[Two patients with renal medullary hyperechogenicity]. / Zwei Patientinnen mit echoreichen Markpyramiden.
Dtsch Med Wochenschr
; 137(30): 1510, 2012 Jul.
Article
em De
| MEDLINE
| ID: mdl-22869509
ABSTRACT
HISTORY AND ADMISSION FINDINGS:
We report two patients with medullary nephrocalcinosis and nephrolithiasis. The first patients had unspecific symptoms of a systemic inflammatory disease, the second patient was asymptomatic. INVESTIGATIONS Both patients had normocalcemia and normal parathyroid hormone levels, very high 1,25(OH)(2)-vitamin D and normal to low 25-OH-vitamin D levels. On renal ultrasound, both patients displayed nephrocalcinosis and nephrolithiasis. DIAGNOSIS Both patients showed dysregulation of the 1α-hydroxylase activity. The first patient suffered from a systemic inflammatory disease with consecutive activation of macrophages with extrarenal α-hydroxylase activity. The second patient had a "loss of function" mutation of the calcium sensing receptor with - for the situation - inadequatly high parathyroid hormone levels and consequently a renal dysregulation of the 1α-hydroxylase. TREATMENT AND COURSE After immunosuppressive treatment with prednisolone and hydroxychloroquin there was complete remission of the systemic inflammatory disease in the first patient. In consequence the 1,25-(OH)(2)-vitamin D levels regressed and renal function stabilized. The second patient was completely asymptomatic with normal renal function, so far we did not initiate any treatment.CONCLUSIONS:
Nephrocalcinosis and nephrolithiasis can result from hypercalciuria due to dysregulated hydroxylation of vitamin D. For both renal and extrarenal sources causal treatment is available.
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Nefrolitíase
/
Medula Renal
/
Nefrocalcinose
Idioma:
De
Ano de publicação:
2012
Tipo de documento:
Article