Ultrasonic diagnosis of primary hyperoxaluria in infancy.

Brennan, James N.; Diwan, R V; Makker, Sudesh P; Cromer, Barbara A.; Bellon, E M

doi:10.1148/radiology.145.1.7122872

Cited by 21 publications

(5 citation statements)

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“…This precipitation most probably leads to the characteristic lowering of the serum calcium levels in these patients ). The similar patterns demonstrated by renal ultrasonography in primary oxalosis and ethylene glycol intoxication also points to a similar aetiology of the renal damage in these conditions (Brennan et al 1982;Walker et al 1983). Calcium oxalate precipitation may be an important pathogenetic factor in the renal toxicity of ethylene glycol poisoning, as it is in the development of a similar renal failure in oxalate poisoning (Jacobsen et al 1981;James 1972) and in primary oxalosis (Boquist et al 1973;Enger et al 1965).…”

Section: Role Of Oxalate In Ethylene Glycol Toxicitysupporting

confidence: 60%

Methanol and Ethylene Glycol Poisonings

Jacobsen¹,

McMartin²

1986

Medical Toxicology

419

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Methanol and ethylene glycol poisonings share many characteristics both clinically and biochemically. Both alcohols are metabolised via alcohol dehydrogenase to their toxic metabolites. Methanol is slowly metabolised to formaldehyde which is rapidly metabolised to formate, the metabolite mainly responsible for methanol toxicity. Formate metabolism depends upon the folate pool which is small in primates compared with other animals. Therefore, formate accumulates in primates during methanol intoxication and is mainly responsible for the metabolic acidosis in the early stage of intoxication. In late stages lactate may also accumulate, mainly due to formate inhibition of the respiratory chain. This tissue hypoxia caused by formate may explain the ocular as well as the general toxicity. Ethylene glycol is metabolised more rapidly than methanol, via alcohol dehydrogenase to glycolaldehyde which is rapidly metabolised to glycolate, the metabolite mainly responsible for the metabolic acidosis in ethylene glycol poisoning. Glycolate is metabolised by various pathways, including one to oxalate which rapidly precipitates with calcium in various tissues and in the urine. Ethylene glycol toxicity is complex and not fully understood, but is mainly due to the severe metabolic acidosis caused by glycolate and to the calcium oxalate precipitation. The clinical course in both poisonings is initially characterised by the development of metabolic acidosis following a latent period, which is more pronounced in methanol poisoning and is the time taken for both alcohols to be metabolised to their toxic metabolites. In methanol poisoning there are usually visual symptoms progressing to visual impairment, whereas ethylene glycol victims develop renal and cardiopulmonary failure. Prognosis is excellent in both poisonings provided that there is early treatment with alkali to combat acidosis, ethanol as an antimetabolite, and haemodialysis to remove the alcohols and their toxic metabolites. Ethanol is also metabolised by alcohol dehydrogenase, but has a much higher affinity for this enzyme than methanol and ethylene glycol. Presence of ethanol will therefore inhibit formation of toxic metabolites from methanol and ethylene glycol. Due to competition for the enzyme, the therapeutic ethanol concentration depends on the concentration of the other two alcohols, but a therapeutic ethanol concentration around 22 mmol/L (100 mg/dl) is generally recommended. Most patients are, however, admitted at a late stage to hospitals not capable of performing analyses of these alcohols or their specific metabolites on a 24-hour basis.(ABSTRACT TRUNCATED AT 400 WORDS)

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Section: Role Of Oxalate In Ethylene Glycol Toxicitysupporting

confidence: 60%

Methanol and Ethylene Glycol Poisonings

Jacobsen¹,

McMartin²

1986

Medical Toxicology

419

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“…Although increased echogenicity has been associated with acute tubular necrosis . renal vein thrombosis, and primary hyperoxaluria of infancy, 6 as we have demonstrated. bilateral increased echogenicity is not, b~· itself, diagnostic of renal disease in the premature infimt.…”

Section: Discussionsupporting

confidence: 69%

A sonographic assessment of neonatal renal parameters.

Erwin¹,

Carroll²,

Müller³

1985

Journal of Ultrasound in Medicine

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“…In infants, failure to thrive is a prominent feature of the disease and ultrasound to detect the nephrocalcinosis may be of special value [10,64]. The classical way to establish the diagnosis of primary hyperoxaluria is the measurement of the excretion of oxalate and glycollate in the urine by various methods [29].…”

Section: Diagnosismentioning

confidence: 99%

Primary hyperoxaluria type I

1990

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Primary hyperoxaluria type I is a metabolic disorder caused by the deficiency of the peroxisomal alanine:glyoxylate aminotransferase. The disease is inherited as an autosomal recessive trait. The clinical course is outlined based on data from 330 published cases. Diagnostic cornerstones are clinical parameters, urinary excretion of oxalate and glycolate, and the determination of enzyme activity in liver tissue. Principles of conservative treatment, e.g. volume load and pyridoxine substitution, are described as well as experience with different modes of dialysis and transplantation. Kidney transplantation is associated with a high rate of recurrence of the original disease despite excellent management resulting in many instances in early graft loss. Liver transplantation offers the possibility to correct the metabolic defect and to prevent the progression of crystal deposition in the body.

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Ultrasonic diagnosis of primary hyperoxaluria in infancy.

Cited by 21 publications

References 0 publications

Methanol and Ethylene Glycol Poisonings

Methanol and Ethylene Glycol Poisonings

A sonographic assessment of neonatal renal parameters.

Primary hyperoxaluria type I

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