{"title":"非肥胖胰岛素抵抗型糖尿病患者造血干细胞移植后严重肝脂肪变性导致肝功能障碍。","authors":"Haruki Yamano, Reiko Kagawa, Yoko Saito, Ryosei Iemura, Eriko Adachi, Gau Maki, Takeru Yamauchi, Ryuichi Nakagawa, Akito Sutani, Shigeru Takishima, Fukiko Kitahata, Miyako Murakawa, Takeshi Isoda, Takahiro Kamiya, Satoshi Okada, Tomohiro Morio, Kei Takasawa, Kenichi Kashimada","doi":"10.1111/ped.70192","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Non-obese survivors of childhood hematopoietic stem cell transplantation (HSCT) often exhibit severe insulin resistance and mild hepatic dysfunction. Although the exact pathophysiology remains unclear, one possible explanation is the increasingly recognized condition known as HSCT-associated partial lipodystrophy. In lipodystrophy, hepatic dysfunction is characterized by fibrosis and severe steatosis, resembling nonalcoholic steatohepatitis. Accordingly, detailed hepatic assessment would improve our overall understanding of the condition. This study aimed to evaluate hepatic dysfunction using a non-invasive method (FibroScan) in patients who developed glucose intolerance post-HSCT.</p><p><strong>Methods: </strong>Fourteen non-obese childhood HSCT survivors from two institutions underwent FibroScan. They were classified according to the presence or absence of diabetes mellitus (DM) with insulin resistance (HOMA-IR >2.5 or insulin therapy >1.5 U/kg/day). FibroScan provided controlled attenuation parameter (CAP) for hepatic steatosis and liver stiffness measurements (LSM) for fibrosis. Serum hepatic fibrosis markers (collagen type IV and hyaluronic acid) were also measured.</p><p><strong>Results: </strong>Seven patients developed insulin-resistant DM while seven had normal glucose/lipid metabolism. All DM patients showed \"Dunnigan\"-type subcutaneous fat distribution. CAP values were higher in the DM group [307 (261.0-343.5) vs. 237 (216.5-271.0), p = 0.041], as were LSM scores [6.7 (5.9-9.9) vs. 4.1 (3.5-4.7), p = 0.007], indicating more severe steatosis and increased liver stiffness. However, serum hepatic fibrosis markers did not differ significantly.</p><p><strong>Conslusions: </strong>Non-obese HSCT survivors with insulin-resistant DM exhibited severe hepatic fat accumulation and progression of liver fibrosis attributable to lipodystrophy. FibroScan may have potential utility for monitoring liver health in this population.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e70192"},"PeriodicalIF":0.9000,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Severe hepatic steatosis contributes to liver dysfunction in non-obese patients with insulin-resistant diabetes mellitus after hematopoietic stem cell transplantation.\",\"authors\":\"Haruki Yamano, Reiko Kagawa, Yoko Saito, Ryosei Iemura, Eriko Adachi, Gau Maki, Takeru Yamauchi, Ryuichi Nakagawa, Akito Sutani, Shigeru Takishima, Fukiko Kitahata, Miyako Murakawa, Takeshi Isoda, Takahiro Kamiya, Satoshi Okada, Tomohiro Morio, Kei Takasawa, Kenichi Kashimada\",\"doi\":\"10.1111/ped.70192\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Non-obese survivors of childhood hematopoietic stem cell transplantation (HSCT) often exhibit severe insulin resistance and mild hepatic dysfunction. Although the exact pathophysiology remains unclear, one possible explanation is the increasingly recognized condition known as HSCT-associated partial lipodystrophy. In lipodystrophy, hepatic dysfunction is characterized by fibrosis and severe steatosis, resembling nonalcoholic steatohepatitis. Accordingly, detailed hepatic assessment would improve our overall understanding of the condition. This study aimed to evaluate hepatic dysfunction using a non-invasive method (FibroScan) in patients who developed glucose intolerance post-HSCT.</p><p><strong>Methods: </strong>Fourteen non-obese childhood HSCT survivors from two institutions underwent FibroScan. They were classified according to the presence or absence of diabetes mellitus (DM) with insulin resistance (HOMA-IR >2.5 or insulin therapy >1.5 U/kg/day). FibroScan provided controlled attenuation parameter (CAP) for hepatic steatosis and liver stiffness measurements (LSM) for fibrosis. Serum hepatic fibrosis markers (collagen type IV and hyaluronic acid) were also measured.</p><p><strong>Results: </strong>Seven patients developed insulin-resistant DM while seven had normal glucose/lipid metabolism. All DM patients showed \\\"Dunnigan\\\"-type subcutaneous fat distribution. CAP values were higher in the DM group [307 (261.0-343.5) vs. 237 (216.5-271.0), p = 0.041], as were LSM scores [6.7 (5.9-9.9) vs. 4.1 (3.5-4.7), p = 0.007], indicating more severe steatosis and increased liver stiffness. However, serum hepatic fibrosis markers did not differ significantly.</p><p><strong>Conslusions: </strong>Non-obese HSCT survivors with insulin-resistant DM exhibited severe hepatic fat accumulation and progression of liver fibrosis attributable to lipodystrophy. FibroScan may have potential utility for monitoring liver health in this population.</p>\",\"PeriodicalId\":20039,\"journal\":{\"name\":\"Pediatrics International\",\"volume\":\"67 1\",\"pages\":\"e70192\"},\"PeriodicalIF\":0.9000,\"publicationDate\":\"2025-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Pediatrics International\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1111/ped.70192\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"PEDIATRICS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatrics International","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1111/ped.70192","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"PEDIATRICS","Score":null,"Total":0}
Severe hepatic steatosis contributes to liver dysfunction in non-obese patients with insulin-resistant diabetes mellitus after hematopoietic stem cell transplantation.
Background: Non-obese survivors of childhood hematopoietic stem cell transplantation (HSCT) often exhibit severe insulin resistance and mild hepatic dysfunction. Although the exact pathophysiology remains unclear, one possible explanation is the increasingly recognized condition known as HSCT-associated partial lipodystrophy. In lipodystrophy, hepatic dysfunction is characterized by fibrosis and severe steatosis, resembling nonalcoholic steatohepatitis. Accordingly, detailed hepatic assessment would improve our overall understanding of the condition. This study aimed to evaluate hepatic dysfunction using a non-invasive method (FibroScan) in patients who developed glucose intolerance post-HSCT.
Methods: Fourteen non-obese childhood HSCT survivors from two institutions underwent FibroScan. They were classified according to the presence or absence of diabetes mellitus (DM) with insulin resistance (HOMA-IR >2.5 or insulin therapy >1.5 U/kg/day). FibroScan provided controlled attenuation parameter (CAP) for hepatic steatosis and liver stiffness measurements (LSM) for fibrosis. Serum hepatic fibrosis markers (collagen type IV and hyaluronic acid) were also measured.
Results: Seven patients developed insulin-resistant DM while seven had normal glucose/lipid metabolism. All DM patients showed "Dunnigan"-type subcutaneous fat distribution. CAP values were higher in the DM group [307 (261.0-343.5) vs. 237 (216.5-271.0), p = 0.041], as were LSM scores [6.7 (5.9-9.9) vs. 4.1 (3.5-4.7), p = 0.007], indicating more severe steatosis and increased liver stiffness. However, serum hepatic fibrosis markers did not differ significantly.
Conslusions: Non-obese HSCT survivors with insulin-resistant DM exhibited severe hepatic fat accumulation and progression of liver fibrosis attributable to lipodystrophy. FibroScan may have potential utility for monitoring liver health in this population.
期刊介绍：
Publishing articles of scientific excellence in pediatrics and child health delivery, Pediatrics International aims to encourage those involved in the research, practice and delivery of child health to share their experiences, ideas and achievements. Formerly Acta Paediatrica Japonica, the change in name in 1999 to Pediatrics International, reflects the Journal''s international status both in readership and contributions (approximately 45% of articles published are from non-Japanese authors). The Editors continue their strong commitment to the sharing of scientific information for the benefit of children everywhere.
Pediatrics International opens the door to all authors throughout the world. Manuscripts are judged by two experts solely upon the basis of their contribution of original data, original ideas and their presentation.
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