Usefulness of Kawasaki disease risk scoring systems to the Turkish population

Objective: Kawasaki disease (KD) is the most common cause of coronary artery aneurysm (CAA) in children. The available risk scores to predict intravenous immunoglobulin (IVIG) resistance and CAA were developed in Asian populations in whom their effectiveness has been proven, but data on non-Asian children are limited. This study aimed to evaluate the ability of 5 risk scoring systems to predict IVIG resistance and CAA in Turkey patients with KD

Novel Variant Syndrome Associated With Congenital Hepatic Fibrosis

Congenital hepatic fibrosis is part of many different malformation syndromes, of which oculo-encephalohepato-renal syndrome is the most common. These syndromes largely overlap, and so accurate classification of individual patients may be difficult. We present herein three syndromic siblings who were products of a consanguineous marriage. We investigated in detail at least six organ systems in these patients, namely the liver, brain, eye, kidneys, skeleton, and gonads. The common features observed in these three cases were congenital hepatic fibrosis, retinitis pigmentosa, truncal obesity, rotatory nystagmus, mental retardation, advanced myopia, and high-arched palate. The clinical dysmorphology in these patients was distinct and lacked the major features of the known syndromes associated with congenital hepatic fibrosis. Although some features of these presented cases are similar to those found in Bardet-Biedl syndrome (BBS), the absence of some major criteria of BBS (polydactyly, renal abnormality, and hypogonadism) suggests that this may be a new syndrome. All three patients remain under follow-up in the departments of Gastroenterology, Ophthalmology, and Neurology at Hacettepe University.Wo

Turkish translation, cross-cultural adaptation, and assessment of psychometric properties of the Ottawa sitting scale for the intensive care unit survivors

To translate and cross-culturally adapt the Ottawa Sitting Scale (OSS) developed for acute care patients into Turkish and to examine its psychometric properties in intensive care unit (ICU) survivors. After translation process the Turkish version of the scale was adminstered to eighty-one patients aged 39–82 years after discharge from the ICU and videotape was recorded. Two physiotherapists watched the videotaped records during the evaluation and scored. Test-retest reliability was assesed by scoring the same video recordings 15 days after the initial scoring. Correlations of the OSS with Berg Balance Scale (BBS), Functional Independence Measurement (FIM), the ICU length of stay and, total mechanical ventilation duration were assessed for convergent validity. The intra-class correlation coefficient for inter and intra-rater reliability was 0.989–0.994 and 0.998 respectively. The internal consistency was excellent (Cronbach’s α = 0.998). The OSS score was highly correlated with the BBS total score (r = 0.716), the BBS unsupported sitting item score (r = 0.863), and moderately correlated with the total score of the FIM (r = 0.602), the number of days in ICU (r= −0.545), and total mechanical ventilation duration (r = −0.518). The Turkish version of OSS has been found to be valid and reliable in assessment of sitting balance in patients discharged from intensive care unit.Implications For RehabilitationPhysiotherapeutic measurements are crucial and necessary to determine the functional status of the patients.Ottawa Sitting Scale was translated and culturally adapted to Turkish and showed good psychometric propertiesOttawa Sitting Scale is valid and reliable tool to evaluate sitting balance in intensive care unit survivors.Ottawa Sitting Scale can guide clinicians in establishing evaluation and rehabilitation programs for patients discharged from intensive care unit. Physiotherapeutic measurements are crucial and necessary to determine the functional status of the patients. Ottawa Sitting Scale was translated and culturally adapted to Turkish and showed good psychometric properties Ottawa Sitting Scale is valid and reliable tool to evaluate sitting balance in intensive care unit survivors. Ottawa Sitting Scale can guide clinicians in establishing evaluation and rehabilitation programs for patients discharged from intensive care unit.</p

Is it all about age? Clinical characteristics of Kawasaki disease in the extremely young: PeRA research group experience.

Objectives In the evaluation of children with Kawasaki disease (KD), the age of onset is important and complications may occur if the distinctive features are not assessed accordingly. The objective of the study is to define the clinical and laboratory presentations and treatment outcomes of KD in infants 6 months multicentrically. Methods This retrospective study reviewed the medical records of the patients diagnosed with KD and followed up between January 2009 and January 2019. Results A total of 204 KD patients were enrolled and grouped according to age as Group I (6 months, n = 173). Except for cervical adenopathy (19.3% vs. 47.4%, p = 0.03), the major clinical manifestations of KD were similar between groups I and II. However, the frequency of incomplete and atypical KD was higher in Group I (38.7% vs. 24.8%, p = 0.04, 38.7% vs. 8.1% p < 0.001, respectively). Clinical features such as vomiting/diarrhea (19.3% vs. 1.1% p < 0.001), aseptic meningitis (19.3% vs. 2.3%, p = 0.001) were more common in Group I. Percentage of neutrophils (45.5 vs. 36, p = 0.004) and hemoglobin levels (8 vs. 10.5 gr/dL, p = 0.02) were statistically lower and platelet count (737,000 vs 400,000/mm(3), p = 0.004) was statistically higher in group I. Coronary artery lesions (CALs) were more common in Group I (48% vs. 20%, p < 0.001). Harada and Kobayashi scores appear to be effective in predicting coronary artery lesions (CALs) and IVIG resistance in the entire cohort. There was no diagnostic delay in group I (5.5 vs 6.5 days, p = 0.88). Conclusions Since clinical presentations and laboratory features of KD may vary with age, and the frequency of atypical and incomplete presentations is high, awareness of KD in young children should be raised among pediatricians

Clinical features and outcomes of 76 patients with COVID-19-related multi-system inflammatory syndrome in children

Objectives Multi-system inflammatory syndrome in children (MIS-C) is a less understood and a rare complication of coronavirus disease-2019 (COVID-19). Given the scarce data regarding this novel disease, we aimed to describe the clinical features and outcomes of our patients with MIS-C and to evaluate the associated factors for the pediatric intensive care unit (PICU) admission