Copyright ©The Author(s) 2015.
World J Nephrol. May 6, 2015; 4(2): 235-244
Published online May 6, 2015. doi: 10.5527/wjn.v4.i2.235
Table 1 Comparison between primary and secondary hyperoxaluria
Clinical featurePrimary hyperoxaluriaSecondary hyperoxaluria
EtiologyInborn error of metabolism with specific enzymatic defectsIncreased dietary intake of oxalate or precursors
PH 1: Alanine glyoxalate aminotransferaseIncreased intestinal absorption
PH 2: Glyoxalate/hydroxypyruvate reductaseAltered intestinal microflora
PH 3: 4-hydroxy 2-oxoglutarate aldolase
Clinical presentationPH 1: Recurrent stones, nephrocalcinosis, ESRD commonRecurrent renal stones, nephrocalcinosis, CKD and ESRD
Clinical heterogeneity in presentation, varies from an infantile to an adult onset form
PH 2: Recurrent stones, nephrocalcinosis less common, ESRD has been reported (approximately 20% cases)
PH 3: Hypercalciuria with hyperoxaluria is reported, no reports to date of ESRD
Systemic oxalosisFrequent part of the presentationLess common but may occur in severe cases of inflammatory bowel disease or short bowel syndrome
Diagnosis: HistoryFamily history is often suggestive with other affected relativesDietary history may be an important pointer towards the diagnosis
Urinary excretion> 1.0 mmol/1.73 m2 BSAUsually < 1.0 mmol/1.73 m2 BSA but in some cases of enteric hyperoxaluria may extend into the primary range
Composition of renal stones95% calcium oxalate monohydrate (whewellite)Mixed stones (whewellite and weddellite)
Other diagnostic pointsPlasma oxalate levels in ESRD are > 60-80 mmol/L as compared from non-PH causes of ESRD14C test can be used to assess for increased intestinal absorption
General measures:Daily fluid intake > 3.0 L/dHydration and urinary alkalinization
Pyridoxine in PH1Renal replacement therapy when ESRD occurs
Urinary alkalinization
Thiazides for PH3
Renal replacement therapy when ESRD occurs
Specific measures:No role as dietary absorption is < 5%Important role as dietary absorption is > 40%
Dietary management
O. formigenesNo role in managementNo role demonstrated in human studies
TransplantationPH1: Liver kidney transplant (combined or sequential)Limited data available regarding transplants for treatment of SH
Isolated kidney transplant in pyridoxine sensitive adult patients
PH2: Isolated kidney transplant
PH3: No role of kidney transplant