Left and right ventricular function by echocardiography, tissue doppler imaging, carotid intima media thickness, and asymmetric dimethylarginine levels in female adolescents with vitamin D deficiency.

BACKGROUND: The aim of our study was to assess left and right ventricle systolic and diastolic functions in female adolescents with vitamin D deficiency using conventional echocardiography and pulsed-wave tissue Doppler imaging and to investigate carotid intima media thickness and asymmetric dimethylarginine levels. METHODS: Sixty-six female adolescents were enrolled in this study. The female adolescents were divided into a vitamin D deficiency group (n: 34) and a control group (n: 32). All subjects underwent laboratory blood tests, including asymmetric dimethyl arginine, complete two-dimensional, pulse, and tissue Doppler echocardiography, and measurement of the carotid intima-media thickness. RESULTS: The vitamin D-deficient female adolescent group had normal left and right ventricle systolic and diastolic functions and normal global systolic and diastolic myocardial performance. In the patients with vitamin D deficiency, the carotid intima-media thickness was higher than that in the controls. In the patients within the vitamin D deficiency group, vitamin D was found to be positively correlated with magnesium and negatively correlated with phosphorus and left atrial dimension. CONCLUSIONS: The results of this study demonstrate that vitamin D deficiency in female adolescence is associated with normal myocardial geometry and function. Although it has been associated with normal levels of asymmetric dimethyl arginine concentration, high measured carotid intima-media thickness may reflect endothelial dysfunction.

One-Year Follow-Up Results of MIS-C Patients with Coronary Artery Involvement: A Multi-center Study.

Multisystem inflammatory syndrome (MIS-C) in children is a rare complication of SARS-CoV-2 infection. Knowing the course of the affected or unaffected coronary arteries in the patients under follow-up is important in terms of defining the long-term prognosis of the disease and determining the follow-up plan. This is a multicenter and retrospective study. The data were obtained from nine different centers. Between May 2020 and August 2022, 68 of 790 patients had coronary artery involvement. One-year echocardiographic data of 67 of 789 MIS-C patients with coronary artery involvement were analyzed. Existing pathologies of the coronary arteries were grouped as increased echogenicity, dilatation and aneurysm according to Z scores, and their changes over a 1-year period were determined. The data of all three groups are defined as frequency. SPSS Statistics version 22 was used to evaluate the data. In our study, aneurysm was observed in 16.4%, dilatation in 68.7% and increased echogenicity in 13.4% of the patients. All of the patients with involvement in the form of increased echogenicity recovered without sequelae by the end of the first month. No progression to aneurysm was observed in any of the patients with dilatation. No new-onset involvement was observed in patients with previously healthy coronary arteries during the convalescent period. In addition, from the sixth month follow-up period, there was no worsening in the amount of dilatation in any of the patients. At least 94% of the patients who completed the 12th month control period returned to normal.

Oral health-related quality of life of preschool-aged Turkish children with congenital heart disease.

BACKGROUND: Dental caries and poor oral hygiene can affect the quality of life (QoL) of patients with congenital heart disease (CHD). Information about the oral health-related quality of life (OHRQoL) of Turkish preschool children with CHD is scarce. OBJECTIVES: The aim of the present study was to assess the OHRQoL, and the presence of caries, plaque and gingivitis in Turkish preschool children with CHD as compared to children without CHD (control group). MATERIAL AND METHODS: Children aged 3-6 years with CHD (n = 75) and a control group (n = 75) were included in the study. Examinations were conducted using the plaque index (PI), the gingival index (GI) and the World Health Organization (WHO) caries diagnostic criteria. The Early Childhood Oral Health Impact Scale (ECOHIS) questionnaire was completed by the children's families. RESULTS: The amount of caries and plaque, as well as the number of missing teeth were higher in children with CHD. The OHRQoL was lower in children with CHD. However, the differences between the 2 groups were not statistically significant (p > 0.05). The number of filled teeth was significantly higher in the control group (p < 0.05). CONCLUSIONS: According to the findings of the present study, the high amount of caries and plaque in both groups demonstrates that caries continues to be a major public health problem. Although there was no significant difference in terms of QoL scale scores between the 2 groups, the study showed that OHRQoL was lower in children with CHD.

COVID-19 associated multisystemic inflammatory syndrome in 614 children with and without overlap with Kawasaki disease-Turk MIS-C study group.

Multisystemic inflammatory syndrome (MIS-C) diagnosis remains difficult because the clinical features overlap with Kawasaki disease (KD). The study aims to highlight the clinical and laboratory features and outcomes of patients with MISC whose clinical manifestations overlap with or without KD. This study is a retrospective analysis of a case series designed for patients aged 1 month to 18 years in 28 hospitals between November 1, 2020, and June 9, 2021. Patient demographics, complaints, laboratory results, echocardiographic results, system involvement, and outcomes were recorded. A total of 614 patients were enrolled; the median age was 7.4 years (interquartile range (IQR) 3.9-12 years). A total of 277 (45.1%) patients with MIS-C had manifestations that overlapped with KD, including 92 (33.3%) patients with complete KD and 185 (66.7%) with incomplete KD. Lymphocyte and platelet counts were significantly lower in patients with MISC, overlapped with KD (lymphocyte count 1080 vs. 1280 cells × µL, p = 0.028; platelet count 166 vs. 216 cells × 103/µL, p < 0.001). The median serum procalcitonin levels were statistically higher in patients overlapped with KD (3.18 vs. 1.68 µg/L, p = 0.001). Coronary artery dilatation was statistically significant in patients with overlap with KD (13.4% vs. 6.8%, p = 0.007), while myocarditis was significantly more common in patients without overlap with KD features (2.6% vs 7.4%, p = 0.009). The association between clinical and laboratory findings and overlap with KD was investigated. Age > 12 years reduced the risk of overlap with KD by 66% (p < 0.001, 95% CI 0.217-0.550), lethargy increased the risk of overlap with KD by 2.6-fold (p = 0.011, 95% CI 1.244-5.439), and each unit more albumin (g/dl) reduced the risk of overlap with KD by 60% (p < 0.001, 95% CI 0.298-0.559). CONCLUSION: Almost half of the patients with MISC had clinical features that overlapped with KD; in particular, incomplete KD was present. The median age was lower in patients with KD-like features. Lymphocyte and platelet counts were lower, and ferritin and procalcitonin levels were significantly higher in patients with overlap with KD. WHAT IS KNOWN: • In some cases of MIS-C, the clinical symptoms overlap with Kawasaki disease. • Compared to Kawasaki disease, lymphopenia was an independent predictor of MIS-C. WHAT IS NEW: • Half of the patients had clinical features that overlapped with Kawasaki disease. • In patients whose clinical features overlapped with KD, procalcitonin levels were almost 15 times higher than normal. • Lethargy increased the risk of overlap with KD by 2.6-fold in MIS-C patients. • Transient bradycardia was noted in approximately 10% of our patients after initiation of treatment.

Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery: A case report.

BACKGROUND: Cornelia de Lange syndrome (CdLS) is a congenital multisystemic genetic disorder. The expected lifespan of children with this disorder has been prolonged in parallel with the advances in medicine in recent years. However, they still more frequently undergo cardiac surgery. There are some challenges for clinicians when faced with CdLS patients. We present the perioperative management of a child with CdLS undergoing open-heart surgery. CASE SUMMARY: Severe pulmonic and subpulmonic valvular stenosis, enlargement of the right side of the heart, mild tricuspid regurgitation, atrial septal defect, and patent ductus arteriosus were diagnosed in a 14-month-old boy with manifested cyanosis, developmental delay, and malnutrition. Attempted balloon valvuloplasty was unsuccessful due to a severe stenotic pulmonary valve, therefore it was decided to perform an open surgical repair. Following a successful and uncomplicated intraoperative course, the patient was extubated on postoperative day 5, and adrenalin and dopamine infusions were gradually decreased and stopped on postoperative days 6 and 10, respectively. Moderate laryngomalacia and suboptimal vocal cord movements were diagnosed, and tracheotomy and percutaneous endoscopic gastrostomy were performed under general anesthesia in the same session at postoperative day 32. The patient was discharged on postoperative day 85 after a challenging postoperative period with additional airway and nutritional problems. CONCLUSION: This is the first report of the perioperative anesthetic and clinical management of a CdLS patient undergoing open-heart surgery.

Evaluation of hematological parameters and inflammatory markers in children with COVID-19.

BACKGROUND: The aim of this study was to evaluate hematological parameters in children with COVID-19 and determine the effects of inflammatory biomarkers on the assessment of hospitalization. METHODS: This retrospective single-center study was performed on 633 children with COVID-19 between March 2020 and January 2021. The study population was separated into two groups: inpatients (n = 83) and outpatients (n = 550). Univariate and multivariate logistic regression was applied to identify risk factors for hospitalization. RESULTS: Lymphopenia (n = 228, 36%) was found mainly to be a hematological abnormality in all cases. Compared with outpatients, inpatients had significantly higher white blood cell (WBC) (p = 0.005), lymphocyte (p < 0.001), and platelet counts (p = 0.036), and significantly higher red cell distribution width (p = 0.001), C-reactive protein (CRP) (p = 0.003), procalcitonin (p = 0.001), D-dimer (p < 0.001), and lymphocyte to monocyte ratio values (p = 0.004). On the other hand, they had significantly lower values of hemoglobin (p < 0.001), neutrophil to lymphocyte ratio (p = 0.024), platelet lymphocyte ratio (p = 0.001), derivated neutrophil to lymphocyte ratio (p = 0.037), and mean platelet volume to lymphocyte ratio (p < 0.001). ROC analysis showed that WBC, CRP, and procalcitonin cutoff values were the best discriminated between inpatients and outpatients. The results for the areas under the curve of WBC, CRP, and procalcitonin used to assess patients' hospitalization were 0.595 (95% CI 0.519-0.670, p = 0.005), 0.599 (95% CI 0.527-0.672, p = 0.003), and 0.599 (95% CI 0.525-0.673, p = 0.004), respectively. CONCLUSION: We suggest that high WBC and procalcitonin levels can be used as independent predictors of hospitalization in children with COVID-19.

Clinical features and outcome of MIS-C patients: an experience from Central Anatolia.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a new clinical condition characterized by signs of inflammation and multiorgan dysfunction due to cytokine storm associated with SARS-CoV-2. The clinical spectrum of MIS-C ranges from mild to severe, and even to mortal multisystem involvement. To guide clinicians, we evaluated detailed demographic characteristics, clinical features, laboratory findings, and outcomes of MIS-C cases. METHODS: We performed a retrospective study of patients with MIS-C who were managed in the Department of Pediatric Infectious Disease in the Selcuk University Faculty of Medicine, Konya, Turkey. MIS-C patients were divided into three clinical severity groups (mild, moderate, and severe) and separated into three age groups (< 5 years, 5-10 years, > 10 years). We compared the characteristics of MIS-C cases according to the severity of the disease and by age groups. RESULT: Thirty-six children with MIS-C were evaluated (52.8% male, median age of 7.8 years). A clinical spectrum overlapping with Kawasaki disease (KD) was the most common presentation (69.4%) in all age groups. The most common clinical symptoms were fever (100%), mucocutaneous rash (69.4%), and gastrointestinal symptoms (66.6%). There was no statistically significant difference in echocardiographic abnormality between KD-like and the other clinical spectra (p > 0.05). All life-threatening rhythm disturbances were observed in severe cases. No patients died. CONCLUSION: It is important to increase the awareness of physicians about the MIS-C disease, which can present with different combinations of different systemic findings, so that patients can be diagnosed and treated in a timely manner. Key Points • A single tertiary centre study shows that children with MIS-C can present with different clinic spectra other than Kawasaki diseases. • Electrocardiographic and echocardiographic evaluation is important in early diagnosis of children with MIS-C. • Pro-BNP can be used as a screening test in the emergency room for children with prolonged and unexplained fever for determine early cardiac involvement of MIS-C. • The lack of require biological agents and favourable outcomes in children with MIS-C may be related with administration of steroid therapy with IVIG in early stage of disease.

Endocarditis infecciosa de la válvula tricúspide asociada con meningitis aséptica: presentación infrecuente en una niña / Tricuspid valve infective endocarditis associated with aseptic meningitis: a rare presentation in a child

La endocarditis infecciosa es infrecuente pero potencialmente mortal. Las presentaciones atípicas retrasan el diagnóstico. El compromiso neurológico es habitual en la endocarditis de la válvula mitral, aunque infrecuente en la endocarditis de la válvula tricúspide. Si bien se han informado algunos casos e el del lado derecho con síntomas neurológicos en adultos, en la bibliografía no se ha descripto en niños. Se presenta una niña de 9 años con comunicación interventricular (CIV) congénita con fiebre, cefalea y rigidez de nuca. Sus síntomas clínicos y los hallazgos en el líquido cefalorraquídeo respaldaron el diagnóstico de meningitis aséptica. El día 3 del tratamiento con ceftriaxona, se resolvieron los síntomas; tras nueve días, reingresó con fiebre y rigidez de nuca. Un ecocardiograma mostró endocarditis de la válvula tricúspide. Recibió tratamiento antibiótico durante 6 semanas. Se realizó una cirugía cardíaca para la CIV y la insuficiencia de la válvula tricúspide.

Infective endocarditis (IE) is a rare but a potentially life-threatening infectious disease. Atypical presentations cause delays in the diagnosis. Neurological involvement such as meningitis or meningismus, are especially common in mitral valve endocarditis, but unusual in tricuspid valve endocarditis. Although few cases of right-sided IE have been reported with neurological symptoms in adults, children have not been described in literature. A nine-year-old girl with congenital ventricular septal defect (VSD) was admitted with fever, headache and neck stiffness. Her clinical symptoms and cerebrospinal fluid findings supported the aseptic meningitis. On ceftriaxone therapy day 3, her complaints were resolved; nine days later she was admitted with fever and neck stiffness again. Further investigation for fever source with echocardiogram revealed a tricuspid valve endocarditis. Antibiotic therapy was completed after 6 weeks. Cardiac surgery was performed for VSD and tricuspid valvular insufficiency.

Tricuspid valve infective endocarditis associated with aseptic meningitis: a rare presentation in a child. / Endocarditis infecciosa de la válvula tricúspide asociada con meningitis aséptica: presentación infrecuente en una niña.

Infective endocarditis (IE) is a rare but a potentially life-threatening infectious disease. Atypical presentations cause delays in the diagnosis. Neurological involvement such as meningitis or meningismus, are especially common in mitral valve endocarditis, but unusual in tricuspid valve endocarditis. Although few cases of right-sided IE have been reported with neurological symptoms in adults, children have not been described in literature. A nine-year-old girl with congenital ventricular septal defect (VSD) was admitted with fever, headache and neck stiffness. Her clinical symptoms and cerebrospinal fluid findings supported the aseptic meningitis. On ceftriaxone therapy day 3, her complaints were resolved; nine days later she was admitted with fever and neck stiffness again. Further investigation for fever source with echocardiogram revealed a tricuspid valve endocarditis. Antibiotic therapy was completed after 6 weeks. Cardiac surgery was performed for VSD and tricuspid valvular insufficiency.

La endocarditis infecciosa es infrecuente pero potencialmente mortal. Las presentaciones atípicas retrasan el diagnóstico. El compromiso neurológico es habitual en la endocarditis de la válvula mitral, aunque infrecuente en la endocarditis de la válvula tricúspide. Si bien se han informado algunos casos e el del lado derecho con síntomas neurológicos en adultos, en la bibliografía no se ha descripto en niños. Se presenta una niña de 9 años con comunicación interventricular (CIV) congénita con fiebre, cefalea y rigidez de nuca. Sus síntomas clínicos y los hallazgos en el líquido cefalorraquídeo respaldaron el diagnóstico de meningitis aséptica. El día 3 del tratamiento con ceftriaxona, se resolvieron los síntomas; tras nueve días, reingresó con fiebre y rigidez de nuca. Un ecocardiograma mostró endocarditis de la válvula tricúspide. Recibió tratamiento antibiótico durante 6 semanas. Se realizó una cirugía cardíaca para la CIV y la insuficiencia de la válvula tricúspide.

Management of acute mitochondriopathy and encephalopathy syndrome in pediatric intensive care unite: a new clinical entity.

Acute mitochondriopathy and encephalopathy syndrome (AMES) is described differently by different authors in the literature. As a new clinical entity, we aimed to present the clinical signs and symptoms, diagnosis and treatment algorithm of our patients with AMES. 56 patients aged between 2 months and 18 years who were followed up in pediatric intensive care units of Konya Training and Research Hospital and Selcuk University Medical Faculty Hospital, between January 2010 and June 2017 were included. Patients' data were obtained retrospectively from the intensive care unit patient files. 34 (60.7%) of the patients were male and 22 (39.3%) were female. The median age of our patients was 10.0 months. At the time of admission, 42 (75%) of the patients had fever, 35 (62.5%) vomiting, 27 (48.2%) abnormal behaviour and agitation and 28 (50%) convulsion. The etiological classification of patients with AMES was divided into four groups as infection, metabolic disorder, toxic, and hypoxic-ischemic. 39 (69.6%) patients were found to have infection, 10 (17.9%) patients hypoxia, 7 (12.5%) patients metabolic disorders. AMES occurs rarely, but should be kept in mind in the differential diagnosis of patients with any encephalopathy of unknown origin especially in those with a history of ingestion of drugs, previous viral infection and vomiting. Early recognition and treatment is imperative to reduce morbidity and mortality in children with AMES.