[From pediatric care to adult medicine: Transition of sickle cell patients, a French monocentric study]. / De la pédiatrie à la médecine adulte : transition des patients drépanocytaires, une étude monocentrique française.

Sickle cell disease, a hemoglobin disorder with autosomal recessive transmission, is one of the most common genetic diseases screened in France. Thanks to early management, 95% of sickle cell patients reach adulthood and require transition from pediatric care to adult care. Through a retrospective study of records from serious sickle cell patients over 17 years old, followed in the hematology-oncology pediatric unit of Reims University Hospital Center in France, we analyzed transition conditions, compared pediatric and adult management, and proposed a plan for transition care. As of 1 January 2016, out of 19 sickle cell patients meeting the inclusion criteria, 12 had made the transition from pediatric care to adult medicine. Among the transition group, the transition was proposed by the pediatrician in 92% of cases. The average age of transition was 19.4 years. The time between receiving the information and the last pediatric visit was 2.4 months. Seven out of the 12 patients were informed of their transition during the last pediatric visit. The age of the first adult visit was 20.3 years. There was no alternate or joint consultation. The treatments prescribed during the last pediatric visit were not modified during the first adult visit. The average number of hospitalizations per patient was 2.7 in pediatric care and 3.4 in adult care with a median value of 2 in both groups. Three out of 12 patients died, the average age of death being 26.7 years. Transition is an important milestone in chronic disease patients. More than age, the maturity of the patient must be taken into account. The transition to the adult structure requires early preparation in the teenage years and investment of the adolescent and his family as well as investment of pediatric and adult caregivers. This study points out the need to establish a transition plan within our hospital in collaboration with adult physicians. Continuity of care is necessary to increase the quality of managing patients and cannot be done without a close relationship between pediatric specialists and adult physicians.

[Cardiogenic shock due to spontaneous neonatal thrombosis of the abdominal aorta]. / Choc cardiogénique dû à une thrombose spontanée néonatale de l'aorte abdominale.

Neonatal arterial thrombosis is unusual and generally associated with an arterial umbilical catheter. Spontaneous aortic thrombosis is exceptional but its severity is related to high mortality rate and renovascular morbidity. We report here the observation of a 10-day-old term infant showing a large abdominal aortic thrombosis revealed by cardiogenic shock induced by systemic arterial hypertension. The resolution was fast following anticoagulant and antihypertensive therapy. Etiologic investigations showed renal failure and moderate hyperhomocysteinemia controlled by a vitamin supplement. Following this observation, we did a brief review of the neonatal spontaneous arterial thrombosis literature to discuss the neonatal hemostasis specific aspects. Management of infants presenting an arterial thrombosis varies depending on the hospital and there are no guidelines at this time concerning the etiologic investigation and treatment in France or internationally.