Improvement of lactic acidosis in pyruvate dehydrogenase complex deficiency using custom-made amino acid-based dialysate for peritoneal dialysis.

IF 2.6 3区 医学 Q1 PEDIATRICS Pediatric Nephrology Pub Date : 2025-07-01 Epub Date: 2025-01-29 DOI:10.1007/s00467-025-06679-4
Manson Chon In Kuok, Chi Kwan Jasmine Chow, Ngai Man Chan, Winnie Kwai Yu Chan
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Abstract

This case report presents a newborn with pyruvate dehydrogenase complex deficiency who developed significant lactic acidosis and acute kidney injury after birth. Peritoneal dialysis with glucose-based peritoneal dialysis fluid was initially started, but the patient had worsening hyperglycemia and lactic acidosis, likely related to excess glucose reabsorption with shunting to lactate due to the underlying metabolic disorder. As amino acid-based dialysis solution was not available in our formulary, a dialysis fluid was manually created with Vaminolact, which was commonly used in neonatal parenteral nutrition. Switching to the new dialysis fluid led to significant biochemical improvement.

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定制氨基酸基腹膜透析液改善丙酮酸脱氢酶复合物缺乏症乳酸性酸中毒。
本病例报告介绍了一个新生儿丙酮酸脱氢酶复合物缺乏症,出生后出现明显的乳酸酸中毒和急性肾损伤。最初开始使用以葡萄糖为基础的腹膜透析液进行腹膜透析,但患者出现高血糖和乳酸性酸中毒恶化,可能与潜在代谢紊乱导致的过量葡萄糖重吸收并分流至乳酸有关。由于我们的处方中没有以氨基酸为基础的透析液,所以我们用Vaminolact手工配制了透析液,这通常用于新生儿肠外营养。改用新的透析液导致了显著的生化改善。
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来源期刊
Pediatric Nephrology
Pediatric Nephrology 医学-泌尿学与肾脏学
CiteScore
4.70
自引率
20.00%
发文量
465
审稿时长
1 months
期刊介绍: International Pediatric Nephrology Association Pediatric Nephrology publishes original clinical research related to acute and chronic diseases that affect renal function, blood pressure, and fluid and electrolyte disorders in children. Studies may involve medical, surgical, nutritional, physiologic, biochemical, genetic, pathologic or immunologic aspects of disease, imaging techniques or consequences of acute or chronic kidney disease. There are 12 issues per year that contain Editorial Commentaries, Reviews, Educational Reviews, Original Articles, Brief Reports, Rapid Communications, Clinical Quizzes, and Letters to the Editors.
期刊最新文献
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