Eight-Year Follow-up of a Girl with McCune-Albright Syndrome

McCune-Albright syndrome (MAS) is characterized by the triad of fibrous dysplasia (FD), cafe-au-lait spots and precocious puberty (PP). We report a 14-year-old girl with MAS who has been followed-up for 8 years. She was referred for multiple fractures and vaginal bleeding at age 5.9 years. She had peripheral PP, FD, and osteoporosis and was diagnosed as MAS. The patient was treated with aromatase inhibitors and bisphosphonates. She had no menses during aromatase inhibitor treatment. Her growth rate and bone maturation were in normal ranges while on treatment. She had one new fracture on the seventh year of follow- up in spite of bisphosphonate treatment

Immunogenicity, safety and clinical outcomes of the SARS-CoV-2 BNT162b2 vaccine in adolescents with type 1 diabetes

IntroductionThe mRNA-based BNT162b2 (Pfizer-BioNTech) vaccine has been shown to elicit robust systemic immune response and confer substantial protection against the severe coronavirus disease (COVID-19), with a favorable safety profile in adolescents. However, no data exist regarding immunogenicity, reactogenicity and clinical outcomes of COVID-19 vaccines in adolescents with type 1 diabetes (T1D). In this prospective observational cohort study, we examined the humoral immune responses and side effects induced by the BNT162b2 vaccine, as well as, the rate and symptomatology of laboratory-confirmed COVID-19 vaccine breakthrough infections after completion of dual-dose BNT162b2 vaccination in adolescents with T1D and compared their data with those of healthy control adolescents. The new data obtained after the vaccination of adolescents with T1D could guide their further COVID-19 vaccination schedule.MethodsA total of 132 adolescents with T1D and 71 controls were enrolled in the study, of whom 81 COVID-19 infection-naive adolescents with T1D (patient group) and 40 COVID-19 infection-naive controls (control group) were eligible for the final analysis. The response of participants to the BNT162b2 vaccine was assessed by measuring their serum IgG antibodies to the spike protein of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), 4–6 weeks after the receipt of first and second vaccine doses. Data about the adverse events of the vaccine was collected after the receipt of each vaccine dose. The rate of COVID-19 vaccine breakthrough infections was evaluated in the 6-month period following second vaccination.ResultsAfter vaccinations, adolescents with T1D and controls exhibited similar, highly robust increments in anti-SARS-CoV-2 IgG titers. All the participants in the patient and control groups developed anti-SARS-CoV-2 IgG titers over 1,050 AU/ml after the second vaccine dose which is associated with a neutralizing effect. None of the participants experienced severe adverse events. The rate of breakthrough infections in the patient group was similar to that in the control group. Clinical symptomatology was mild in all cases.ConclusionOur findings suggest that two-dose BNT162b2 vaccine administered to adolescents with T1D elicits robust humoral immune response, with a favorable safety profile and can provide protection against severe SARS-CoV-2 infection similar to that in healthy adolescents

Çocuklarda pnömokok enfeksiyonlarında üriner antijen tarama testinin tanı koymadaki yeri

S. pneumoniae çocukluk çağındaki menenjit, bakteriyemi, pnömoni ve otitlerin en önemli nedenidir. Pnömokokkal enfeksiyonların tanısı halen problemdir. Binax NOW, idrarda S. pneumoniae C- polisakkarit antijenini tarayan bir hızlı immunkromotografik testtir (İKT). Biz çalışmamızda, çocuklardaki pnömokok enfeksiyonlarının tanısında İKT in kullanılabilirliğini araştırdık. Çalışmamıza 50 sağlıklı çocuk (kontrol grubu) ile sepsis, pnömoni, otit ve menenjit tanılı 50 çocuk (hasta grubu) alındı. Tüm olgulardan idrar örnekleri ve nasofaringeal kültürler elde edildi. Ayrıca hasta grubunda uygun olan olgulardan kan, transtrekeal aspirat, idrar ve BOS örnekleri alındı. Kontrol grubunda 7/50 olguda ve hasta grubunda da 10/50 olguda üriner antijen pozitifliği bulduk. Nazofarinkslerinde S. pneumoniae taşıyan 3 çocuktan (1 kontrol grubunda, 2 hasta grubunda) hepsinde pozitif IKT vardı. Biz 1 kan kültüründe, 2 BOS kültüründe olmak üzere 3 olguda pnömokok saptadık. Hepsinde İKT pozitifti. Antimikrobial tedavinin, aşılamanın ve akut faz reaktan düzeylerinin üriner antijen tarama testi üzerine etkisi yoktu. Biz, çocuklardaki pnömokoksik enfeksiyonların tanısında yüksek taşıyıcılık oranlarının yanlış pozitifliğe neden olması nedeniyle bu testin kullanılabilir olmadığı sonucuna vardık. Bu test, sadece diğer geleneksel mikrobiyolojik yöntemlerle birlikte destekleyici yöntem olarak denenebilir

Urinary antigen screening in pneumococcal infections in children

Streptococcus pneumoniae is a leading cause of meningitis, bacteremia, pneumonia and otitis media in childhood. The diagnosis of pneumococcal infections still remains problematic. Binax NOW;reg; is a rapid immunochromatographic test (ICT) that detects S. pneumoniae C-polysaccharide antigen in the urine. We evaluated the usefulness of ICT in the diagnosis of pneumococcal infections in children in our study. Fifty children with sepsis, pneumonia, otitis or meningitis (patient group) and 50 healthy children (control group) were enrolled in the study. Urine samples and nasopharyngeal cultures were obtained from all cases. Blood, transtracheal aspirate, urine and CSF (cerebrospinal fluid) cultures were also taken from the patient group when possible. We found urinary antigen positivity in 7 cases in the control group and 10 cases in the patient group. All the children who carried S. pneumoniae in their nasopharynx (1 in the control group and 2 in the patient group for a total of 3 cases) had positive ICT. We detected S. pneumoniae in 3 patients (1 blood culture and 2 CSF cultures) and ICT was positive in all. There was no effect of antimicrobial treatment, vaccination and acute phase reactant levels on the urinary antigen detection test. We concluded that this test is not useful in the diagnosis of pneumococcal infections in children as high carrier rates cause false positivity. This test can only be used with other conventional microbiological tests as a supplementary method.Streptococcus pneumoniae çocukluk çağındaki menenjit, bakteriyemi, pnömoni ve otitlerin en önemli nedenidir. Pnömokokkal enfeksiyonların tanısı halen problemdir. Binax NOW, idrarda S. pneumoniae C- polisakkarit antijenini tarayan hızlı bir immunkromotografik testtir (İKT) . Biz çalışmamızda, çocuklardaki pnömokok enfeksiyonlarının tanısında İKT in kullanılabilirliğini araştırdık. Çalışmamıza 50 sağlıklı çocuk (kontrol grubu) ile sepsis, pnömoni, otit ve menenjit tanılı 50 çocuk (hasta grubu) alındı. Tüm olgulardan idrar örnekleri ve nasofaringeal kültürler elde edildi. Ayrıca hasta grubunda uygun olan olgulardan kan, transtrekeal aspirat, idrar ve BOS (beyin omurilik sıvısı) örnekleri alındı. Kontrol grubunda 7 olguda ve hasta grubunda da 10 olguda üriner antijen pozitifliği bulduk. Nazofarinkslerinde S. pneumoniae taşıyan 3 çocuktan (1 kontrol grubunda, 2 hasta grubunda) hepsinde IKT pozitifti. Biz 1 kan kültüründe, 2 BOS kültüründe olmak üzere 3 olguda pnömokok saptadık. Hepsinde İKT pozitifti. Antimikrobial tedavinin, aşılamanın ve akut faz reaktan düzeylerinin üriner antijen tarama testi üzerine etkisi yoktu. Biz, çocuklardaki pnömokoksik enfeksiyonların tanısında yüksek taşıyıcılık oranlarının yanlış pozitifliğe yol açması nedeniyle bu testin kullanılabilir olmadığı sonucuna vardık. Bu test, sadece diğer geleneksel mikrobiyolojik yöntemlerle birlikte destekleyici yöntem olarak denenebilir

Cranial MRI abnormalities and long-term follow-up of the lesions in 770 girls with Central Precocious Puberty.

Context: Central precocious puberty (CPP) may arise from central nervous system (CNS) lesions in a few affected girls. Recently, the incidence of girls with CPP has increased mostly in 6-8 year olds, in whom the necessity of magnetic resonance imaging (MRI) is debated