Wilson J.,University of Texas Southwestern Medical Center |
Rivarola M.,Garrahan Pediatric Hospital |
Mendonca B.,University of Sao Paulo |
Warne G.,Royal Melbourne Hospital |
And 4 more authors.
Seminars in Reproductive Medicine | Year: 2012
The birth of a child with ambiguous genitalia is a challenging and distressing event for the family and physician and one with life-long consequences. Most disorders of sexual differentiation (DSD) associated with ambiguous genitalia are the result either of inappropriate virilization of girls or incomplete virilization of boys. It is important to establish a diagnosis as soon as possible, for psychological, social, and medical reasons, particularly for recognizing accompanying life-threatening disorders such as the salt-losing form of congenital adrenal hyperplasia. In most instances, there is sufficient follow-up data so that making the diagnosis also establishes the appropriate gender assignment (infants with congenital adrenal hyperplasia, those with androgen resistance syndromes), but some causes of DSD such as steroid 5α-reductase 2 deficiency and 17β-hydroxysteroid dehydrogenase deficiency are associated with frequent change in social sex later in life. In these instances, guidelines for sex assignment are less well established. Copyright © 2012 by Thieme Medical Publishers, Inc.
Vazquez N.M.,CONICET |
Fiorilli G.,Garrahan Pediatric Hospital |
Caceres Guido P.A.,Garrahan Pediatric Hospital |
Caceres Guido P.A.,Maimónides University |
And 2 more authors.
Phytomedicine | Year: 2016
Background Methicillin-resistant Staphylococcus aureus (MRSA) is resistant to different commonly used antibiotics, stressing the need for further strategies to treat this human pathogen with worldwide prevalence. The use of phytochemicals within the current pharmacology is a promising approach to enhance the antimicrobial activity of common antibiotics in the battle against these bacteria. Purpose The purpose of this study was to determine the antimicrobial effectiveness of carnosic acid, the major constituent of Rosmarinus officinalis L. leaves, in combination with gentamicin against multi-drug resistant MRSA clinical isolates obtained from pediatric patients with bacteremia. Materials and methods Anti-MRSA activity was studied using the broth microdilution assay and time–kill method. Combinations of subinhibitory concentrations of carnosic acid and gentamicin were examined using the checkerboard method. Results Carnosic acid exhibited a good antibacterial activity against all multidrug-resistant MRSA clinical isolates tested, which are resistant to four up to nine antibiotics. In addition, the combination of carnosic acid with gentamicin not only decreased the minimal inhibitory concentration (MIC) of both by 4- to 5-fold, but also improved the bactericidal potency of the common antibiotic by 32- to 40-fold against both gentamicin-susceptible and gentamicin-resistant MRSA clinical isolates. A clear bactericidal synergistic activity between carnosic acid and gentamicin in killing multidrug-resistant MRSA clinical isolates with a fractional bactericidal concentration index (FBCI) of 0.28–0.35 was demonstrated. Conclusions Our findings show the potential use of carnosic acid in combination with gentamicin as a promising alternative for the control of healthcare-associated infections caused by multidrug-resistant MRSA. © 2016 Elsevier GmbH
Abiuso A.M.B.,CONICET |
Berensztein E.,Garrahan Pediatric Hospital |
Pagotto R.M.,CONICET |
Pagotto R.M.,Institute Pasteur Of Montevideo |
And 9 more authors.
Journal of Endocrinology | Year: 2014
The histamine H4 receptor (HRH4), discovered only 13 years ago, is considered a promising drug target for allergy, inflammation, autoimmune disorders and cancer, as reflected by a steadily growing number of scientific publications and patent applications. Although the presence of HRH4 has been evidenced in the testis, its specific localization or its role has not been established. Herein, we sought to identify the possible involvement of HRH4 in the regulation of Leydig cell function. We first evaluated its expression in MA-10 Leydig tumor cells and then assessed the effects of two HRH4 agonists on steroidogenesis and proliferation. We found that HRH4 is functionally expressed in MA-10 cells, and that its activation leads to the inhibition of LH/human chorionic gonadotropin-induced cAMP production and StAR protein expression. Furthermore, we observed decreased cell proliferation after a 24-h HRH4 agonist treatment. We then detected for the sites of HRH4 expression in the normal rat testis, and detected HRH4 immunostaining in the Leydig cells of rats aged 7-240 days, while 21-day-old rats also presented HRH4 expression in male gametes. Finally, we evaluated the effect of HRH4 activation on the proliferation of normal progenitor and immature rat Leydig cell culture, and both proved to be susceptible to the anti-proliferative effect of HRH4 agonists. Given the importance of histamine (2-(1H-imidazol-4-yl)ethanamine) in human (patho)physiology, continued efforts are directed at elucidating the emerging properties of HRH4 and its ligands. This study reveals new sites of HRH4 expression, and should be considered in the design of selective HRH4 agonists for therapeutic purposes. © 2014 Society for Endocrinology.
Moreno F.,National Cancer Institute |
Lopez Marti J.,Garrahan Pediatric Hospital |
Palladino M.,Garrahan Pediatric Hospital |
Lobos P.,Italian Hospital |
And 2 more authors.
Pediatric Blood and Cancer | Year: 2016
Background: There are reports indicating a low incidence of neuroblastoma (NB) in some developing countries but no conclusive data are available from population-based studies at a national level. Purpose: To describe the incidence and survival of 971 patients with NB in Argentina with data from the National Pediatric Cancer Registry (ROHA), and the impact of age, gender, stage, regional, and socioeconomic indicators on outcome. Methods: All cases of NB reported to ROHA (2000–2012) were the subject of the analysis. Annual-standardized incidence rate (ASR) was calculated using the National Vital Statistics and survival was estimated. The extended human development index (EHDI) was used as the socioeconomic indicator. Results: ASR was 8.3/1,000,000 children (0–14 years) and remained stable along this period. Regional variation in ASR ranged from 3.4 in the Northwest to 9.8 in the Central region, being most marked in the first year of life. Five-year survival rate (SR) was 47%, with no sex difference. For patients older than 18 months, it was 36%, for stage IV 23%, for those born in the Northeast region 38%, and for those with an amplified MYCN 15%. Residents in provinces with a higher EHDI had a better 5-year survival (57% vs. 41% for lower EHDI) and higher ASR (12.3 vs. 5.6 for lower EHDI). Stage and MYCN status showed an independent inferior prognosis. Conclusions: ASR of NB in Argentina is lower than in developed countries, with considerable regional variation. SRs are also lower than in developed countries. © 2016 Wiley Periodicals, Inc.
PubMed | National Academy of Medicine, Garrahan Pediatric Hospital, Italian Hospital and National Cancer Institute
Type: Journal Article | Journal: Pediatric blood & cancer | Year: 2016
There are reports indicating a low incidence of neuroblastoma (NB) in some developing countries but no conclusive data are available from population-based studies at a national level.To describe the incidence and survival of 971 patients with NB in Argentina with data from the National Pediatric Cancer Registry (ROHA), and the impact of age, gender, stage, regional, and socioeconomic indicators on outcome.All cases of NB reported to ROHA (2000-2012) were the subject of the analysis. Annual-standardized incidence rate (ASR) was calculated using the National Vital Statistics and survival was estimated. The extended human development index (EHDI) was used as the socioeconomic indicator.ASR was 8.3/1,000,000 children (0-14 years) and remained stable along this period. Regional variation in ASR ranged from 3.4 in the Northwest to 9.8 in the Central region, being most marked in the first year of life. Five-year survival rate (SR) was 47%, with no sex difference. For patients older than 18 months, it was 36%, for stage IV 23%, for those born in the Northeast region 38%, and for those with an amplified MYCN 15%. Residents in provinces with a higher EHDI had a better 5-year survival (57% vs. 41% for lower EHDI) and higher ASR (12.3 vs. 5.6 for lower EHDI). Stage and MYCN status showed an independent inferior prognosis.ASR of NB in Argentina is lower than in developed countries, with considerable regional variation. SRs are also lower than in developed countries.
Ciaccio M.,Garrahan Pediatric Hospital |
Gil S.,Garrahan Pediatric Hospital |
Guercio G.,Garrahan Pediatric Hospital |
Vaiani E.,Garrahan Pediatric Hospital |
And 5 more authors.
Hormone Research in Paediatrics | Year: 2010
Background: GH deficiency (GHD) and spine irradiation (SI) have been implicated in the mechanism of reduced adult height (AH) in childhood survivors of medulloblastoma. However, growth dynamics after tumor diagnosis and the effectiveness of rhGH on AH in comparison with rhGH-untreated survivors have not been reported. Aim: To follow height (H) SDS (HSDS) since tumor diagnosis and the effect of rhGH in GHD patients, comparing with GH-untreated GHD patients. Methods: 14 patients received rhGH treatment until AH (medulloblastoma GH-treated group, MGHGr). 19 patients refused rhGH therapy (GH-untreated control medulloblastoma group, MCGr). Standing H and sitting H (SitH) were measured. Results: In MGHGr, mean ± SD HSDS decreased from 0.09 ± 0.63 at tumor diagnosis to -1.38 ± 0.91 at diagnosis of GHD, and to -1.90 ± 0.72 at the onset of rhGH, p < 0.01, but it remained unchanged during rhGH (AH -2.12 ± 0.55). MCGr HSDS (-0.25 ± 0.88) was not different from MGHGr at tumor diagnosis, but it was -3.40 ± 0.88 at AH, significantly lower than in MGHGr, p = 0.001. SitH SDS at AH (-4.56 ± 0.82) was significantly lower than at the onset of rhGH (-2.86 ± 0.75), p = 0.003, and it was not different from MCGr (-4.85 ± 1.77). Conclusions: rhGH treatment improves AH in GH-deficient childhood medulloblastoma survivors but not spinal growth. Copyright © 2010 S. Karger AG, Basel.
Perez M.F.,University of Buenos Aires |
de Torres M.E.,University of Buenos Aires |
Bujan M.M.,Garrahan Pediatric Hospital |
Lanoel A.,Garrahan Pediatric Hospital |
And 2 more authors.
Anais Brasileiros de Dermatologia | Year: 2011
Neonatal lupus erythematosus is a very rare disease, clinically characterized by skin lesions that resemble those of subacute or discoid lupus erythematosus and/or congenital heart block. Generally, when patients have skin manifestations, they have no cardiac defects and vice-versa; however, in 10% of cases these manifestations may coexist. Other findings may include hematologic, hepatic and neurological abnormalities. This condition is caused by the transplacental passage of maternal autoantibodies against Ro (95%), La and, less frequently, U1-ribonucleoprotein (U1-RNP). The present case report describes four patients with clinical, histopathological and immunological findings compatible with neonatal lupus erythematosus, their treatment and progress. © 2011 by Anais Brasileiros de Dermatologia.
Pagani S.,University of Pavia |
Chaler E.A.,Garrahan Pediatric Hospital |
Meazza C.,University of Pavia |
Maceiras M.,Garrahan Pediatric Hospital |
And 8 more authors.
Journal of Pediatric Endocrinology and Metabolism | Year: 2010
We analyzed the ability of the BaF3 cell line bioassay to select patients with biologically inactive GH. We first evaluated the biological response of the Ba/F3-hGHR cells to rhGH additional doses from 10 to 5000 pg/ml. The concentration points corresponding to the linear part of the curve were selected. We then analyzed a group of sera, diluted like the standard, including the entire range of GH concentrations that can be analyzed by bioassay. The serum/standard area below the curve ratio was calculated. Serum GH immunoactivity determined by IMMULITE/ GH bioactivity ratios was calculated. Our experimental data showed that GH-bioactivity/GH-immunoactivity ratios below 0.303 are indicative of a bioinactive GH molecule. This bioassay would recognize only extreme cases of GH bioinactivity, and it would not be a useful tool in the search for patients with altered forms of GH. © Freund Publishing House Ltd.
PubMed | Garrahan Pediatric Hospital
Type: Journal Article | Journal: Journal of pediatric endocrinology & metabolism : JPEM | Year: 2010
Serum IGF-I and IGFBP-3 assays are used to monitor rhGH treatment. Some discrepancies in results obtained by means of different assays have been reported. The aim of this study was to establish normal ranges for circulating IGF-I and IGFBP-3 in children and adolescents of Hispanic and Italian origin. Circulating levels of IGF-I and IGFBP-3 were measured in 169 Hispanic and Italian prepubertal children and 66 adolescents of both sexes, using a chemiluminescent assay. Serum levels of IGF-I and IGFBP-3 increased from early childhood into adolescence. After pubertal peaks of IGF-I and IGFBP-3, slight decreases were observed with increasing age. Furthermore, serum IGF-I levels were significantly higher in girls than in boys, suggesting a sexual dimorphism in serum IGF-I values in late prepuberty and early puberty. Differences in IGF-I and IGFBP-3 absolute values between our study and previous studies suggest the need to establish reference ranges for each ethnic group.