[Juvenil autoimmune myasthenia]. / Myasthénie auto-immune juvénile.

We report the story of an 11-year-old girl admitted to the emergency room for diplopia and divergent squint. Promptly apparead a fluctuating ptosis, a nasal voice and swallowing disorders, evoking the diagnosis of autoimmune myasthenia. The latter has been confirmed with electromyogram. Treatment with corticoids, plasmapheresis and pyridostigmine allowed symptoms control. Thymectomy by left thoracoscopy, non-robot assisted, was performed 6 months after the appearance of the first clinical signs for the purpose of remission.

Nous rapportons l'histoire d'une enfant de 11 ans admise aux urgences pour diplopie et strabisme divergent. Rapidement sont apparus un ptosis fluctuant, une voix nasonnée et ensuite des troubles de déglutition faisant évoquer un diagnostic de myasthénie auto-immune. Ce dernier a été confirmé à l'électromyogramme. Le traitement associant corticoïdes, plasmaphérèse et pyridostigmine a permis un contrôle des symptômes. Une thymectomie par thoracoscopie gauche non assistée par un robot a été réalisée 6 mois après l'apparition des premiers signes cliniques dans le but d'obtenir une rémission.

[Fellowship in Canada: instructions]. / Fellowship au Canada : mode d'emploi.

In North America, postdoctoral fellowships are proposed to physicians and surgeons after their residency to obtain an expertise in a specific domain of their speciality. In obstetrics and gynecology, three fellowship programs are accredited by the Royal College of Physicians and Surgeons of Canada: maternal fetal medicine, gynaecological oncology and reproductive endocrinology and infertility. A two-year fellowship in Canada provides a great professional and personal experience. We present here the organization of these programs and the conditions to be admitted in a fellowship program in Canada.

Ultrasonographically guided direct gene transfer in utero: successful induction of beta-galactosidase in a rabbit model.

OBJECTIVE: We sought to determine whether the transfer of enzyme-encoding genes in utero can be detected after birth. STUDY DESIGN: An adenoviral vector carrying the gene for beta-galactosidase was injected under ultrasonographic guidance into the livers of 4 rabbit fetuses per litter (3 litters total) at 27 days' gestation. On delivery of the pups 2 to 3 days later, the livers were analyzed for beta-galactosidase activity by using 5-bromo-4-chloro-3-indolyl-beta-D -galactopyranoside (X-gal) staining. Polymerase chain reaction was also performed on liver extracts as an additional independent measure of successful vector delivery. RESULTS: Successful targeting of the livers of fetal rabbits was demonstrated by beta-galactosidase activity in the nuclei of liver serosal cells, parenchymal hepatocytes, or columnar cells of the gallbladder in 7 (58%) of 12 injected pups and by polymerase chain reaction in liver extracts from 10 (83%) of 12 injected pups. CONCLUSIONS: These results suggest that vectors that carry genes for specific enzymes can be delivered to fetal organs in utero and that expression of the enzyme can be detected after delivery.

An animal model for hemolytic disease of the fetus or newborn in New Zealand white and New Zealand red rabbits: newborn effects.

OBJECTIVE: Our purpose was to study the neonatal effects of red blood cell alloimmunization in a rabbit model. STUDY DESIGN: Eighteen does were alloimmunized to incompatible red blood cells. Does were bred twice, once with a homozygous buck of incompatible blood type and once with a homozygous buck of compatible blood type. Fetal blood sampling was undertaken on day 27 of gestation (term 28 to 31 days). Does were delivered on day 30 and the neonatal pups were anesthetized. Direct cardiac samplings were performed for hemoglobin, reticulocyte count, and direct Coombs' test. Hepatic, splenic, and renal wet weights were measured. RESULTS: Twenty-two pregnancies (12 compatible and 10 incompatible) were studied. Neonatal hemoglobin was higher in the compatible litters (11.1 gm/dL [7.7 to 12.6 gm/dL] vs 4.9 gm/dL [2.1 to 9.1 gm/dL], P <.001), whereas no difference could be detected between the respective reticulocyte counts (34.0/100 red blood cells [27.3 to 36.1/100 red blood cells] vs 32.6/100 red blood cells [26.8 to 43.5/100 red blood cells], P =.55). The direct Coombs' assay was negative in 23 pups from 8 compatible litters and false positive (weakly positive result) in 2 pups of a ninth compatible litter. The Coombs' assay was positive in all 22 incompatible pups tested. Hepatosplenomegaly was noted in affected pups but not in controls. CONCLUSIONS: A disease analogous to human hemolytic disease of the newborn can be induced in the rabbit neonate.

Long-term neurodevelopmental outcome after intrauterine transfusion for the treatment of fetal hemolytic disease.

OBJECTIVE: The aim of the study was to assess the developmental outcome of neonatal survivors of hemolytic disease of the neonate treated with modern intrauterine transfusion techniques. STUDY DESIGN: In this prospective, observational study, auditory evoked-response tests were performed in the nursery. Neurodevelopmental evaluation with the Gesell Developmental Schedules was performed between 9 and 18 months of corrected age to assess motor skills, language development, comprehension capacity, and social skills. The McCarthy Scales of Children's Abilities were administered between 36 and 62 months. RESULTS: Forty children who survived severe fetal hemolytic disease were followed up until 62 months old. Demographic data included gestational age at first intrauterine transfusion (26.4 +/- 3.7 weeks), median number of intrauterine transfusions (4, range 1-8), lowest fetal hematocrit (20.2% +/- 7.8%), peak fetal bilirubin (7.1 +/- 2.1 mg/dL), incidence of hydrops fetalis (45%), and mean gestational age at delivery (35.6 +/- 2.2 weeks). One case of severe bilateral deafness and 1 case of right spastic hemiplegia were diagnosed. The Gesell Developmental Schedules score was assessed between 9 and 18 months of corrected age in 22 infants. The global developmental quotient was 101.9 +/- 9.5 (mean for normal population is 100). Regression analysis revealed no correlation between the global developmental quotient and gestational age at the first intrauterine transfusion, gestational age at birth, or the severity of the fetal hemolytic disease (fetal hematocrit, fetal bilirubin, presence of hydrops fetalis, total number of intrauterine transfusions, duration of neonatal phototherapy, and number of neonatal exchange transfusions). Eleven of the 40 children were followed up until they were 62 months old, and the McCarthy Scales of Children's Abilities were administered. The mean cognitive index was 107.6 +/- 9.4 (90-109 is considered average). CONCLUSION: Despite severe fetal hemolytic disease, normal developmental outcome can be expected for children treated with intrauterine transfusions.

Diagnosis and management of placenta percreta: a review.

A review of the clinical decisions, diagnostic, and surgical methods in managing patients with placenta percreta was done by conducting a MEDLINE computerized search from January 1991 to January 1997 using the key words "placenta percreta," "placenta previa," "acute normovolemic hemodilution," and "erythropoietin use." Additional sources were identified through cross-referencing. We reviewed all published reports and articles regarding the clinical and surgical management of placenta percreta and nontraditional ways to treat or prevent anemia in these cases (including acute normovolemic hemodilution and erythropoietin use). The diagnosis of placenta percreta using different ultrasonographic criteria is reliable. Clinical and surgical methods of managing placenta previa with a high risk of percreta are all based on prevention of uncontrolled hemorrhage. Ninety percent of these patients will lose more than 3000 ml intraoperatively and will require blood transfusion. To avoid serious maternal morbidity secondary to hypovolemia, several options are available: erythropoietin use, acute normovolemic hemodilution, selective arterial embolization, prophylactic uterine, or hypogastric artery ligation. With the increasing incidence of placenta percreta, the clinician must use all available methods to accurately diagnose this condition. Adequate preparation and good surgical technique will help reduce maternal mortality and morbidity related to this condition.

Automated patient acuity. Linking nursing systems and quality measurement with patient outcomes.

Traditional patient classification systems no longer provide a useful function for nursing management. Value from these systems can only be realized through major revisions and integration with other systems. The resulting transformed system, linking patient outcomes, cost of care, and quality measurements together, provides a valuable measurement tool and enables nursing to demonstrate their value to the industry. The authors describe their rationale and experience in designing such a system.