEGF-A, Transferrin, and Angiotensinogen Levels in Normoalbuminuric Children and Adolescents with Type 1 Diabetes: Can They Be Early Markers of Diabetic Kidney Disease?

OBJECTIVE: We hypothesized that diabetic kidney disease (DKD) begins early, before albuminuria occurs. We therefore aimed to assess potential early urinary biomarkers of DKD in normoalbuminuric and normotensive children and adolescents with type 1 diabetes (T1D) to evaluate the relationship between these markers and clinical and laboratory risk factors for DKD. METHODS: This cross-sectional study included 75 children and adolescents with T1D (62% females, mean age 13.9 ± 3.2 years) with normoalbuminuria (an albumin/creatinine ratio [ACR] below 30 mg/g creatinine). Fifty-five age- and sex-matched healthy children and adolescents served as controls. For the assessment of early DKD, urinary levels of angiotensinogen (AGT), transferrin, nephrin, vascular endothelial growth factor-A (VEGF-A), and kidney injury molecule-1 (KIM-1) were measured in adequately collected 24-h urine samples using enzyme-linked immunoassays. RESULTS: The mean disease duration was 7.3 ± 3.2 (range 2.1-15.7) years, and the mean HbA1c level was 8.8 ± 1.4%. The median levels of urine VEGF-A/Cr, AGT/Cr, and transferrin/Cr were significantly higher in normoalbuminuric patients with T1D, compared with those of controls (p < 0.001, p = 0.02, and p = 0.001, respectively), but there was no difference in nephrin/Cr and KIM-1/Cr between the 2 groups. Although none of the patients had albuminuria, the median level of urine ACR was significantly higher in the patient group than the control group (p = 0.003). The ACR was positively correlated with glomerular filtration rate (GFR). Urinary transferrin/Cr, AGT/Cr, and VEGF-A/Cr were significantly correlated with ACR, but not with either GFR or diabetic risk factors including HbA1c or disease duration. CONCLUSION: Normoalbuminuric and normotensive children and adolescents with T1D have elevated urinary VEGF, AGT, and transferrin levels, which may indicate the development of DKD before albuminuria occurs.

Rituximab treatment for difficult-to-treat nephrotic syndrome in children: a multicenter, retrospective study

Background/aim: This study aimed to evaluate the efficacy of rituximab in children with difficult-to-treat nephrotic syndrome, considering the type of disease (steroid-sensitive or ­resistant) and the dosing regimen. Materials and methods: This multicenter retrospective study enrolled children with difficult-to-treat nephrotic syndrome on rituximab treatment from 13 centers. The patients were classified based on low (single dose of 375 mg/m2) or high (2-4 doses of 375 mg/m2) initial dose of rituximab and the steroid response. Clinical outcomes were compared. Results: Data from 42 children [20 steroid-sensitive (frequent relapsing / steroid-dependent) and 22 steroid-resistant nephrotic syndrome, aged 1.9­17.3 years] were analyzed. Eleven patients with steroid-sensitive nephrotic syndrome (55%) had a relapse following initial rituximab therapy, with the mean time to first relapse of 8.4 ± 5.2 months. Complete remission was achieved in 41% and 36% of steroid-resistant patients, with the median remission time of 3.65 months. At Year 2, eight patients in steroid-sensitive group (40%) and four in steroid-resistant group (18%) were drug-free. Total cumulative doses of rituximab were higher in steroid-resistant group (p = 001). Relapse rates and time to first relapse in steroid-sensitive group or remission rates in steroid-resistant group did not differ between the low and high initial dose groups. Conclusion: The current study reveals that rituximab therapy may provide a lower relapse rate and prolonged relapse-free survival in the steroid-sensitive group, increased remission rates in the steroid-resistant group, and a significant number of drug-free patients in both groups. The optimal regimen for initial treatment and maintenance needs to be determined.

Natural history of patients with infantile nephrolithiasis: what are the predictors of surgical intervention?

BACKGROUND: We evaluated the risk factors for the requirement of surgical intervention in infants with nephrolithiasis. METHODS: The medical records of 122 (156 kidney units (KU)) infants were reviewed. The clinical features, stone characteristics, changes in stone status, and treatment protocols were noted. The stone status of the KU was categorized into 3 groups according to the change in size between the first and last ultrasound: resolution, unchanged, and growth. RESULTS: The median age was 8 months (r: 2-12). The median length of follow-up was 16 months (r: 10-36). Resolution was detected in 94 KUs (60%). Stone growth was detected in 39 KUs (25%), and stone size was unchanged in 23 KUs (15%). Surgical intervention was required in 26 patients (17%). A history of intensive care unit (ICU) follow-up and a stone size > 5 mm at time of diagnosis were defined as independent risk factors for stone growth (p = 0.005, < 0.001, respectively). The surgical intervention rate was higher in stones > 5 mm and stones with pelvic localization (p = 0.018, 0.021, respectively). Stone resolution was higher in patients with stone size ≤ 5 mm (p = 0.018). CONCLUSION: A stone size > 5 mm at the time of diagnosis and a history of ICU follow-up are independent risk factors for stone growth. Pelvic localization of stones and stones > 5 mm are associated with an increased risk of surgical intervention.

Axillary and Tympanic Temperature Measurement in Children and Normal Values for Ages.

AIM: The aim of the study was define the normal values of tympanic and axillary body temperature in healthy children. METHODS: This observational cross-sectional study was performed in healthy children aged 0 to 17 years who visited the ambulatory general pediatric of Istanbul Medical Faculty. RESULTS: Of 1364 children, 651 (47.7%) were girls and 713 were boys, the mean (SD, range) age was 72.5 (53.6, 1-204) months. The mean (SD) axillary body temperature was 36.04°C (0.46°C; minimum, 35.0°C; maximum, 37.6°C). The 95th and 99th percentiles were 36.8°C and 37.0°C, respectively. The mean (SD) tympanic body temperature was 36.91°C (0.46°C; minimum, 35.15°C; maximum, 37.9°C). The 95th and 99th percentiles were 37.6°C and 37.8°C, respectively. There were statistically significant differences between sexes for only tympanic body temperatures. Both axillary and tympanic body temperatures were statistically higher in 0 to 2 months compared with other age groups. For this age group, the 99th percentile was 37.5°C for axillary and 37.85°C for tympanic temperature. CONCLUSIONS: Axillary and tympanic body temperatures should be considered as fever when they are more than 37.0°C and 37.8°C, respectively. For 0 to 2 months, fever is 37.5°C and 37.85°C in axillary and tympanic temperatures, respectively.