RESUMO
Neonatal hyperinsulinism is a life-threatening disease that, when treated by total pancreatectomy, leads to diabetes and pancreatic insufficiency. A more conservative approach is now possible since the separation of the disease into a nonrecurring focal form, which is cured by partial surgery, and a diffuse form, which necessitates total pancreas removal only in cases of medical treatment failure. The pathogenesis of the disease is now divided into K-channel disease (hyperinsulinemic hypoglycemia, familial [HHF] 1 and 2), which can mandate surgery, and other metabolic causes, HHF 3 to 6, which are treated medically in most patients. The diffuse form is inherited as a recessive gene on chromosome 11, whereas most cases of the focal form are caused by a sulfonylurea receptor 1 defect inherited from the father, which is associated with a loss of heterozygosity on the corresponding part of the mother's chromosome 11. The rare bifocal forms result from a maternal loss of heterozygosity specific to each focus. Paternal disomy of chromosome 11 is a rare cause of a condition similar to Beckwith-Wiedemann syndrome. A preoperative PET scan with fluorodihydroxyphenylalanine and perioperative frozen-section confirmation are the types of studies done before surgery when needed. Adult variants of the disease are less well defined at the present time.
Assuntos
Hiperinsulinismo Congênito , Transportadores de Cassetes de Ligação de ATP/genética , Biópsia , Hiperinsulinismo Congênito/genética , Hiperinsulinismo Congênito/patologia , Hiperinsulinismo Congênito/fisiopatologia , Hiperinsulinismo Congênito/terapia , Secções Congeladas , Humanos , Lactente , Recém-Nascido , Células Secretoras de Insulina/patologia , Ilhotas Pancreáticas/patologia , Síndrome de Munchausen/diagnóstico , Nesidioblastose/patologia , Pâncreas/embriologia , Canais de Potássio/genética , Canais de Potássio Corretores do Fluxo de Internalização/genética , Receptores de Droga/genética , Receptores de SulfonilureiasRESUMO
OBJECTIVE: Assess the radiation exposure of surgical staff during sentinel node surgery in gynecology using a radiotracer, the (99m)Tc-microalbumin. MATERIALS AND METHODS: A monocentric, prospective study was conducted during 3 months representing 40 sentinel node surgical procedures with different dosimetric measurements. Dosimeters were used to evaluate the whole body and the fingers radiation exposure for all exposed workers (surgeon, nurse and surgical assistant). Another dosimeter was used to estimate the atmospheric radiation level. The activity of (99m)Tc-microalbumin was 50.1±2.4MBq when the surgery was performed the same day and 90.4±3.2MBq when the surgery was performed the day after. RESULTS: Radioactive doses received during each procedure by the surgeon, surgical assistant and nurse are 5, 3.75 and 0µSv for whole body exposure and 17.5, 15.6 and 16.2µSv for extremities respectively. Atmosphere dosimeter does not detect any radiation over this period. On average, 200 procedures are performed each year in our hospital by 7 surgeons. Surgeon's radiation exposure remains below the threshold of 1mSv annual for whole body and 50mSv annual for fingers set for public by the International Commission on Radiological Protection. DISCUSSION AND CONCLUSIONS: During sentinel node surgery radiation exposure of surgical staff is weak. Everyone, including the surgeon, receives a dose below the limits of the public radiation exposure. There is no need for special dosimetric monitoring or use radiation protective devices during the sentinel node surgery using (99m)Tc-microalbumin injection.