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Severe rickets in Lowe syndrome: treatment with continuous nasogastric infusion.

作者信息

Redfield V A, Mimouni F, Strife F C, Tsang R C

机构信息

Department of Pediatrics, Children's Hospital Medical Center, University of Cincinnati College of Medicine, OH 45267-0541.

出版信息

Pediatr Nephrol. 1991 Nov;5(6):696-9. doi: 10.1007/BF00857875.

DOI:10.1007/BF00857875
PMID:1768581
Abstract

A boy with Lowe syndrome who manifested renal Fanconi syndrome by severe hypophosphatemic rickets, failure to thrive, and metabolic acidosis failed to improve with conventional bolus therapy of phosphate and bicarbonate. He was then placed on home continuous nasogastric infusion of phosphate and bicarbonate in addition to caloric supplementation. Rapid reversal of metabolic acidosis and hypophosphatemia was achieved. There was significant improvement in clinical and radiological signs of rickets and in bone mineral content. After 9 months therapy, his ponderal age improved dramatically from 7.5 to 29.5 months and his statural age from 16 to 26 months. We conclude that continuous nasogastric infusion of phosphorus and bicarbonate is a useful alternative mode of therapy in patients with renal Fanconi syndrome who are resistant to conventional bolus therapy.

摘要

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本文引用的文献

1
Continuous nasogastric phosphorus infusion in hypophosphatemic rickets of prematurity.持续性经鼻胃管输注磷治疗早产儿低磷血症性佝偻病
Am J Dis Child. 1984 Feb;138(2):172-5. doi: 10.1001/archpedi.1984.02140400054013.
2
Hyperparathyroidism in patients with X-linked dominant hypophosphatemic rickets--application of the calcium infusion test as an indicator for parathyroidectomy.X连锁显性低磷性佝偻病患者的甲状旁腺功能亢进——钙输注试验作为甲状旁腺切除术指标的应用
Int J Pediatr Nephrol. 1984 Mar;5(1):39-43.
3
Bone response to phosphate salts, ergocalciferol, and calcitriol in hypophosphatemic vitamin D-resistant rickets.
低磷性维生素D抵抗性佝偻病中骨骼对磷酸盐、麦角钙化醇和骨化三醇的反应
N Engl J Med. 1980 Oct 30;303(18):1023-31. doi: 10.1056/NEJM198010303031802.
4
Synthesis and sulfation of glycosaminoglycans in fibroblasts from a patient with Lowe's syndrome.来自一名患有劳氏综合征患者的成纤维细胞中糖胺聚糖的合成与硫酸化
Connect Tissue Res. 1984;13(1):89-98. doi: 10.3109/03008208409152146.
5
Oculo-cerebro-renal syndrome. A review.
Am J Dis Child. 1968 Feb;115(2):145-68. doi: 10.1001/archpedi.1968.02100010147003.
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Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome.
J Clin Invest. 1988 Feb;81(2):549-60. doi: 10.1172/JCI113353.
7
Elevated nucleotide pyrophosphatase activity in cultured skin fibroblasts from patients with Lowe's syndrome.洛氏综合征患者培养的皮肤成纤维细胞中核苷酸焦磷酸酶活性升高。
Clin Chim Acta. 1988 Sep 30;177(1):41-8. doi: 10.1016/0009-8981(88)90305-1.
8
Idiopathic hypercalciuria in children: prevalence and metabolic characteristics.儿童特发性高钙尿症:患病率及代谢特征
J Pediatr. 1978 Jun;92(6):906-10. doi: 10.1016/s0022-3476(78)80358-8.