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Брой: 3/2013
Автор: Лозанов Л. Б., Горчева Д., Лозанов Б. С., Кадурина М., Цанков Н.
Абстракт:
Жена на 41 г, оперирана преди 19 год за Базедова болест, с постоперативен хипотиреоидизъм, суплементиран с Л-Тироксин. Около 2 години по-късно е бил диагностициран захарен диабет тип 1, с последващо развитие на диабетна микроангиопатия, нефропатия и ХБН. От няколко месеци е с изява на генерализирани отоци, изливи в серозните кухини, хипоалбуминемия, високостепенна протеинурия и влошаване на метаболитния контрол, наложило високи дози аналогови инсулини в интензифициран режим. Болната постъпи в клиниката в много тежко общо състояние, с клинични прояви на тежък хипотиреоиден синдром на фона на 150 мкг дн Л-тироксин (ТСХ 71 mIU/L). С увеличаване дозата на Л-Тироксина до 300 мкг в комбинация с Т3 50 мкг/24 ч се посгигна нормализиране на серумните ТСХ, СТ4 и рязко подобрение на общото състояние. В резултат на комплексното лечение бе постигнато компенсиране на въглехидратните и електролитните нарушения, резорбция на серозните изливи и отоците, но кожата по крайниците остана много плътна, необичайно задебелена. Направената кожна биопсия показа хистологични промени в долните и средни слоеве на дермата, характерни за Склеродермия на Бушке, представляваща рядък генетично детерминиран синдром, най-често е свързан със захарен диабет. Същият се характеризира с натрупване на глюкозоаминогликани в дермата, най-вероятно вследствие нарушения в алдолазо-редуктазната система. Описаният случай може да бъде отнесен към Автоимунните Полигландуларни Синдроми-тип 3. Рязкото влошаване на хипотиреоидизма най-вероятно се дължи на нарушения в периферния метаболизъм и в транспорта на тиреоидните хормони вследствие тежка бъбречна недостатъчност.
Title: A Rare Case of Autoimmune Polyglandular Syndrome, Inclunding Diabetes Mellitus – Type 1, Graves Disease and Cleroderma Adultorum Buschke
Author: Lozanov L. B., Gorcheva D., Lozanov B. S., Kadurina M., Tzankov N.
Abstract:
A woman aged 41 years underwent thyroidectomy for Graves’ disease 19 yr ago with postoperative hypothyroidism, supplemented by L-Thyroxin. Diabetes mellitus – type 1 was diagnosed two years later with consecutive development of diabetic microvascular complications and chronic renal failure. Within several months before addmission the patient manifested generalized edema and serose exudates, hypolbuminemia, high grade proteinuria and worsening of the metabolic control of diabetes which required high doses of insulin analogues in the intensive regimen. She was addmitted to The Departement with clinical manifestation of very severe hypothyroidism, TSH 71 mIU/L on the phone of L-Thyroxin 150 mig/24 h. After increasing of dose up to 300 mcg L-T4 in combination with Triiodothyronine 50 mcg/24 h the clinical signs of hypothyroidism disappeared. In result of the complex management the carbohydrate and electrolyte abnormalities were compensated, serose exudates and edema disappeared but the skin remained too thick and consistent. The skin biopsy revealed histological features of a remarkable change of the middle and deep layers of derma appearing indicative of Scleroderma adultorum of Buschke which represents a rare genetic determined disorder. It is inherited dominantly and appeared most frequently in diabetic patients, characterized by accumulation of glucosaminoglycans in the derma. It was supposed that pathogenetic mechanisms were the disturbances of aldolase/reductase enzymes. The clinical case described here might be related to Autoimmune Polyglandulare Syndrome type 3. The rapidly worsening of hypothyroidism in the patient most likely was due to disturbances in peripheral metabolism and transport as a thyroid hormones in result of the severe renal failure.
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Issue: 3/2013
Author: Lozanov L. B., Gorcheva D., Lozanov B. S., Kadurina M., Tzankov N.
Abstract:
A woman aged 41 years underwent thyroidectomy for Graves’ disease 19 yr ago with postoperative hypothyroidism, supplemented by L-Thyroxin. Diabetes mellitus – type 1 was diagnosed two years later with consecutive development of diabetic microvascular complications and chronic renal failure. Within several months before addmission the patient manifested generalized edema and serose exudates, hypolbuminemia, high grade proteinuria and worsening of the metabolic control of diabetes which required high doses of insulin analogues in the intensive regimen. She was addmitted to The Departement with clinical manifestation of very severe hypothyroidism, TSH 71 mIU/L on the phone of L-Thyroxin 150 mig/24 h. After increasing of dose up to 300 mcg L-T4 in combination with Triiodothyronine 50 mcg/24 h the clinical signs of hypothyroidism disappeared. In result of the complex management the carbohydrate and electrolyte abnormalities were compensated, serose exudates and edema disappeared but the skin remained too thick and consistent. The skin biopsy revealed histological features of a remarkable change of the middle and deep layers of derma appearing indicative of Scleroderma adultorum of Buschke which represents a rare genetic determined disorder. It is inherited dominantly and appeared most frequently in diabetic patients, characterized by accumulation of glucosaminoglycans in the derma. It was supposed that pathogenetic mechanisms were the disturbances of aldolase/reductase enzymes. The clinical case described here might be related to Autoimmune Polyglandulare Syndrome type 3. The rapidly worsening of hypothyroidism in the patient most likely was due to disturbances in peripheral metabolism and transport as a thyroid hormones in result of the severe renal failure.
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