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)

An ectopic pancreas rarely transforms into a malignancy, and the symptoms vary from patient to patient. The most commonly observed site of an ectopic pancreas is the antrum of the stomach. A 59-year-old male patient with severe abdominal pain underwent CT. A 9.6 cm-sized well-defined exophytic huge mass with heterogenic density was located between the stomach distal antrum and duodenum. A malignant submucosal tumor was suspected because of the exophytic dirty huge mass. Initially, surgery was considered to confirm the histological evaluation. After 2 months, the abdominal pain disappeared, and the follow-up MRI scan showed a decrease in size, which contained a necrotic component inside. It was confirmed that the parenchymal tissue was the pancreas. The pathology through EUS-guided fine needle aspiration (EUS-FNA) was normal pancreatic acinar cells, smooth muscle fragments, squamous cyst, and some neutrophils (abscess). Walled-off necrosis occurs as a complication of acute pancreatitis with parenchymal tissues and surrounding tissues, but complications of ectopic pancreatitis occurred in this case. Abdominal pain due to ectopic pancreas leading to the formation of a giant abscess has been reported as a very rare case. Diagnosis through biopsy is most important when a malignant submucosal tumor is suspected. In addition, it is important to determine the clinical features, examination findings, such as EUS, CT, and MRI, and the changes according to the follow-up period. This paper reports a case of ectopic pancreas, resulting in necrotic tissue and walled-off necrosis, abdominal pain, and spontaneous improvement.

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.

Objective: This study aimed to clarify the relative prognostic value of each History, Electrocardiography, Age, Risk Factors, and Troponin (HEART) score component for major adverse cardiac events (MACE) within 3 months and validate the modified HEART (mHEART) score. Methods: This study evaluated the HEART score components for patients with chest symptoms visiting the emergency department from November 19, 2018 to November 19, 2019. All components were evaluated using logistic regression analysis and the scores for HEART, mHEART, and Thrombolysis in Myocardial Infarction (TIMI) were determined using the receiver operating characteristics curve. Results: The patients were divided into a derivation (809 patients) and a validation group (298 patients). In multivariate analysis, age did not show statistical significance in the detection of MACE within 3 months and the mHEART score was calculated after omitting the age component. The areas under the receiver operating characteristics curves for HEART, mHEART and TIMI scores in the prediction of MACE within 3 months were 0.88, 0.91, and 0.83, respectively, in the derivation group; and 0.88, 0.91, and 0.81, respectively, in the validation group. When the cutoff value for each scoring system was determined for the maintenance of a negative predictive value for a MACE rate >99%, the mHEART score showed the highest sensitivity, specificity, positive predictive value, and negative predictive value (97.4%, 54.2%, 23.7%, and 99.3%, respectively). Conclusion Our study showed that the mHEART score better detects short-term MACE in high-risk patients and ensures the safe disposition of low-risk patients than the HEART and TIMI scores.

Background/Aims: Biliary strictures remain one of the most challenging aspects after living donor liver transplantation (LDLT). The aim of this study was to assess long-term outcome of endoscopic treatment of biliary strictures occurring after LDLT and to identify risk factors of recurrent biliary strictures following endoscopic retrograde biliary drainage (ERBD) in LDLT. Methods: A total of 1,106 patients underwent LDLT from May 1995 to May 2014. We compared the risk factors between patients with and without recurrent biliary strictures. Results: Biliary strictures developed in 24.0% of patients. Technical success rate of ERBD for biliary stricture after LDLT was 66.2% (145/219). Among 145 patients managed by endoscopic drainage, stricture resolution occurred in 69 with median duration of stent indwelling of 13.6 months (range, 0.5 to 67.3 months), and stricture recurrence was seen in 20 (21.3%) out of 94. The median recurrence-free duration after final endoscopic success was 13.1 months (range, 0.5 to 67.3 months). Older donor age (hazard ratio [HR], 1.10; 95% confidence interval [CI], 1.03 to 1.17; p=0.004) and non-B, non-C liver cirrhosis (HR, 5.10; 95% CI, 1.10 to 25.00; p=0.043) were associated with higher recurrence of biliary stricture. Conclusions Long-term stricture resolution rate after ERBD insertion for biliary stricture occurring after LDLT was 73.4%. Clinicians should pay careful attention during following-up to decide when to remove ERBD in patients who have factors associated with recurrent biliary strictures.

PURPOSE: This study was designed to assess the outcome of the extracorporeal membrane oxygenation (ECMO) in liver transplantation (LT) recipients with refractory septic shock and predict the prognosis of those cases. METHODS: From February 2005 to October 2012, ECMO was used in 8 cases of refractory septic shock. Laboratory values including lactate and total bilirubin level just before starting ECMO were obtained and sepsis-related organ failure assessment (SOFA) score, acute physiology and chronic health evaluation (APACH) II score and simplified acute physiology score (SAPS) 3 were calculated. Subsequent peak serum lactate and total bilirubin level, and SOFA score after 24 hours of starting ECMO were measured. RESULTS: Comparisons were made between survivors and nonsurvivors. ECMO was weaned off successfully in 3 patients (37.5%) and 2 patients (25%) survived to hospital discharge. Clinical scores including SOFA, APACH II, and SAPS3 and laboratory results including lactate, total bilirubin and CRP were not significantly different between survivor and nonsurvivor groups. Lactate level and SOFA score tended to decrease after ECMO support in survivor group and total bilirubin and CRP level tended to increase in nonsurvivor group. CONCLUSION: Our findings suggest that the implantation of ECMO might be considered in highly selected LT recipients with refractory septic shock.
APACHE ; Bilirubin ; Extracorporeal Membrane Oxygenation* ; Humans ; Lactic Acid ; Liver Transplantation* ; Liver* ; Physiology ; Prognosis ; Shock, Septic* ; Survivors

BACKGROUND: From May to July 2015, the Republic of Korea experienced the largest outbreak of Middle East respiratory syndrome (MERS) outside the Arabian Peninsula. A total of 186 patients, including 36 deaths, had been diagnosed with MERS-coronavirus (MERS-CoV) infection as of September 30th, 2015. MATERIALS AND METHODS: We obtained information of patients who were confirmed to have MERS-CoV infection. MERS-CoV infection was diagnosed using real-time reverse-transcriptase polymerase chain reaction assay. RESULTS: The median age of the patients was 55 years (range, 16 to 86). A total of 55.4% of the patients had one or more coexisting medical conditions. The most common symptom was fever (95.2%). At admission, leukopenia (42.6%), thrombocytopenia (46.6%), and elevation of aspartate aminotransferase (42.7%) were observed. Pneumonia was detected in 68.3% of patients at admission and developed in 80.8% during the disease course. Antiviral agents were used for 74.7% of patients. Mechanical ventilation, extracorporeal membrane oxygenation, and convalescent serum were employed for 24.5%, 7.1%, and 3.8% of patients, respectively. Older age, presence of coexisting medical conditions including diabetes or chronic lung disease, presence of dyspnea, hypotension, and leukocytosis at admission, and the use of mechanical ventilation were revealed to be independent predictors of death. CONCLUSION: The clinical features of MERS-CoV infection in the Republic of Korea were similar to those of previous outbreaks in the Middle East. However, the overall mortality rate (20.4%) was lower than that in previous reports. Enhanced surveillance and active management of patients during the outbreak may have resulted in improved outcomes.
Antiviral Agents ; Aspartate Aminotransferases ; Coronavirus Infections* ; Disease Outbreaks ; Dyspnea ; Extracorporeal Membrane Oxygenation ; Fever ; Humans ; Hypotension ; Leukocytosis ; Leukopenia ; Lung Diseases ; Middle East Respiratory Syndrome Coronavirus ; Middle East* ; Mortality ; Pneumonia ; Polymerase Chain Reaction ; Republic of Korea* ; Respiration, Artificial ; Thrombocytopenia

Esophageal and gastric varix, portal hypertensive gastropathy, Mallory-Weiss tear and gastric ulcer are common causes of bleeding in patients with liver cirrhosis. However, spontaneous arterial bleeding without a history of trauma is a rare cause of bleeding which can be fatal. We report a case of a 55-year-old woman with alcoholic liver cirrhosis who developed spontaneous bleeding of multiple right lumbar arteries and died in spite of repetitive transfusion and embolization.
Arteries ; Female ; Gastrointestinal Hemorrhage/*etiology/therapy ; Hematoma/diagnosis ; Humans ; Liver Cirrhosis/complications/*diagnosis ; Lung Injury/pathology ; Middle Aged ; Tomography, X-Ray Computed

Microcystic adnexal carcinoma is a rare type of tumor, with about 300 cases reported globally. Due to its similar histology with other tumors, it is occasionally misdiagnosed as desmoplastic trichoepithelioma, basal cell carcinoma, syringoma, and so on. We present a patient with a mass on the perioral area who was preoperatively diagnosed with trichoepithelioma. Microcystic adnexal carcinoma was diagnosed after excisional biopsy and a wide excision. Defects were reconstructed with a mucosal advancement flap. There was no recurrence and there were no significant complications during the 18-month follow-up period. Because superficial punch biopsy has limitations in width and depth, surgeons should always consider the possibility of malignancy of a mass even if a biopsy shows a benign result.
Biopsy* ; Carcinoma, Basal Cell ; Diagnostic Errors ; Follow-Up Studies ; Humans ; Recurrence ; Skin Neoplasms ; Syringoma