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Multiple kidney cysts in thin basement membrane disease with proteinuria and kidney function impairment.

Sevillano AM, Gutierrez E, Morales E, Hernandez E, Molina M, Gonzalez E, Praga M - Clin Kidney J (2014)

Bottom Line: Little is known about factors associated with this unfavourable outcome.Multiple bilateral kidney cysts were found in a majority (56%) of these patients.Further studies are needed to investigate the pathogenesis and the influence on long-term outcome of this TBMD-associated multiple kidney cysts.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology , 12 de Octubre University Hospital , Madrid , Spain.

ABSTRACT

Background: Some patients with thin basement membrane disease (TBMD) develop proteinuria, hypertension and different degrees of CKD, besides the persistent microhaematuria characteristic of the disease. Little is known about factors associated with this unfavourable outcome.

Methods: We reviewed clinical, pathological and radiological features of 32 patients with biopsy-proven TBMD. Patients were divided in two groups: those with persistent normal kidney function and negative or minimal proteinuria (n = 16) and those with persistent proteinuria >0.5 g/day (n = 16).

Results: Patients with proteinuria had a worse kidney function at baseline than those with negative proteinuria. Global or segmental glomerulosclerosis, together with interstitial fibrosis, was found in 37% of patients with proteinuria. All proteinuric patients were treated with renin-angiotensin system blockers. At the end of follow-up (198 months in proteinuric patients and 210 months in patients with negative proteinuria) the prevalence of hypertension was 68% in proteinuric patients (12% at baseline), compared with 12 and 6%, respectively, in non-proteinuric patients. A slow decline of renal function was observed in proteinuric patients, although no patient developed end-stage kidney disease. Ultrasound studies showed bilateral kidney cysts in nine patients (56%) with proteinuria. Cysts were bilateral and countless in six patients, and bilateral but with a limited number of cysts in the three remaining patients. No cysts were found in patients with negative proteinuria.

Conclusions: Some patients with TBMD develop hypertension, proteinuria and CKD. Multiple bilateral kidney cysts were found in a majority (56%) of these patients. Further studies are needed to investigate the pathogenesis and the influence on long-term outcome of this TBMD-associated multiple kidney cysts.

No MeSH data available.


Related in: MedlinePlus

Kidney ultrasound in a patient with TBMD, proteinuria and CKD, showing bilateral kidney cysts.
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SFU033F1: Kidney ultrasound in a patient with TBMD, proteinuria and CKD, showing bilateral kidney cysts.

Mentions: A majority of patients with proteinuria showed multiple kidney cysts on ultrasound abdominal examination (Figure 1). Bilateral kidney cysts were detected in nine patients (56%) with proteinuria, whereas no patient with negative proteinuria showed kidney cysts or other radiological abnormalities (P = 0.002). Individualized main radiological findings in patients with TBMD and proteinuria are shown in Table 4. Multiple and bilateral renal cysts were found in six patients, and the other three patients showed a limited number of cysts. Normal size kidneys were observed, with the exception of Patient 1 (Table 4), who showed multiple and bilateral kidney cysts with a kidney length pole to pole of 15 cm in both kidneys. Kidney size was slightly reduced in those patients with a more advanced CKD. When comparing proteinuric TBMD patients with or without kidney cysts, we found that patients with cysts were significantly older than those without cysts (Table 5). Kidney function tended to be worse in patients with cysts, both at baseline and at the end of follow-up, when compared with patients without cysts, but these differences were not statistically significant.Table 4.


Multiple kidney cysts in thin basement membrane disease with proteinuria and kidney function impairment.

Sevillano AM, Gutierrez E, Morales E, Hernandez E, Molina M, Gonzalez E, Praga M - Clin Kidney J (2014)

Kidney ultrasound in a patient with TBMD, proteinuria and CKD, showing bilateral kidney cysts.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4377753&req=5

SFU033F1: Kidney ultrasound in a patient with TBMD, proteinuria and CKD, showing bilateral kidney cysts.
Mentions: A majority of patients with proteinuria showed multiple kidney cysts on ultrasound abdominal examination (Figure 1). Bilateral kidney cysts were detected in nine patients (56%) with proteinuria, whereas no patient with negative proteinuria showed kidney cysts or other radiological abnormalities (P = 0.002). Individualized main radiological findings in patients with TBMD and proteinuria are shown in Table 4. Multiple and bilateral renal cysts were found in six patients, and the other three patients showed a limited number of cysts. Normal size kidneys were observed, with the exception of Patient 1 (Table 4), who showed multiple and bilateral kidney cysts with a kidney length pole to pole of 15 cm in both kidneys. Kidney size was slightly reduced in those patients with a more advanced CKD. When comparing proteinuric TBMD patients with or without kidney cysts, we found that patients with cysts were significantly older than those without cysts (Table 5). Kidney function tended to be worse in patients with cysts, both at baseline and at the end of follow-up, when compared with patients without cysts, but these differences were not statistically significant.Table 4.

Bottom Line: Little is known about factors associated with this unfavourable outcome.Multiple bilateral kidney cysts were found in a majority (56%) of these patients.Further studies are needed to investigate the pathogenesis and the influence on long-term outcome of this TBMD-associated multiple kidney cysts.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology , 12 de Octubre University Hospital , Madrid , Spain.

ABSTRACT

Background: Some patients with thin basement membrane disease (TBMD) develop proteinuria, hypertension and different degrees of CKD, besides the persistent microhaematuria characteristic of the disease. Little is known about factors associated with this unfavourable outcome.

Methods: We reviewed clinical, pathological and radiological features of 32 patients with biopsy-proven TBMD. Patients were divided in two groups: those with persistent normal kidney function and negative or minimal proteinuria (n = 16) and those with persistent proteinuria >0.5 g/day (n = 16).

Results: Patients with proteinuria had a worse kidney function at baseline than those with negative proteinuria. Global or segmental glomerulosclerosis, together with interstitial fibrosis, was found in 37% of patients with proteinuria. All proteinuric patients were treated with renin-angiotensin system blockers. At the end of follow-up (198 months in proteinuric patients and 210 months in patients with negative proteinuria) the prevalence of hypertension was 68% in proteinuric patients (12% at baseline), compared with 12 and 6%, respectively, in non-proteinuric patients. A slow decline of renal function was observed in proteinuric patients, although no patient developed end-stage kidney disease. Ultrasound studies showed bilateral kidney cysts in nine patients (56%) with proteinuria. Cysts were bilateral and countless in six patients, and bilateral but with a limited number of cysts in the three remaining patients. No cysts were found in patients with negative proteinuria.

Conclusions: Some patients with TBMD develop hypertension, proteinuria and CKD. Multiple bilateral kidney cysts were found in a majority (56%) of these patients. Further studies are needed to investigate the pathogenesis and the influence on long-term outcome of this TBMD-associated multiple kidney cysts.

No MeSH data available.


Related in: MedlinePlus