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Chemes H.E.,CONICET | Venara M.,CONICET | del Rey G.,CONICET | Arcari A.J.,Hospital de Ninos Dr. Ricardo Gutierrez | And 4 more authors.
Andrology | Year: 2015

All malignant testicular germ cell tumors (TGCT) of adult men are preceded by an in situ stage (CIS) of protracted evolution. The adult CIS is well characterized, but there is debate on the phenotype of infantile CIS, its distinction from delayed maturation of germ cells and prognostic potential. A large series of 43 patients with Disorders of Sex Development (DSD) and dysgenetic testes (90% ranging from neonates to 12 years, mean age 4.7 years), was studied by quantifying dysgenetic features, degree of germ cell abnormalities/atypia (GCA), expression of OCT 3/4 (a pluripotency-undifferentiation marker), germ cell ploidy and evolution to CIS and invasive TGCT. Findings were compared with those of normal testes. The type of gonads present defined three groups of patients: bilateral testes (BT-DSD, n = 21), one testis and one streak gonad (CT-DSD, C for combined, n = 13), and ovarian-testicular combinations (OT-DSD, n = 9). There were 5 boys with infantile CIS, bilateral in 3 (total of 8 infantile CIS) and two patients with adult CIS, bilateral in one (total of 3 adult CIS). Two patients had bilateral seminomas one at 12-17 and the other at 23 years. Histological dysgenesis was significantly higher in CT-DSD (p < 0.05), that had only 1 CIS. The highest frequency of GCA was in BT-DSD (p < 0.05), which coincided with a total of 11CIS + Seminomas. In all patients, aneuploidy was significantly higher (63%) than diploidy (p < 0.02), and GCA were more frequent in aneuploid than in diploid samples (p < 0.02). All CIS and TGCT were OCT 3/4 positive. Finally, there was a significant association between the triad Aneuploidy + GCA + OCT 3/4 positivity and the incidence of CIS (Fisher Exact test p < 0.002, relative risk 7.0). The degree of testicular dysgenesis (derived from abnormal organization of Sertoli cells in fetal testicular cords) is inversely related to the incidence of CIS. Our data demonstrate that the combined use of OCT 3/4 expression, quantification of germ cell abnormalities-atypia and ploidy in dysgenetic testes can satisfactorily identify infantile CIS with high risk of malignant evolution and set it aside from delayed germ cell maturation with lower or nil neoplastic potential. © 2014 American Society of Andrology and European Academy of Andrology.

Kreutzer G.O.,Hospital de Ninos Dr. Ricardo Gutierrez
Revista Argentina de Cardiologia | Year: 2011

The evolutionary process that led to the development of the Fontan-Kreutzer (FK) procedure was determined by the evolution in thought in pediatric cardiology since the pioneer Dr. Rodolfo O. Kreutzer created the department of cardiology, with the help of Dr. Alberto Rodríguez Coronel in the catheterization laboratory and Dr. Luis Becú in charge of department of anatomic pathology. The first atriopulmonary anastomosis (APA) was performed in our environment ignoring that Fontan had described his procedure, that consisted of "ventricularization" of the right atrium (RA) inserting a valve in inferior vena cava (IVC) inlet. Our technique was different and based on the concept that end-diastolic pressure of the main ventricular chamber is the power source necessary to serve the pulmonary circulation. We introduced a valve in the IVC and developed the concept of atrial baffle fenestration. In 1971 we described two techniques of APA (one using a homograft and the other with the patient's own pulmonary artery), and in 1978 we developed a largest, posterior, nonvalved direct APA. Since 1987, new and improved techniques have been developed: lateral tunnel (LT) and extracardiac conduit (EC), which minimize kinetic energy loss as opposed to the RA, a volume chamber. Evidently, the FK procedure is the best option to treat single ventricle (SV); yet life with a SV is not easy. Many patients evolve with progressive impairment of the system due to chronic low volume, increased central venous pressure and edema. However, these abnormalities are not present in all cases, as demonstrated by the longest-lived survivor of the world after 35 years of follow-up. Indeed, the FK procedure is a palliative surgical procedure and one of the achievements in congenital heart surgery.

Bardach A.,Institute for Clinical Effectiveness and Health Policy | Ciapponi A.,Institute for Clinical Effectiveness and Health Policy | Garcia-Marti S.,Institute for Clinical Effectiveness and Health Policy | Glujovsky D.,Institute for Clinical Effectiveness and Health Policy | And 4 more authors.
International Journal of Pediatric Otorhinolaryngology | Year: 2011

Background: Acute otitis media (AOM) is one of the most common childhood diseases requiring antimicrobial prescription in pre-school children. This systematic review aimed to estimate the AOM incidence, bacterial etiology and use of resources in children aged <6 years in Latin America and the Caribbean (LA&C). Methods: A systematic search using keywords otitis or middle ear and inflammation was performed for articles published during 1988-2008 in MEDLINE, Cochrane Library, EMBASE, LILACS, generic and academic internet searches, Ministries of Health, PAHO, regional proceedings, reference lists and consulting experts. Pairs of reviewers independently selected articles and assessed their methodological quality with a checklist of essential items from the STROBE statement according to pre-specified criteria. Studies involving immune-competent children with AOM were considered. Arcsine transformations were used for proportion meta-analyses. Results: Annual AOM incidence in four studies in children aged <5 years ranged from 1,171-36,000 episodes/100,000 children. Meta-analysis on etiology and pneumococcal serotypes included 18 studies and 125, 519 children with AOM from six LA&C countries. Meta-analysis per serotype showed that Streptococcus pneumoniae (32.4%; 95%CI. =27.1-38.0%) and Haemophilus influenzae (26%; 95%CI. =19.5-33.1%), including non-typeable H. influenzae (18.3%; 95%CI. =9.5-33.1%) were the most prevalent. The most commonly observed pneumococcal serotype was 19F (24.0%; 95% CI 17.0-32.0%). Data on use of health resources were scarce. Conclusions: Streptococcus pneumoniae and H. influenzae were the most frequent AOM bacterial pathogens, consistent with the international literature from other regions. Future studies on AOM incidence and health resources usage will help better define the impact of this disease. © 2011 Elsevier Ireland Ltd.

Cox T.M.,University of Cambridge | Drelichman G.,Hospital de Ninos Dr. Ricardo Gutierrez | Cravo R.,State Institute Of Haematology Arthur Of Siqueira Cavalcanti | Balwani M.,Mount Sinai Hospital | And 7 more authors.
The Lancet | Year: 2015

Background The mainstay of treatment for Gaucher's disease type 1 is alternate-week infusion of enzyme replacement therapy (ERT). We investigated whether patients stable on such treatment would remain so after switching to oral eliglustat, a selective inhibitor of glucosylceramide synthase. Methods In this phase 3, randomised, multinational, open-label, non-inferiority trial, we enrolled adults (aged 18 years) who had received ERT for 3 years or more for Gaucher's disease. Patients were randomly allocated 2:1 at 39 clinics (stratified by ERT dose; block sizes of four; computer-generated centrally) to receive either oral eliglustat or imiglucerase infusions for 12 months. Participants and investigators were aware of treatment assignment, but the central reader who assessed organ volumes was masked. The composite primary efficacy endpoint was percentage of patients whose haematological variables and organ volumes remained stable for 12 months (ie, haemoglobin decrease not more than 15 g/L, platelet count decrease not more than 25%, spleen volume increase not more than 25%, and liver volume increase not more than 20%, in multiples of normal from baseline). The non-inferiority margin was 25% for eliglustat relative to imiglucerase, assessed in all patients who completed 12 months of treatment. This trial is registered with ClinicalTrials.gov, number NCT00943111, and EudraCT, number 2008-005223-28. Findings Between Sept 15, 2009, and Nov 9, 2011, we randomly allocated 106 (66%) patients to eliglustat and 54 (34%) to imiglucerase. In the per-protocol population, 84 (85%) of 99 patients who completed eliglustat treatment and 44 (94%) of 47 patients who completed imiglucerase treatment met the composite primary endpoint (between-group difference -8·8%; 95% CI -17·6 to 4·2). The lower bound of the 95% CI of -17·6% was within the prespecified threshold for non-inferiority. Dropouts occurred due to palpitations (one patient on eliglustat), myocardial infarction (one patient on eliglustat), and psychotic disorder (one patient on imiglucerase). No deaths occurred. 97 (92%) of 106 patients in the eliglustat group had treatment-emergent adverse events, as did 42 (79%) of 53 in the imiglucerase group (mostly mild or moderate in severity). Interpretation Oral eliglustat maintained haematological and organ volume stability in adults with Gaucher's disease type 1 already controlled by intravenous ERT and could be a useful therapeutic option. Funding Genzyme, a Sanofi company. © 2015 Elsevier Ltd.

Gentile A.,Hospital de Ninos Dr. Ricardo Gutierrez
Archivos Argentinos de Pediatria | Year: 2010

Physiopathological, clinical, and epidemiological aspects of whooping cough, an acute highly contagious respiratory infection caused by Bordetella pertussis, are described. Different vaccination schedules are mentioned, since the vaccine was introduced, more than 40 years ago, until present; and the outbreacks ocurred in Argentina during different periods. Moreover, the recrudescense of the disease observed in several countries during last years is highlighted, particularly in preschool children, and even more in adolescents, and young adults.

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