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Hepatomegaly and hyperlipidemia in a toddler.

作者信息

Pai Gautham, Achutha Divya

机构信息

Department of Pediatrics, Yenepoya Medical College Hospital, Mangalore, 575 018, India.

Department of Pathology, Yenepoya Medical College Hospital, Mangalore, 575 018, India.

出版信息

Indian J Gastroenterol. 2024 May 23. doi: 10.1007/s12664-024-01609-y.

DOI:10.1007/s12664-024-01609-y
PMID:38780879
Abstract
摘要

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1
Hepatomegaly and hyperlipidemia in a toddler.一名幼儿出现肝肿大和高脂血症。
Indian J Gastroenterol. 2024 May 23. doi: 10.1007/s12664-024-01609-y.
2
A toddler with hepatomegaly.一名患有肝肿大的幼儿。
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Fenofibrate-promoted hepatomegaly and liver regeneration are PPAR-dependent and partially related to the YAP pathway.非诺贝特促进的肝肿大和肝再生是过氧化物酶体增殖物激活受体（PPAR）依赖性的，并且部分与Yes相关蛋白（YAP）途径有关。
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本文引用的文献

1
Clinical and Histologic Liver Improvement in Siblings With Lysosomal Acid Lipase Deficiency After Enzyme Replacement.酶替代治疗后溶酶体酸性脂肪酶缺乏症患者的兄弟姐妹的临床和组织学肝脏改善。
J Pediatr Gastroenterol Nutr. 2020 May;70(5):635-639. doi: 10.1097/MPG.0000000000002671.
2
Liver histology in cholesteryl ester storage disease.
Indian J Pathol Microbiol. 2018 Apr-Jun;61(2):302-304. doi: 10.4103/IJPM.IJPM_260_17.
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Lysosomal acid lipase deficiency allograft recurrence and liver failure- clinical outcomes of 18 liver transplantation patients.溶酶体酸性脂肪酶缺乏症肝移植复发和肝功能衰竭-18 例肝移植患者的临床结局。
Mol Genet Metab. 2018 May;124(1):11-19. doi: 10.1016/j.ymgme.2018.03.010. Epub 2018 Mar 27.
4
Sebelipase alfa over 52 weeks reduces serum transaminases, liver volume and improves serum lipids in patients with lysosomal acid lipase deficiency.在52周的时间里， sebelipase alfa可降低溶酶体酸性脂肪酶缺乏症患者的血清转氨酶、肝脏体积，并改善血脂水平。
J Hepatol. 2014 Nov;61(5):1135-42. doi: 10.1016/j.jhep.2014.06.022. Epub 2014 Jun 30.
5
Cholesteryl ester storage disease: review of the findings in 135 reported patients with an underdiagnosed disease.胆固醇酯贮积症：135 例报道患者的发现回顾——一种被低估的疾病。
J Hepatol. 2013 Jun;58(6):1230-43. doi: 10.1016/j.jhep.2013.02.014. Epub 2013 Feb 26.
6
Cholesteryl ester storage disease: protean presentations of lysosomal acid lipase deficiency.胆固醇酯贮积症：溶酶体酸性脂肪酶缺乏的多样表现。
J Pediatr Gastroenterol Nutr. 2013 Jun;56(6):682-5. doi: 10.1097/MPG.0b013e31828b36ac.
7
Intractable ascites as a manifestation of Wolman's disease: report of two sibs.以沃曼病表现的顽固性腹水：两例同胞病例报告。
Indian J Gastroenterol. 2012 Dec;31(6):343-5. doi: 10.1007/s12664-012-0253-6. Epub 2012 Sep 25.
8
Distinctive histopathological features that support a diagnosis of cholesterol ester storage disease in liver biopsy specimens.在肝活检标本中具有支持胆固醇酯贮积病诊断的独特组织病理学特征。
Histopathology. 2012 Jun;60(7):1107-13. doi: 10.1111/j.1365-2559.2011.04164.x.
9
Cholesterol ester storage disease with unusual neurological manifestations in two siblings: a report from South India.
J Child Neurol. 2007 Dec;22(12):1401-4. doi: 10.1177/0883073807307104.