Variable phenotype and genotype of pediatric patients with HNF1B nephropathy.

AIMS: Hepatocyte nuclear factor 1ß (HNF1B) mutations are the most common monogenic cause of congenital anomalies of the kidney and urinary tract (CAKUT). We aimed to investigate clinical and genetic characteristics of patients with HNF1B nephropathy to expand its phenotypic and genetic spectrum. MATERIALS AND METHODS: This retrospective cohort study included 16 unrelated pediatric patients (6 females, 10 males) from 13 families with genetically confirmed HNF1B-related nephropathy. RESULTS: Abnormal prenatal kidney abnormalities were present in 13 patients (81.3%). The most common antenatal kidney abnormality was kidney cysts, which were observed in 8 patients (61.5%). Urinary system abnormalities (vesicoureteral reflux (VUR) and ureteropelvic junction obstruction (UPJO)) were present in 4 patients (25%). HNF1B analysis uncovered missense variants in 4 families (30.8%) as the most common genetic abnormality. In addition, 4 novel pathological variations have been defined. During follow-up, hypomagnesemia and hyperuricemia were observed in 7 (43.8%) and 5 patients (31.3%), respectively. None of the patients with a missense variant had hypomagnesemia. However, 7 out of 12 patients (58.3%) with a non-missense variant had hypomagnesemia (p = 0.09). None of the patients had an HNF1B score below 8, and the mean score was 15.3 ± 4.4. The mean follow-up period was 7.4 ± 5.0 years. While 100% of patients (n = 4) with missense variants were in various stages of CKD (CKD2: 2 patients, CKD3: 2 patients), 25% of those with non-missense variants had CKD (CKD2, 3, and 5; 1 patient, respectively) (p = 0.026). CONCLUSION: Patients with HNF1B-associated disease have concomitant urinary system abnormalities such as VUR or UPJO. Missense variants seem to be the most common pathological variations in HNF1B gene and have higher risk of CKD.

The Effect of "Unclassified" Blood Pressure Phenotypes on Left Ventricular Hypertrophy.

OBJECTIVE: We aimed to evaluate the clinical significance of the "unclassified" blood pressure phenotypes on left ventricular hypertrophy in children. MATERIALS AND METHODS: All children evaluated with ambulatory blood pressure monitoring in the pediatric nephrology department between October 2018 and January 2021 were included in the study. Prehypertension, normotensive, white coat hypertension, masked hypertension, ambulatory hypertension groups and 2 other groups including increased blood pressure load, normal ambulatory blood pressure measurements, but normal (unclassified group 1) or high (unclassified group 2) office blood pressure measurements were defined according to the American Heart Association 2014 statement. Left ventricular mass index, left ventricular mass index/95 percentile values, and left ventricular hypertrophy ratios were compared between the groups separately to establish the influence of the unclassified cases. RESULTS: A total of 497 children were included. There were 52 cases in normotensive, 47 cases in unclassified group 1, 50 cases in masked hypertension, 79 cases in white coat hypertension, 104 cases in unclassified group 2, and 165 cases in the ambulatory hypertension group. Left ventricular mass index/95 percentile and left ventricular hypertrophy in masked hypertension were significantly higher than normotensive but similar between normotensive and unclassified group 1 groups. Left ventricular hypertrophy was significantly higher in the ambulatory hypertension group compared to white coat hypertension, and similar between white coat hypertension and unclassified group 2 groups. CONCLUSION: Independent of age, we have found that interpretation of blood pressure load not only has a limited predictable effect on left ventricular hypertrophy but also causes a large group of patients to be unclassified. Cite this article as: Kasap-Demir B, Basaran C, Demircan T, et al. The effect of "unclassified" blood pressure phenotypes on left ventricular hypertrophy. Turk Arch Pediatr. 2024;59(1):43-48.

Trimethylamine N-oxide and kidney diseases: what do we know? / N-óxido de trimetilamina e doenças renais: o que sabemos?

Abstract In the human gut, there is a metabolically active microbiome whose metabolic products reach various organs and are used in the physiological activities of the body. When dysbiosis of intestinal microbial homeostasis occurs, pathogenic metabolites may increase and one of them is trimethyl amine-N-oxide (TMAO). TMAO is thought to have a role in the pathogenesis of insulin resistance, diabetes, hyperlipidemia, atherosclerotic heart diseases, and cerebrovascular events. TMAO level is also associated with renal inflammation, fibrosis, acute kidney injury, diabetic kidney disease, and chronic kidney disease. In this review, the effect of TMAO on various kidney diseases is discussed.

Resumo No intestino humano, existe um microbioma metabolicamente ativo cujos produtos metabólicos alcançam diversos órgãos e são utilizados nas atividades fisiológicas do corpo. Quando ocorre disbiose da homeostase microbiana intestinal, os metabólitos patogênicos podem aumentar, e um deles é o N-óxido de trimetilamina (TMAO). Acredita-se que o TMAO tenha um papel na patogênese da resistência à insulina, diabetes, hiperlipidemia, doenças cardíacas ateroscleróticas e eventos cerebrovasculares. O nível de TMAO também está associado à inflamação renal, fibrose, lesão renal aguda, doença renal diabética e doença renal crônica. Nesta revisão, discute-se o efeito do TMAO em diversas doenças renais.

Pediatric kidney care experience after the 2023 Turkey/Syria earthquake.

BACKGROUND/AIMS: Two earthquakes on February 6th, 2023 destroyed ten cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome related-acute kidney injury (Crush-AKI) and death. METHOD: A web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were asked. RESULTS: 903 injured children (median age: 11.62 years) were evaluated. Mean TUR was 13 h (Interquartile range-IQR: 32.5), max 240 h). 31 of 32 patients with a TUR of >120 h survived. The patient who rescued after ten days survived.Two-thirds of the patients were given 50 mEq/L sodium-bicarbonate in 0.45% sodium-chloride solution on admission day. 58% of patients were given intravenous fluid (IVF) at a volume of 2000-3000 mL/m2 body surface area (BSA), 40% of 3000-4000 mL/m2 BSA, and only 2% of >4000 mL/m2 BSA. 425 patients had surgeries, 48 suffered from major bleeding. Amputations were recorded in 96 patients. Eighty-two and 66 patients required ventilator and inotropic support, respectively.Crush-AKI developed in 314 patients (36% of all patients). 189 patients were dialyzed. Age > 15 years, creatine phosphokinase (CK)≥20 950 U/L, TUR≥10 h, and the first-day IVF volume < 3000-4000 mL/m2 BSA were associated with Crush-AKI development. 22 deaths were recorded, 20 of 22 occurred in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived. CONCLUSIONS: This is the most extensive pediatric kidney disaster data after an earthquake. Serum CK level was significantly associated with Crush-AKI at the levels of >20 950 U/L, but not with death. Adolescent age and initial IVF of less than 3000-4000 mL/m2 BSA were also asscoiated with Crush-AKI. Given that mildly injured victims can survive longer periods in the disaster field, we suggest uninterrupted rescue activity for at least 10 days.

Characteristics and predictors of chronic kidney disease in children with myelomeningocele: a nationwide cohort study.

BACKGROUND: Myelomeningocele (MMC) is highly prevalent in developing countries, and MMC-related neurogenic bladder is an important cause of childhood chronic kidney disease (CKD). This nationwide study aimed to evaluate demographic and clinical features of pediatric patients with MMC in Turkey and risk factors associated with CKD stage 5. METHODS: Data from children aged 0-19 years old, living with MMC in 2022, were retrospectively collected from 27 pediatric nephrology centers. Patients > 1 year of age without pre-existing kidney abnormalities were divided into five groups according to eGFR; CKD stages 1-5. Patients on dialysis, kidney transplant recipients, and those with eGFR < 15 ml/min/1.73 m2 but not on kidney replacement therapy at time of study constituted the CKD stage 5 group. RESULTS: A total of 911 (57.8% female) patients were enrolled, most of whom were expectantly managed. Stages 1-4 CKD were found in 34.3%, 4.2%, 4.1%, and 2.4%, respectively. CKD stage 5 was observed in 5.3% of patients at median 13 years old (range 2-18 years). Current age, age at first abnormal DMSA scan, moderate-to-severe trabeculated bladder on US and/or VCUG, and VUR history were independent risk factors for development of CKD stage 5 (OR 0.752; 95%; CI 0.658-0.859; p < 0.001; OR 1.187; 95% CI 1.031-1.367; p = 0.017; OR 10.031; 95% CI 2.210-45.544; p = 0.003; OR 2.722; 95% CI 1.215-6.102; p = 0.015, respectively). Only eight CKD stage 5 patients underwent surgery related to a hostile bladder between 1 and 15 years old. CONCLUSION: MMC-related CKD is common in childhood in Turkey. A proactive approach to neurogenic bladder management and early protective surgery in selected cases where conservative treatment has failed should be implemented to prevent progressive kidney failure in the pediatric MMC population in our country.

Trimethylamine N-oxide and kidney diseases: what do we know?

In the human gut, there is a metabolically active microbiome whose metabolic products reach various organs and are used in the physiological activities of the body. When dysbiosis of intestinal microbial homeostasis occurs, pathogenic metabolites may increase and one of them is trimethyl amine-N-oxide (TMAO). TMAO is thought to have a role in the pathogenesis of insulin resistance, diabetes, hyperlipidemia, atherosclerotic heart diseases, and cerebrovascular events. TMAO level is also associated with renal inflammation, fibrosis, acute kidney injury, diabetic kidney disease, and chronic kidney disease. In this review, the effect of TMAO on various kidney diseases is discussed.

Pulse Wave Analysis in Obese Children with and without Metabolic Syndrome

Objective: To compare pulse wave analysis (PWA) of obese children with and without metabolic syndrome (MS) with healthy, non-obese children and to evaluate the association between PWA findings and additional risk factors present in children with MS and obesity. Methods: From the obese patients examined between June 2019 and June 2021, 41 patients with MS, 36 obese patients without MS, and 34 healthy non-obese children of similar age and gender were evaluated retrospectively. Anthropometric measurements, biochemical evaluation, 24-hour ambulatory blood pressure (BP) measurement (ABPM), left ventricular mass index (LVMI) and PWA measurements were compared. Results: When the three groups were compared, weight standard deviation score (SDS), height SDS and body mass index SDS were all significantly higher in the MS group (p<0.05). The following measurements were significantly higher in both MS and non-MS obese patients compared to the control group: from ABPM measures, the systolic and mean arterial pressure BP SDSs load; from PWA, the night central systolic BP, 24-hour, day and night pulse pressure values and 24-hour, day and night pulse wave velocity (PWV) rates; and from cardiac evaluations, the LVMI and relative wall thickness measurements (all p<0.05). Furthermore, the 24-hour and daytime central systolic (cSBP) and diastolic BP (cDBP) values were significantly different between the three groups, being the highest in the MS group (p<0.05). Conclusion: Obesity causes higher office, ambulatory and central BP, PWV and LVMI. However our results suggest that additional risk factors associated with MS do not contribute to these parameters, except for 24-hour and daytime cSBP and cDBP values.

Blood pressure parameters affecting ventricular repolarization in obese children.

BACKGROUND: Ventricular repolarization (VR) increases the risk of sudden cardiac death due to ventricular arrhythmia. We aimed to evaluate the blood pressure (BP) parameters affecting VR in obese children. METHODS: Obese (BMI ≥ 95p) and healthy children ≥ 120 cm between January 2017 and June 2019 were included. Demographic and laboratory data, peripheral and central BPs evaluated by a device capable of ambulatory blood pressure monitoring (ABPM), and pulse wave analysis were assessed. Electrocardiographic ventricular repolarization indices, left ventricular mass index (LVMI), and relative wall thickness (RWT) were calculated. RESULTS: A total of 52 obese and 41 control patients were included. Uric acid, triglyceride, total cholesterol, LDL, and ALT values, systolic and diastolic office BPs, 24-h, daytime and nighttime systolic and mean arterial BPs, daytime diastolic BP SDS levels, daytime and nighttime systolic loads, daytime diastolic load, 24-h, daytime and nighttime central systolic and diastolic BPs, and pulse wave velocity values were significantly higher, whereas 24-h, daytime and nighttime AIx@75 were similar between the groups. fT4 levels of obese cases were significantly lower. QTcd and Tp-ed were higher in obese patients. Although RWT was higher in obese cases, LVMI values and cardiac geometry classifications were similar. The independent factors affecting VR in obese cases were younger age and higher diastolic load at night (B = - 2.83, p = 0.010; B = 0.257, p = 0.007, respectively). CONCLUSION: Obese patients have higher peripheral and central BP, arterial stiffness, and higher VR indices that develop before an increase in LVMI. It would be useful to prevent obesity from an early age and follow up nighttime diastolic load to control VR associated sudden cardiac death in obese children. A higher resolution version of the Graphical abstract is available as Supplementary information.

NETosis and kidney disease: what do we know?

Neutrophils are the most abundant leukocytes in the blood. They are rapidly mobilized from the circulation to sites of inflammation and/or infection. In affected tissues, neutrophils exhibit some dramatic antimicrobial functions, including degranulation, reactive oxygen species (ROS) production, phagocytosis, and formation of neutrophil extracellular traps (NETs). Like other cells of the immune system, after fulfilling their biological duties, they enter the path of death. Depending on the conditions, they may undergo different types of cell death (apoptosis, necrosis, necroptosis, autophagy, NETosis, and pyroptosis) that require the participation of multiple signaling pathways. NETosis is a unique neutrophil cell death mechanism that gives rise to different inflammatory and autoimmune pathological conditions. Recent studies have shown that NETosis also plays a role in the formation and/or progression of kidney diseases. This review discusses the underlying mechanism of NETosis and its relationship with some major kidney diseases in light of the current knowledge.

The Influence of Non-E. Coli or Extended-Spectrum ß-Lactamase-Producing Bacterial Growth on the Follow-Up Procedure of Infants with the First Febrile Urinary Tract Infection.

OBJECTIVE: To evaluate the effects of non-E. coli or extended-spectrum ß-lactamase-positive (ESBL-positive) microorganism growth in the first febrile urinary tract infection (UTI) of infants on laboratory findings or renal parenchymal damage presenting the severity of inflammation, anatomic abnormalities defined by imaging studies, and recurrent UTIs in the follow-up period. METHODS: The data of patients aged between 2 and 24 mo and followed up for at least 6 mo with febrile UTI guideline of the authors' pediatric-nephrology clinic, were retrospectively analyzed. Ultrasonography was performed in all the cases at the time of UTI and dimercaptosuccinic-acid (DMSA) at least 4 mo after the infection. Voiding cystourethrography (VCUG) was performed only if ultrasonography findings were abnormal, the uptake deformity was detected in DMSA scan, or the patients experienced recurrent UTIs. The patients were grouped concerning E. coli or non-E. coli and ESBL-PB or non-ESBL-BP growth in the urine cultures. RESULTS: There were 277 infants followed up for 28.55 ± 15.24 (6-86) mo. The causative microorganisms were non-E. coli in 73 (26.4%) and ESBL-PB in 58 (20.9%) cases. CRP values, pyuria, and leukocyte-esterase positivity were significantly higher in UTIs caused by E. coli compared to non-E. coli bacteria. All clinical and laboratory findings were similar between the ESBL-PB and non-ESBL groups, but abnormal ultrasonography findings were more common in non-E. coli group. CONCLUSION: E. coli causes more severe inflammation, but non-E. coli infections are more frequently associated with ultrasound abnormalities. However, ESBL production did not affect either laboratory or radiological findings in the present cohort.

Risk Assessment for Arrhythmia in Pediatric Renal Transplant Recipients.

OBJECTIVES: Renal transplant recipients are at risk for ventricular arrhythmia and sudden death. To assess that risk, we compared the ventricular repolarization markers of pediatric renal transplant recipients with those of healthy children. MATERIALS AND METHODS: We included 30 children and adolescents who were followed for at least 6 months after renal transplant; 30 age- and sex-matched children were included for the control group. Demographic features, medications, and laboratory findings were recorded. Blood pressure measurements, ventricular repolarization indexes including QT dispersion, corrected QT dispersion, T-wave peak-to-end interval dispersion, the T-wave peak-to-end interval∕QT ratio, the T-wave peak-to-end interval∕corrected QT ratio, left ventricular mass index, and relative wall thickness were compared between groups. In addition, the correlations of ventricular repolarization indexes with other variables were evaluated. RESULTS: Blood pressure standard deviation scores, the mean heart rate, QT dispersion, corrected QT dispersion, the T-wave peak-to-end interval∕QT ratio, the T-wave peak-to-end interval/corrected QT ratio, left ventricular mass index, and relative wall thickness values were significantly higher in renal transplant patients, whereas T-wave peak-to-end interval dispersion, ejection fraction, and fractional shortening were similar between groups. Although ventricular repolarization indexes were similar in patients with and without left ventricular hypertrophy, only corrected QT dispersion was significantly higher in patients with hypertension (P = .006). The only variable that significantly predicted prolonged corrected QT dispersion was the systolic blood pressure standard deviation score (P = .005, ß = .403). CONCLUSIONS: Ventricular repolarization anomalies, hypertension, left ventricular hypertrophy, and cardiac geometry irregularity may be observed after renal transplant in pediatric recipients despite acceptable allograft functions and normal serum electrolyte levels. Control of systolic blood pressure would decrease the risk of ventricular repolarization abnormalities, namely, the corrected QT dispersion. Follow-up of cardiovascular risks with noninvasive methods is recommended in all pediatric renal transplant recipients.

Evaluation of Pediatric Patients with a Diagnosis of Ureterocele.

Aim: The presence and clinical importance of vesicoureteric reflux in patients with a double collecting system are being questioned. Therefore, the role of voiding cystourethrography in the management of patients with ureterocele is unclear. This study aimed to evaluate patients with a ureterocele in terms of urinary tract infection (UTI) and vesicoureteral reflux (VUR). Material Methods: The cases who were admitted to the Pediatric Nephrology Clinic of Health Sciences University Tepecik Training and Research Hospital between 2012 and 2022 and were diagnosed with ureterocele were evaluated retrospectively. Demographic, clinical, and laboratory data were obtained from file records. Results: All patients diagnosed with ureterocele and voiding cystourethrography (VCUG) were evaluated. A total of 24 (female 13 (54.2%)) children were included. The reasons for admission were antenatal hydronephrosis in 13 (54.2%) patients, UTI in 9 (37.5%) patients, and incidentally diagnosed ureterocele in 2 (8.3%) patients. Urinary tract infection was observed in 20 patients at admission, recurrent UTI in 21 patients at follow-up, preoperative pyelonephritis in 12 patients. VUR was found in 11 patients, and severe VUR (≥stage 3) was found in 9 patients. Ten patients had ipsilateral hydronephrosis, and 14 patients had a double collecting system. The presence of VUR was found to be associated with female gender, UTI at admission, and recurrent UTI at follow-up (p < 0.05). However, there was no difference between groups with or without VUR in terms of ipsilateral hydronephrosis, scar formation, and the need for surgery (p > 0.05). Conclusions: We could not demonstrate any criteria to select patients to receive VCUG; on the other hand, VUR did not cause more kidney damage. Our study supports the need for more scientific data to determine management in patients with ureterocele.

Epicardial adipose tissue and risk of arrhythmia in nephrotic syndrome.

BACKGROUND: Patients with nephrotic syndrome (NS) are at a high risk of cardiovascular disease, obesity, and dyslipidemia. The aim of this study was to evaluate the formation of epicardial adipose tissue (EAT) and investigate electrocardiographic (ECG) parameters in patients. METHODS: Thirty-two patients aged 0-18 years and 15 control patients were compared. In the patient group, physical examination and laboratory parameters were recorded. Atrial depolarization and ventricular repolarization parameters in ECG were compared between the groups. EAT was evaluated with M-mode measurements on echocardiography. RESULTS: There was no difference between the groups in terms of sex, age, body mass index, systolic and diastolic BP. EAT was found to be significantly higher in the patient group. In ECG evaluations it was determined that atrial depolarization and ventricular repolarization parameters increased in the patient group. CONCLUSIONS: Cardiovascular morbidity and mortality are high in kidney diseases. Measurement and follow-up of EAT and ECG findings as a noninvasive parameter can provide information in NS.

Predictors of febrile urinary tract infection caused by extended-spectrum beta-lactamase-producing bacteria.

BACKGROUND: We aimed to evaluate the predictability of extended-spectrum beta-lactamase (ESBL)-producing bacteria (PB) with inflammation markers and hemogram parameters as neutrophil-lymphocyte-ratio (NLR), platelets-lymphocyte-ratio (PLR) and mean-platelet-volume (MPV) in infants with febrile urinary tract infection until the urine cultures are resulted. METHODS: Infants between 2-24 months hospitalized for the first febrile urinary tract infections were grouped as those infected with ESBL-PB and non-ESBL-PB. The demographic and laboratory data (inflammation markers and hemogram parameters) and the ultrasonographical findings were compared between the two groups. RESULTS: A total of 232 patients were included in the study. The mean age was 8.82 ± 5.68 (2-23) months and 114 (49%) of them were female. Escherichia coli was the most common isolated bacteria (79%) followed by Klebsiella pneumoniae (15.5%) in urine cultures. There were 88 patients in ESBL-PB infected group and 144 patients in the non-ESBL-PB group. The hematologic parameters such as white blood cell count (WBC) count, NLR, PLR, MPV and procalcitonin (PCT) were similar between the two groups. Only the rate of ultrasonographic abnormalities was significantly higher in infants infected with ESBL-PB (p=0.012). The risk of ESBL-PB positivity in urine cultures increased with age (OR 1.068, 95% CI 1.002-1.139, p=0.045), PCT (OR 1.094, 95% CI 1.011-1.184, p=0.025), and ultrasonographic abnormalities (OR 3.981, 95% CI 1.792-8.845, p=0.001). CONCLUSIONS: Platelet counts, WBC, MPV, NLR, PLR, and PCT were not reliable markers, however having an ultrasonographic abnormality is the most important independent risk factor for prediction of infection with ESBL-PB.

A rare endocrinological complication of chronic kidney disease.

BACKGROUND: Chronic kidney disease (CKD) may lead to increase in serum levels of peptide hormones as a result of changes in peripheral metabolism. The pathogenesis of uremic hyperprolactinemia in CKD is not fully understood. Plasma prolactin levels are elevated in women, pubertal girls, and also in men with chronic kidney disease. But this is not comon in prepubertal boys. Also in prepubertal children and postmenopausal women, hyperprolactinemia rarely results in galactorrhea. We aimed to discuss hyperprolactinemia and galactorrhea in a 12-year-old male with CKD. CASE: A twelve-year-old boy with chronic kidney disease (CKD) suffered from bilateral galactorrhea. He was on follow-up at Pediatric Nephrology Department from the age of two due to bilateral dysplastic kidney. On physical examination, his weight was - 0.59 SDS, height was -2.82 SDS, Blood pressure was 115 / 72 (75p), stretched penis length was 6 cm, testicular volume was 3mL / 3mL, pubic hair was Tanner Stage 1, breast examination did not reveal plaque on bilateral breast. He was receiving recombinant erythropoietin, sodium bicarbonate, polystyrene sulfonate, calcium acetate, and calcitriol treatments. Glomerular filtration rate was 23ml/min/1.73 m2 (CKD stage IV). Serum prolactin (PRL) was > 200 µg/L (N, 2.64-13.13). The pituitary adenoma was excluded with pituitary and cranial magnetic resonance imaging (gadolinium). Cabergoline (0.5 mg/ twice weekly) was initiated to decrease PRL levels and reduce galactorrhea. In the second week of treatment, serum PRL level was suppressed (0.4 µg/L) and galactorrhea was completely resolved. CONCLUSIONS: Although uremic hyperprolactinemia is very rarely seen in childhood, it is important to evaluate, and initiate an appropriate treatment since it is associated with delayed puberty and infertility in adulthood in many cases.

Re-evaluating hypertension in children according to different guidelines: a single-center study.

We aimed to evaluate the agreements between the guidelines used for both office blood pressure (OBP) and ambulatory blood pressure monitoring (ABPM). Our secondary aim was to define the best threshold to assess children at risk of left ventricular hypertrophy (LVH). Thresholds proposed by the Fourth Report (FR), European Society of Hypertension (ESH), and American Academy of Pediatrics (AAP) for OBP and the Wühl, ESH, and American Heart Association (AHA) for ABPM were used, and nine different BP phenotype combinations were created. The agreements between the thresholds, the sensitivity of the thresholds, and the BP phenotypes used to predict LVH were determined in 949 patients with different ages and body mass indices (BMIs). The agreements between the guidelines for OBP and ABPM were "good" and "very good" (κ = 0.639; 95% CI, 0.638-0.640, κ = 0.986; 95% CI, 0.985-0.988), respectively. To classify OBP and ABPM into BP phenotypes, we obtained nine different combinations, which had "very good" agreement (κ = 0.880; 95% CI, 0.879-0.880). The sensitivity of AAP for detecting LVH was the highest in <12-year-old obese children (S = 75.8, 95% CI, 56.4-89.7). The sensitivity of ABPM in detecting LVH was similar among different age and BMI groups. The sensitivity of different BP phenotypes tended to be higher in the groups where OBP was evaluated according to AAP. The highest sensitivity was detected in the 13- to 15-year-old normal weight group.(S: 88.8, 95% CI, 51.7-99.7). The AAP guideline is more sensitive and decisive for BP phenotypes to detect LVH, especially in normal-weight children ≤ 15 years, while ABPM thresholds for children have limited effect.

Effects of ambulatory blood pressure monitoring parameters on left ventricular mass index in hypertensive children.

OBJECTIVE: We aimed to evaluate the effects of blood pressure (BP) values obtained by 24-h ambulatory BP monitoring (ABPM) of hypertensive children and adolescents on left ventricular mass index (LVMI). METHODS: Patients diagnosed with HT with BP measurements confirmed with ABPM and evaluated with echocardiography for LVMI were included. The patients were divided into two groups according to their BMI as obese and nonobese. SDSs of ABPM parameters were compared between the groups. RESULTS: A total of 158 children with HT were included in the study. Ninety of these patients were obese. In obese and nonobese cases, mean SDS levels were similar in ABPM parameters, whereas LVMI was significantly higher in obese patients ( P = 0.049). There was a significant correlation between LVMI and 24-h SBP SDS, daytime SBP SDS, 24-h SBP load and daytime BP load. In obese cases, there was a statistically significant correlation between LVMI and 24-h SBP SDS, daytime SBP SDS, 24-h SBP load, daytime SBP load as well as nighttime SBP SDS and nighttime SBP load. When the whole group was evaluated, 24-h SBP SDS was the most effective parameter influencing LVMI ( P = 0.001). Similarly, the most effective ABPM parameter on LVMI in obese patients was 24-h SBP SDS ( P = 0.001). CONCLUSION: A significantly higher rate of LVMI in obese patients suggests that obesity itself is an effective factor on LVMI. In addition, systolic hypertension is more effective on cardiac functions compared with DBP measurements and systolic-DBP dipping ratios.

Kidney disease profile and encountered problems during follow-up in Syrian refugee children: a multicenter retrospective study.

BACKGROUND: Children are one of the most vulnerable groups in conflict zones, especially those with chronic diseases. This study aimed to investigate kidney disease profiles and problems during follow-up in a population of Syrian refugee children residing in Turkey. METHODS: Syrian refugee children aged between 0 and 18 years were included in the study. Demographic data, diagnosis, particular interventions due to nephrological problems, and problems encountered during follow-up were obtained from all participating pediatric nephrology centers. RESULTS: Data from 633 children from 22 pediatric nephrology centers were included. Mean age of the children was 94.8 ± 61.7 months and 375 were male (59%). 57.7% had parental consanguinity and 23.3% had a close relative(s) with kidney disease. The most common kidney diseases were congenital anomalies of the kidney and urinary tract (CAKUT) (31.0%), glomerular disease (19.9%), chronic kidney disease (CKD) (14.8%), and urolithiasis (10.7%). Frequent reasons for CAKUT were nonobstructive hydronephrosis (23.0%), vesico-ureteral reflux (18.4%), and neurogenic bladder (15.8%). The most common etiology of glomerular diseases was nephrotic syndrome (69%). Ninety-four children had CKD, and 58 children were on chronic dialysis. Six children had kidney transplantation. Surgical intervention was performed on 111 patients. The language barrier, lack of medical records, and frequent disruptions in periodic follow-ups were the main problems noted. CONCLUSIONS: CAKUT, glomerular disease, and CKD were highly prevalent in Syrian refugee children. Knowing the frequency of chronic diseases and the problems encountered in refugees would facilitate better treatment options and preventive measures.