• Болезнь Горема у 11-летнего мальчика с поражением грудного и поясничного отделов позвоночника, ребер, с двусторонним гидротораксом. Клинический случай

Болезнь Горема у 11-летнего мальчика с поражением грудного и поясничного отделов позвоночника, ребер, с двусторонним гидротораксом. Клинический случай

SOVREMENNAYA PEDIATRIYA.2018.7(95):51-58; doi 10.15574/SP.2018.95.51

Дорош О. И., Дудаш П. И., Петрончак О. А., Масинник Ю. С., Селезнев А. А., Щуровский А. М., Киричук И. В., Мелько И. П.
Коммунальное некоммерческое предприятие Львовского областного совета «Западноукраинский специализированный детский медицинский центр», Украина
Коммунальное учреждение Львовского областного совета «Львовское областное патологоанатомическое бюро», Украина
Центр медицинских инноваций «NOVO», г. Львов, Украина
Коммунальная 4я городская клиническая больница, г.Львов, Украина
Патоморфологическая лаборатория «CSD Health Care», г. Киев, Украина

Болезнь Горема (Gorham–Stout disease (GSD)) — это редкое заболевание неясной этиологии, характеризующееся спонтанным и прогрессирующим идиопатическим массивным остеолизом одной или нескольких костей скелета без корреляции с полом, рассой, типом наследования и географического места рождения пациентов. В международной литературе описано около 200 случаев заболевания. Рентгенографические изменения при GSD довольно специфические, в некоторых случаях возможна полная резорбция пораженной кости с признаками так называемой «фантомной кости» или исчезновения костей. Целью сообщения является ознакомление медицинского сообщества с этим редким заболеванием и обсуждение гистологической и клинической презентации болезни Горема с поражением грудного и поясничного отделов позвоночника, ребер, с двусторонним гидротораксом, особенностями радиологических находок с кратким обзором литературы.

Ключевые слова: Gorham–Stout disease, остеолизис, гемангиоматоз, болезни костей, дети, редкие болезни.

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Статья поступила в редакцию 12.05.2018, принята к печати 15.11.2018.

 

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