Article

Glomerulocystic kidney disease presenting as acute renal failure in an adult patient

University of São Paulo, San Paulo, São Paulo, Brazil
Nephrology Dialysis Transplantation (Impact Factor: 3.58). 11/2005; 20(10):2293. DOI: 10.1093/ndt/gfh995
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Available from: Regina C R M Abdulkader, Feb 26, 2016
Advance Access publication 19 July 2005
Glomerulocystic kidney disease presenting as acute
renal failure in an adult patient
Sir,
The diagnosis of glomerulocystic kidney disease (GCKD)
in adults is usually an incidental finding during the evalua-
tion of other working diagnoses. Hereby we report the case
of a 55-year-old woman admitted for an elective second
prosthetic mitral valve replacement for rheumatic mitral
valvulopathy who presented acute renal failure (ARF).
Four months before, when a diagnosis of granuloma
annulare was made, her plasma creatinine was 1.8 mg/dl
and urinanalysis showed haematuria without proteinuria.
Her father, aged 60 years, had died of chronic renal failure
of unknown cause. Twenty days before hospital admis-
sion she had pneumonia and was treated with penicillin
and diclofenac for 10 days. She was regularly taking
atenolol and hydroclorthiazide. On admission she was
normotensive. Urinary volume was 1000 ml/day.
Laboratory tests showed plasma creatinine ¼ 6 mg/dl, blood
urea nitrogen ¼ 90 mg/dl, plasma sodium ¼ 140 mEq/l,
plasma potassium ¼ 5.2 mEq/l, ionic calcium ¼ 4.4 mg/dl,
phosphate ¼ 10 mg/dl, haemoglobin ¼ 9.6 g/l, leukocytes ¼
6600/mm
3
and platelets ¼ 120 000/mm
3
. Urinalysis showed
pH ¼ 6, gravity ¼1014, leukocytes ¼144 000/ml, red blood
cells ¼ 145 000/ml. Proteinuria was 1.6 g/24 h. Urine culture
was negative. Ultrasonography showed normal-sized
kidneys with mild hyperechogenicity and a cortical cyst of
2.4 cm in the left kidney. The patient’s renal function
worsened and she required haemodialysis for 2 weeks.
Renal biopsy showed marked dilatation of Bowman’s
space in 40% of glomeruli with the correspondent glomerular
tufts collapsed (see Figure 1). No periglomerular fibrosis
was found. Dilated tubuli with flattened epithelial cells
or with regenerative changes were seen in some areas.
The arteries had no alterations. The final diagnosis was
GCKD plus regenerating acute tubular necrosis. In the
40th hospital day she was discharged, having undergone
uneventful heart surgery on the 20th hospital day, with
plasma creatinine ¼ 2.5 mg/dl. Thirty days after discharge,
she maintained the same creatinine and microscopic
haematuria.
GCDK is more often described in infants and young
children than in adults. Nowadays GCDK is categorized
into three groups according its clinical presentation: (i) spo-
radic or heritable non-syndromal forms found in children or
adults; (ii) heritable syndromal forms; and (iii) glomerular
cysts found in dysplasic kidneys [1]. The sporadic form
of GCKD associated with other renal diseases has been
designated acquired [2,3]. Although hypercalcaemic ARF
had been reported in association with annular granuloma,
our patient did not have hypercalcemia that points to
the presence of annular granuloma be incidental [4]. Her
father having had renal failure suggests a heritable
non-syndromal form of GCKD, where urinanalysis can be
normal or show non-nephrotic proteinuria and haematuria
as in the case of our patient. Ultrasonography can show
characteristic findings, such as a hypoechoic cortical rim
or less characteristic patterns, such as exclusive cortical
cysts, loss of corticomedullary differentiation, hypoplastic
or diffusely echogenic kidneys [5]. The patient’s ultra-
sound showed mild cortical hyperechogenicity and only a
cortical cyst. Although there was no consensus on the
histological criteria to define GCKD, Berstein’s definition
is the most strict: presence of dilatation of Bowman’s space
of two to three times in the plane of section in at least
5% of the glomeruli [1]. Our patient certainly fits this
definition: 40% of her glomeruli showed marked dilatation
of Bowman’s space. The finding of superimposed acute
tubular necrosis can explain the worsening of renal function
and subsequent partial recovery in our patient. ARF was
attributed to the prolonged use of diclofenac. The reported
case points to the importance of performing renal biopsy
in ARF of uncertain cause and, to the best of our
knowledge, this is the first report of GCKD associated with
acute tubular necrosis.
Conflict of interest statement. None declared.
Hospital das Clı
´
nicas Fla
´
vio Teles de Farias Filho
Nephrology Division Ame
´
rico Cuvello Neto
University of Sa
˜
o Paulo Regina C. R. M. Abdulkader
Medical School
Sa
˜
o Paulo, SP
Brazil
Email: kader@usp.br
1. Bernstein J. Glomerulocystic kidney disease nosological
considerations. Pediatr Nephrol 1993; 7: 464–470
2. Crowe AV, Woolfson RG, Griffiths MH, Neild GH.
Glomerulocystic kidney disease associated with Wegener’s
granulomatosis and membranous glomerulonephritis: a case
report. Nephrol Dial Transplant 1995; 10: 888–890
3. Miyazaki K, Miyazaki M, Yoshikuza N et al. Glomerulocystic
kidney disease (GCKD) associated with Henoch-Schoenlein
purpura: a case report and a review of adult cases of GCKD.
Clin Nephrol 2002; 57: 386–391
4. Ehehalt R, Bommer J, Andrassy K, Waldherr R, Ritz E.
Hypercalcemic acute renal failure a complication of
granuloma annulare elastolyticum. Nephrol Dial Transplant
1998; 13: 2125–2127
5. Sharp CK, Bergman SM, Stockwin JM, Robbin ML, Galliani
C, Guay-Woodford LM. Dominantly transmitted glomerulo-
cystic kidney disease: a distinct genetic entity. J Am Soc
Nephrol 1997; 8: 77–84
doi:10.1093/ndt/gfh995
Fig. 1. Renal biopsy showing glomeruli with marked dilation of
Bowman’s space and glomerular tuft collapsed. Dilated tubuli with
flattened epithelium, focal interstitial fibrosis and lympho-histiocytic
infiltrate can also be observed (Masson’s trichromatic stain).
Nephrol Dial Transplant (2005) 20: 2293 2293
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