Glomerulocystic kidney disease presenting as acute renal failure in an adult patient
University of São Paulo, San Paulo, São Paulo, BrazilNephrology Dialysis Transplantation (Impact Factor: 3.58). 11/2005; 20(10):2293. DOI: 10.1093/ndt/gfh995
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Advance Access publication 19 July 2005
Glomerulocystic kidney disease presenting as acute
renal failure in an adult patient
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
and platelets ¼ 120 000/mm
. 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
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 . 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 . 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 . 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 . 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
vio Teles de Farias Filho
Nephrology Division Ame
rico Cuvello Neto
University of Sa
o Paulo Regina C. R. M. Abdulkader
o Paulo, SP
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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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- [Show abstract] [Hide abstract] ABSTRACT: The patient was a 28-year-old man with chronic kidney disease in stage 5 and in the course of chronic membranoroliferative glomerulonephritis. The patient was treated for a period of 2 months using peritoneal dialysis. In September 2014, he had a kidney transplant from a deceased donor. Four months after transplantation the patient was admitted to the hospital for a protocol biopsy. His creatinine was 1.5 mg/dL and urea was 59 mg/dL, urinalysis was normal in blood count with a normocytic anemia-hemoglobin level of 7.8 mmol/L. We obtained a histopathological evaluation of the cortex and medulla of the kidney. Glomeruli dilatation of Bowman space with reduced glomerular capillary tufts was found in the section. Histopathological evaluation indicated gromerulocystic kidney disease in a transplanted kidney.