[Two cases of Werdnig-Hofmann disease]. / Enfermedad de Werdnig-Hofmann. Dos casos clínicos.

Spinal muscular atrophy (SMA) is an autonomic recessive disorder that affects the anterior horn cells of the spinal cord, degeneration of which results in proximal muscle weakness. It is classified into three types: I and II (Werdnig-Hoffmann disease) and III (Kugelberg-Welander disease). With an incidence of 1/10,000. We report two cases of infants with hypotonic syndrome, that were diagnose with SMA, in the first case by muscular biopsy, and in the second by electromyography and chromosomes study. It is import that the physicians know about this disease, and its complications.

[Burnout syndrome associated factors in gynecologists]. / Factores relacionados con el síndrome de burnout en ginecólogos.

OBJECTIVE: To determine risk factors associated to Burnout Syndrome in medical gynecologist staff who works at the Gynecology-pediatrics with Family Medicine Hospital number 31 of the IMSS at Mexicali city, Baja California. MATERIAL AND METHOD: A cross sectional study was performed in 27 gynecologists (100% of the total gynecologist that works at that particular hospital). A validated survey instrument developed by Maslach was applied to them in order to identify the presence of the syndrome and a general questionnaire including sociodemographic information was also applied to the doctors. The statistical analysis was both descriptive and inferential including chi square, Student t and logistic regression. RESULTS: 74.1% of the doctors who showed signs of Burnout Syndrome were males and 25.9% were females. The average doctors age of the group who developed Burnout was 44.81 +/- 6.11 years. The mean time in years of work for the doctors with the syndrome was 15.56 +/- 7.11 years. Only one doctor was divorced and she presented Burnout Syndrome, on the other hand, 62.5% of the married doctors showed evidence of Burnout Syndrome (p > 0.05). The general prevalence was 59.2%. Of those who showed signs of burnout, 87.5% presented it in low degree, 6.2% presented moderate degree, 6.2% presented high burnout degree. Out of all the organizational factors type, none resulted significative. CONCLUSIONS: The prevalence of the reported syndrome is apparently high when compared to the medical anesthesiology staff of the same hospital; however this difference is not statistically significative. In the unvaried and multivariate analyses, no organizational factor shows significance as a potential risk. It is necessary to consider psychological support to the affected doctors and to value the organization of the service, looking for an administrative reengineering.

[Moebius syndrome. Clinical case report]. / Síndrome de Moebius. Informe de un caso clínico.

BACKGROUND: Moebius syndrome (MBS) is an infrequent disease, having an incidence of 1 in 10,000 births, mainly characterized by a congenital bilateral facial paralysis due to an agenesia of the sixth and seventh cranial nerves. In addition, orofacial and limb anomalies are frequently found in these patients. The diagnosis is fundamentally based on different clinical manifestations of the disorder. CLINICAL CASE: a female newborn with the clinical picture of Moebius syndrome is presented, and genetic or environmental aspects are discussed. Since the use of misoprostol for abortion and inducing uterine activity in combination with NSAIDs, the number of newborns with MBS associated with this drug has increased. Nowadays, either genetic or environmental factors are associated with MBS. CONCLUSIONS: it is necessary that the general and medical community be aware of the risk of teratogenic effects of misoprostol, and the usefulness of genetic counseling whenever there is a newborn with Moebius syndrome.

Introducción: el síndrome de Moebius (MBS) es una enfermedad poco frecuente que tiene una incidencia de 1 por cada 10 000 nacimientos. Se caracteriza principalmente por parálisis facial bilateral congénita debido a una agenesia de los núcleos de los nervios craneales del VI y VII par. Además, es frecuente encontrar anomalías orofaciales y de extremidades torácicas y pélvicas en estos pacientes. El diagnóstico se basa fundamentalmente en las distintas manifestaciones clínicas del trastorno. Caso clínico: niña recién nacida con el cuadro clínico de síndrome de Moebius. Se discuten los aspectos genéticos y medioambientales asociados a esta patología. Desde la introducción del misoprostol y su uso inadecuado como agente abortivo e inductor de actividad uterina, y en combinación con antiinflamatorios no esteroideos, se ha observado un incremento en la frecuencia de recién nacidos con síndrome de Moebius asociados al uso de este medicamento. Actualmente, el factor genético y el medioambiental se encuentran asociados a este síndrome. Conclusiones: es importante alertar a la comunidad médica y a la población en general acerca del riesgo teratogénico del misoprostol y de la importancia del consejo de los genetistas cuando nace un paciente con síndrome de Moebius.

[Prevalence of burnout syndrome in nurses in 2 Mexican hospitals]. / Prevalencia del síndrome de burnout en el personal de enfermería de 2 hospitales mexicanos.

OBJECTIVE: To determine the prevalence of burnout syndrome in nursing staff in 2 different second level hospitals in the city of Mexicali, Baja California, Mexico. METHOD: A cross-sectional study was conducted in all nursing staff (n = 698) in 2 second level hospitals (the Gynecology, Pediatrics and Family Medicine Hospital No. 31 and the General Specialist Hospital No. 30 in Mexicali). A stratified random sample was obtained from the 698 nurses, with a sample size of 184 nurses from each hospital. The Maslach Burn-out Inventory and a general questionnaire on demographic factors were used. Statistical analysis consisted of descriptive statistics and analysis of proportions using the chi-squared test. RESULTS: A total of 368 nurses were studied, of whom 345 (93.75%) were women. The mean age was 41.21 SD (standard deviation) = 6.59 years. The mean length of service was 15.87 (SD = 6.12) years. In both hospitals, the prevalence of burnout syndrome was 6.79% (25). CONCLUSIONS: Previous publications on burn-out have included studies performed in Mexico and in nurses in the emergency department of a tertiary hospital in Barcelona, as well as in other health care professionals. In comparison with these studies, the prevalence of burn-out observed in the present study was significantly lower.