Background: Diabetes and hepatosteatosis are dramatically increasing in childhood. Non-alcoholic fatty liver disease (NAFLD) is defined as a common disorder in adulthood, especially with type-2 diabetes and metabolic syndrome, while very few studies are available on liver health in children with type-1 diabetes. Patients and methods: One hundred and ten (52 males and 58 females) patients with type-1 diabetes aged between 8 and 18 years were examined. The lipid profile, liver enzymes and hepatobiliary ultrasound findings of patients were investigated in terms of hepatopathies. Patients diagnosed with fatty liver were evaluated by pediatric gastroenterology specialists for the differential diagnosis and exclusion of other etiologies. The relationships between hepatopathy and age, pubertal status, the duration of diabetes and glycemic control were evaluated. Results: Hepatopathy was found in 17 (15.5%) patients. The levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST) were normal and did not correlate with the ultrasonography (USG) findings. Hyperechogenicity detected by USG, whether it is true fat or glycogen hepatopathy, was found to be associated with “poor glycemic control” independently of age, puberty status and the duration of diabetes. Conclusions: This study contributes to the literature in terms of the relationship between liver health and glycemic control in pediatric type-1 diabetes. Hepatopathies were releated with poor glycemic control independently of the duration of diabetes. This suggested that liver disorders should be considered as one of the subacute complications of diabetes. It was concluded that routine screening for comorbidities and complications in type-1 diabetes should also include hepatobiliary USG, as liver enzymes alone are inadequate for detecting hepatopathies.

Objectives: In this study, we present our clinical severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) experience in patients with childhood rheumatic disease during novel coronavirus-2019 (COVID-19) pandemic. Patients and methods: A total of 87 patients (50 males, 37 females; median age: 12 years; range, 6.6 to 16 years) suspected of having COVID-19 at our pediatric rheumatology clinic between March 11th and October 15th 2020 were retrospectively analyzed. Demographic and clinical features, treatments, laboratory results, imaging findings, and clinical outcomes of the patients diagnosed with COVID-19 and/or multisystem inflammatory syndrome in children (MIS-C) were retrieved from the medical records. The diagnosis of SARS-CoV-2 infection was made based on the reverse transcriptase-polymerase chain reaction test. Results: The most common rheumatic diseases were juvenile idiopathic arthritis and familial Mediterranean fever (35.6% and 34.5%, respectively). Twenty-six of these patients were treated with biological disease-modifying anti-rheumatic drugs. SARS-CoV-2 infection was tested as positive in 84 (96.5%) patients. Also, 51 (58.6%) patients had an epidemiological contact to a person with COVID-19. Eighteen patients met the clinical criteria and diagnosed with MIS-C. The COVID-19 outbreak also caused exacerbation of systemic disease in 56 children due to medication cessation, postponed drug switch, or recurrent viral infection. Conclusion: Children with rheumatic disease do not appear to present a higher risk of severe COVID-19. The immunosuppressive treatments can be adjusted in case of infection; otherwise, it is not recommended to interrupt the treatments. Physicians should be cautious about the hyperinflammatory syndrome associated with COVID-19 in rheumatic children, which may be severe in this group of patients and may be confused with primary diseases.

Y Respiratory syncytial virus (RSV) is one of the most common causes of acute respiratory tract infections among children. 1%-2% of RSV infections require hospitalization. In addition to the respiratory system, cardiovascular system may be also affected by the RSV infection. A 7-year-old, previously healthy, female patient presenting with respiratory difficulties was admitted to the paediatric intensive care unit. The patient was intubated and connected to a mechanical ventilator because of acute respiratory failure. Her tracheal aspirate was studied for viral multiplex polymerase chain reaction (PCR), and RSV positivity was detected. Her echocardiogram revealed left ventricular dysfunction. She was put on fluid restriction, intravenous furosemide, and inotropic support. Her cranial magnetic resonance examination showed the signs of acute haemorrhagic encephalopathy. She underwent five sessions of therapeutic plasma exchange with fresh frozen plasma. She was extubated on the 18th day of admission and provided with respiratory support with high-flow oxygen therapy thereafter. On the 23rd day, when her clinical status remained stable, she was transferred to the paediatrics ward. An RSV infection should be considered in cases with acute necrotising encephalitis and myocarditis.

Generalized arterial calcification of infancy (GACI) is a rare autosomal-recessive disorder, characterized by calcification of the internal elastic lamina, fibrotic myointimal proliferation of muscular arteries and resultant arterial stenosis. Treatment with bisphosphonates has been proposed as a means of reducing arterial calcifications in GACI patients, although there is no formalized treatment approach. The case reported here was a patient with severe GACI diagnosed at three months of age who had no response to bisphosphonate treatment, but clinically improved after the initiation of magnesium and anti-phosphate (using calcium carbonate) treatments. In patients unresponsive to bisphosphonate, magnesium and anti-phosphate treatment may be attempted.

Objective: To retrospectively evaluate the imaging and clinical findings of patients diagnosed with multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. Methods: The clinical, laboratory and radiological data of 110 patients (74 male and 36 female) diagnosed with COVID-19-related MIS-C between June 2020 and November 2021 were evaluated retrospectively. Cases with a diagnosis of MIS-C based on a positive real time polymerase chain reaction (RT-PCR) test or serology results according to the WHO criteria were included in the study. All the radiological data were evaluated by a pediatric radiologist with 14 years of radiology experience. Results: Peribronchial thickening and hyperinflation were the most common findings on chest X-ray, while atelectasis and pleural effusion were often present in thoracic CT. Cardiac involvement was detected in 30% of the patients, mainly with valve insufficiency and systolic dysfunction, and 7.2% of these patients had sequalae findings. The most common abdominal findings were hepatosplenomegaly, mesenteric inflammation, lymphadenomegaly, thickening of the intestinal walls and free fluid. 23 of the patients had comorbidities. Neurological radiological findings observed in a total of six patients were reversible splenial lesion syndrome, posterior reversible encephalopathy syndrome, meningitis, and cerebral edema. 37 patients were followed up in the intensive care unit and 2 of them died. Conclusion: Radiological findings seen in MIS-C vary according to the affected system. There is no specific radiologic finding for this disease, but radiological findings can assist in the evaluation of affected systems and guide treatment. Advances in knowledge: Since there are few studies with a limited number of patients in the literature, data on this subject are limited. We aimed to contribute to the literature with our large patient data.

Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory disease characterized by a tumor-like infiltration of IgG4 positive plasma cells and fibrosis in various organs. The exact pathogenesis remains unknown. In this article, we discuss the diagnostic management of IgG4-RD with reference to clinical, serologic, pathological and radiological data on a 17-year-old male patient with lumbar vertebral involvement.