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.

Clinical Profile and Renal Ultrasound Characteristics of Children With Nutcracker Syndrome in Turkey.

OBJECTIVE: We aimed to evaluate the clinical profile and radiological findings of children with nutcracker syndrome (NCS) and to assess the association between the parameters. METHODS: A retrospective analysis of the clinical, laboratory and radiological parameters of children diagnosed with NCS between January, 2011 and October, 2017 was done. RESULTS: Of a total of 29 patients [19 girls, 65.5%] with NCS, having a mean (SD) age of 10.8 years, 72.4% had BMI <-2SD. Approximately half of the patients (51.7%) were asymptomatic. Left flank pain was commonest (9/29; 31%) symptom, followed by macroscopic hematuria (4/29; 13.8%). Isolated proteinuria was seen in 9 children. There was no significant difference between the symptomatic and asymptomatic patients in terms of Doppler ultrasonography findings. All patients were followed up conservatively, 5 received enalapril therapy for moderate proteinuria. CONCLUSION: NCS should be considered in children, especially with low BMI, presenting with orthostatic proteinuria and hematuria, with or without left flank pain after ruling out the common causes.

Severity of self-reported depressive symptomatology and relevant factors in children with primary monosymptomatic nocturnal enuresis and their mothers.

BACKGROUND: Nocturnal enuresis (NE) may negatively affect social and emotional life as well as mood in both children and their mothers. The aim of this study is to evaluate severity of self-reported depressive symptomatology and determine the relevant factors in children with primary monosymptomatic nocturnal enuresis (MNE) and their mothers by using depression inventories. METHODS: Children Depression Inventory (CDI) for children and Beck Depression Inventory (BDI) for mothers were administered to the study group. The children and mothers in the patient and control groups were compared according to the depression inventory scores. The relationship of various sociodemographic factors with those scores was also investigated. RESULTS: BDI scores of the mothers of children with primary MNE demonstrated minor depressive symptomatology and were significantly higher than the mothers in the control group (p = 0.002). Moreover, although within the normal range, CDI scores of the children with primary MNE were also significantly higher than the controls (p = 0.031). Main factors associated with BDI scores were the presence of primary MNE, maternal educational level, and CDI scores. School achievement of the children, monthly income of the family, and BDI scores were found to be correlated to the CDI scores. CONCLUSIONS: Primary MNE was found to be associated with negative mood of the mothers in the present study. As misinformed parental attitudes adversely affect family dynamics, improved awareness of, and maternal education regarding primary MNE is vital in improving the holistic outcome of families affected by MNE.

An adolescent boy progressing insidiously to end-stage renal disease: Questions.

Hinman syndrome (HS), or non-neurogenic neurogenic bladder, is a voiding dysfunction of the bladder of neuropsychological origin that is characterized by functional bladder outlet obstruction in the absence of neurologic deficits. The bladder-sphincter discoordination causes damage to the bladder and upper urinary tract if it is not timely diagnosed and adequately treated. This case emphasizes the following important message; nighttime wetting is not a benign condition in every child. Parental awareness should be raised about voiding disorders, so it may be possible to prevent some important renal diseases such as Hinman syndrome.

An adolescent boy progressing insidiously to end-stage renal disease: Answers.

Hinman syndrome was a non-neurogenic neurogenic bladder and the most severe form of dysfunctional voiding disorder. The bladder-sphincter discoordination causes damage to the bladder and upper urinary tract if it is not diagnosed early and treated adequately. This case emphasizes the following important message: nighttime wetting is not a benign condition in every child. Parental awareness should be raised about voiding disorders, as it may be possible to prevent important renal diseases such as Hinman syndrome.

Bisphosphonates in juvenile dermatomyositis with dystrophic calcinosis.

OBJECTIVES: Our primary objective was to retrospectively analyze whether bisphosphonates initiated in combination with immunosuppressive drugs and/or intravenous immunoglobulin (IVIG) resulted in a radiological and clinical improvement of dystrophic calcinosis in six female juvenile dermatomyositis (JDM) patients. METHODS: Medical records of the patients were reviewed. All six patients met the Bohan and Peter diagnostic criteria for JDM. RESULTS: A resolution of calcinosis was observed in four of the six patients with JDM following the use of bisphosphonates and intensive immunosuppressive therapy with or without IVIG. Bisphosphonates were unable to either decelerate the progression of calcinosis or improve calcinosis in cases 5 and 6. In case 5, it took a relatively long time to control the disease activity despite the appropriate immunsuppressive treatment and she experienced multiple flares of active JDM. Case 6 had a long duration of severe active disease before treatment initiation. No important adverse event was observed. CONCLUSIONS: Early commencement of aggressive immunosuppressive agents in combination with bisphosphonate is a choice for the treatment of calcinosis in JDM patients. Concomitant use of IVIG may have an additional effect on the resolution of calcinosis.

Midstream clean-catch urine collection in newborns: a randomized controlled study.

UNLABELLED: We aimed to evaluate a recently defined technique based on bladder stimulation and paravertebral lumbar massage maneuvers in collecting a midstream clean-catch urine sample in newborns. A total of 127 term newborns were randomly assigned either to the experimental group or the control group. Twenty-five minutes after feeding, the genital and perineal areas of the babies were cleaned. The babies were held under the armpits with legs dangling. Bladder stimulation and lumbar paravertebral massage maneuvers were only applied to the babies in the experimental group. Success was defined as collection of a urine sample within 5 min of starting the stimulation maneuvers in the experimental group and of holding under the armpits in the control group. The success rate of urine collection was significantly higher in the experimental group (78%) than in the control group (33%; p < 0.001). The median time (interquartile range) for sample collection was 60 s (64.5 s) in the experimental group and 300 s (95 s) in the control group (p < 0.0001). Contamination rates were similar in both groups (p = 0.770). CONCLUSION: We suggest that bladder stimulation and lumbar paravertebral massage is a safe, quick, and effective way of collecting midstream clean-catch urine in newborns.