A case of Herlyn-Werner-Wunderlich syndrome: a rare, congenital genitourinary anomaly in a 12-year-old girl

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare, congenital genitourinary anomaly involving the Müllerian and Wolffian structures, and is characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in adolescent girls in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Here, we report a case of a 12-year-old girl who presented to the emergency department with lower abdominal pain and mass that had lasted for 2 weeks. After the confirmation of HWW syndrome with magnetic resonance imaging, hysteroscopic septostomy was carried out as a definitive treatment. When we evaluate adolescent girls with lower abdominal pain and mass, we should consider the possibility of HWW syndrome

Ultrasound-guided central venous catheter insertion in pediatric patients

In pediatric patients, central venous catheterization (CVC) is necessary for administration of fluids, drugs, high concentration electrolytes, vasopressors or inotropic drugs, transfusion, intravenous nutrition, and dialysis. Using an anatomical landmark for the CVC insertion may have a low success rate in children due to the positional variation between the deep vein and the landmarks, the small size of body and blood vessels, low insertion frequency, and operator skill. In order to improve the success rate, ultrasound guided CVC insertion is recommended in critically ill children. It is also expected to reduce mechanical complications, which are more common with subclavian CVC insertion. However, the association between the insertion site and the infection or thrombosis is unclear. Since thrombosis is relatively common, further studies are needed on the association between the incidence rate and insertion sites

Clinical outcomes of spontaneous bacterial peritonitis due to extended-spectrum beta-lactamase-producing Escherichia coli and Klebsiella species: A retrospective matched case-control study

<p>Abstract</p> <p>Background</p> <p>Clinical outcomes of spontaneous bacterial peritonitis (SBP) due to extended-spectrum β-lactamase-producing <it>Escherichia coli </it>and <it>Klebsiella </it>species (ESBL-EK) have not been adequately investigated.</p> <p>Methods</p> <p>We conducted a retrospective matched case-control study to evaluate the outcomes of SBP due to ESBL-EK compared with those due to non-ESBL-EK. Cases were defined as patients with liver cirrhosis and SBP due to ESBL-EK isolated from ascites. Control patients with liver cirrhosis and SBP due to non-ESBL-EK were matched in a 3:1 ratio to cases according to the following five variables: age (± 5 years); gender; species of infecting organism; Child-Pugh score (± 2); Acute Physiological and Chronic Health Evaluation II score (± 2). 'Effective initial therapy' was defined as less than 72 hours elapsing between the time of obtaining a sample for culture and the start of treatment with an antimicrobial agent to which the EK was susceptible. Cephalosporin use for ESBL-EK was considered 'ineffective', irrespective of the minimum inhibitory concentration. ESBL production was determined according to the Clinical and Laboratory Standards Institute guidelines on stored isolates.</p> <p>Results</p> <p>Of 1026 episodes of SBP in 958 patients from Jan 2000 through Dec 2006, 368 (35.9%) episodes in 346 patients were caused by SBP due to EK, isolated from ascites. Of these 346 patients, twenty-six (7.5%) patients with SBP due to ESBL-EK were compared with 78 matched controls. Treatment failure, evaluated at 72 hours after initial antimicrobial therapy, was greater among the cases (15/26, 58% <it>vs</it>. 10/78, 13%, <it>P </it>= .006); 30-day mortality rate was also higher than in the controls (12/26, 46% <it>vs</it>. 11/78, 15%, <it>P </it>= .001). When the case were classified according to the effectiveness of the initial therapy, 'ineffective initial therapy' was associated with higher 30-day mortality rate (11/18, 61% <it>vs</it>. 1/8, 13%, <it>P </it>= .036).</p> <p>Conclusion</p> <p>SBP due to ESBL-EK had poorer outcomes than SBP due to non-ESBL-EK. Ineffective initial therapy seems to be responsible for the higher rate of treatment failure and mortality in SBP due to ESBL-EK.</p

Performances of serum creatinine, C-reactive protein and white blood cell to predict urinary tract infection in febrile children younger than 24 months of age

Purpose Differentiation of urinary tract infection (UTI) from viral infection is a critical challenge in febrile children in emergency departments (EDs). This study aimed to assess the predicting performances of creatinine, C-reactive protein (CRP), and white blood cell (WBC) for predicting UTI in the children. Methods This study was a retrospective analysis of a prospectively enrolled cohort of febrile children who presented to our children’s hospital ED from August 2016 through February 2018. We included previously healthy, febrile (≥ 38。C) children younger than 24 months whose urine cultures were obtained. Accuracy of creatinine, CRP, and WBC were assessed by optimal cutoffs, which were calculated using receiver operating characteristic curves. Results Among the total 33,013 children to the ED, 7,847 (23.8%) febrile children were registered to the fever registry. Finally, 506 children were included, and UTI was diagnosed in 127 (25.1%). The areas under the curve of creatinine, CRP, and WBC to predict UTI were 0.41 (95% confidence interval [CI], 0.35-0.46), 0.71 (95% CI, 0.66-0.77), and 0.66 (95% CI, 0.60-0.72), respectively. The cutoffs were 0.26 mg/dL for creatinine, 2.3 mg/dL for CRP, and 14.4 × 103 cells/μL for WBC. Creatinine showed worse performance than the other variables. The application of creatinine added to the other variables led to an increase only in the sensitivity, but at the expense of a lower specificity, positive predictive value, and negative predictive value. Conclusion Serum creatinine showed a poor performance in predicting UTI in the febrile young children. Since a single biomarker can neither rule in nor rule out UTI in the children, the prediction of UTI can be achieved by the interpretation of both clinical and laboratory findings

Estimating contact-adjusted immunity levels against measles in South Korea and prospects for maintaining elimination status.

Measles has been reemerging in South Korea since December 2018 resulting in 185 cases by September 2019. We calculated contact-adjusted immunity levels against measles in South Korea using national seroprevalence data in 2014, vaccination uptake rates, and an age-specific contact matrix. We further explored options to achieve a contact-adjusted immunity level of 93% for herd immunity. The assessed contact-adjusted immunity level has increased from 86% in 2014 to 92% in 2018. Herd immunity could be achieved with immunizing 50% of susceptibles among birth cohorts 1999-2003 in 2018. Contact-adjusted immunity levels against measles have increased recently in South Korea, although they might not yet be high enough to guarantee herd immunity

The corrected QT (QTc) prolongation in hyperthyroidism and the association of thyroid hormone with the QTc interval

PurposeVentricular repolarization is assessed using the QT interval corrected by the heart rate (QTc) via an electrocardiogram (ECG). Prolonged QTc is associated with an increased risk of arrhythmias and cardiac mortality. As there have been few reports regarding the effects of hyperthyroidism on ventricular repolarization, we studied the association between serum free thyroxine (free T4 [fT4]) and thyroid stimulating hormone (TSH) levels and the QTc interval.MethodsThirty-eight patients with hyperthyroidism (<30 years old) were included, and we used their clinical records and available ECGs (between August 2003 and August 2011) to evaluate the association between their fT4 and TSH levels and their QTc interval. In addition, we studied the ECGs of 72 age-matched patients with no hyperthyroidism (control group) and compared their data with that from the patients group.ResultsThe QTc duration in patients with hyperthyroidism was significantly prolonged compared to that in the control subjects (P<0.001). In addition, the number of hyperthyroid patients with abnormal prolonged QTc was significantly higher than that in the control group (P<0.001). Among the patients with hyperthyroidism, patients with prolonged QTc and borderline QTc had higher fT4 levels and there was positive correlation between their fT4 levels and their QTc interval (P<0.05). However, no correlation was observed between their TSH levels and their QTc interval.ConclusionWe report that hyperthyroidism is associated with QTc prolongation. The correlation between the fT4 levels and the QTc interval suggests that thyroid status is associated with QTc values and the risk of cardiac mortality

Tumor immune profiles noninvasively estimated by FDG PET with deep learning correlate with immunotherapy response in lung adenocarcinoma

Rationale: The clinical application of biomarkers reflecting tumor immune microenvironment is hurdled by the invasiveness of obtaining tissues despite its importance in immunotherapy. We developed a deep learning-based biomarker which noninvasively estimates a tumor immune profile with fluorodeoxyglucose positron emission tomography (FDG-PET) in lung adenocarcinoma (LUAD). Methods: A deep learning model to predict cytolytic activity score (CytAct) using semi-automatically segmented tumors on FDG-PET trained by a publicly available dataset paired with tissue RNA sequencing (n = 93). This model was validated in two independent cohorts of LUAD: SNUH (n = 43) and The Cancer Genome Atlas (TCGA) cohort (n = 16). The model was applied to the immune checkpoint blockade (ICB) cohort, which consists of patients with metastatic LUAD who underwent ICB treatment (n = 29). Results: The predicted CytAct showed a positive correlation with CytAct of RNA sequencing in validation cohorts (Spearman rho = 0.32, p = 0.04 in SNUH cohort; spearman rho = 0.47, p = 0.07 in TCGA cohort). In ICB cohort, the higher predicted CytAct of individual lesion was associated with more decrement in tumor size after ICB treatment (Spearman rho = -0.54, p < 0.001). Higher minimum predicted CytAct in each patient associated with significantly prolonged progression free survival and overall survival (Hazard ratio 0.25, p = 0.001 and 0.18, p = 0.004, respectively). In patients with multiple lesions, ICB responders had significantly lower variance of predicted CytActs (p = 0.005). Conclusion: The deep learning model that predicts CytAct using FDG-PET of LUAD was validated in independent cohorts. Our approach may be used to noninvasively assess an immune profile and predict outcomes of LUAD patients treated with ICB.

Incidence of Atazanavir-associated Hyperbilirubinemia in Korean HIV Patients: 30 Months Follow-up Results in a Population with Low UDP-glucuronosyltransferase1A1*28 Allele Frequency

Hyperbilirubinemia is frequently observed in Caucasian HIV patients treated with atazanavir. UDP-glucuronosyltransferase 1A1 polymorphism, UGT1A1*28, which is associated with atazanavir-induced hyperbilirubinemia, is less common in Asians than in Caucasians. However, little is known about the incidence of atazanavir-associated hyperbilirubinemia in Asian populations. Our objective was to investigate the incidence of and tolerability of atazanavir-associated hyperbilirubinemia in Korean HIV patients. The prevalence and cumulative incidence of atazanavir-associated hyperbilirubinemia and UGT1A1*28 allele frequency was investigated in 190 Korean HIV-infected patients treated with atazanavir 400 mg per day. The UGT1A1*28 were examined by direct sequencing of DNA from peripheral whole blood. The UGT1A1*28 allele frequency was 11%. The cumulative incidence of any grade of hyperbilirubinemia was 77%, 89%, 98%, and 100%, at 3, 12, 24, and 30 months, respectively. The cumulative incidence of severe (grade 3-4) hyperbilirubinemia was 21%, 41%, 66%, and 75%, at 3, 12, 24, and 30 months, respectively. However, the point prevalence of severe hyperbilirubinemia did not increase with time and remained around 25%. Our data suggest that atazanavir-associated hyperbilirubinemia is common but transient in a population with low UGT1A1*28 allele frequency

Development of a clinical scoring system for appendicitis in children with presumed appendicitis

Purpose To develop a clinical scoring system for children with presumed appendicitis who visit the emergency department. Methods A registry based-retrospective study was conducted in the pediatric emergency department between September 2015 and December 2016. Patients aged 4 to 17 years who had a > 1 of 5 Likert scale for possibility of appendicitis were included. Multiple logistic regressions based on Akaike information criterion were performed using variables regarding clinical features and inflammatory markers to develop the clinical scoring system. Results A total of 233 patients were included, and 93 (39.9%) had the final diagnosis of appendicitis. The final model with the lowest Akaike information criterion (171.7) consisted of 5 variables, including vomiting (1 point), absence of watery diarrhea (1 point), duration of symptoms ≤ 3 days (1 point), rebound tenderness (1 point), and white blood cell count > 10.0 × 109/L (2 points). If the clinical score was ≥ 4 of 6 points, the area under the receiver operating characteristic curve was 0.78 (95% confidence interval, 0.71-0.86) with a 78.9% sensitivity, 66.7% specificity, positive and negative predictive values of 70.0% and 76.2%, respectively, and positive and negative likelihood ratios of 2.4 and 0.3, respectively. Conclusion The 5-item clinical scoring system shows a fair performance for prediction of pediatric appendicitis. This simple tool could be applied to predict the pediatric appendicitis, and to avoid the use of potentially unnecessary computed tomography