Научная статья на тему 'TRANSFORMATION OF PROLACTINOMA INTO CORTICOTROPIN-SECRETING ADENOMA IN PATIENT WITH MEN 1 SYNDROME: A CASE REPORT'

TRANSFORMATION OF PROLACTINOMA INTO CORTICOTROPIN-SECRETING ADENOMA IN PATIENT WITH MEN 1 SYNDROME: A CASE REPORT Текст научной статьи по специальности «Клиническая медицина»

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Журнал
Проблемы эндокринологии
WOS
Scopus
ВАК
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Ключевые слова
MEN 1 TYPE / WERMER’S SYNDROME / PITUITARY TUMOR / GENE ANALYSIS
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Текст научной работы на тему «TRANSFORMATION OF PROLACTINOMA INTO CORTICOTROPIN-SECRETING ADENOMA IN PATIENT WITH MEN 1 SYNDROME: A CASE REPORT»

proinflammatory cytokines synthesis leads to increased bone fragility without decreasing BMD.

KEYWORDS: fracture risk, osteoporosis. ★ ★ ★

TRANSFORMATION OF PROLACTINOMA INTO CORTICOTROPIN-SECRETING ADENOMA IN PATIENT WITH MEN 1 SYNDROME: A CASE REPORT

Ludmila Rozhinskaya, Zhanna Belaya, Patimat Khandaeva

Endocrinology Research Centre, Moscow, Russia

Multiple endocrine neoplasia syndrome type 1 (MEN 1 type, Wermer's syndrome) is a group of heterogeneous inherited diseases, pathogenesis of which is based on hyperplasia or neoplasia of several endocrine glands, characterized by autosomal dominant mode of inheritance, high penetrance and similar prevalence among males and females. Prevalence of MEN 1 is estimated as 2—10 people per 100,000 of population. The patient turned to the doctor for the first time at the age of 20 with her primary amenorrhea. Examination revealed pituitary adenoma with pronounced secretion of prolac-tin (11,370 IU/L). She underwent transspenoidal ade-nomectomy, followed by drug treatment with dopamine agonists which normalized prolactin level, and restored menstrual function. At the age of 31 the acute gastrointestinal bleeding was the reason for further investigations and subsequent surgery. There were found gastrin-se-creting tumor of the pancreatic gland and small tumors in the spleen area, as well as carcinoid in the mesentery area. At the age of 39 primary hyperparathyroidism (hypercalcemia, osteoporosis, high PTH level and parathyroid adenoma) was diagnosed. Parathyroidectomy was performed. Genetic analysis has revealed nonsense mutation Y77X in the Gene Menin in that patient and in her brother, thus MEN 1 type was confirmed. Nodular hyperplasia of both adrenals was visualized on CT. Disturbance of adrenocorticotrophic hormone secretion (in the morning 27 pg/mL, in the evening 33, 8 pg/mL) and cortisol secretion (in the morning 581 nmol/L, in the evening 338 nmol/L), high urinary free cortisol to 2,178 nmol/day, no suppression of cortisol secretion by 1 mg of dexamethasone were measured, and at the same time no clinical symptoms were detected. Cushing's disease was confirmed by inferior petrosal sinus sampling and functional tests. Pituitary surgery was not performed due to the absence of clinical manifestations of hypercorti-solism. For the next 7 years active hypercortisolism was persisting, however clinical features appeared only last year. The patient underwent neurosurgical intervention in March 2017, remission of hypercortisolism was achieved. The clinical case may be called unique due the following reasons: multiple lesions of endocrine organs, of gastrointestinal tract; absolute synchronism of tumor

development in various organs similar to those observed in her brother except hypercortisolism, as well as transformation of tumorigenesis in pituitary from prolactin-secreting tumor to ACTH-secreting tumor.

KEYWORDS: MEN 1 type; Wermer's syndrome;

pituitary tumor; gene analysis.

★ ★ ★

MOBILE APP ELECTRONIC DIARY IMPROVES THE MOTIVATION IN PATIENTS WITH GESTATIONAL DIABETES

Evgenii Pustozerov1,2, Polina Popova1,2, Aleksandra Tkachuk1, Yana Bolotko1, Andrei Gerasimov1, Elena

Grineva1,2

1Almazov Federal North-West Medical Research Centre, Saint-Petersburg, Russia; 2Saint Petersburg State Electrotechnical University, Saint-Petersburg, Russia

Introduction. In the course of gestational diabetes (GDM) treatment, it is significantly important to keep track on records in a personal diary, which helps physicians and patients to understand the problems appearing during blood glucose (BG) compensation. At the same time, the lack of motivation can lead patients to stop making records and exchanging them with their doctor. In our study we analyzed, how the electronic diary app can improve the motivation of patients in comparison with traditional means of perceiving data on blood glucose. The aim of the study is to improve the motivation in gestational diabetes patients by providing them with helpful tools to keep track on their records. Material and methods. Android and desktop application DiaCompan-ion was developed and given to a group of patients with diagnosed GDM. Another group of GDM patients received a traditional diary via Excel spreadsheet. Patients from both groups were asked to fill the diaries with the data on BG levels, insulin injections (when prescribed) and, if possible, food intakes, physical activity, sleep and ketones (when prescribed). Results. By the middle of march 2017, a total of 179 patients with GDM received an application DiaCompanion and 36 patients recorded their BG levels via Excel spreadsheet. A total of 24914 and 4247 BG records were analyzed correspondingly. A significant difference was shown in the amount of women quitting keeping records (6.0% vs 19.4% patients with less than 2 weeks of reports, p=0.029) and the average number of days with records (53.0 days against 40.2 days, p=0.006). Considerable amount of patients used an app to track additional records, while patients with traditional diary rarely reported any (98.6% against 44.4% reported food intakes (p=9.97-e-8), 59.6% vs 22.2% reported physical activity (p=6.64-e-6), 37.2% vs 5.6% sleep (p=6.37-e-9) and 21.6% vs 11.1% ketones (p=0.044) correspondingly). Average fasting BG levels during the whole course of monitoring were lower in women who used the app (4.88 vs 5.01 correspondingly, p=0.048), while postprandial BG values didn't show significant dif-

PROBLEMS OF ENDOCRINOLOGY, 1, 2017, SUPPL. 2

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