Clinical Manifestations of Primary Hyperoxaluria

Hall, Edward G.; Scowen, E. F.; Watts, R. W. E.

doi:10.1136/adc.35.179.108

Cited by 24 publications

(7 citation statements)

References 12 publications

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“…Hockaday et al (1965) using an isotope dilution technique have shown that normal values in children are similar to adults when corrected for body surface area. The level of oxalate excretion in our patients does not appear to be related to the clinical course of the disease, a finding previously noted by Hall et al (1960). Moreover it has fluctuated widely during follow-up, and twofold or even threefold changes have been found in consecutive specimens.…”

Section: Discussionsupporting

confidence: 60%

“…Thus of 40 typical cases presenting before the age of 5, 20 (50%) had died after an average follow-up of 8 years (ranging from early death to living after 26 years). Of the 8 patients described by Hall, Scowen, and Watts (1960), 3 had died within 5 years of the onset of their first symptoms, and 2 were stone free and symptom free after 1 and 4 years. By contrast all patients in the present series are alive and clinically well after an average period of 9 years since first presentation.…”

Section: Discussionmentioning

confidence: 99%

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Treatment of Primary Hyperoxaluria

Dent¹,

Stamp²

1970

Archives of Disease in Childhood

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735. Treatment of primary hyperoxaluria. Nine patients with primary hyperoxaluria have been followed regularly for 1 to 11 years, and their treatment and progress are discussed in relation to the known natural history of the disease. 6 of them probably have the usual form of primary hyperoxaluria associated with increased glycollic acid excretion, while 3 who are sibs have the recently described variant associated with L-glyceric aciduria and normal glycollic acid excretion.All 9 patients have been on regimens designed to increase the urinary solubility of calcium oxalate, with or without the simultaneous lowering of urinary calcium and raising of urinary phosphate excretions. 8 patients have been treated for 1 -7j years (average duration 4 years) with oral magnesium hydroxide, and 2 patients have been treated with sodium phosphate. One of the latter was changed after 31 years to magnesium hydroxide and the other has been on sodium phosphate combined with a low calcium diet and cellulose phosphate continuously for 5j years. 2, not at first diagnosed as hyperoxalurics, were first given sodium bicarbonate for their presumably secondary renal tubular acidosis.The over-all progress of the whole group is felt to have been better than could be expected from the known natural history of primary hyperoxaluria.

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Section: Discussionsupporting

confidence: 60%

Section: Discussionmentioning

confidence: 99%

Treatment of Primary Hyperoxaluria

Dent¹,

Stamp²

1970

Archives of Disease in Childhood

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“…He was an asymptomatic 12-year-old boy, height 133 cm., weight 30 7 kg., with a right renal calculus, blood urea 40 mg./100 ml., and grossly raised urinary oxalate excretion. (He was reported as 'Case 4' in 'Family 4' of Hall et al (1960).) His diet was unrestricted except that the high oxalate foods and citrus fruits were excluded.…”

Section: Methodsmentioning

confidence: 99%

“…The increased urinary oxalate excretion which is characteristic of primary hyperoxaluria causes recurrent urinary calculi and nephrocalcinosis, with death at an early age (Hall, Scowen, and Watts, 1960;Hockaday, Clayton, Frederick, and Smith, 1964), and no effective treatment for the disease has been reported. Glyoxylate is the main immediate metabolic precursor of oxalate, and if the enzyme or enzymes which catalyse the oxidation CHO COOH COOH COOH could be inhibited in vivo the urinary oxalate excretion should be reduced and renal damage averted.…”

mentioning

confidence: 99%

Biochemical studies on the treatment of primary hyperoxaluria.

Gibbs¹,

Watts²

1967

Archives of Disease in Childhood

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“…Dr. E. G. Hall has kindly given permission for reference to a further member, born in 1962, of family No. 4 of Hall, Scowen, and Watts (1960). This child was in renal failure in his first month and died in his first year, yet never had renal calculi despite renal and bony oxalate deposits.…”

mentioning

confidence: 96%