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Errante P.R.,University of Sao Paulo | Franco J.L.,University of Antioquia | Espinosa-Rosales F.J.,National Institute of Pediatrics | Sorensen R.,Louisiana State University Health Sciences Center | Condino-Neto A.,University of Sao Paulo
Annals of the New York Academy of Sciences | Year: 2012

Primary immunodeficiencies (PIDs) are genetic disorders of the immune system comprising many different phenotypes. Although previously considered rare, recent advances in their clinical, epidemiological, and molecular definitions are revealing how much we still need to learn about them. For example, geographical and ethnic variations as well as the impact of certain practices influence their frequency and presentation, making it necessary to consider their study in terms of regions. The Latin American Society for Immunodeficiencies was established as an organization dedicated to provide scientific support for basic and clinical research and to develop tools and educational resources to promote awareness in the medical community. Initiatives such as these are positively influencing the way PIDs are tackled in these countries, as shown by recent reports and publications. This paper provides a historical compilation and a current view of the many issues faced by scientists studying these diseases in these countries, highlighting the diverse scientific contributions and offering a promising perspective for the further developments in this field in Latin America. © 2012 New York Academy of Sciences.


Narvaez-Rosales V.,National Institute of Pediatrics
Skinmed | Year: 2013

A 33 year-old woman presented with numerous 3- to 5-mm red-brown and yellow-brown dome-shaped nodules, primarily located on the scalp, dorsal aspects of the forearms, and lower extremities (Figure 1 and Figure 2). Her lesions started to appear 5 years prior to her consultation with increasing number and without spontaneous regression. Findings from a previous biopsy revealed epithelioid dermatofibroma. The remainder of the physical examination was unremarkable. There were no familial cases of this condition (both the mother and two older sisters were examined).


Orozco-Covarrubias L.,National Institute of Pediatrics | Lara-Carpio R.,National Institute of Pediatrics | Saez-De-Ocariz M.,National Institute of Pediatrics | Duran-Mckinster C.,National Institute of Pediatrics | And 2 more authors.
Pediatric Dermatology | Year: 2013

Dermoid cysts (DCs) are benign cutaneous tumors that tend to persist and grow. The aim of this study was to examine the clinicopathologic features of congenital DCs. We present a case series of 75 children with a clinicopathologic diagnosis of DC. Seventy-two cysts were located on the head, one on the neck, and two on the trunk. Six cysts were located along the midline. Eight patients had symptoms other than changes in cyst size. Imaging studies were performed on 15 patients. Surgical excision was the primary treatment in all 75 cases. Neurosurgery and ophthalmology services were involved in the care of some patients. Histopathologic studies reported a foreign body giant cell reaction in 17 of the cysts. No recurrence was documented. DCs can remain stable for years, but they can become symptomatic as a result of enlargement and rupture or, more rarely, as a result of extension into surrounding tissues. Physicians should be aware that certain locations have a higher risk of DC extension, and adequate diagnostic investigations should be performed before their complete resection. © 2013 Wiley Periodicals, Inc.


Rodriguez-Jurado R.,National Institute of Pediatrics | Morales S.S.,National Institute of Pediatrics
Journal of Pediatric Surgery | Year: 2010

Polypoid arteriovenous malformations, which are localized in the colon, are extremely rare in adults, with only 7 cases published to date. Here we present the case of a 6-year-old girl with a jejunal polypoid tumor that a sonogram and a computed tomographic scan have shown to resemble intussusception. Histologically, numerous large ectatic veins intermixed with small-caliber arteries, venules, arterioles, and capillaries were observed in the intestinal wall. A retrospective computed tomographic scan 3-dimensional angiographic reconstruction demonstrated that this may be a diagnostic characteristic. Clinical and morphologic comparisons with previously reported cases were discussed. © 2010 Elsevier Inc. All rights reserved.


Garcia-Romero M.T.,National Institute of Pediatrics | Arenas R.,Hospital General Dr Manuel Gea Gonzalez
Journal of Investigative Dermatology | Year: 2015

Fungal infections in humans are among the most prevalent diseases globally. Superficial invasion by dermatophytes leads to skin, hair, and nail infection. Even though they have usually been associated with extrinsic conditions such as immunosuppression, environmental factors, and contaminated individuals, objects, or surfaces; people are not equally susceptible to dermatophyte infection, even when they have the same risk factors. This commentary summarizes findings that provide evidence of familial or genetic predisposition to fungal infection, mediated by innate and/or adaptive immunity. © 2015 The Society for Investigative Dermatology.


Garcia-Contreras R.,National Institute of Cardiology | Perez-Eretza B.,National Institute of Cardiology | Lira-Silva E.,National Institute of Cardiology | Jasso-Chavez R.,National Institute of Cardiology | And 4 more authors.
Pathogens and Disease | Year: 2014

The novel antimicrobial gallium is a nonredox iron III analogue with bacteriostatic and bactericidal properties, effective for the treatment of Pseudomonas aeruginosa in vitro and in vivo in mouse and rabbit infection models. It interferes with iron metabolism, transport, and presumably its homeostasis. As gallium exerts its antimicrobial effects by competing with iron, we hypothesized that it ultimately will lead cells to an iron deficiency status. As iron deficiency promotes the expression of virulence factors in vitro and promotes the pathogenicity of P. aeruginosa in animal models, it is anticipated that treatment with gallium will also promote the production of virulence factors. To test this hypothesis, the reference strain PA14 and two clinical isolates from patients with cystic fibrosis were exposed to gallium, and their production of pyocyanin, rhamnolipids, elastase, alkaline protease, alginate, pyoverdine, and biofilm was determined. Gallium treatment induced the production of all the virulence factors tested in the three strains except for pyoverdine. In addition, as the Ga-induced virulence factors are quorum sensing controlled, co-administration of Ga and the quorum quencher brominated furanone C-30 was assayed, and it was found that C-30 alleviated growth inhibition from gallium. Hence, adding both C-30 and gallium may be more effective in the treatment of P. aeruginosa infections. © 2013 Federation of European Microbiological Societies. Published by John Wiley & Sons Ltd. All rights reserved.


Rojas P.,National Institute of Neurology and Neurosurgery | Montes P.,National Institute of Neurology and Neurosurgery | Rojas C.,National Autonomous University of Mexico | Serrano-Garcia N.,National Institute of Neurology and Neurosurgery | Rojas-Castaneda J.C.,National Institute of Pediatrics
Nutrition | Year: 2012

Ginkgo Biloba extract 761 (EGb 761) is a patented and well-defined mixture of active compounds extracted from Ginkgo biloba leaves. This extract contains two main groups of active compounds, flavonoids (24%) and terpenoids (6%). EGb 761 is used clinically to treat dementia and vaso-occlusive and cochleovestibular disorders. This extract has neuroprotective effects, exerted probably by means of its antioxidant function. Parkinson's disease (PD) is a neurodegenerative disorder that affects 2% of the population older than 60 y. It produces a progressive loss of dopaminergic neurons and depletion of dopamine (DA), leading to movement impairment. The production of reactive oxygen species, which act as mediators of oxidative damage, is linked to PD. This disease is routinely treated with the DA precursor, L-3,4-dihydroxyphenylalanine. However, this produces severe side effects, and its neurotoxic properties can be due to a free radical production. Thus, administration of antioxidant drugs might be used to prevent neuronal death produced by oxidative mechanisms. The use of synthetic antioxidants has decreased because of their suspected activity as carcinogenic promoters. We describe the studies related to the antioxidant effect of EGb 761 in an animal model of PD. It has been shown that EGb761 can provide a neuroprotective/neurorecovery effect against the damage to midbrain DA neurons in an animal model of PD. EGb 761 also has been found to lessen the impairment of locomotion, correlating with an increase of DA and other morphologic and biochemical parameters related to its antioxidant effect in an animal model of PD. These studies suggest it as an alternative in the future treatment of PD. © 2012 Elsevier Inc.


Sanchez-Huerta K.,Escuela Nacional de Ciencias Biologicas | Sanchez-Huerta K.,National Institute of Pediatrics | Pacheco-Rosado J.,Escuela Nacional de Ciencias Biologicas | Gilbert M.E.,U.S. Environmental Protection Agency
Journal of Neuroendocrinology | Year: 2015

Thyroid hormone (TH) is essential for a number of physiological processes and is particularly critical during nervous system development. The hippocampus is strongly implicated in cognition and is sensitive to developmental hypothyroidism. The impact of TH insufficiency in the foetus and neonate on hippocampal synaptic function has been fairly well characterised. Although adult onset hypothyroidism has also been associated with impairments in cognitive function, studies of hippocampal synaptic function with late onset hypothyroidism have yielded inconsistent results. In the present study, we report hypothyroidism induced by the synthesis inhibitor propylthiouracil (10 p.p.m., 0.001%, minimum of 4 weeks), resulted in marginal alterations in excitatory postsynaptic potential (EPSP) and population spike (PS) amplitude in the dentate gyrus measured in vivo. No effects were seen in tests of short-term plasticity, and a minor enhancement of long-term potentiation of the EPSP slope was observed. The most robust synaptic alteration evident in hypothyroid animals was an increase in synaptic response latency, which was paralleled by a failure to maintain normal body temperature under anaesthesia, despite warming on a heating pad. Latency shifts could be reversed in hypothyroid animals by increasing the external heat source and, conversely, synaptic delays could be induced in control animals by removing the heat source, with a consequent drop in body and brain temperature. Thermoregulation is TH- dependent, and anaesthesia necessary for surgical procedures posed a thermoregulatory challenge that was differentially met in control and hypothyroid animals. Minor increases in field potential EPSP slope, decreases in PS amplitudes and increased latencies are consistent with previous reports of hypothermia in naive control rats. We conclude that failures in thyroid-dependent temperature regulation rather than direct action of TH in synaptic physiology are responsible for the observed effects. These findings stand in contrast to the synaptic impairments observed in adult offspring following developmental TH insufficiency, and emphasise the need to control for the potential unintended consequences of hypothermia in the interpretation of hypothyroid-induced changes in physiological systems, most notably synaptic transmission. © 2014 British Society for Neuroendocrinology.


Gonzalez Saldana N.,National Institute of Pediatrics
BMC research notes | Year: 2012

Infectious mononucleosis (IM) or Mononucleosis syndrome is caused by an acute infection of Epstein-Barr virus. In Latin American countries, there are little information pertaining to the clinical manifestations and complications of this disease. For this reason, the purpose of this work was to describe the clinical and laboratory characteristics of infection by Epstein-Barr virus in Mexican children with infectious mononucleosis. A descriptive study was carried out by reviewing the clinical files of patients less than 18 years old with clinical and serological diagnosis of IM by Epstein-Barr virus from November, 1970 to July, 2011 in a third level pediatric hospital in Mexico City. One hundred and sixty three cases of IM were found. The most frequent clinical signs were lymphadenopathy (89.5%), fever (79.7%), general body pain (69.3%), pharyngitis (55.2%), hepatomegaly (47.2%). The laboratory findings were lymphocytosis (41.7%), atypic lymphocytes (24.5%), and increased transaminases (30.9%), there were no rupture of the spleen and no deaths among the 163 cases. Our results revealed that IM appeared in earlier ages compared with that reported in industrialized countries, where adolescents are the most affected group. Also, the order and frequency of the clinical manifestations were different in our country than in industrialized ones.


Cardenas-Rodriguez N.,National Institute of Pediatrics | Huerta-Gertrudis B.,National Institute of Pediatrics | Rivera-Espinosa L.,National Institute of Pediatrics | Montesinos-Correa H.,National Institute of Pediatrics | And 3 more authors.
International Journal of Molecular Sciences | Year: 2013

Oxidative stress, a state of imbalance in the production of reactive oxygen species and nitrogen, is induced by a wide variety of factors. This biochemical state is associated with systemic diseases, and diseases affecting the central nervous system. Epilepsy is a chronic neurological disorder with refractoriness to drug therapy at about 30%. Currently, experimental evidence supports the involvement of oxidative stress in seizures, in the process of their generation, and in the mechanisms associated with refractoriness to drug therapy. Hence, the aim of this review is to present information in order to facilitate the handling of this evidence and determine the therapeutic impact of the biochemical status for this pathology. © 2013 by the authors; licensee MDPI, Basel, Switzerland.

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