Evaluation of a prophylactic transfusion program on obstetric outcomes in pregnant women with sickle cell disease: A single centre retrospective cohort study.

OBJECTIVE: To evaluate the effects of a prophylactic transfusion program (TP) on obstetric and perinatal outcomes in pregnant women with sickle cell disease (SCD). METHODS: This retrospective cohort study included all singleton pregnancies among women with SCD in a French university tertiary care center between 1 January 2004 and 31 December 2017. The TP group included patients selected according to the French guidelines who received regular red blood cell transfusions during pregnancy until delivery. The factors associated with TP indication [year of birth, SCD genotype, history of acute chest syndrome and delayed hemolysis transfusion reaction (DHTR) risk score] were taken into account in a propensity score. A composite obstetric adverse outcome was defined associating birth before 34 gestational weeks and/or pre-eclampsia and/or small for gestational age and/or abruption and/or stillbirth and/or maternal death and/or neonatal death. RESULTS: In total, 246 pregnancies in 173 patients were analyzed. Twenty-two pregnancies with a history of DHTR were excluded. A higher frequency of TP was found before 2013 [119/148 (80.4%) vs 38/76 (50%); p < 0.001]. Rates of preterm birth before 34 gestational weeks (5.6% vs 19.7%; p = 0.001), vaso-occlusive crisis (36.5% vs. 61.8%; p < 0.001), and acute chest syndrome (6.1% vs. 14.5%; p = 0.04) during pregnancy were decreased significantly in the TP group. Among the groups with and without composite obstetric adverse outcomes, the frequency of TP was 52.6% and 74.7%, respectively [odds ratio (OR) 0.30, 95% confidence interval (CI) 0.09-1.02]. The multivariate analysis shows that the TP was associated with a significant reduction in the risk of composite obstetric adverse outcomes (OR 0.28, 95% CI 0.08-0.97; p = 0.04). CONCLUSION: A red blood cell TP may have an independent protective effect on maternal and perinatal adverse outcomes during pregnancy in women with SCD.

[Use of pocket-sized ultrasound in internal medicine (hospitalist) practice: Feedback and perspectives]. / Échographie ultraportable en médecine interne : retour d'expérience et point de vue.

INTRODUCTION: Point of care ultrasound (POCUS) is routinely used by intensivists and emergency physicians for many years. Its interest is not arguable any more for these specialists, despite the large variety of diseases they care. Hospitalists and internists also should find some interest in POCUS, which convenience and wide range of indications responds well to the variety of their practice. However, it is still not widely used in internal medicine departments. METHODS: We here report our experience of using a pocket-sized ultrasound device in a French internal medicine department. The device used was a Vscan Dual Probe, GE, whose two probes and presets allow for cardiac, abdominal, pulmonary, obstetric, vascular, pulmonary, and superficial soft tissue exploration. One physician of the ward received a course for POCUS that was initially dedicated for emergency physicians. This study reports on the results of the examinations made between January and September 2015. For each examination performed, clinical usefulness was assessed at the time of patient discharge, by two independent physicians who reviewed the clinical course and the results of conventional imaging and rated their evaluation on a Likert scale. RESULTS: One hundred and four examinations were evaluated. The mean duration of the ultrasound examination was 9±5minutes. The POCUS conclusions were corrected by disease course or the results of conventional imaging in 10 (9.6%) cases. The presets of the device: heart, soft tissue, lung, abdomen and vascular were used respectively in 32, 30, 21, 12 and 5% of the examinations. The main indications of POCUS examination were for identification of pleural, pericardial or peritoneal effusion, and to assess the central venous pressure by inferior vena cava examination. Eighteen examinations were performed for puncture of effusion. The retrospectively evaluated clinical benefit was clearly demonstrated in 78% of cases. The agreement between the two blinded assessors was good (kappa coefficient at 0.82). CONCLUSION: Pocket-sized ultrasound device could be used in internal medicine wards. However, its limited performance compared to more sophisticated echography limits the possible explorations and their reliability, which encourages caution and makes critical the question of the initial training of doctors and medical students.

[Neurological impairment in children with HIV infection]. / Afectación neurológica en niños con infección VIH.

We retrospectively checked 53 paediatric patients suffering from infection with human immunodeficiency virus (HIV) registered in our Centre between the years 1987 and 1993, and evaluated the appearance of HIV encephalopathy. We noted important neurological signs in eleven patients (20.7%) ten of whom had HIV infection via vertical transmission and one as a result of contamination from haemoderivatives. In this review we give a detailed description of neurological signs, the moment of onset of these signs and their possible relationship with the state of the HIV infection. We also analyzed the resulting neuroradiological findings as well as any abnormalities in cerebrospinal fluid. Follow-up period ranged from one month to two and a half years from the moment of onset of the appearance of encephalopathy. Although most of our patients showed a clear improvement after oral or intravenous treatment with zidovudine, this improvement generally proved to be short-lived. The mortality rate in our HIV encephalopathy series was 81.8%, this figure being reached two and a half years after encephalopathy. The appearance of neurological signs in HIV patients therefore represents a very gloomy prognostic factor in the evolution of the disease.

[Neuromuscular gait disorders in pediatric age]. / Trastornos de la marcha de origen neuromuscular en la edad pediátrica.

UNLABELLED: Alterations in gait are a frequent cause of consultation in neuropaediatric units. They have several different causes. One subgroup of special interest concerns those which have their origin in a neuromuscular disease. We studied the experience of the Child Neurology Unit at the Hospital 12 October from 1980 to 1993. MATERIAL AND METHODS: between 1980 and 1993 a total of 176 were studied because they showed alterations in their gait caused by a neuromuscular disease. Frequency and clinical features were analysed (sex, age at the beginning of the deterioration, duration of the latter etc.). RESULTS AND CONCLUSIONS: the alterations in gait were separated into two groups: Group I: backward or non-acquired gait. Group II: deterioration in gait. IIa: temporary. IIb: definitive. The most frequent cause in Group I was spinal muscular atrophy and in Group II, muscular dystrophy linked to sex. To sum up, muscular diseases are far more important than the neuropathic ones, except for Group IIa where acute demyelinating motor polyneuritis was to be found at the top of the list of frequent occurrences.

Rapid identification of Salmonella typhimurium, S. enteritidis and S. virchow isolates by polymerase chain reaction based fingerprinting methods.

In this study we used and evaluated three rapid molecular typing methods for the identification of three frequent, clinically significant Salmonella serovars on the basis of the ease, simplicity and reproducibility of the chosen methods. We determined the genetic diversity among several isolates of Salmonella enteritidis, S. typhimiurium and S. virchow, and compared them with other enterobacteria by using the repetitive extragenic palindromic (REP) sequences, the enterobacterial repetitive intergenic consensus (ERIC) sequences, and the 16S-23S rDNA intergenic spacer region (ITS 1). The objective was to evaluate their potential application to discriminate among members of the species Salmonella enterica subspecies enterica using the genetic diversity of the group found by genomic fingerprinting. The three different serovars of Salmonella studied gave reproducible and distinguishable profiles using whichever of the above mentioned polymerase chain reaction (PCR) methods assayed. The conserved patterns in each serovar allowed for easy differentiation from other serovars of Salmonella.

[Multicystic encephalomalacia. Review of 19 cases]. / Encefalomalacia multiquística. Revisión de 19 casos.

OBJECTIVE: Multicystic encephalomalacia (EMQ) is a pediatric entity where the brain tissue is substituted by cavities of variably sizes. PATIENTS AND METHODS: Nineteen children diagnosed of EMQ were studied retrospectively. We analyzed the etiology, diagnosis and the clinical and radiological evolution. RESULTS: There were three different etiologies: twin pregnancy, 12 cases; perinatal hypoxia, 4 cases; perinatal infection, 1 case; cardiac arrest, 1 case; and 1 patient with multiple embolism of unknown cause. The diagnosis of EMQ was made with cerebral ultrasonography (14 patients), computed tomography (19 patients) and magnetic resonance imaging (5 patients). We stress the utility of cerebral ultrasonography in early and reliable diagnosis of EMQ. The clinical development was unfavorable. We studied the outcome of 15 patients. Two children died. Eleven patients (73%) show severe consequences (mental retardation, microcephaly, spastic tetraplegia, and in 7 children epilepsy). In 2 cases, the radiological development was hydranencephaly. Examination of the 4 remaining children (26.66%) revealed a spastic hemiplegia. CONCLUSIONS: EMQ is a rare disease in childhood and has a very poor outcome, for this reason early diagnosis is very important. To this effect, cerebral ultrasonography is the imaging modality of choice.

[Symptomatic Chiari type-II malformation in the neonatal period: a review of 4 cases]. / Malformación de Chiari tipo II sintomática en periodo neonatal: revisión de cuatro casos.

Four newborn patients with symptomatic Chiari II malformations were studied retrospectively. Clinical manifestations and surgical results are presented. All of these patients had hydrocephalus, which was symptomatic in two patients from the first day of life. Three of them had bradycardia and apnea spells. Two patients had lower cranial nerve palsies. Cranial sonography was the first neuroimaging procedure used and it played an important role during the follow-up period, especially for the study of the hydrocephalus. Two newborns could be studied with Magnetic Resonance Imaging (MRI). It defined the level of the decent of the fossa posterior structures and the associated malformations. All of the patients were treated with surgical repair of the spinal dysraphism, ventricular shunt and decompressive surgery. Two patients died before the age of three months. Two other patients showed better evolution after their two year and four year check-ups, respectively. Bradycardia and apnea spells are features that worsen the prognosis as compared with other manifestations.

La metodología cualitativa como una alternativa en los estudios sobre embarazo a nivel comunitario / Qualitative methdology as an alternative procedure in pregnanacy studies at community level

Este trabajo tiene como objetivos centrales, presentar parte de la información cualitativa de un estudio realizado con mujeres de dos zonas rurales, una mestiza y otra indígena, y discutir algunas de las ventajas de la metodología cualitativa en los estudios de salud reproductiva en general y embarazo en particular. La investigación se llevó a cabo con 10 mujeres de Malinalco, Edo. de México y 10 de la Sierra Juárez en Oaxaca. La técnica utilizada con las madres fueron las entrevistas a profundidad sobre los temas de sus vivencias, percepciones y prácticas en relación con los eventos del embarazo y parto. Esto es, su "experiencia vivida", en torno a algunos aspectos de la maternidad. El promedio de edad de las madres fue de 26 años en Malinalco, y de 30 años en Oaxaca. Todas las mujeres tenían pareja y mientras que las del estado de México se dedicaban a las labores domésticas y/o al comercio ambulante, las madres de Oaxca todas eran agricultoras. El embarazo y el parto en las dos zonas, se percibe como "algo natural", es decir, es parte del deber ser. El apoyo recibido durante el embarazo en Malinalco es escaso, a diferencia del que reciben las mujeres de la Sierra Juárez. Mientras que las madres del estado de México atendieron su último parto en clínicas del sector salud, las madres entrevistadas en Oaxaca se atendieron con la partera, lo que marcó una gran diferencia entre los grupos. En ninguna de las comunidades se encontró una relación entre la práctica anticonceptiva y la planeación de un nuevo hijo. Finalmente, se discuten las ventajas que tiene la metodología cualitativa en los estudios de salud reproductiva, que conllevan a un mayor acercamiento hacia los problemas y en la necesidad de crear nuevas categorías que permitan entender más claramente las vivencias y percepciones de los eventos relacionados con la maternidad en las mujeres que viven en el medio rural