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The Omenn syndrome is a form of severe combined immune deiciency that is inherited autosomal recessively and characterized by growth retardation, erythrodermia, lymphadenopathy, hepatosplenomegaly and severe recurrent infections. Although circulating T lymphocytes on the lymphocyte panel are normal, their functions are abnormal. On the other hand, circulating B lymphocytes are decreased or absent. Our case was a baby girl who was born to the second pregnancy of a 29-year-old mother on the 37th gestational week. She had a history of a sister's death on the 51st day of life due to Omenn syndrome. There was no consanguinity between parents. Her physical examination was normal expect generalized exfoliative erythrodermia. Routine laboratory investigations revealed eosinophilia. X-ray, echocardiography, abdominal and transfontanel ultrasonography indings were normal. IgG was at the lowest boundaries of the normal values. IgA and IgM were low as well. While T lymphocytes were elevated, B lymphocytes were extremely low. The family had a history of a baby death with the homozygote RAG1 g.854C>T (p.Q248X) mutation and parents were heterozygote carriers of the same mutation. Our patient also had the same homozygote RAG1 g.854C>T mutation. Since the parents' immunoglobulin levels and lymphocyte panels were normal, haploidentical stem cell transplantation from the father was performed. Unfortunately our patient passed away due to pulmonary infection on the posttransplantation 12th day. Genetic counseling has a great signiicance as untreated Omenn syndrome is fatal but the disorder is treatable with appropriate bone marrow or umbilical cord blood stem cell transplantion. [ABSTRACT FROM AUTHOR]

Background: Healthcare-associated infections results in increased health care costs and mortality. There are limited studies concerning the distribution of the etiologic agents and the resistance patterns of the microorganisms causing healthcare-associated urinary tract infections (HA-UTI) in pediatric settings., Objectives: The aim of this study was to evaluate the distribution and antibiotic susceptibility patterns of pathogens causing HA-UTI in children., Material and Methods: Isolates from 138 children with UTI who were hospitalized in pediatric, neonatal and pediatric surgery intensive care units were reviewed., Results: Most common isolated organism was Klebsiella pneumoniae (34.1%) and Escherichia coli (26.8%). Among the Pseudomonas aeruginosa, Meropenem and imipenem resistance rates were 46.2% and 38.5%. Extended-spectrum beta-lactamase (ESBL) production was present in 48 Klebsiella species (82.8%). Among ESBL positive Klebsiella species, the rate of meropenem and imipenem resistance was 18.8%, and ertapenem resistance was 45.9%. Extended spectrum beta-lactamase production was present in 27 (72.9%) Escherichia coli species. Among ESBL positive E. coli, the rate of meropenem and imipenem resistance was 7.4%, and ertapenem resistance was 14.8., Conclusions: Emerging meropenem resistance in P. aeruginosa, higher rates of ertapenem resistance in ESBL positive ones in E. coli and Klebsiella species in pediatric nosocomial UTI are important notifying signs for superbug infections., Competing Interests: Competing interests: The authors have declared that no competing interests exist.