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© 2022 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).Aim: Multisystem inflammatory syndrome in children (MIS-C) may cause shock and even death in children. The aim of this study is to describe the clinical features, laboratory characteristics and outcome of children diagnosed with MIS-C in 25 different hospitals in Turkey. Methods: The retrospective study was conducted between 8 April and 28 October 2020 in 25 different hospitals from 17 cities. Data were collected from patients' medical records using a standardised form. Clinical and laboratory characteristics and outcomes according to different age groups, gender and body mass index percentiles were compared using multivariate logistic regression analysis. Results: The study comprised 101 patients, median age 7 years (interquartile range (IQR) 4.6–9.3); 51 (50.5%) were boys. Reverse-transcriptase polymerase chain reaction (PCR) assay was positive in 21/100 (21%) patients; 62/83 (74.6%) patients had positive serology for SARS-CoV-2. The predominant complaints were fever (100%), fatigue (n = 90, 89.1%), and gastrointestinal symptoms (n = 81, 80.2%). Serum C-reactive protein (in 101 patients, median 165 mg/L; range 112–228), erythrocyte sedimentation rate (73/84, median 53 mm/s; IQR 30–84) and procalcitonin levels (86/89, median 5 μg/L; IQR 0.58–20.2) were elevated. Thirty-eight patients (37.6%) required admission to intensive care. Kawasaki disease (KD) was diagnosed in 70 (69.3%) patients, 40 of whom had classical KD. Most patients were treated with intravenous immunoglobulin (n = 92, 91%) and glucocorticoids (n = 59, 58.4%). Seven patients (6.9%) died. Conclusion: The clinical spectrum of MIS-C is broad, but clinicians should consider MIS-C in the differential diagnosis when persistent fever, fatigue and gastrointestinal symptoms are prominent. Most patients diagnosed with MIS-C were previously healthy. Immunomodulatory treatment and supportive intensive care are important in the management of cases with MIS-C. Glucocorticoids and intravenous immunoglobulins are the most common immunomodulatory treatment options for MIS-C. Prompt diagnosis and prompt treatment are essential for optimal management.

Background Regarding the difficulties in recognition and management of the malignancies in primary immune deficiencies (PIDs), we aimed to present the types, risk factors, treatment options, and prognosis of the cancers in this specific group. Methods Seventeen patients with PID who developed malignancies or malignant-like diseases were evaluated for demographics, clinical features, treatment, toxicity, and prognosis. Results The median age of malignancy was 12.2 years (range, 2.2-26). Lymphoma was the most frequent malignancy (n = 7), followed by adenocarcinoma (n = 3), squamous cell carcinoma (n = 2), cholangiocarcinoma (n = 1), Wilms tumor (n = 1), and acute myeloid leukemia (n = 1). Nonneoplastic lymphoproliferation mimicking lymphoma was observed in five patients. The total overall survival (OS) was 62.5% ± 12.1%. The OS for lymphoma was 62.2% ± 17.1% and found to be inferior to non-PID patients with lymphoma (P = 0.001). Conclusion In patients with PIDs, malignancy may occur and negatively affect the OS. The diagnosis can be challenging in the presence of nonneoplastic lymphoproliferative disease or bone marrow abnormalities. Awareness of susceptibility to malignant transformation and early diagnosis with multidisciplinary approach can save the patients' lives.

Background Tuberculosis (TB) is an important worldwide ongoing health issue. To be able to control TB, one should not only cure active TB but also identify childhood TB patients who have the possibility to develop active disease in the future. The aim of this study was to compare a century-old tuberculin skin test (TST) and QuantiFERON-TB Gold In-Tube (QFT-GIT) test, developed as an alternative to TST and which has been claimed to be superior to TST in several ways, in the diagnosis of childhood TB. Methods Fifty-three children with TB between 5 months and 17.5 years of age and 92 healthy children from the same age group with no risk factors for TB were recruited into the study. All children underwent TST and QFT-GIT test and their demographic, clinic and laboratory data were recorded. Data were analyzed using SPSS 14. Results A total of 53 patients were diagnosed with TB. Mean patient age was 8.5 ± 4.3 years (range, 5 months–17.5 years). A total of 41.7% of the patients were female. Sixteen of 53 patients had confirmation on culture. QFT-GIT test was positive in 16 and TST was positive in 15 among 16 children with culture-confirmed TB. The sensitivity of TST and QFT-GIT were 93.8% and 100.0%, and the specificity of TST and QFT-GIT were 100.0% and 97.8%, respectively. With regard to the 53 TB children including those without bacteriological confirmation, QFT-GIT was positive in 33 children, and TST was positive in 44 children. The sensitivity of TST and QFT-GIT was then 83.0% and 62.3%, and the specificity, 100.0% and 97.8%, respectively. Conclusion Although positive QFT-GIT test is very significant for TB, negative results will not exclude TB infection. TST and QFT-GIT used together may provide more efficient results.

WOS: 000350167200008, PubMed ID: 25703555, Tuberculosis (TB), caused by Mycobacterium tuberculosis (M.tb) and a few related mycobacteria, is a devastating disease, killing more than a million individuals per year worldwide. However, its pathogenesis remains largely elusive, as only a small proportion of infected individuals develop clinical disease either during primary infection or during reactivation from latency or secondary infection. Subacute, hematogenous, and extrapulmonary disease tends to be more frequent in infants, children, and teenagers than in adults. Life-threatening primary TB of childhood can result from known acquired or inherited immunodeficiencies, although the vast majority of cases remain unexplained. We review here the conditions conferring a predisposition to childhood clinical diseases caused by mycobacteria, including not only M.tb but also weakly virulent mycobacteria, such as BCG vaccines and environmental mycobacteria. Infections with weakly virulent mycobacteria are much rarer than TB, but the inherited and acquired immunodeficiencies underlying these infections are much better known. Their study has also provided genetic and immunological insights into childhood TB, as illustrated by the discovery of single-gene inborn errors of IFN- immunity underlying severe cases of TB. Novel findings are expected from ongoing and future human genetic studies of childhood TB in countries that combine a high proportion of consanguineous marriages, a high incidence of TB, and an excellent clinical care, such as Iran, Morocco, and Turkey., European Research CouncilEuropean Research Council (ERC) [ERC-2010-AdG-268777]; French National Research Agency (ANR)French National Research Agency (ANR) [ANR-10-IAHU-01, ANR-08-MIEN-014-02]; Institut National de la Sante et de la Recherche Medicale, Universite Paris DescartesInstitut National de la Sante et de la Recherche Medicale (Inserm); St. Giles Foundation; Rockefeller University from the National Center for Research Resources [8ULTR000043]; National Center for Advancing Sciences (NCATS) of the National Institute of Health; Rockefeller University; National Institute of Allergy and Infectious DiseasesUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute of Allergy & Infectious Diseases (NIAID) [U01AI088685, R01AI089970, R37AI095983], We thank all clinicians from many countries around the world for their help and contribution in the investigation of TB patients. Special thanks to A. Strickler and J. F. Emile for providing the pictures. We also thank all members of the Necker and Rockefeller branches of the Laboratory of Human Genetics of Infectious Diseases. This work was supported by grants from the European Research Council (ERC-2010-AdG-268777), the French National Research Agency (ANR) under "the Investments for the Future" grant number ANR-10-IAHU-01, ANR-08-MIEN-014-02, Institut National de la Sante et de la Recherche Medicale, Universite Paris Descartes, the St. Giles Foundation, the Rockefeller University grant number 8ULTR000043 from the National Center for Research Resources and the National Center for Advancing Sciences (NCATS) of the National Institute of Health, the Rockefeller University, the National Institute of Allergy and Infectious Diseases grant number U01AI088685, R01AI089970, and R37AI095983. The authors have no financial or commercial conflict of interest to declare.

Brucellosis is considered the most widespread zoonosis in the world. In endemic regions of brucellosis, childhood brucellosis includes up to one-third of all cases of human brucellosis. Brucellosis constitutes a public health problem in Turkey. A boy aged 12 yr who had PFIC2 had undergone deceased-donor liver transplantation in 2008 at the age of seven. The boy presented with fatigue, fever, and pain in the right leg and hip and was admitted to the hospital. Brucella melitensis grew in the blood culture, and the SAT was positive at a titer of 1:640. The patient was treated with oral doxycycline and rifampicin for eight wk. After treatment, the patient recovered and his blood cultures became negative. The patient's mother also had a high Brucella agglutination titer of 1:320 positive and was treated in the internal medicine department with spiramycin and doxycycline. Brucella infection should be suspected in liver transplant recipients with fever of unknown origin, especially in recipients who live in an endemic area.

Background. With the aim of establishing the role of blunt trauma, a trauma without any fracture or obvious wound, as a risk factor in children who were admitted for community-acquired severe staphylococcal septicemia with various organ involvement, 63 patients were investigated during a seven-year study period. All children were previously healthy. Results: Blunt trauma was present in 12 (46%) of 26 septicemic patients in whom skeletal infections were diagnosed and the relative risk of trauma was high (odds ratio 15.0; confidence interval 2.9–75.8, P< 0.001) in these children. Patients with multiple organ involvement and with multifocal skeletal infections were more frequent among traumatized patients. Children with skeletal involvement were significantly older than others (P< 0.001). The period between the first symptom of infection and the diagnosis was shorter in traumatized children (P < 0.001). None of the children with staphylococcal pulmonary or soft tissue infection had any history of trauma. We conclude that blunt trauma is one of the important risk factors for skeletal involvement in staphylococcal septicemia. Its immunosuppressive nature, especially in local damage of the skin barrier, may be the cause of seeding and multifocal infection.

Purpose Ceftriaxone is a widely used third-generation cephalosporin. In this prospective study, we used sonography to investigate the incidence and outcome of biliary complications in children receiving ceftriaxone therapy. Methods Ceftriaxone was administered intravenously at a dosage of 100 mg/kg/day for 1–3 weeks to 118 children hospitalized for severe infection. Serial gallbladder sonograms were obtained on days 1, 5–7, and 10–14 of therapy and the day after therapy ended if it had lasted more than 2 weeks. When sonographic abnormalities were found, additional sonograms were obtained every 3 days until the abnormalities had completely resolved. Results Twenty patients (17%), all asymptomatic, demonstrated sonographic abnormalities: 8 had gallbladder sludge, defined as echogenic material without associated acoustic shadowing, and 12 had pseudolithiasis, defined as echogenic material with acoustic shadowing. These abnormalities spontaneously resolved within 2 weeks of stopping the ceftriaxone (mean time to disappearance, 8.2 ± 3.4 days). No significant differences were found between patients with normal versus abnormal sonographic findings in sex, age, duration of treatment, or other risk factors for drug precipitation. Conclusions Ceftriaxone-associated biliary pseudolithiasis is usually asymptomatic and was rapidly reversible after cessation of therapy in this group of Turkish children. © 2000 John Wiley & Sons, Inc. J Clin Ultrasound 28:166–168, 2000.