Literature DB >> 1768581

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

V A Redfield1, F Mimouni, F C Strife, R C Tsang.   

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.

Entities:  

Mesh:

Substances:

Year:  1991        PMID: 1768581     DOI: 10.1007/bf00857875

Source DB:  PubMed          Journal:  Pediatr Nephrol        ISSN: 0931-041X            Impact factor:   3.714


  8 in total

1.  Elevated nucleotide pyrophosphatase activity in cultured skin fibroblasts from patients with Lowe's syndrome.

Authors:  K Horie; T Yano; I Funakoshi; I Yamashina
Journal:  Clin Chim Acta       Date:  1988-09-30       Impact factor: 3.786

Review 2.  Oculo-cerebro-renal syndrome. A review.

Authors:  V Abbassi; C U Lowe; P L Calcagno
Journal:  Am J Dis Child       Date:  1968-02

3.  Synthesis and sulfation of glycosaminoglycans in fibroblasts from a patient with Lowe's syndrome.

Authors:  P V Donnelly; P Reed; N DiFerrante
Journal:  Connect Tissue Res       Date:  1984       Impact factor: 3.417

4.  Bone response to phosphate salts, ergocalciferol, and calcitriol in hypophosphatemic vitamin D-resistant rickets.

Authors:  F H Glorieux; P J Marie; J M Pettifor; E E Delvin
Journal:  N Engl J Med       Date:  1980-10-30       Impact factor: 91.245

5.  Continuous nasogastric phosphorus infusion in hypophosphatemic rickets of prematurity.

Authors:  W W Koo; G Antony; L H Stevens
Journal:  Am J Dis Child       Date:  1984-02

6.  Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome.

Authors:  W A Gahl; I Bernardini; M Dalakas; W B Rizzo; G S Harper; J M Hoeg; O Hurko; J Bernar
Journal:  J Clin Invest       Date:  1988-02       Impact factor: 14.808

7.  Idiopathic hypercalciuria in children: prevalence and metabolic characteristics.

Authors:  E S Moore; F L Coe; B J McMann; M J Favus
Journal:  J Pediatr       Date:  1978-06       Impact factor: 4.406

8.  Hyperparathyroidism in patients with X-linked dominant hypophosphatemic rickets--application of the calcium infusion test as an indicator for parathyroidectomy.

Authors:  U Alon; H Newsome; J C Chan
Journal:  Int J Pediatr Nephrol       Date:  1984-03
  8 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.