Pubertal Suppression, Bone Mass, and Body Composition in Youth With Gender Dysphoria.

BACKGROUND AND OBJECTIVES: Puberty onset and development contribute substantially to adolescents' bone mass and body composition. Our objective with this study was to examine the effects of gonadotropin-releasing hormone agonists (GnRHa) on these puberty-induced changes among youth with gender dysphoria (GD). METHODS: Medical records of the endocrine diversity clinic in an academic children's hospital were reviewed for youth with GD seen from January 2006 to April 2017 with at least 1 baseline dual-energy radiograph absorptiometry measurement. RESULTS: At baseline, transgender females had lower lumbar spine (LS) and left total hip (LTH) areal bone mineral density (aBMD) and LS bone mineral apparent density (BMAD) z scores. Only 44.7% of transgender youth were vitamin D sufficient. Baseline vitamin D status was associated with LS, LTH aBMD, and LS BMAD z scores. Post-GnRHa assessments revealed a significant drop in LS and LTH aBMD z scores (transgender males and transgender females) without fractures and LS BMAD (transgender males), an increase in gynoid (fat percentage), and android (fat percentage) (transgender males and transgender females), and no changes in BMI z score. CONCLUSIONS: GnRHa monotherapy negatively affected bone mineral density of youth with GD without evidence of fractures or changes in BMI z score. Transgender youth body fat redistribution (android versus gynoid) were in keeping with their affirmed gender. The majority of transgender youth had vitamin D insufficiency or deficiency with baseline status associated with bone mineral density. Vitamin D supplementation should be considered for all youth with GD.

Primary immunodeficiencies associated with eosinophilia.

BACKGROUND: Eosinophilia is not an uncommon clinical finding. However, diagnosis of its cause can be a dilemma once common culprits, namely infection, allergy and reactive causes are excluded. Primary immunodeficiency disorders (PID) are among known differentials of eosinophilia. However, the list of PIDs typically reported with eosinophilia is small and the literature lacks an inclusive list of PIDs which have been reported with eosinophilia. This motivated us to review the literature for all PIDs which have been described to have elevated eosinophils as this may contribute to an earlier diagnosis of PID and further the understanding of eosinophilia. METHODS: A retrospective PubMed, and Google Scholar search using the terms "eosinophilia" and "every individual PID" as classified by Expert Committee of the International Union of Immunological Societies with the limit of the English language was performed. Results were assessed to capture case(s) which reported eosinophilia in the context of PID conditions. Absolute eosinophil counts (AEC) were retrieved from manuscripts whenever reported. RESULTS: In addition to the typical PID conditions described with eosinophilia, we document that MHC class II deficiency, CD3γ deficiency, STAT1 deficiency (AD form), Kostmann disease, cyclic neutropenia, TCRα deficiency, Papillon-Lefevre syndrome, CD40 deficiency, CD40L deficiency, anhidrotic ectodermal dysplasia with immune deficiency, ataxia-telangiectasia, common variable immunodeficiency disorders (CVID), Blau syndrome, CARD9 deficiency, neonatal onset multisystem inflammatory disease or chronic infantile neurologic cutaneous and articular syndrome (NOMID/CINCA), chronic granulomatous disease, MALT1 deficiency and Roifman syndrome have been noted to have elevated eosinophils. Severe eosinophilia (>5.0 × 10(9)/L) was reported in Omenn syndrome, Wiskott Aldrich syndrome, ADA deficiency, autoimmune lymphoproliferative syndrome, immunodysregulation polyendocrinopathy enteropathy X-linked, STAT3 deficiency, DOCK8 deficiency, CD40 deficiency, MHC II deficiency, Kostmann disease, Papillon-Lefevre syndrome, and CVID. CONCLUSIONS: This literature review shows that there is an extensive list of PIDs which have been reported with eosinophilia. This list helps clinicians to consider an extended differential diagnoses when tasked with exclusion of PID as a cause for eosinophilia.

An overview and mapping of childhood tuberculosis: prevalence, scientific production and citation analysis.

AIM: The study aimed to analyze publications in the field of "pediatric tuberculosis" and associate them with regional Tuberculosis (TB) profile. METHODS: A schematic analysis of scientific production in the field of pediatric tuberculosis between 1990 and 2010 using ISI web of science was carried out. Terms used for searches were each as a combination of "Mycobacterium Tuberculosis", or "Tuberculos*" and "Child", or "Infant", or "New born", or "Neonatal", or "Adolescent", or "Pediatric". Features including year of publication, citation per year, country of origin, funding state, contributing university, language, leading journals and authors, and highly cited articles, main journal publishing these articles were taken into account. RESULTS: The search retrieved 3417 articles (of 4559 total) with an almost gradual annually progressive pattern from 20 (in 1990) to 302 (in 2009) which have been cited totally 48459 times and 14.18 times per article. The greatest contribution originated from United States of America (25.11%) followed by South Africa (12.17%), and England (11.18%). Interestingly, 82.4% of all South African articles were from Stellenbosch University and Cape Town University on contrary. CONCLUSIONS: International Journal of Tuberculosis and Lung Disease ranked as the first with regard to the number of articles and Lancet with regard to the number of highly cited articles. Developing countries excluding South Africa despite their high prevalence scarcely contribute to the field and USA is the leading country in the field.

Frequency and Susceptibility of Bacteria Caused Urinary Tract Infection in Neonates: Eight-Year Study at Neonatal Division of Bahrami Children's Hospital, Tehran Iran.

BACKGROUND: Susceptibility pattern of organisms causing urinary tract infection (UTI) in neonate would potentially improve the clinical management by enabling clinicians to choose most reasonable first line empirical antibiotics. This study aimed to this end by studying isolated organisms from neonates with UTI in an inpatient setting. METHODS: Current retrospective study has recruited all cases of neonatal UTI diagnosed through a suprapubic/catheterized sample, admitted to Neonatal Division of Bahrami Children's Hospital, Tehran, Iran, from June 2004 to June 2012. RESULTS: Escherichia coli was the dominant (64.4%) bacteria among a total of 73 cases (69.9% boys and 30.1% girls; aged 14.14 ± 7.68 days; birth weight of 3055.85 ± 623.00 g) and Enterobacter (19.2%), Klebsiella (12.3%), and Staphylococcus epidermdisis (4.1%) were less frequent isolated bacteria. E. coli was mostly resistant to ampicillin (93.6%), cefixime (85.7%) and cephalexin (77.3%), and sensitive to cefotaxime (63.6%). Enterobacter found to be most resistant to amikacin (100%), ampicillin (92.85%), and most sensitive to ceftizoxime (71.4%). CONCLUSION: A high ratio (> 92.85%) of resistance toward ampicillin was observed among common neonatal UTI bacterial agents. Having this finding along with previous reports of emerging resistance of neonatal uropathogensto ampicillin could be a notion that a combination of a third generation cephalosporin and an aminoglycoside would be a more reasonable first choice than ampicillin plus an aminoglycoside.

Neonatal urinary tract infection: clinical response to empirical therapy versus in vitro susceptibility at Bahrami Children's Hospital- Neonatal Ward: 2001-2010.

Urinary tract infection (UTI) is a neonatal life threatening infection which is usually treated with ampicillin plus an aminoglycoside or a third-generation cephalosporin. Recently, growing number of Escherchia coli species resistant to ampicillin and aminoglycosides have raised concerns regarding the necessity to change the empirical therapy. This motivates us to determine neonatal UTI clinical response to the used empirical antibiotics. This study was designed as a Case Series. All neonates admitted to Bahrami Children Hospital, Tehran, Iran, during 2001- 2010 with a diagnosis of UTI surveyed by simple non-random sampling. Totally, 97 cases (including 83 (85.6%) term, 8 (8.2%) post-term and 6 (6.2%) preterm neonates) with a mean age of 15.85 ± 7.05 days at admission ,average weight of 3195.57 ± 553g at birth and 3276.29 ± 599.182 g at admission were studied. Ampicillin resistance in 93 cases (95.9%), gentamicin resistance in 51 cases (52.6%) and trimethoprim- sulfamethoxazole resistance in 44 cases (45.4%) were the leading resistances in this study. Escherichia coli was the dominant organism in 76.3% (74 patients) of study population which was resistant to ampicillin in 95.9% (71 cases). Despite the observed resistant to initial empirical regimen antibiotics (especially ampicillin), 81.4% of patients responded to empirical therapy. However, we believe till conductance of more detailed studies regarding the relationship between empirical therapy and antibiogram concordance, physicians take ampicillin-resistant E coli infection issue into accounts from the first steps of management of critically ill neonates.

An estimation of steroid responsiveness of idiopathic nephrotic syndrome in Iranian children.

OBJECTIVE: Idiopathic Nephrotic syndrome (INS) is the most common form of nephrotic syndrome (NS) in children with the potential of progression to end stage renal disease (ESRD). INS is steroid-responsive in most children, but not all patients respond to it. The aim of this study was to determine the rate of steroid responsiveness in children with INS that referred to Children's Medical Center since 1995 to 2007. METHODS: In as a cross sectional study, the medical records of all children with INS aged 1 to 15 years who were referred to our referral hospital was reviewed. All patients with onset of disease less than 1 year of age, spontaneous remission, secondary forms of NS associated with systemic diseases, and follow up duration of less than 12 months were excluded from the study. Patients were categorized into 6 groups: Group 1 needed biopsy prior to any treatment, group 2 non-relapsing NS, group 3 infrequently relapsing NS, Group 4 frequently relapsing NS, group 5 steroid dependent NS and group 6 steroid resistant NS. FINDINGS: A total of 238 patients were enrolled in the study. Kidney biopsy was performed in 79 cases. Minimal change lesion (MCL) was the most common (36.7%) pathological diagnosis. Steroid responsiveness was found in 81.5% of all cases including: 96% of MCL (consisting of biopsy proven cases and presumed ones), 32% of focal and segmental glomerulosclerosis, 73% of diffuse mesangial proliferation and 58% of membranoproliferative glomerulonephritis patients. During minimal follow up period of 12 months, there were 194 patients in remission, 32 patients with active NS, and 12 patients in ESRD. CONCLUSION: Our study results showed that 81.5% of all patients, 96.2% of MCL and 32% of FSGS patients initially responded to steroid therapy.