Characteristics of pediatric rhabdomyolysis and the associated risk factors for acute kidney injury: a retrospective multicenter study in Korea

Background@#The clinical features of pediatric rhabdomyolysis differ from those of the adults with rhabdomyolysis; however, multicenter studies are lacking. This study aimed to investigate the characteristics of pediatric rhabdomyolysis and reveal the risk factors for acute kidney injury (AKI) in such cases. @*Methods@#This retrospective study analyzed the medical records of children and adolescents diagnosed with rhabdomyolysis at 23 hospitals in South Korea between January 2007 and December 2016. @*Results@#Among 880 patients, those aged 3 to 5 years old composed the largest subgroup (19.4%), and all age subgroups were predominantly male. The incidence of AKI was 11.3%. Neurological disorders (53%) and infection (44%) were the most common underlying disorder and cause of rhabdomyolysis, respectively. The median age at diagnosis in the AKI subgroup was older than that in the non-AKI subgroup (12.2 years vs. 8.0 years). There were no significant differences in body mass index, myalgia, dark-colored urine, or the number of causal factors between the two AKI-status subgroups. The multivariate logistic regression model indicated that the following factors were independently associated with AKI: multiorgan failure, presence of an underlying disorder, strong positive urine occult blood, increased aspartate aminotransferase and uric acid levels, and reduced calcium levels. @*Conclusions@#Our study revealed characteristic clinical and laboratory features of rhabdomyolysis in a Korean pediatric population and highlighted the risk factors for AKI in these cases. Our findings will contribute to a greater understanding of pediatric rhabdomyolysis and may enable early intervention against rhabdomyolysis-induced AKI.

Intellectual Functioning of Pediatric Patients with Chronic Kidney Disease:Results from the KNOW-Ped CKD

Background@#Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the cognitive function of children and adolescents with CKD. This study therefore aimed to investigate the mean intelligence and risk factors for low intelligence in children and adolescents with CKD. @*Methods@#Eighty-one patients with CKD under 18 years old were included in the KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease (KNOW-Ped CKD). Participants completed either the Wechsler Intelligence Scale for Children (6–16 years), or Wechsler Adult Intelligence Scale (> 16 years). @*Results@#The mean full-scale intelligence quotient (IQ) was 91 ± 19; 24.7% of participants scored a full-scale IQ below 80. Participants with a short stature (height Z scores < −1.88), failure to thrive (weight Z scores < −1.65), more severe CKD stage (≥ IIIb), longer duration of CKD (≥ 5 years), and those who were Medicare or Medicaid beneficiaries, had significantly lower mean full-scale IQs. @*Conclusion@#On linear regression analysis, the association between the full-scale IQ, and longer duration of CKD and growth failure, remained significant after controlling for demographic and clinical variables. It is therefore necessary to investigate cognitive impairment in pediatric patients with CKD who exhibit growth failure or for a longer postmorbid period. It is believed that early interventions, such as kidney transplantation, will have a positive effect on IQ in children with CKD, as the disease negatively affects IQ due to poor glomerular filtration rate over time.

Intellectual Functioning of Pediatric Patients with Chronic Kidney Disease:Results from the KNOW-Ped CKD

Background@#Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the cognitive function of children and adolescents with CKD. This study therefore aimed to investigate the mean intelligence and risk factors for low intelligence in children and adolescents with CKD. @*Methods@#Eighty-one patients with CKD under 18 years old were included in the KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease (KNOW-Ped CKD). Participants completed either the Wechsler Intelligence Scale for Children (6–16 years), or Wechsler Adult Intelligence Scale (> 16 years). @*Results@#The mean full-scale intelligence quotient (IQ) was 91 ± 19; 24.7% of participants scored a full-scale IQ below 80. Participants with a short stature (height Z scores < −1.88), failure to thrive (weight Z scores < −1.65), more severe CKD stage (≥ IIIb), longer duration of CKD (≥ 5 years), and those who were Medicare or Medicaid beneficiaries, had significantly lower mean full-scale IQs. @*Conclusion@#On linear regression analysis, the association between the full-scale IQ, and longer duration of CKD and growth failure, remained significant after controlling for demographic and clinical variables. It is therefore necessary to investigate cognitive impairment in pediatric patients with CKD who exhibit growth failure or for a longer postmorbid period. It is believed that early interventions, such as kidney transplantation, will have a positive effect on IQ in children with CKD, as the disease negatively affects IQ due to poor glomerular filtration rate over time.

Predictive Markers for Screening Renal Damagein Children with Urinary Tract infections andVesicoureteral Reflux

Purpose@#Urinary tract infections (UTIs) are the most common and serious bacterialinfections in children. Therefore, early diagnosis of vesicoureteral reflux (VUR)for treatment planning and the identification of noninvasive markers that canpredict renal injury are important in patients with UTIs. We analyzed the clinicalfeatures of pediatric UTIs commonly encountered by general practitioners and reinterpretedthe blood tests and imaging findings to identify the important clinicalpredictive markers of VUR in order to selectively perform VCUG. @*Methods@#This retrospective study was performed among 183 children diagnosedwith a UTI or acute pyelonephritis. @*Results@#The most significant predictor of high grade and bilateral VUR identifiedusing area under the curve analyses was hydronephrosis on kidney ultrasoundimages with renal cortical defects on dimercaptosuccinic acid (DMSA) kidney scansimultaneously, followed by hydronephrosis only on kidney ultrasound. @*Conclusion@#The presence of hydronephrosis on kidney ultrasound images or corticaldefects or asymmetric kidneys on the DMSA kidney scans can be predictivemarkers of VUR, reducing the need for VCUG. Our study can thus help minimizethe exposure to radiation among patients through selective VCUG.

Prediction Model for Massive Transfusion in Placenta Previa during Cesarean Section

0.05). The area under the receiver operating characteristics curve (AUC) was 0.922 [95% confidence interval (CI) 0.89–0.95]. In external validation, the discrimination was good, with an AUC value of 0.833 (95% CI 0.70–0.92) for this model. Nomogram calibration plots indicated good agreement between the predicted and observed outcomes, exhibiting close approximation between the predicted and observed probability.CONCLUSION: We constructed a scoring model for predicting massive transfusion during cesarean section in women with placenta previa. This model may help in determining the need to prepare an appropriate amount of blood products and the optimal timing of blood transfusion.

Localization of the genicular arteries under ultrasound guidance

BACKGROUND: The genicular arteries (GAs) can be utilized for genicular nerve block. We aimed to evaluate the ability to localize GAs under ultrasound in patients with chronic knee pain. METHODS: Twenty-four knees from 14 osteoarthritic patients were enrolled. The target GAs included the superomedial GA (SMGA), superolateral GA (SLGA), and inferomedial GA (IMGA). GAs were observed at the relevant adductor tubercle and epicondyle-shaft transition under ultrasound. Distribution of the SMGA at the adductor tubercle was evaluated using defined zones in transverse and longitudinal ultrasound images. SLGA and IMGA were also categorized using defined zones in longitudinal images. Distance from bony cortex to the relevant GA was then estimated. RESULTS: Among 24 knees, 91.7% of SMGAs were located at the upper part of the adductor tubercle. The distances between the SMGA and bony cortex on transverse view (dSMGAt) and on longitudinal view (dSMGAl) were directly correlated (rs = 0.6539, P = 0.0005). CONCLUSIONS: Under ultrasound guidance, the SMGA was found to be mainly localized to the upper part of the adductor tubercle. Likewise, the SLGA and IMGA were mainly localized at the distal and proximal parts of the epicondyle-shaft transition, respectively. Our results support the feasibility of ultrasound guidance for GA localization in patients with knee osteoarthritis.

Bilateral Ureteral Obstruction Related to Pelvic Rhabdomyosarcoma Presenting with Acute Kidney Injury: A Case Report

Bilateral renal obstruction is a rare critical condition, requiring a prompt diagnostic approach and treatment to restore the renal function. The most commonly observed obstructive uropathy in children is congenital malformation, such as posterior urethral valves and bilateral ureteropelvic junction obstruction. Malignant pelvic masses obstructing the ureter are widely reported in adults but are rarely observed in children. The treatment of ureteral obstruction related to pelvic malignancy is a therapeutic challenge with a median survival duration of 3–7 months in adults; however, pediatric patients with pelvic malignancy leading to ureteral obstruction had better outcomes, with a reported 5-year mortality rate of 20%, than the adult patients. Here, we report a rare case of bilateral ureteral obstruction associated with pelvic rhabdomyosarcoma presenting with acute kidney injury treated by ureteral diversion with double J stent, and concommittent emergency hemodialysis, leading to restoration of good renal function. We suggest that bilateral ureteral obstruction should be released as soon as possible using surgical or interventional approach to minimize the obstruction period, and subsequential chemotherapy may contribute to improvement of survival and recovery of renal function.

De novo mutations in COL4A5 identified by whole exome sequencing in 2 girls with Alport syndrome in Korea / 소아과

Alport syndrome (ATS) is an inherited glomerular disease caused by mutations in one of the type IV collagen novel chains (α3, α4, and α5). ATS is characterized by persistent microscopic hematuria that starts during infancy, eventually leading to either progressive nephritis or end-stage renal disease. There are 3 known genetic forms of ATS, namely X-linked ATS, autosomal recessive ATS, and autosomal dominant ATS. About 80% of patients with ATS have X-linked ATS, which is caused by mutations in the type IV collagen α5 chain gene, COL4A5. Although an 80% mutation detection rate is observed in men with X-linked ATS, some difficulties do exist in the genetic diagnosis of ATS. Most mutations are point mutations without hotspots in the COL4A3, COL4A4, and COL4A5 genes. Further, there are insufficient data on the detection of COL4A3 and COL4A4 mutations for their comparison between patients with autosomal recessive or dominant ATS. Therefore, diagnosis of ATS in female patients with no apparent family history can be challenging. Therefore, in this study, we used whole-exome sequencing (WES) to identify mutations in type IV collagen in 2 girls with glomerular basement membrane structural changes suspected to be associated with ATS; these patients had no relevant family history. Our results revealed de novo c.4688G>A (p.Arg1563Gln) and c.2714G>A (p.Gly905Asp) mutations in COL4A5. Therefore, we suggest that WES is an effective approach to obtain genetic information in ATS, particularly in female patients without a relevant family history, to detect unexpected DNA variations.

Is the Diagnostic Trial with Proton Pump Inhibitors Reasonable for School Age Children with Gastroesophageal Reflux Symptoms? / 대한소아소화기영양학회지

PURPOSE: On the basis of evidence, we aimed to reevaluate the necessity of the empirical proton pump inhibitor (PPI) trial for children with suspected gastroesophageal reflux disease (GERD). METHODS: We analyzed the frequency of GERD in 85 school-age children with gastroesophageal reflux (GER) symptoms, who received 24-hour esophageal pH monitoring and/or upper endoscopy. According to the reflux index (RI), the children were classified into normal (RI <5%), intermediate (5%≤ RI <10%), or abnormal (RI ≥10%) groups. RESULTS: Fifty six were female and 29 were male. Their mean age was 12.6±0.5 (±standard deviation) years (range: 6.8–18.6). The RI analysis showed that the normal group included 76 patients (89.4%), the intermediate group included 6 patients (7.1%), and the abnormal group included 3 patients (3.5%). The DeMeester score was 5.93±4.65, 14.68±7.86 and 40.37±12.96 for the normal, intermediate and abnormal group, respectively (p=0.001). The longest reflux time was 5.56±6.00 minutes, 9.53±7.84 minutes, and 19.46±8.35 minutes in the normal, intermediate, and abnormal group, respectively (p=0.031). Endoscopic findings showed reflux esophagitis in 7 patients. On the basis of the Los Angeles Classification of Esophagitis, 5 of these patients were included in group A, 1 patient, in group B and 1 patient, in group C. CONCLUSION: The incidence of GERD was very low in school-age children with GER symptoms. Therefore, injudicious diagnostic PPI trials would be postponed until the actual prevalence of GERD is verified in future prospective studies.

Scheduled injection of ramosetron for prevention of nausea and vomiting following single-port access total laparoscopic hysterectomy: a prospective randomized study

OBJECTIVE: The purpose of this study was to evaluate the effectiveness of scheduled ramosetron injections for controlling postoperative nausea and vomiting (PONV) after single-port access total laparoscopic hysterectomy (SPA-TLH). METHODS: Ninety patients who underwent SPA-TLH at the Korean National Health Insurance Service Ilsan Hospital between June 2013 and July 2014 were enrolled in this prospective, randomized, double-blinded, placebo-controlled study. The patients were divided into 2 groups as follows: the ramosetron group (0.3 mg intravenously [IV]; n=45) and the placebo group (normal saline IV; n=45). Both groups received their respective injections 12 and 24 hours post surgery. The incidence and severity of PONV (numerical rating scale, 0–10), and the use of rescue antiemetics post surgery were evaluated. RESULTS: Demographic and perioperative statistically significant differences were not observed between the 2 groups. The incidence of PONV in the ramosetron and placebo groups was 46.7% and 51.1%, respectively (P=0.51). We found significant differences in the severity of PONV between the 24- to 48-hour postoperative periods in both groups (ramosetron group, P=0.04 and placebo group, P=0.03). The use of rescue antiemetics was significantly lower in the ramosetron group than in the placebo group (P=0.02). CONCLUSION: After general anesthesia, scheduled injections of ramosetron 12 and 24 hours after SPA-TLH reduced the severity of PONV and the use of rescue antiemetics. Administration of ramosetron can be considered not only immediately after SPA-TLH but also during the first 24-hour recovery period. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT 02011659

Prevalence of Operation Room-Related Syncope and Presyncope among Medical Students / 의학교육논단

Attending the operation room is an essential part of surgical clerkships. Syncope or presyncopal attacks in the operation room may negatively affect students' learning and career development. This study set out to identify the prevalence of syncope and presyncopal attacks in the operation room during medical students' surgical clerkships. Data from 420 medical students (303 men and 117 women) in their 3rd year of clerkship were collected between 2014 and 2017. An anonymous questionnaire was distributed to assess the prevalence and degree of syncope and presyncopal symptoms. A total of 27% of the respondents had experienced syncope or presyncopal symptoms, 49.6% of the female students and 18.8% of the male students (p < 0.001). Fifty students (43.5%) had been attending as observers at the time of the syncopal attack, while 65 students (56.5%) had been participating as assistants. Thirty-four students (29.6%) had recently eaten at the time of the syncopal attack, while 81 students (70.4%) had not recently eaten. Prodromal symptoms included the urge to sit down (21.2%), sweating (19.3%), nausea (16.9%), a feeling of warmth (13.3%), darkened vision (12.6%), yawning (11.7%), palpitation (11.0%), ear fullness (10.2%), black spots in one's vision (7.6%), and hyperventilation (7.1%). This study showed the prevalence of syncope and presyncopal symptoms in the operation room during surgical clerkships. For students' safety and effective clerkship learning, thorough proactive education on syncopal attacks is required.

Concurrence of Diabetic Ketoacidosis and Acute Ischemic Stroke due to Moyamoya Disease / 대한소아신경학회지

Although the etiology of moyamoya disease (MMD) remains unknown, autoimmunity is one of the proposed pathogeneses. Unlike other autoimmune disorders that are associated with cerebral arteritis, concurrence of MMD and diabetes mellitus (DM) is rare. However, we encountered a patient with concurrent diabetic ketoacidosis (DKA) and acute ischemic stroke due to MMD. Our patient was diagnosed with glutamic acid decarboxylase antibody-positive type 2 DM (T2DM) based on laboratory and physical examination findings. Brain magnetic resonance images revealed an acute ischemic stroke in the left cerebral hemisphere and bilateral diffuse stenosis/occlusion in the middle cerebral artery and multiple collaterals. Thus, here, we report a patient with both T2DM and MMD who developed an acute ischemic stroke that was complicated by DKA.

The Influence of Urinary Catheter Materials on Forming Biofilms of Microorganisms

Biofilms are commonly associated with an increased risk of catheter-associated infection. To study the efficacy of materials designed to reduce biofilm formation, microbial biofilms on clinically used urinary catheter were examined. We performed 2, 3-bis (2-methyoxy-4-nitro-5-sulfo-phenyl)-2H-tetrazolium-5-carboxanilide (XTT) reduction assay to determine of biofilm formation ability and observed with scanning electron microscopy (SEM) to analyze biofilm architecture. Additionally, we calculated relative cell surface hydrophobicity (CSH) to measure hydrophobicity of microorganisms. On SEM, catheter surfaces made of latex or anti-infective (IC)-latex were rough but those of silicone, hydrogel-coated silicone (HCS), or silver-alloy-coated silicone (SCS) were relatively smoother. According to XTT reduction assay, biofilm formation was reduced on the surface of smooth silicone-based catheters compared to rough latex-based catheters. The greatest to lowest formation of microbial biofilm were as follows for these material types: silicone-elastomer-coated (SEC) latex > latex > silicone > IC-latex > HCS > SCS. Catheter materials can affect the microbial biofilm formations. First, rougher surfaces on the catheter made the microbial attachment easier and a greater amount of biofilm was formed. Second, when chemicals that inhibit growth and attachment of microorganisms on the inner and outer surfaces of the catheters were applied, the biofilm formation was inhibited. SCS was found to be the most effective in reducing the microbial biofilm formation. These results indicate that microbial biofilm formation may be closely related to the surface roughness and microbial CSH.

Kawasaki Disease Shock Syndrome with Acute Kidney Injury and Hypertension

Kawasaki disease (KD) is an acute febrile mucocutaneous lymph node syndrome that commonly presents with stable hemodynamic status during the acute phase. An 8-year-old boy initially presented with severe hypotension and acute kidney injury. He was placed in the intensive care unit and was diagnosed with KD. Observed clinical features were defined as KD shock syndrome. His coronary artery was dilated during the subacute phase. Furthermore, he was given anti-hypertensive medications, owing to hypertension as an unusual complication of KD. We knew the importance of monitoring for blood pressure considering vasculitis as an aspect of the main pathogenesis of KD.

Peripheral T-cell Lymphoma Presenting with Chylothorax / 대한내과학회지

A 72-year-old male presented with respiratory discomfort. A simple chest X-ray and abdominal computed tomography showed pleural effusion and multiple lymph node enlargement. The pleural effusion was determined by thoracentesis to be chylothorax. An inguinal lymph node biopsy showed peripheral T-cell lymphoma. Following three cycles of cyclophospamide, hydroxyl doxorubicin, vincristine, prednisolone (CHOP) chemotherapy, a partial response was observed. Chylothorax is an extremely rare complication of T-cell lymphoma. We present a case of peripheral T-cell lymphoma presenting with chylothorax. We suggest that clinicians should consider chylothorax when examining patients with lymphoma who present with atypical pleural effusion.

A nonsense PAX6 mutation in a family with congenital aniridia / 소아과

Congenital aniridia is a rare ocular malformation that presents with severe hypoplasia of the iris and various ocular manifestations. Most cases of congenital aniridia are known to be related to mutations in the paired box gene-6 (PAX6), which is an essential gene in eye development. Herein, we report a familial case of autosomal dominant congenital aniridia with four affected members in 3 consecutive generations and describe the detailed ophthalmologic findings for one of these members. As expected, mutational analysis revealed a nonsense mutation (p.Ser122*) in the PAX6 gene. Thus, our findings reiterate the importance of PAX6 mutations in congenital aniridia.

Sonographic Growth Charts for Kidney Length in Normal Korean Children: a Prospective Observational Study

Kidney length is the most useful parameter for clinical measurement of kidney size, and is useful to distinguish acute kidney injury from chronic kidney disease. In this prospective observational study of 437 normal children aged between 0 and < 13 years, kidney length was measured using sonography. There were good correlations between kidney length and somatic values, including age, weight, height, and body surface area. The rapid growth of height during the first 2 years of life was intimately associated with a similar increase in kidney length, suggesting that height should be considered an important factor correlating with kidney length. Based on our findings, the following regression equation for the reference values of bilateral kidney length for Korean children was obtained: kidney length of the right kidney (cm) = 0.051 × height (cm) + 2.102; kidney length of the left kidney (cm) = 0.051 × height (cm) + 2.280. This equation may aid in the diagnosis of various kidney disorders.

Bilateral iliac and popliteal arterial thrombosis in a child with focal segmental glomerulosclerosis / 소아과

Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%-5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and femoral arteries, and is associated with the use of steroids and diuretics. Popliteal artery thrombosis in children has been described in cases of traumatic dissection, osteochondroma, Mycoplasma pneumoniae infection, and fibromuscular dysplasia. We report of a 33-month-old girl with bilateral iliac and popliteal arterial thrombosis associated with steroid-resistant NS due to focal segmental glomerulosclerosis. Her treatment involved thrombectomy and intravenous heparinization, followed by oral warfarin for 8 months. Herein, we report a rare case of spontaneous iliac and popliteal arterial thrombosis in a young child with NS.

Pathogenesis of minimal change nephrotic syndrome: an immunological concept / 소아과

Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia. Minimal change nephrotic syndrome (MCNS) is the most common form of INS in children. The pathogenesis of MCNS still remains unclear, however, several hypotheses have been recently proposed. For several decades, MCNS has been considered a T-cell disorder, which causes the impairment of the glomerular filtration barrier with the release of different circulating factors. Increased levels of several cytokines are also suggested. Recently, a "two-hit" theory was proposed that included the induction of CD80 (B7-1) and regulatory T-cell (Treg) dysfunction, with or without impaired autoregulatory functions of the podocyte. In contrast to the well-established involvement of T cells, the role of B cells has not been clearly identified. However, B-cell biology has recently gained more attention, because rituximab (a monoclonal antibody directed against CD20-bearing cells) demonstrated a very good therapeutic response in the treatment of childhood and adult MCNS. Here, we discuss recent insights into the pathogenesis of MCNS in children.

Incomplete Kawasaki Disease in a 5-Month-Old Girl Associated with Cerebrospinal Fluid Pleocytosis and Epidural Fluid Collection

Cases of incomplete Kawasaki disease (KD), wherein the patient does not fulfill the full diagnostic criteria for KD, are often detected in infants younger than 6 months of age. The clinical manifestations in infants with incomplete KD may resemble other infectious diseases, including meningitis. For this reason, clinicians may have difficulty differentiating incomplete KD from other infectious diseases in this population. Various neurological features are associated with KD, including aseptic meningitis, subdural effusion, facial nerve palsy, cerebral infarction, encephalopathy, and reversible corpus callosum splenial lesions on magnetic resonance imaging. We report a case of a 5-month-old girl with incomplete KD, associated with cerebrospinal fluid pleocytosis and an epidural fluid collection. Echocardiography indicated dilatation of the main coronary arteries. The girl made a complete recovery, with resolution of both the epidural fluid collection and coronary artery aneurysms. In this case, the child is well, and showed normal developmental milestones at the 7-month follow-up.