Prolonged high‐dose phosphate treatment: a risk factor for tertiary hyperparathyroidism in X‐linked hypophosphatemic rickets

Mäkitie, Outi; Kooh, Sang Whay; Sochett, Etienne

doi:10.1046/j.1365-2265.2003.01685.x

Cited by 61 publications

(44 citation statements)

References 29 publications

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“…Among them, we include secondary and tertiary hyperparathyroidism and nephrocalcinosis. Doses of phosphate above 50 mg/kg/day can induce PTH stimulation and give rise to secondary hyperparathyroidism with reduction of calcemia through the direct action of phosphate over the PTH secretion (59,70). The persistency of hyperparathyroidism for long periods of time can lead to autonomous function of parathyroid, characterizing the tertiary hyperparathyroidism, which, although rare, is severe, leading to intense bone resorption, nephrocalcinosis, and renal insufficiency.…”

Section: Complicationsmentioning

confidence: 99%

“…The persistency of hyperparathyroidism for long periods of time can lead to autonomous function of parathyroid, characterizing the tertiary hyperparathyroidism, which, although rare, is severe, leading to intense bone resorption, nephrocalcinosis, and renal insufficiency. Tertiary hyperparathyroidism can lead to hypercalcemia, while in the secondary form, calcium levels are normal or decreased (70). In the treatment of XHR the following factors favor the progression to tertiary hyperparathyroidism: early start of treatment, longer duration of treatment, high doses of elementary phosphorus (100 mg/kg/day), and very high PTH plasma levels (around 400 pg/mL) (70).…”

Section: Complicationsmentioning

confidence: 99%

“…Tertiary hyperparathyroidism can lead to hypercalcemia, while in the secondary form, calcium levels are normal or decreased (70). In the treatment of XHR the following factors favor the progression to tertiary hyperparathyroidism: early start of treatment, longer duration of treatment, high doses of elementary phosphorus (100 mg/kg/day), and very high PTH plasma levels (around 400 pg/mL) (70).…”

Section: Complicationsmentioning

confidence: 99%

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Hypophosphatemic rickets and osteomalacia

Filho

Castro²,

Damiani

2006

Arq Bras Endocrinol Metab

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The hypophosphatemic conditions that interfere in bone mineralization comprise many hereditary or acquired diseases, all of them sharing the same pathophysiologic mechanism: reduction in the phosphate reabsorption by the renal tubuli. This process leads to chronic hyperphosphaturia and hypophosphatemia, associated with inappropriately normal or low levels of calcitriol, causing osteomalacia or rickets in children and osteomalacia in adults. X-linked hypophosphatemic rickets, autosomaldominant hypophosphatemic rickets, and tumor-induced osteomalacia are the main syndromes involved in the hypophosphatemic rickets. Although these conditions exhibit different etiologies, there is a common link among them: increased activity of a phosphaturic factor, being the fibroblast growth factor 23 (FGF-23) the most studied one and to which is attributed a central role in the pathophysiology of the hyperphosphaturic disturbances. Activating mutations of FGF-23 and inactivating mutations in the PHEX gene (a gene on the X chromosome that codes for a Zn-metaloendopeptidase proteolytic enzyme which regulates the phosphate) involved in the regulation of FGF-23 have been identified and have been implicated in the pathogenesis of these disturbances. Genetic studies tend to show that the phosphorus homeostasis depends on a complex osteo-renal metabolic axis, whose mechanisms of interaction have been poorly understood so far. This paper reviews the current knowledge status concerning the pathophysiology of phosphate metabolism regulation and the pathophysiologic basis of hypophosphatemic rickets. It also analyzes the clinical picture and the therapeutic aspects of these conditions as well. RESUMORaquitismo Hipofosfatêmico e Osteomalácia. Os distúrbios hipofosfatêmicos que comprometem a mineralização óssea englobam várias doenças, hereditárias e adquiridas, as quais compartilham um mesmo mecanismo fisiopatológico: a diminuição da reabsorção de fosfato nos túbulos renais. Este processo promove hiperfosfatúria e hipofosfatemia crônicas, associadas a níveis inapropriadamente normais ou baixos de 1,25 (OH) 2 D 3 , com conseqüente desordem do metabolismo ósteo-mineral, resultando em raquitismo e osteomalá-cia na faixa etária pediátrica e em osteomalácia nos adultos. O raquitismo hipofosfatêmico ligado ao X, o raquitismo hipofosfatêmico autossômico dominante e a osteomalácia induzida por tumor são as principais síndromes que constituem os raquitismos hipofosfatêmicos. Apesar de estas doenças apresentarem etiopatogenias distintas, as evidências bioquímico-moleculares indicam uma base fisiopatológica em comum: maior atividade de um agente fosfatúrico, sendo o fator de crescimento do fibroblasto 23 (FGF-23) o mais estudado e ao qual é atribuído um papel central na fisiopatologia destes distúrbios. Várias mutações ativadoras do gene do FGF-23 e mutações inativadoras do

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Section: Complicationsmentioning

confidence: 99%

Section: Complicationsmentioning

confidence: 99%

Section: Complicationsmentioning

confidence: 99%

See 1 more Smart Citation

Hypophosphatemic rickets and osteomalacia

Filho

Castro²,

Damiani

2006

Arq Bras Endocrinol Metab

View full text Add to dashboard Cite

show abstract

“…A review of the previously reported cases (Table 1) 5, [7][8][9][10][11][12][13] shows us the different treatments, the recurrence, complications, and hospital stay. The literature presents the resection of a single or double adenoma as an effective treatment, but many authors defend that subtotal parathyroidectomy is necessary for operative cure.…”

Section: Wjoesmentioning

confidence: 99%

“…[14][15][16][17][18][19] The largest series of surgical management of tertiary hyperparathyroidism associated with XLHR contains six patients: Three had total parathyroidectomy, two had three parathyroid glands resected, one of them required completion parathyroidectomy for recurrent disease, and one had two abnormal parathyroid glands resected, dying in the follow-up. 13 The size of the resected parathyroid tissue maybe is a predictor of the HBS in patients with XLHR after parathyroidectomy Surgical management of tertiary hyperparathyroidism secondary to XLHR is still unclear, but it is mandatory for a thorough neck exploration. …”

Section: Wjoesmentioning

confidence: 99%