Renal Function in Wilson??s Disease: Response to Penicillamine Therapy

Leu, Ming-Sheng; Strickland, G. Thomas; Gutman, Robert

doi:10.1097/00000441-197012000-00007

Cited by 44 publications

(30 citation statements)

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“…When sought, evidence of defective tubular reabsorptive capacity for glucose and phosphate, defective urinary acidification and hypercalciuria and uricosuria can usually be detected. Reversal of a number of these features following penicillamine therapy has been recorded [79,106].…”

Section: Renal Tubular Disordersmentioning

confidence: 99%

Abnormal Renal Urate Homeostasis in Systemic Disorders

Emmerson

Ravenscroft

1975

Nephron

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Abnormalities of renal handling of urate occur in a wide variety of physiological and pathological conditions and are mediated by factors including renal blood flow, glomerular filtration rate, urine flow rate, urinary constituents, metabolites, hormones and drugs. The determination of the aetiological factors in each abnormal situation is complex and the problem is discussed in relation to a variety of conditions including renal tubular disorders and metal intoxications, hypertension, toxaemia of pregnancy, glycogen storage disease, fructose administration, hereditary fructose intolerance, as well as obesity, regular alcohol consumption and hyperlipoproteinaemia. Apart from those diseases, usually genetically determined, which are associated with excessive production of urate, the most common causes of hyperuricaemia act at a renal level and result in a reduction in the net renal excretion of urate.

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Section: Renal Tubular Disordersmentioning

confidence: 99%

Abnormal Renal Urate Homeostasis in Systemic Disorders

Emmerson

Ravenscroft

1975

Nephron

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“…Therefore, it is thought that mutation of ATP7B gene may induce an abnormality of copper metabolism in the kidney. Indeed, patients with Wilson's disease are known to develop renal dysfunction [15]. Increased Cu levels [36] and tissue damage [35] were shown in LEC rat kidneys.…”

Section: Introductionmentioning

confidence: 99%

“…Leu et al [15] reported that D-penicillamine therapy improves renal function of Wilson's disease patients. Santon et al [26] reported that Zn treatment might provide cellular protection against Cu toxicity in LEC rat kidneys.…”

Section: Introductionmentioning

confidence: 99%

Inhibitory Effects of Trientine, a Copper-Chelating Agent, on Induction of DNA Strand Breaks in Kidney Cells of Long-Evans Cinnamon (LEC) Rats

et al. 2005

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“…With the demonstration of ami noaciduria in WD. it has been recognized that some patients with WD may exhibit derangement of renal func tion [10][11][12][13]. In a study of 27 Chinese patients with WD.…”

Section: Resultsmentioning

confidence: 99%

Recurrent Hypokalemic Muscle Weakness as an Initial Manifestation of Wilson’s Disease

Nakazawa

Kaneko²,

Hoshi³

et al. 1996

Nephron

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A 24-year-old man had several episodes of hypokalemic muscle weakness of undetermined etiology since the age of 13 years. Wilson’s disease (WD) was not diagnosed until the age of 18 when wing-beating tremor in the left upper limb was noted. Renal function study revealed incomplete proximal renal tubular acidosis. The hypokalemic muscle weakness and wing-beating tremor had subsided after long-term penicillamine therapy. The present case indicates that recurrent hypokalemic paralysis due to renal tubular acidosis is a rare initial presentation of WD and may respond to penicillamine therapy.

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Renal Function in Wilson??s Disease: Response to Penicillamine Therapy

Cited by 44 publications

References 0 publications

Abnormal Renal Urate Homeostasis in Systemic Disorders

Abnormal Renal Urate Homeostasis in Systemic Disorders

Inhibitory Effects of Trientine, a Copper-Chelating Agent, on Induction of DNA Strand Breaks in Kidney Cells of Long-Evans Cinnamon (LEC) Rats

Recurrent Hypokalemic Muscle Weakness as an Initial Manifestation of Wilson’s Disease

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