Clinical Analysis Of 18 Cases Of Pituitary Stalk Interruption Syndrome And Literature Review

Objective:To analyze the clinical data of 18 patients with pituitary stalk interruption syndrome and combine with literature learning to improve the clinicians' understanding of the disease so as to achieve early detection,early diagnosis,early treatment and improve the prognosis.Method:A retrospective analysis of the clinical data of 18 patients diagnosed with pituitary stalk interruption syndrome from the Department of Endocrinology of Fujian Provincial Hospital between January 1,2013 and December 31,2017 was conducted to study the clinical manifestations and characteristics of the disease.,laboratory tests,imaging examinations,treatment methods and prognosis,and related literature review.Results:1.Medical history: 18 patients includes 2 women(11.11%)and 16 men(88.89%),and the rate of male to female is 8:1,age of them is between 4-15(8.56±3.518)years.12 people(66.67%)had breech delivery or foot delivery.2.Clinical manifestations: All 18 patients had different degrees of growth retardation compared with their peers,with 14 patients(77.78%)with hypothyroidism and 1 person(5.56%)with disappeared rhythm of adrenocortical function,14 patients with adrenocortical insufficiency(77.78%),1 of whom developed pituitary crisis(5.56%).Osteoporosis was found in 15 of the 18 cases,and the bone turnover markers were elevated.Of these,12 ones were found to have 25 OH vitamin D,inculding 9 ones(75.00%)with vitamin D deficiency.3.diagnosis: 18 cases involved in the pituitary secretion axis of varying degrees,and the cranial MRI are pituitary stenosis absence or thinning,which can diagnose the pituitary stalk interruption syndrome.4.treatment and prognosis: According to the patient's personal conditions and involved the pituitary axis of the situation,to be the appropriate hormone supplement.Telephone follow-up was performed on 18 patients,1 patient refused to visit,and 2 patients could not contact.Among the 15 patients who were successfully followed,high self-intervention rates,poor compliance,and inadequate knowledge of the disease were found.In the early,adequate,long course of growth hormone replacement therapy,the patient's lifetime is up to-2 SD within the same sex peer group.In SPSS 24.0,13 patients with clear sex hormone replacement therapy and successful follow-up were analyzed statistically and divided into drug treatment effective group and invalid group.The treatment age was between the two groups(rank sum test,Z=-0.934,P).(0.350>0.05)and treatment duration(rank sum test,Z=-0.360,P=0.719>0.05)were not statistically different,and these 13 patients were divided into single sex hormone replacement therapy group and multiple sex hormone replacement therapy group,The treatment efficiency in the hormone replacement therapy group(83.33%)was higher than that in the single hormone replacement therapy group(14.29%),and there was a statistical difference(chi-square test,P=0.029<0.05).In patients with concurrent corticosteroid deficiency and thyroid hormone deficiency,it is also necessary to add adrenocortical hormone and thyroid hormone.Fifteen patients had no symptoms of hypoglycemia,adrenocortical crisis,hypothyroidism,bone pain,and pathological fractures after discharge.Conlusion:The pituitary stalk interruption syndrome is a rare disease in men more than in women,manifested as growth retardation,with deficiency of single or multiple hypophysial hormone,mostly lacking of growth hormone,and always of sex hormone,thyroxine and adrenocorticotropic hormone.In the supplement of growth hormone should be early,adequate,full course,can improve the prognosis.In the alternative treatment of sex hormones,it is recommended to give a variety of drug treatment,treatment efficiency can be higher than drug treatment.This study observed that in addition to hormone deficiency in patients with pituitary stalk blocking syndrome,but also more associated with osteoporosis and vitamin D deficiency,osteoporosis may be considered related to sex hormone deficiency,to be improved after sexual hormone replacement therapy,and vitamin D deficiency Considering that it may be related to IGF-1 deficiency,vitamin D supplementation is needed.