Educational Impact of Targeted Neonatal Echocardiography and Hemodynamics Programs on Neonatal-Perinatal Medicine Fellows.

Objective: Targeted neonatal echocardiography (TNE) is a real-time cardiac imaging modality used by a hemodynamics program to aid in diagnosis, treatment, and monitoring of neonatal cardiovascular illness. This study aimed to describe trainees' perspectives on existing hemodynamics education and perceived impacts of TNE and hemodynamics services on their education.

Study Design: This was a mixed quantitative and qualitative study that surveyed neonatal-perinatal medicine (NPM) fellow trainees in Canada and the United States, at programs both with and without a hemodynamics service.

TP63-mutation as a cause of prenatal lethal multicystic dysplastic kidneys.

Background: Ectrodactyly-ectodermal dysplasia-clefting syndrome 3 (EEC) is one of the six overlapping syndromes caused by mutations in the tumor protein p63 gene (TP63). EEC is suspected when patients have cleft hands or feet, polydactyly, and syndactyly, abnormal development of the ectodermally derived structures, and orofacial clefting. Genitourinary (GU) anomalies have been identified in patients with EEC, yet these are often under-recognized and under-reported.

Paroxysmal nocturnal haemoglobinuria in a patient with primary Budd-Chiari syndrome: a contraceptive challenge.

Case Report: In this report, we describe the unique case of a 21 year-old woman, gravida 1, para 1, with paroxysmal nocturnal haemoglobinuria (PNH) and Budd-Chiari syndrome, as well as severe vaginismus and cervical stenosis, in need of contraception. Herein, we present the clinical considerations and implications taken to arrive at the right contraceptive choice for the patient.

Discussion: Budd-Chiari syndrome is defined by the presence of hepatic venous outflow tract obstruction, which may be due to a number of underlying causes.

Gestational weight gain and the risk of infant mortality amongst women with normal prepregnancy BMI: the Friedmann-Balayla model.

Objective: The study of the association between gestational weight gain (GWG) and infant mortality is riddled with methodological concerns, particularly with limitations in accounting for gestational age-specific weight gain. In our study, we developed a new model, which accounts for gestational age, to determine whether insufficient or excessive GWG is associated with an increased risk of infant death amongst women with normal prepregnancy BMI (18.5-24.

Maternal and obstetrical predictors of sudden infant death syndrome (SIDS).

Objective: Public Health initiatives, such as the "Safe to Sleep" campaign, have traditionally targeted infants' risk factors for the prevention of Sudden Infant Death Syndrome (SIDS). However, controversy remains regarding maternal and obstetrical risk factors for SIDS. In our study, we sought out to determine both modifiable and non-modifiable obstetrical and maternal risk factors associated with SIDS.

Systems Architecture for a Nationwide Healthcare System.

From a national level to give Internet technology support, the Nationwide Integrated Healthcare System in Uruguay requires a model of Information Systems Architecture. This system has multiple healthcare providers (public and private), and a strong component of supplementary services. Thus, the data processing system should have an architecture that considers this fact, while integrating the central services provided by the Ministry of Public Health.

Synergistic effects of valproic acid and mitomycin C in adenocarcinoma cell lines and fresh tumor cells of patients with colon cancer.

Valproic acid has been demonstrated to mediate cytotoxic effects against tumor cells by acting as a histone-deacetylase inhibitor. However, to date, there are only limited data on the effects of valproic acid in colon cancer. Moreover, information regarding combinations of the drug with chemotherapeutic agents is very limited.

T cell-mediated neuroprotection involves antithrombin activity.

Functional loss after injury to the mammalian central nervous system (CNS) has been attributed not only to the immediate loss of neurons but also to secondary neuronal degeneration caused by the toxicity of physiological compounds present in abnormally high amounts as a result of the injury. One such compound appears to be the protease thrombin. Here we show that the beneficial effect of T cells directed against myelin self-antigens can be attributed, at least in part, to the ability of these 'autoimmune' T cells to produce antithrombin III.

Calcium oxalate granuloma of the nose of a chronically dialysed nephritic patient.

The patient was a 54-year-old woman who had been suffering from chronic tubulo-interstitial nephritis for about seven years, requiring haemodialysis. More recently, she developed a polypoid mass in the left nasal cavity causing discomfort on breathing and slight epistaxis. The tumour was of gritty consistency and measured 28 x 8 x 5 mm.

Self-Protective mechanism awakened by glutamate in retinal ganglion cells.

The progression of degeneration in chronic optic neuropathies or in animal models of optic nerve injury is thought to be caused, at least in part, by an increase in glutamate to abnormally high concentrations. We show here that glutamate, when injected in subtoxic amounts into the vitreal body of the rat eye, transduces a self-protecting signal that renders the retinal ganglion cells resistant to further toxicity, whether glutamate-derived or not. This neuroprotective effect is attained within 24 h and lasts at least 4 days.

Extraskeletal Ewing's sarcoma of the nose.

A 20-year-old woman presented with nasal obstruction and slight epistaxis. The obstructing lesion was excised and microscopy showed a neoplasm composed of comparatively uniform undifferentiated cells forming solid nests. The cytoplasm of the cells was clear but poorly demarcated, partly vacuolated and contained much glycogen.

Thrombin attenuation is neuroprotective in the injured rat optic nerve.

The functional loss that often follows injury of the mammalian CNS has been attributed not only to the immediate neural loss, but also to secondary neuronal degeneration caused by toxic biochemical mediators in the environment of the injured nerve. We report here that a high thrombin content, produced as a result of injury-induced activation of prothrombin, appears to be an important mediator of secondary damage. Measurement of post-traumatic neuronal survival in vivo revealed that post-traumatic local application of the thrombin inhibitor N-alpha-(2-naphthylsulphonylglycyl)-4-(D,L)-amidinophenylalanine piperidide acetate in the rat optic nerve subjected to mild partial crush injury left twice as many retinal ganglion cells with functioning axons as in controls.

Injury-induced gelatinase and thrombin-like activities in regenerating and nonregenerating nervous systems.

It is now widely accepted that injured nerves, like any other injured tissue, need assistance from their extracellular milieu in order to heal. We compared the postinjury activities of thrombin and gelatinases, two types of proteolytic activities known to be critically involved in tissue healing, in nonregenerative (rat optic nerve) and regenerative (fish optic nerve and rat sciatic nerve) neural tissue. Unlike gelatinases, whose induction pattern was comparable in all three nerves, thrombin-like activity differed clearly between regenerating and nonregenerating nervous systems.

Actinomycosis of the middle ear.

Another case of actinomycosis of the ear is described in a nine-year-old boy, drawing attention to the increasing incidence of diseases of the ear considered to be rare. The patient presented with the clinical signs of chronic purulent otitis media, not responding to conservative treatment. After the diagnosis of actinomycosis was established the patient was treated by surgery and long-term antibiotic medication, responding favourably.

Pregnancy and malignant neoplasms of the head and neck.

This paper, based on a literature review, deals with the occasional development of a head and neck neoplasm in the pregnant woman. This rare event makes for some challenging problems in patient management, insofar as the otolaryngologist is actually responsible for taking care of 2 patients -- the mother and her unborn child. In particular, 4 tumor types have figured prominently among those head and neck tumors arising in pregnant women: cancer of the larynx, cancer of the thyroid, malignant melanomas, and malignant lymphomas of the head and neck.

GTP-dependent conformational changes associated with the functional switch between Galpha and cross-linking activities in brain-derived tissue transglutaminase.

GTP and Ca2+, two well-known modulators of intracellular signaling pathways, control a structural/functional switch between two vital and mutually exclusive activities, cross-linking and Galpha activity, in the same enzyme. The enzyme, a brain-derived tissue-type transglutaminase (TGase), was recently cloned by us in two forms, one of which (s-TGN) lacks a C-terminal region that is present in the other (l-TGN). Immunoreaction with antibodies directed against a peptide present in the C-terminus of l-TGN but missing in s-TGN suggested that this site, which is located in the C-terminal fourth domain, undergoes conformational changes as a result of interaction between l-TGN and GTP.