Urinary tract dilation classification system for predicting surgical management and urinary tract infection in neonates and young infants: a systematic review and meta-analysis.

BACKGROUND: The urinary tract dilation classification system has recently been developed to ensure a unified approach to describe urinary tract dilation in neonates and young infants. However, the predictive value of this system for surgical intervention or urinary tract infection (UTI) has not yet been evaluated in a meta-analysis. OBJECTIVE: This systematic review and meta-analysis aimed to evaluate the utility of a postnatal urinary tract dilation classification system for predicting surgical management or a UTI occurrence. MATERIALS AND METHODS: As the urinary tract dilation classification system was introduced in 2014, we searched Embase and PubMed databases for studies published between January 2014 and December 2022. Original articles that reported surgical interventions or UTI episodes according to postnatal urinary tract dilation grades were included. The pooled odds ratio (OR) was calculated, using either the fixed-effects or random-effects model, given the lower urinary tract dilation grades as the base category. The quality of the included studies was evaluated using the Newcastle-Ottawa scale. RESULTS: Of the 285 articles reviewed, eight (comprising 2,165 children) were included in the analysis. The studies were of medium-to-high quality. Pooled analysis demonstrated that urinary tract dilation P3 (combined OR, 21.41; 95% confidence interval [CI], 15.72-29.17) and urinary tract dilation P2-P3 (combined OR, 65.17; 95% CI, 33.08-128.38) were associated with surgical intervention. The urinary tract dilation P3 (combined OR, 2.11; 95% CI, 1.56-2.85) and urinary tract dilation P2-P3 (combined OR, 3.36; 95% CI, 2.43-4.63) were associated with UTI episodes. CONCLUSION: The postnatal urinary tract dilation classification system is useful for predicting the need for surgical management and UTI episodes in infants with hydronephrosis.

Erratum: efficacy and tolerability of anticholinergics in korean children with overactive bladder: a multicenter retrospective study.

[This corrects the article on p. 1550 in vol. 29, PMID: 25408588.].

Desmopressin alone versus desmopressin and an anticholinergic in the first-line treatment of primary monosymptomatic nocturnal enuresis: a multicenter study.

BACKGROUND: The aim of this study was to compare the efficacy of combination therapy with desmopressin and an anticholinergic to desmopressin monotherapy for the first-line treatment of children with primary monosymptomatic nocturnal enuresis (PMNE). METHODS: A total of 98 children with PMNE (male:female 71:27) aged 5-16 (mean age 7.18 ± 1.8) years were retrospectively analyzed. The patients were divided into two groups: the monotherapy group (n = 49) was given oral desmopressin alone, and the combination therapy group (n = 49) was given desmopressin plus an anticholinergic (propiverine 10 mg) as a first-line treatment. The two groups were matched according to the following criteria: age, gender, and baseline frequency of nocturnal enuresis. The efficacy was evaluated by International Children's Continence Society criteria at 1 and 3 months after treatment initiation. RESULTS: The combination therapy group showed a higher rate of complete response than the monotherapy group (20.4 vs. 6.1% at 1 month of treatment; 46.9 vs. 22.4% at 3 months of treatment). In terms of success (response and complete response), there was a significant difference between the two groups after 3 months of treatment (P = 0.002). CONCLUSIONS: Our results indicate that combination therapy with desmopressin plus an anticholinergic is quicker and more effective than desmopressin monotherapy in reducing PMNE.

Efficacy and tolerability of anticholinergics in Korean children with overactive bladder: a multicenter retrospective study.

We investigated the efficacy and tolerability of various anticholinergics in Korean children with non-neurogenic overactive bladder (OAB). A total of 326 children (males:females= 157:169) aged under 18 yr (mean age 7.3±2.6 yr) who were diagnosed with OAB from 2008 to 2011 were retrospectively reviewed. The mean duration of OAB symptoms before anticholinergic treatment was 16.9±19.0 months. The mean duration of medication was 5.6±7.3 months. Urgency urinary incontinence episodes per week decreased from 1.9±3.1 to 0.4±1.5 times (P<0.001). The median voiding frequency during daytime was decreased from 9.2±5.4 to 6.3±4.2 times (P<0.001). According to 3-day voiding diaries, the maximum and average bladder capacity were increased from 145.5±66.9 to 196.8±80.3 mL and from 80.8±39.6 to 121.8±56.5 mL, respectively (P<0.001). On uroflowmetry, maximum flow rate was increased from 17.6±8.4 to 20.5±8.2 mL/sec (P<0.001). Adverse effects were reported in 14 (4.3%) children and six children (1.8%) discontinued medication due to adverse effects. Our results indicate that anticholinergics are effective to improve OAB symptoms and tolerability was acceptable without severe complications in children.

Mechanical stimulation by ultrasound enhances chondrogenic differentiation of mesenchymal stem cells in a fibrin-hyaluronic acid hydrogel.

Chondrogenic differentiation and cartilage tissue formation derived from stem cells are highly dependent on both biological and mechanical factors. This study investigated whether or not fibrin-hyaluronic acid (HA) coupled with low-intensity ultrasound (LIUS), a mechanical stimulation, produces an additive or synergistic effect on the chondrogenesis of rabbit mesenchymal stem cells (MSCs) derived from bone marrow. For the purpose of comparison, rabbit MSCs were first cultured in fibrin-HA or alginate hydrogels, and then subjected to chondrogenic differentiation in chondrogenic-defined medium for 4 weeks in the presence of either transforming growth factor-beta3 (TGF-ß3) (10 ng/mL) or LIUS treatment (1.0 MHz and 200 mW/cm(2) ). The resulting samples were evaluated at 1 and 4 weeks by histological observation, chemical assays, and mechanical analysis. The fibrin-HA hydrogel was found to be more efficient than alginate in promoting chondrogenesis of the MSCs by producing a larger amount of sulfated glycosaminoglycans (GAGs) and collagen, and engineered constructs made with the hydrogel demonstrated higher mechanical strength. At 4 weeks of tissue culture, the chondrogenesis of the MSCs in fibrin-HA were shown to be further enhanced by treatment with LIUS, as observed by analyses for the amounts of GAGs and collagen, and mechanical strength testing. In contrast, TGF-ß3, a well-known chondrogenic inducer, showed a marginal additive effect in the amount of collagen only. These results revealed that LIUS further enhanced chondrogenesis of the MSCs cultured in fibrin-HA, in vitro, and suggested that the combination of fibrin-HA and LIUS is a useful tool in constructing high-quality cartilage tissues from MSCs.

A nationwide epidemiological study of nocturnal enuresis in Korean adolescents and adults: population based cross sectional study.

We performed a nationwide epidemiological study to evaluate the prevalence and characteristics of nocturnal enuresis (NE) in Korean adolescents and adults. A questionnaire was sent via e-mail to 51,073 people aged 16-40 yr by stratified sampling according to age, sex, and region among a 200,000 internet survey panel pool. The questionnaire included following information; presence or absence of NE, frequency of NE, possible risk factors for NE, self-esteem scale score and depression score results, and measures for the treatment of NE. Among the 2,117 responders, 54 (2.6%) had NE (≥1 enuretic episode within 6 months). Of 54 bedwetters, 9.3% wet ≥1 night per week and 20.5% wet ≥1 per month. The prevalence rates remained relatively stable with no apparent trend of reduction with age. The presence of sleep disturbance, family history, urgency, or urge incontinence increased the probability of NE episode significantly. The self-esteem score was lower (P=0.053) and the depression scale score was higher (P=0.003) in bedwetters compared with non-bedwetters. Overall 2.6% of Korean aged 16-40 yr have NE. The higher rate of urgency and urge incontinence in adolescent and adult enuretics suggests that bladder function has an important role in adolescent and adult NE.

Long-term outcomes and associated prognostic risk factors of childhood-onset lupus nephritis.

Background: This study investigated the clinical characteristics and kidney outcomes of childhood-onset lupus nephritis (LN), and risk factors associated with prognosis. Methods: We enrolled 216 patients with histologically diagnosed LN during childhood. The Korean Society of Pediatric Nephrology organized a retrospective cohort study of childhood-onset LN in 13 major pediatric nephrology centers in South Korea. Results: The mean age at kidney biopsy was 13.2 ± 3.22 years. The main forms of presentation were nephrotic syndrome and/or hematuria in 152 patients (70.4%), and the most common histological finding was World Health Organization (WHO) class IV in 138 patients (63.9%), followed by WHO class III in 34 patients (15.7%). In the outcome analysis, the mean follow-up period of the patients was 7.8 ± 5.11 years. At last follow-up, 32 patients (14.8%) developed advanced chronic kidney disease (CKD). Male sex and failure to achieve remission at 12 months of treatment were significant risk factors for developing advanced CKD (hazard ratio of 2.57 and 2.29, respectively). Conclusion: Our study demonstrated the clinical characteristics and long-term outcomes of patients with childhood-onset LN. Male sex and failure to achieve remission in the first year of treatment were predictive of advanced CKD. Therefore, prompt awareness and close monitoring of these high-risk patients are needed, which may further improve the prognosis of children with LN.

Genotype-phenotype correlation of X-linked Alport syndrome observed in both genders: a multicenter study in South Korea.

The genotype-phenotype correlation of the X-linked Alport syndrome (XLAS) has been well elucidated in males, whereas it remains unclear in females. In this multicenter retrospective study, we analyzed the genotype-phenotype correlation in 216 Korean patients (male:female = 130:86) with XLAS between 2000 and 2021. The patients were divided into three groups according to their genotypes: the non-truncating group, the abnormal splicing group, and the truncating group. In male patients, approximately 60% developed kidney failure at the median age of 25.0 years, and kidney survival showed significant differences between the non-truncating and truncating groups (P < 0.001, hazard ratio (HR) 2.8) and splicing and truncating groups (P = 0.002, HR 3.1). Sensorineural hearing loss was detected in 65.1% of male patients, while hearing survival periods showed a highly significant difference between the non-truncating and truncating groups (P < 0.001, HR 5.1). In female patients, approximately 20% developed kidney failure at the median age of 50.2 years. The kidney survival was significantly different between the non-truncating and truncating groups (P = 0.006, HR 5.7). Our findings support the presence of genotype-phenotype correlation not only in male patients but also in female patients with XLAS.

Characteristics of patients who visit the emergency department with self-inflicted injury.

During visits to emergency medical facilities, the primary care of and risk identification for individuals who have attempted suicide is considered an important element in suicide prevention. With the ultimate goal of helping to prevent suicide, the aim of the present study was to determine the characteristics of patients with self-inflicted injuries who presented in the emergency department. Patients with self-inflicted injuries who visited 1 of 3 sentinel emergency medical centers from 2007 through 2009 were included in the study. The characteristics, methods, and reasons for suicide attempts were evaluated. Moreover, predictors of severe outcomes were evaluated. A total of 2,996 patients with self-inflicted injuries visited the three centers during a period of 3 yr. The male-to-female suicide ratio was 1:1.38 (P < 0.001). The mean age was 41 yr. Poisoning was the most common method of self-inflicted injury (68.7%) among all age groups. Medication was the primary means of injury in the < 50 age group, and the use of agricultural chemicals was the primary means in the ≥ 50 age group. The reasons for attempting suicide varied among the age groups. The predictors of severe outcome are male gender, older age, and not having consumed alcohol.

Prevalence of primary immunodeficiency in Korea.

This study represents the first epidemiological study based on the national registry of primary immunodeficiencies (PID) in Korea. Patient data were collected from 23 major hospitals. A total of 152 patients with PID (under 19 yr of age), who were observed from 2001 to 2005, have been entered in this registry. The period prevalence of PID in Korea in 2005 is 11.25 per million children. The following frequencies were found: antibody deficiencies, 53.3% (n = 81), phagocytic disorders, 28.9% (n = 44); combined immunodeficiencies, 13.2% (n = 20); and T cell deficiencies, 4.6% (n = 7). Congenital agammaglobulinemia (n = 21) and selective IgA deficiency (n = 21) were the most frequently reported antibody deficiency. Other reported deficiencies were common variable immunodeficiencies (n = 16), X-linked agammaglobulinemia (n = 15), IgG subclass deficiency (n = 4). Phagocytic disorder was mostly chronic granulomatous disease. A small number of patients with Wiskott-Aldrich syndrome, hyper-IgE syndrome, and severe combined immunodeficiency were also registered. Overall, the most common first manifestation was pneumonia. This study provides data that permit a more accurate estimation PID patients in Korea.

Cyclosporin A therapy for Henoch-Schönlein nephritis with nephrotic-range proteinuria.

To evaluate the therapeutic role of cyclosporin A (CyA) for the treatment of Henoch-Schönlein nephritis (HSN), 29 patients (18 boys, 11 girls) with nephrotic-range proteinuria were analyzed retrospectively. Mean age was 8.6 years (range 2.0-15.5 years) at diagnosis of Henoch-Schönlein purpura (HSP). All patients had developed the nephrotic-range proteinuria at a mean interval of 4.4 months (range 0-50.7 months) after the diagnosis of HSP. Mean duration of CyA treatment was 12.3 months (range 2.6-55.0 months). Mean follow-up times were 3.7 years (range 1.2-12.9 years) from the beginning of the CyA treatment. Steroids were tapered off and stopped gradually after initiation of CyA. All patients responded to the CyA treatment within a mean of 1.8 months (range 1 week to 3.5 months). Twenty-three patients achieved stable remission with mean follow-up duration of 3.2 years and 6 patients seemed to become CyA-dependent, since they developed proteinuria when the treatment was stopped. Renal function was preserved in all patients but one who developed end-stage renal disease after poor compliance with CyA. We concluded that CyA treatment for HSN showing nephrotic-range proteinuria is very effective and a safe method, although some patients become CyA-dependent.

Acute peritoneal dialysis in very low birth weight neonates using a vascular catheter.

We report on our experience with acute peritoneal dialysis (APD) in 16 very low birth weight neonates ranging from 24.6 to 30.2 weeks' gestation with a birth weight ranging from 630 g to 1,430 g using a 14-gauge Arrow vascular catheter for APD access. The underlying causes of acute renal failure were: sepsis (7), necrotizing enterocolitis (4), patent ductus arteriosus (3), hydrops fetalis (1), intracranial hemorrhage (3), pulmonary hemorrhage (2), pneumonia (1), and perinatal asphyxia (1). Among 12 patients, the APD was successful for the control of hyperkalemia, fluid overload, and metabolic acidosis. The peritoneal permeability and transport were at their maximum at a short dwell time with rapid exchanges. Complications associated with the APD were: peritonitis (2), leakage (2), hemoperitoneum (1), and hernia (1). During the dialysis, four patients died; there were three episodes of catheter-related complications in these patients. At 60 days after the withdrawal of the APD, 10 patients were alive, and had full recovery of their renal function. Therefore, APD in premature neonates with a 14-gauge Arrow vascular catheter was safe and effective. This procedure helped manage the hemodynamic and metabolic imbalance of acute renal failure and was associated with few complications.

Prevalence of pediculosis capitis among Korean children.

Pediculus humanus capitis is an ectoparasite, which causes scalp pruritus particularly among children. A total of 15,373 children including 8,018 boys and 7,355 girls from 26 primary schools and 15 kindergartens attached to the primary schools and a total of 33 children from an orphanage were examined for head lice infestation (HLI). The overall prevalence of HLI in this study was 4.1% including 3.7% of the urban areas and 4.7% of the rural areas. Head lice were found more frequently in girls than in boys with prevalence of 6.5% and 1.9%, respectively. The infestation rate by school grade was 3.2%, 4.7%, 4.2%, 5.0%, 4.9%, 3.8%, and 2.1% for kindergarten, first, second, third, fourth, fifth, and sixth grades, respectively. The infestation rate of the children in an orphanage was 66.7%. The prevalence of HLI has decreased especially in the rural areas. However, HLI is a still health problem of kindergarteners and primary schoolchildren in Korea.

Partial tetrasomy of chromosome 22q11.1 resulting from a supernumerary isodicentric marker chromosome in a boy with cat-eye syndrome.

The 22q11 region has been implicated in chromosomal rearrangements that result in altered gene dosage, leading to three different congenital malformation syndromes: DiGeorge syndrome, cat-eye syndrome (CES), and der(22) syndrome. Although DiGeorge syndrome is a common genomic disorder on 22q11, CES is quite rare, and there has been no report of Korean CES cases with molecular cytogenetic confirmation. In this study, we present the phenotypic and genetic characteristics of a 3-month-old boy with CES. Clinical findings included micropthalmia, multiple colobomata, and renal and genital anomalies. Cytogenetic analyses showed the presence of a supernumerary marker chromosome, which was identified as a bisatellited and isodicentric chromosome derived from an acrocentric chromosome. The results of array comparative genomic hybridization and fluorescence in situ hybridization studies confirmed the karyotype as 47,XY,+mar.ish idic(22)(q11.1) (D22S43+).arr 22q11.1(15,500,000-15,900,000)x4, resulting in a partial tetrasomy of 22q11.1. To the best of our knowledge, this is the first report in Korea of CES confirmed by cytogenetic and molecular cytogenetic analyses.

Diagnostic performance of contrast-enhanced ultrasound for acute pyelonephritis in children.

The objective of our study was to evaluate the performance of renal contrast-enhanced ultrasound (CEUS) against the 99m-labeled dimercaptosuccinic acid (DMSA) scan and computed tomography (CT) in children for the diagnosis of acute pyelonephritis. We included children who underwent both renal CEUS and the DMSA scan or CT. A total of 33 children (21 males and 12 females, mean age 26 ± 36 months) were included. Using the DMSA scan as the reference standard, the sensitivity, specificity, positive predictive value, and negative predictive value of CEUS was 86.8%, 71.4%, 80.5%, and 80.0%, respectively. When CT was used as the reference standard, the sensitivity, specificity, positive predictive value, and negative predictive value of CEUS was 87.5%, 80.0%, 87.5%, and 80.0%, respectively. The diagnostic accuracy of CEUS for the diagnosis of acute pyelonephritis was 80.3% and 84.6% compared to the DMSA scan and CT, respectively. Inter-observer (kappa = 0.54) and intra-observer agreement (kappa = 0.59) for renal CEUS was moderate. In conclusion, CEUS had good diagnostic accuracy for diagnosing acute pyelonephritis with moderate inter- and intra-observer agreement. As CEUS does not require radiation or sedation, it could play an important role in the future when diagnosing acute pyelonephritis in children.

Renovascular hypertension treated by renal artery embolization.

Renovascular hypertension is caused by narrowing of the arteries supplying the kidneys. There are several methods to treat renal artery stenosis, such as medications, percutaneous transluminal renal angioplasty, and atherosclerosis. A boy presented to our hospital with severe hypertension. Computed tomography angiogram revealed severe narrowing of the left renal artery and hypoplastic left kidney. Total renal artery embolizaton was performed to make a complete occlusion of the left renal artery. Follow-up renin and aldosterone levels were gradually decreased. The main advantage of renal artery embolization is that it is minimally invasive compared with extensive surgical procedures. Therefore, renal artery embolization should be considered as an alternative to surgical nephrectomy in pediatric patients with renovascular hypertension.