Purpose: Angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers (ARBs) are frequently employed to counteract the detrimental effects of proteinuria on glomerular diseases. However, the effects of ARBs remain poorly examined in pediatric patients with immunoglobulin A (IgA) nephropathy. Herein, we evaluated the efficacy and safety of losartan, an ARB, in pediatric IgA nephropathy with proteinuria. Methods: This prospective, single-arm, multicenter study included children with IgA nephropathy exhibiting proteinuria. Changes in proteinuria, blood pressure, and kidney function were prospectively evaluated before and 4 and 24 weeks after losartan administration. The primary endpoint was the difference in proteinuria between baseline and 24 weeks. Results: In total, 29 patients were enrolled and received losartan treatment. The full analysis set included 28 patients who received losartan at least once and had pre- and post-urinary protein to creatinine ratio measurements (n=28). The per-protocol analysis group included 22 patients who completed all scheduled visits without any serious violations during the study period. In both groups, the mean log (urine protein to creatinine ratio) value decreased significantly at 6 months. After 24 weeks, the urinary protein to creatinine ratio decreased by more than 50% in approximately 40% of the patients. The glomerular filtration rate was not significantly altered during the observation period. Conclusions Losartan decreased proteinuria without decreasing kidney function in patients with IgA nephropathy over 24 weeks. Losartan could be safely employed to reduce proteinuria in this patient population. ClinicalTrials.gov trial registration (NCT0223277)

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.

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.

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.

The authors regret that there was an important error in the results in Table 1; the ATP7A mutations detected in Patients 2 and 14 were incorrectly noted.

BACKGROUND: Menkes disease (MD) is a rare X-linked hereditary multisystemic disorder that is caused by dysfunction of copper metabolism. Patients with MD typically present with progressive neurodegeneration, some connective tissue abnormalities, and characteristic “kinky” hair. In addition, various types of urological complications are frequent in MD because of underlying connective tissue abnormalities. In this study, we studied the clinical features and outcomes of MD, focusing on urological complications. METHODS: A total of 14 unrelated Korean pediatric patients (13 boys and 1 girl) with MD were recruited, and their phenotypes and genotypes were analyzed by retrospective review of their medical records. RESULTS: All the patients had early-onset neurological deficit, including developmental delay, seizures, and hypotonia. The girl patient showed normal serum copper and ceruloplasmin levels as well as milder symptoms. Mutational analysis of the ATP7A gene revealed 11 different mutations in 12 patients. Bladder diverticula was the most frequent urological complication: 8 (57.1%) in the 14 patients or 8 (72.7%) in the 11 patients who underwent urological evaluation. Urological imaging studies were performed essentially for the evaluation of accompanying urinary tract infections. Four patients had stage II chronic kidney disease at the last follow-up. CONCLUSION: Urologic problems occurred frequently in MD, with bladder diverticula being the most common. Therefore, urological imaging studies and appropriate management of urological complications, which may prevent or reduce the development of urinary tract infections and renal parenchymal damage, are required in all patients with MD.
Ceruloplasmin ; Connective Tissue ; Copper ; Diverticulum ; Female ; Follow-Up Studies ; Genotype ; Hair ; Humans ; Medical Records ; Menkes Kinky Hair Syndrome* ; Metabolism ; Muscle Hypotonia ; Phenotype ; Renal Insufficiency, Chronic ; Retrospective Studies ; Seizures ; Urinary Bladder ; Urinary Tract Infections

The aim of this study was to investigate the clinical characteristics of acute hepatitis A during a recent outbreak in Korea. Data of patients diagnosed with acute hepatitis A from 2007 to 2009 were collected from 21 tertiary hospitals retrospectively. Their demographic, clinical, and serological characteristics and their clinical outcomes were analyzed. A total of 4,218 patients (mean age 33.3 yr) were included. The median duration of admission was 9 days. The mean of the highest ALT level was 2,963 IU/L, total bilirubin was 7.3 mg/dL, prothrombin time INR was 1.3. HBsAg was positive in 3.7%, and anti-HCV positive in 0.7%. Renal insufficiency occurred in 2.7%, hepatic failure in 0.9%, relapsing hepatitis in 0.7%, and cholestatic hepatitis in 1.9% of the patients. Nineteen patients (0.45%) died or were transplanted. Complications of renal failure or prolonged cholestasis were more frequent in patients older than 30 yr. In conclusion, most patients with acute hepatitis A recover uneventfully, however, complication rates are higher in patients older than 30 yr than younger patients. Preventive strategies including universal vaccination in infants and active immunization of hepatitis A to adult population should be considered for prevention of community-wide outbreaks of hepatitis A in Korea.
Acute Disease ; Adolescent ; Adult ; Age Factors ; Aged ; Child ; Child, Preschool ; Cholestasis/epidemiology/etiology ; Demography ; Hepatitis A/complications/*diagnosis/mortality ; Humans ; Kidney Failure, Chronic/epidemiology/etiology ; Liver Transplantation ; Middle Aged ; Morbidity ; Republic of Korea ; Retrospective Studies ; Tertiary Care Centers ; Young Adult

STATEMENT OF PROBLEM: Crestal bone loss, a common problem associated with dental implant, has been attributed to excessive bone stresses. Design of implant's transgingival (TG) part may affect the crestal bone stresses. PURPOSE: To investigate if concavely designed geometry at a dental implant's TG part reduces peri-implant bone stresses. MATERIAL AND METHODS: A total of five differently configured TG parts were compared. Base model was the ITI one piece implant (Straumann, Waldenburg, Switzerland) characterized by straight TG part. Other 4 experimental models, i.e. Model-1 to Model-4, were designed to have concave TG part. Finite element analyses were carried out using an axisymmetric assumption. A vertical load of 50 N or an oblique load of 50 N acting at 30degrees with the implant's long axis was applied. For a systematic stress comparison, a total of 19 reference points were defined on nodal points around the implant. The peak crestal bone stress acting at the intersection of implant and crestal bone was estimated using regression analysis from the stress results obtained at 5 reference points defined along the mid plane of the crestal bone. RESULTS: Base Model with straight configuration at the transgingival part created highest stresses on the crestal bone. Stress level was reduced when concavity was imposed. The greater the concavity and the closer the concavity to the crestal bone level, the less the crestal stresses. CONCLUSION: The transgingival part of dental implant affect the crestal bone stress. And that concavely designed one may be used to reduce bone stress.
Axis, Cervical Vertebra ; Dental Implants ; Finite Element Analysis ; Models, Theoretical

BACKGROUND: Acid-base imbalances are common in critically ill patients; however, the incidence of acid-base imbalances in the medical intensive care units has not been fully determined. In this study, we investigated the incidence and the type of acid-base imbalances in critically ill patients and we assessed which variables were associated with the patients' outcome. METHODS: One hundred eighty-seven patients (122 men, age: 61.2+/-12.8 years) were enrolled. All the patients were admitted to the medical intensive care unit between January 2005 and December 2005. All the data sets included simultaneous measurements of an arterial blood gas with base excess, the serum electrolytes, the anion gap and the APACHE II scores. RESULTS: The mortality rate was 56.7%. The incidence of acid-base imbalances was as follows: 25.1% were single disorders that existed with only a single primary acid-base imbalance, 48.7% were double disorders, 13.4% were triple disorders and 12.8% were normal (no disorders). The incidence of metabolic acidosis was 57.8% and the mortality rate was not different according to the type of acid-base imbalances. There were significant differences between the nonsurvivors and survivors according to the pH (7.34 vs. 7.41, respectively), HCO(-)3 (20.68 mmol/L vs. 25.90 mmol/L, respectively), ECF base excess (-5.19 vs. 1.19, respectively), the anion gap (18.57 mmol/L vs. 13.77 mmol/L, respectively), the corrected anion gap (23.63 mmol/L vs. 17.96 mmol/L, respectively), the serum albumin (2.37 g/dL vs. 2.74 g/dL, respectively), and the APACHE II scores (20.7 vs 17.2, respectively). However, on the Cox proportional hazard regression analysis, only the APAHCE II scores affected the patients' outcome. CONCLUSIONS: There were diverse acid-base imbalances in the critically ill patients and the incidence of metabolic acidosis was highest among the acid-base imbalances. The best predictor of the patients' outcome was the APACHE II scores.
Acid-Base Equilibrium ; Acid-Base Imbalance ; Acidosis ; APACHE ; Critical Illness* ; Dataset ; Electrolytes ; Humans ; Hydrogen-Ion Concentration ; Incidence ; Intensive Care Units* ; Critical Care ; Male ; Mortality ; Serum Albumin ; Survivors

A patient, with severe chest pain and isolated ST segment elevation, was evaluated in order to differentiate ischemic heart disease, such as acute myocardial infarction. A fishhook was incidentally found during coronary angiography, chest CT and esophagoscopy. With this information, the patient was re-interviewed, and it was learned that the patient had swallowed the fishhook from cooked fish after fishing trip. The patient was diagnosed as traumatic acute pericarditis with acute mediastinitis, associated with esophageal and pericardial perforation. The patient was successfully treated by surgical treatment drainage and adequate antibiotic use. We report a case of traumatic acute pericarditis, which mimicked an acute myocardial infarction, with a review of the literature.
Chest Pain ; Coronary Angiography ; Deglutition* ; Drainage ; Esophagoscopy ; Humans ; Mediastinitis ; Myocardial Infarction* ; Myocardial Ischemia ; Pericarditis* ; Tomography, X-Ray Computed