Nakagawa M, Kise K, Okamoto N, Fujino H, Iwai M, Nomura Y, Sawa H
Department of Pediatrics, Shiga University of Medical Science, Seta, Otsu, Japan.
Clin Nephrol. 2005 Apr;63(4):313-6. doi: 10.5414/cnp63313.
We describe a 40-month-old male infant with renal failure, treated with peritoneal dialysis, who developed massive calcification of soft tissues including the heart and lungs with subsequent cardiopulmonary insufficiency. A diagnosis of Jeune syndrome was made. After starting peritoneal dialysis, the patient exhibited an intractable metabolic acidosis of unknown etiology necessitating treatment with intravenous or oral sodium bicarbonate. Myocardial calcification was first detected by 2-dimensional echocardiography performed 3 months after starting dialysis. The patient was not suitable for renal transplantation because of his cardiac dysfunction and died of cardiac and respiratory failure at the age of 6 years. Although the patient exhibited a variety of risk factors for ectopic calcification including hyperphosphatemia, hyperparathyroidism, high calcium-phosphate product and treatment with vitamin D, the early and massive soft tissue calcification may have been accelerated by correction of the metabolic acidosis. Therefore, the use of sodium bicarbonate may be involved in the etiology of the myocardial calcification.
我们描述了一名40个月大的患有肾衰竭的男婴，接受了腹膜透析治疗，他出现了包括心脏和肺部在内的软组织大量钙化，随后出现心肺功能不全。诊断为耶拿综合征。开始腹膜透析后，患者出现了病因不明的顽固性代谢性酸中毒，需要静脉或口服碳酸氢钠治疗。透析开始3个月后通过二维超声心动图首次检测到心肌钙化。由于心脏功能障碍，该患者不适合进行肾移植，6岁时死于心脏和呼吸衰竭。尽管患者表现出多种异位钙化的危险因素，包括高磷血症、甲状旁腺功能亢进、高钙磷乘积以及维生素D治疗，但代谢性酸中毒的纠正可能加速了早期和大量的软组织钙化。因此，使用碳酸氢钠可能与心肌钙化的病因有关。
