Nephrocalcinosis in a patient with primary hyperoxaluria type 2.
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Nephrocalcinosis in a patient with primary hyperoxaluria type 2. / Kemper, Markus J.; Müller-Wiefel, D E.
in: PEDIATR NEPHROL, Jahrgang 10, Nr. 4, 4, 1996, S. 442-444.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Nephrocalcinosis in a patient with primary hyperoxaluria type 2.
AU - Kemper, Markus J.
AU - Müller-Wiefel, D E
PY - 1996
Y1 - 1996
N2 - Although nephrocalcinosis is a classical finding in primary hyperoxaluria type 1 (PH 1) associated with a poor renal survival it is exceptional in patients with PH type 2 (PH 2), characterized by a more favorable outcome. We describe an 8-month-old girl who suffered from recurrent urinary tract infections. Imaging studies revealed a profound corticomedullary nephrocalcinosis with no evidence of calculi. Urinary oxalate and D-glycerate excretion were massively elevated, while urinary glycolate or glyoxylate could not be detected, confirming the diagnosis of PH 2. Although the nephrocalcinosis progressed radiologically, renal function remained stable for over 2 years. Only further follow-up will show whether the associated nephrocalcinosis worsens the prognosis of our patient and of PH 2 in general.
AB - Although nephrocalcinosis is a classical finding in primary hyperoxaluria type 1 (PH 1) associated with a poor renal survival it is exceptional in patients with PH type 2 (PH 2), characterized by a more favorable outcome. We describe an 8-month-old girl who suffered from recurrent urinary tract infections. Imaging studies revealed a profound corticomedullary nephrocalcinosis with no evidence of calculi. Urinary oxalate and D-glycerate excretion were massively elevated, while urinary glycolate or glyoxylate could not be detected, confirming the diagnosis of PH 2. Although the nephrocalcinosis progressed radiologically, renal function remained stable for over 2 years. Only further follow-up will show whether the associated nephrocalcinosis worsens the prognosis of our patient and of PH 2 in general.
KW - Humans
KW - Female
KW - Child
KW - Hydrogen-Ion Concentration
KW - Glyceric Acids/urine
KW - Glycolates/urine
KW - Glyoxylates/urine
KW - Hyperoxaluria, Primary/complications/radiography/ultrasonography
KW - Kidney/radiography/ultrasonography
KW - Nephrocalcinosis/radiography/ultrasonography/urine
KW - Oxalates/urine
KW - Urinary Calculi/ultrasonography/urine
KW - Humans
KW - Female
KW - Child
KW - Hydrogen-Ion Concentration
KW - Glyceric Acids/urine
KW - Glycolates/urine
KW - Glyoxylates/urine
KW - Hyperoxaluria, Primary/complications/radiography/ultrasonography
KW - Kidney/radiography/ultrasonography
KW - Nephrocalcinosis/radiography/ultrasonography/urine
KW - Oxalates/urine
KW - Urinary Calculi/ultrasonography/urine
M3 - SCORING: Journal article
VL - 10
SP - 442
EP - 444
JO - PEDIATR NEPHROL
JF - PEDIATR NEPHROL
SN - 0931-041X
IS - 4
M1 - 4
ER -
