Pulmonary histocytosis X: clinical observation of early-stage disease

Фролова, И. Г.; Величко, С. А.; Гольдберг, А. В.; Тузиков, С. А.; Miller, SW; Родионов, Е. О.; Ефтеев, Л. А.; Бондарь, Л. Н.; Перельмутер, В. М.

doi:10.20538/1682-0363-2017-2-195-201

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“…Гистиоцитоз из клеток Лангерганса (прежние термины: гистиоцитоз Х, болезнь Хенда-Шюллера-Крисчена, эозинофильная гранулема, болезнь Таратынова, болезнь Леттерера-Сиве) -исключительно разнообразное по клиническим проявлениям и течению заболевание, характеризующееся накоплением и/или пролиферацией в очагах поражения клеток с характеристиками эпидермальных гистиоцитов -клеток Лангерганса [1,2,3,4]. В детском возрасте встречается с частотой 3-4 случая на 1 миллион детского населения в год, у взрослых -еще реже, с частотой, не превышающей 1:560 000 взрослых.…”

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Surgical aspects of treating a child with histiocytosis of the lungs

Стальмахович

Сапухин

et al. 2019

jour

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Objective: to present a case of successful treatment of rare severe lung lesions in a child.Methods: the article analyzes the result of the treatment of lung histiocytosis in a child aged 4 years. The clinical manifestation of the disease began with an increase in the parotid lymph nodes, otitis, pneumonia, and rapidly progressive respiratory failure. On the roentgenogram, bulle transformation of both lungs was noted. The severity of the disease was complicated by bilateral pneumothorax, the formation of bronchopleural fistulas. Histiocytosis of the lungs from Langerhans cells (GCR) was determined morphologically when examining tissue taken at thoracoscopy. Endoscopically, the lung was without signs of inflammation, with multiple subpleural air cavities. The child underwent chemotherapy (vinblastine, prednisone), repeated drainage of the pleural cavity with recurrent intense pneumothorax. For health reasons, a longitudinal sternotomy was performed, total decortication of the costal pleura on both sides (mechanical pleurodesis), which contributed to subsequent fixation of the lung to the chest wall and prevention of recurrent total stressed pneumothorax. Twice developed limited pneumothorax was stopped by drainage and chemical pleurodesis.Results: Against the background of ongoing complex therapy (drainage of the pleural cavity, mechanical and chemical pleurodesis, chemo- and antibacterial therapy), the condition improved (clinically and radiographically). The child was discharged on day 152 of treatment. On the control MSCT of the lungs, eight months after discharge, there are minimal signs of bullous transformation, and the symptoms of pneumosclerosis of both lungs are moderate. There are no manifestations of pulmonary hypertension.Conclusion: verification of the diagnosis of pulmonary GLC is very difficult. The complex treatment requires the participation of a wide range of doctors, including a pediatric surgeon.

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Section: Introductionunclassified

Surgical aspects of treating a child with histiocytosis of the lungs

Стальмахович

Сапухин

et al. 2019

jour

View full text Add to dashboard Cite

show abstract

Pulmonary histocytosis X: clinical observation of early-stage disease

Cited by 1 publication

References 8 publications

Surgical aspects of treating a child with histiocytosis of the lungs

Surgical aspects of treating a child with histiocytosis of the lungs

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