Evaluation of 601 children with multisystem inflammatory syndrome (Turk MISC study).

PURPOSE: Due to its link with the 2019 coronavirus, the multisystem inflammatory syndrome in children (MISC) has garnered considerable international interest. The aim of this study, in which MISC patients were evaluated multicenter, and the data of the third period of the Turk-MISC study group, to compare the clinical and laboratory characteristics and outcomes of MISC patients who did and did not require admission to an intensive care unit (ICU). METHODS: This retrospective multicenter observational study was carried out between June 11, 2021, and January 01, 2022. The demographics, complaints, laboratory results, system involvements, and outcomes of the patients were documented. RESULTS: A total of 601 patients were enrolled; 157 patients (26.1%) required hospitalization in the intensive care unit (ICU). Median age was 8 years (interquartile range (IQR) 4.5-11.3 years. The proportion of Kawasaki disease-like features in the ICU group was significantly higher than in the non-ICU group (56.1% vs. 43.2% p = 0.006). The ICU group had considerably lower counts of both lymphocytes and platelets (lymphocyte count 900 vs. 1280 cells × µL, platelet count 153 vs. 212 cells × 103/ µL, all for p< 0.001). C-reactive protein, procalcitonin, and ferritin levels were significantly higher in the ICU group (CRP 164 vs. 129 mg/L, procalcitonin 9.2 vs. 2.2 µg/L, ferritin 644 vs. 334 µg/L, all for p< 0.001). Being between ages 5-12 and older than 12 increased the likelihood of hospitalization in the ICU by four [95% confidence intervals (CI)1.971-8.627] and six times (95% CI 2.575-14.654), respectively, compared to being between the ages 0-5. A one-unit increase in log D-dimer (µg/L) and log troponin (ng/L) was also demonstrated to increase the need for intensive care by 1.8 (95% CI 1.079-3.233) and 1.4 times (95% CI 1.133-1.789), respectively.     Conclusion: By comparing this study to our other studies, we found that the median age of MISC patients has been rising. Patients requiring an ICU stay had considerably higher levels of procalcitonin, CRP, and ferritin but significantly lower levels of lymphocyte and thrombocyte. In particular, high levels of procalcitonin in the serum might serve as a valuable laboratory marker for anticipating the need for intensive care. WHAT IS KNOWN: • Lymphopenia and thrombocytopenia were an independent predictor factors in patients with MISC who needed to stay in intensive care unit. • The possibility of the need to stay in the intensive care unit in patients with MISC who had Kawasaki disease-like findings was controversial compared with those who did not. WHAT IS NEW: • A one-unit increase log D dimer and log troponin was demonstrated to require for intensive care unit by 1.8 and 1.4 times, respectively. • Serum procalcitonin levels had the best performance to predict stay in the intensive care unit stay.

Adaptation of the professionalism mini-evaluation exercise instrument into Turkish: a validity and reliability study.

BACKGROUND: There is an ongoing search for standardized scales appropriate for each culture to evaluate professionalism, which is one of the basic competencies of a physician. The Professionalism Mini-evaluation Exercise (P-MEX) instrument was originally developed in Canada to meet this need. In this study, it was aimed to adapt the P-MEX to Turkish and to evaluate the validity and reliability of the Turkish version. METHODS: A total of 58 residents at Bakirkoy Dr. Sadi Konuk Training and Research Hospital were assessed with the Turkish version of P-MEX by 24 raters consisting of faculty members, attending physicians, peer residents, and nurses during patient room visits, outpatient clinic and group practices. For construct validity, the confirmatory factor analysis was performed. For reliability, Cronbach's alpha scores were calculated. Generalizibility and decision studies were undertaken to predict the reliability of the validated tool under different conditions. After the administration of P-MEX was completed, the participants were asked to provide feedback on the acceptability, feasibility, and educational impact of the instrument. RESULTS: A total of 696 forms were obtained from the administration of P-MEX. The content validity of P-MEX was found to be appropriate by the faculty members. In the confirmatory factor analysis of the original structure of the 24-item Turkish scale, the goodness-of-fit parameters were calculated as follows: CFI = 0.675, TLI = 0.604, and RMSEA = 0.089. In the second stage, the factors on which the items loaded were changed without removing any item, and the model was modified. For the modified model, the CFI, TLI, and RMSEA values were calculated as 0.857, 0.834, and 0.057, respectively. The decision study on the results obtained from the use of P-MEX in a Turkish population revealed the necessity to perform this evaluation 18 times to correctly evaluate professionalism with this instrument. Cronbach's alpha score was 0.844. All the faculty members provided positive feedback on the acceptability, feasibility, and educational impact of the adapted P-MEX. CONCLUSION: The findings of this study showed that the Turkish version of P-MEX had sufficient validity and reliability in assessing professionalism among residents. Similarly, the acceptability and feasibility of the instrument were found to be high, and it had a positive impact on education. TRIAL REGISTRATION: 2020/249, Bakirkoy Dr. Sadi Konuk Training and Research Hospital.

Prevalence of congenital sucrase-isomaltase deficiency in Turkey may be much higher than the estimates.

Congenital sucrase-isomaltase deficiency (CSID) is a rare autosomal carbohydrate malabsorption disorder caused by mutations in the sucrase-isomaltase gene. While the prevalence of CSID is high in the indigenous populations of Alaska and Greenland, it is imprecise and ambiguous in the Turkish pediatric population. In this cross-sectional case-control study, which is retrospective in nature, next-generation sequencing (NGS) results obtained from records of 94 pediatric patients with chronic nonspecific diarrhea were reviewed. Demographic characteristics, clinical symptoms and treatment responses of those diagnosed with CSID were evaluated. We identified one new, homozygous frame-shift mutation and 10 other heterozygous mutations. Two cases were from the same family and nine were from different families. While the median age at onset of symptoms was 6 months (0-12), median age at diagnosis was 60 months (18-192) with a median delay of 5 years and 5 months (10 months -15 years and 5 months) in diagnosis. Clinical symptoms included diarrhea (100%), abdominal pain (54.5%), vomiting after consuming sucrose (27.2%), diaper dermatitis (36.3%) and growth retardation (81%). Our clinical study revealed that sucrase-isomaltase deficiency may have been underdiagnosed in patients with chronic diarrhea in Turkey. In addition, the frequency of heterozygous mutation carriers was significantly higher than that of homozygous mutation carriers and those with a heterozygous mutations responded well to the treatment.

Imaging of Multisystem Inflammatory Disease in Children (MIS-C) Associated With COVID-19.

RATIONALE AND OBJECTIVES: To retrospectively evaluate imaging findings in multisystem inflammatory disease in children associated with COVID-19 (MIS-C). MATERIALS AND METHODS: The radiological imaging findings of 45 pediatric patients aged between 52 days and 16 years, who were diagnosed with MIS-C according to the World Health Organization (WHO) criteria, were evaluated. All the patients underwent chest X-ray and echocardiography. The findings obtained from 25 abdominal radiographs, 24 abdominal US, 7 abdominal CT, 16 thorax CT, 21 cranial MRI and one spinal MRI, MR cholangiography (MRCP) and cardiac MRI examinations were categorized and evaluated according to the affected systems. RESULTS: While the most common findings in chest X-ray were perihilar opacity and peribronchial thickening, pleural effusion was the most finding in thorax CT. Echocardiography findings of myocarditis were observed in 31% of the cases. The most common findings in abdominal radiological evaluation were hepatomegaly and splenomegaly, edema in the gallbladder wall and periportal area, mesenteric lymph nodes in the right lower quadrant, thickening of the intestinal walls, and free fluid. Reversible splenial lesion syndrome (RESLES) was the most common neurological finding. Acute disseminated encephalomyelitis (ADEM)-like lesions, acute hemorrhagic necrotizing encephalomyelitis, and radiological findings consistent with Guillain-Barré syndrome were found in one case each. CONCLUSION: Radiological findings seen in MIS-C in pediatric cases are correlated with the affected system. According to the system involved, there is no specific finding for this disease. Radiological findings are not the primary diagnostic tool but can assist in the evaluation of the affected systems and to guide treatment.