problendoПроблемы ЭндокринологииProblems of Endocrinology0375-96602308-1430Endocrinology Research Centre10.14341/probl12723problendo-12723Research ArticleДетская эндокринологияPediatric EndocrinologyГепатопульмональный синдром — редкая манифестация цирроза печени у пациентки с диэнцефальным ­ожирением после удаления краниофарингиомыHepatopulmonary syndrome: a rare manifestation of cirrhosis in patient with diencephalic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngiomahttps://orcid.org/0000-0002-0428-0498МазеркинаН. А.MazerkinaN. A.

Мазеркина Надежда Александровна - доктор медицинских наук, профессор.

125047 Москва, 1-я Тверская-Ямская, 16.

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Nadia A. Mazerkina - MD, PhD, Professor.

16, 4 Tverskaya-Yamskaya, 125047, Moscow.

eLibrary SPIN: 1012-2923

nmazer@nsi.ruhttps://orcid.org/0000-0001-6433-1202СаватеевА. Н.SavateevA. N.

Саватеев Александр Николаевич - кандидат медицинских наук, нейрохирург.

Москва.

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Alexander N. Savateev – MD.

Moscow.

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asavateev@nsi.ruhttps://orcid.org/0000-0003-0984-2039ГорелышевС. К.GorelyshevS. K.

Горелышев Сергей Кириллович - доктор медицинских наук, профессор.

125047 Москва, 1-я Тверская-Ямская, 16.

eLibrary SPIN: 6686-3132

Sergey K. Gorelyshev - MD, PhD, Professor.

16, 4 Tverskaya-Yamskaya, 125047, Moscow.

eLibrary SPIN: 6686-3132

sgorel@nsi.ruhttps://orcid.org/0000-0002-0108-0677МаряшевС. А.MariashevS. A.

Маряшев Сергей Алексеевич - доктор медицинских наук.

125047 Москва, 1-я Тверская-Ямская, 16.

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Sergey A. Mariashev - MD, PhD.

16, 4 Tverskaya-Yamskaya, 125047, Moscow.

eLibrary SPIN: 3980-1278

mars@nsi.ruhttps://orcid.org/0000-0002-0192-186XБереговскаяС. А.BeregovskayaS. A.

Береговская Светлана Александровна.

Москва.

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Svetlana A. Beregovskaya.

Moscow.

eLibrary SPIN: 1119-4245

beregsa@mail.ruhttps://orcid.org/0000-0002-1731-6568КоноваловА. Н.KonovalovA. N.

Коновалов Александр Николаевич - доктор медицинских наук, профессор, академик РАН.

125047 Москва, 1-я Тверская-Ямская, 16.

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Alexandre N. Konovalov - MD, PhD, Professor, Acad. RAS.

16, 4 Tverskaya-Yamskaya, 125047, Moscow.

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AKonovalov@nsi.ruНациональный медицинский исследовательский центр нейрохирургии им. Н.Н. БурденкоРоссияBurdenko Neurosurgery InstituteRussian FederationАО «Деловой центр нейрохирургии»РоссияBusiness Center for NeurosurgeryRussian FederationНациональный медицинский исследовательский центр терапии и профилактической медициныРоссияСenter for therapy and preventive medicineRussian Federation2021031020216755866Copyright © Мазеркина Н.А., Саватеев А.Н., Горелышев С.К., Маряшев С.А., Береговская С.А., Коновалов А.Н., 20212021Мазеркина Н.А., Саватеев А.Н., Горелышев С.К., Маряшев С.А., Береговская С.А., Коновалов А.Н.Mazerkina N.A., Savateev A.N., Gorelyshev S.K., Mariashev S.A., Beregovskaya S.A., Konovalov A.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.probl-endojournals.ru/jour/article/view/12723

В статье описан клинический случай пациентки 15 лет, у которой в результате удаления краниофарингиомы, помимо несахарного диабета и пангипопитуитаризма, развилось диэнцефальное ожирение, приведшее к формированию цирроза печени. В данном случае патология печени манифестировала гепатопульмональным синдромом, характеризующимся гипоксемией, причиной которой является патологическое расширение сосудов легких, приводящее к шунтированию крови у пациентов с поражением печени. Несмотря на отсутствие рецидива опухоли после удаления и радиохирургического лечения, а также адекватную заместительную гормональную терапию, пациентка скончалась через 9 лет после операции. Заместительная терапия гормоном роста у пациентов с гипопитуитаризмом и жировым гепатозом может уменьшить аккумуляцию липидов в печени и снизить риск развития цирроза.

We describe a 15-year girl, who developed panhypopituitarism and diencephalic obesity after surgical excision of craniopharyngioma, followed by nonalcoholic fatty liver disease and cirrhosis 5 years after surgery. Cirrhosis in this case manifested by hypoxia due to hepatopulmonary syndrome, and despite cure of craniopharyngioma by surgery and radiosurgery treatment and adequate hormonal substitution therapy patient died 9 years after surgery. Growth hormone substitutional therapy in patients with hypopituitarism, and steatohepatitis may decrease liver triglyceride accumulation and prevent end-stage liver disease.

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The authors declare that there are no conflicts of interest present.