Editorial Hepatic Cysts in Autosomal Dominant Polycystic Kidney Disease
old (N = 18) and 75% of those older t h a n age 70 years (N = 8) had hepatic cysts.'' Hepatic imag ing studies of patients with A D P K D have con firmed this initial impression. With u s e of radio isotope scintigraphy, Milutinovic and colleagues^ In this i s s u e of t h e Proceedings (pages 9 3 3 to found that 10% of patients 2 0 to 29 years of age 942), Telenti and associates describe the clinical and 75% of patients older t h a n 60 years h a d manifestations a n d t r e a t m e n t of infected h e hepatic cysts. Griinfeld and co-workers,^ using patic cysts in patients with autosomal dominant the more sensitive technique of ultrasonography polycystic kidney disease (ADPKD). This article (mainly B-mode), found a higher prevalence of e m p h a s i z e s t h e m u l t i s y s t e m nature of A D P K D hepatic cysts with a linear increase in preva and demonstrates that complications arising in lence from the third (25%) to sixth (80%) decades extrarenal sites m a y lead to substantial morbid of life. T h o m s e n and Thaysen* used computed ity and even mortality. The goals of this editorial tomography and obtained age-related prevalence are twofold: to familiarize t h e reader with n e w figures almost identical to those of Griinfeld and information about risk factors a n d function of associates.^ Although all studies demonstrated hepatic cysts a n d to evaluate t h e clinical ap t h a t t h e prevalence of hepatic cystic disease increased with advancing age, none compared proach to hepatic cyst infection. The primary extrarenal manifestations of the age-related prevalence of hepatic and renal A D P K D are hepatic cysts, mitral valve prolapse, cysts, a n d no study w a s designed to determine cerebral a n e u r y s m s , and, possibly, diverticular w h e t h e r a g e w a s a n independent predictor of disease of t h e colon.'"'' Although t w o genetic hepatic cyst formation. My colleagues a n d I recently conducted a variants of A D P K D exist,^-'"' t h e frequency of each extrarenal manifestation, except perhaps prospective study of risk factors for hepatic cysts'* intracranial aneurysm, is similar within all af in a large number of subjects enrolled in an fected kindreds. Hepatic cysts, t h e most com investigation of the natural history of ADPKD.' mon extrarenal manifestation of A D P K D , are Overall, 2 3 9 patients with A D P K D a n d 189 similar to renal cysts in that t h e y are probably unaffected family members underwent a formal derived from epithelia engaged in transport of history and physical examination, routine blood solutes. For t h e s e reasons, t h e study of hepatic chemistry testing, a n d real-time ultrasonogra cysts in A D P K D m a y yield insight about t h e phy of the liver a n d kidneys. The age-related pathogenesis of not only hepatic cysts b u t also prevalence of hepatic a n d renal cysts differed A D P K D in general. considerably (Fig. 1). Forty-six percent of pa Risk Factors for Hepatic Cystic Disease. tients younger t h a n a g e 3 0 years who were at A g e . — T h e prevalence of hepatic cysts in pa risk for A D P K D had renal cysts, but only 18% of tients with A D P K D increases with advancing the s a m e patients had hepatic cysts. Renal cysts age. Unlike renal cysts, hepatic cysts are not were commonly detected in childhood, b u t no detected in utero and rarely are detected before patient younger t h a n age 16 years h a d detect puberty. In the most extensive autopsy study of able hepatic cysts. B y logistic regression analy A D P K D to date, 17% of patients 2 9 to 3 9 years sis, a g e w a s a n independent predictor of both prevalence and number of hepatic cysts. The increase in hepatic cystic disease with advanc Address reprint requests to Dr. G. T. Everson, Gastroen ing age s u g g e s t s that genetic or environmental terology Division, Box B-158, University of Colorado Health factors m a y somehow exert a cumulative effect Sciences Center, 4200 East Ninth Avenue, Denver, CO over time on t h e growth of hepatic cysts. 80262. Mayo Clin Proc 65:1020-1025, 1990
1020
EDITORIAL 1021
Mayo Clin Proc, J u l y 1990, Vol 65
100%
E3 RENAL CYSTS HEPATIC CYSTS 80
δ ζ sg^O
60
20
1 0-10
10-20
20-30
16
28
80
30-40
40-50
50-60
60+
45
42
AGE (years) ( Ν
137
80
)
Fig. 1. Frequency of renal and hepatic cysts stratified by age in a population at risk for autosomal dominant polycystic kidney disease (ADPKD), as detected by real-time ultra sonography. Population at risk included 239 patients with ADPKD and 189 unaiTected family members. Number of subjects in each decade is indicated at bottom. (From Gabow and associates.^ By permission of the American Association for the Study of Liver Diseases.)
Gender, Pregnancy, and Female Steroid H o r m o n e s . — S e v e r a l authors have suggested that m a s s i v e hepatic cysts are more likely to develop in w o m e n t h a n in men. Griinfeld and colleagues^ found that the prevalence of hepatic cysts in w o m e n w a s almost twice t h a t in m e n in relatively young, nondialyzed patients. In con trast, they found no difference in prevalence of hepatic cysts between m e n and w o m e n a m o n g older patients on long-term hemodialysis. A recent study also found t h a t w o m e n were more likely t h a n m e n to have m a s s i v e hepatic cystic d i s e a s e . I n our study of risk factors,^ we found t h a t the overall prevalence of hepatic cysts in nonazotemic patients w a s similar b e t w e e n m e n (46%) and w o m e n (50%). Women, however, were more likely to have more and larger hepatic cysts. Women with hepatic cysts had more pregnancies t h a n did those without hepatic cysts (2.9 ± 0.3 versus 1.6 ± 0.2; P
old (N = 18) and 75% of those older t h a n age 70 years (N = 8) had hepatic cysts.'' Hepatic imag ing studies of patients with A D P K D have con firmed this initial impression. With u s e of radio isotope scintigraphy, Milutinovic and colleagues^ In this i s s u e of t h e Proceedings (pages 9 3 3 to found that 10% of patients 2 0 to 29 years of age 942), Telenti and associates describe the clinical and 75% of patients older t h a n 60 years h a d manifestations a n d t r e a t m e n t of infected h e hepatic cysts. Griinfeld and co-workers,^ using patic cysts in patients with autosomal dominant the more sensitive technique of ultrasonography polycystic kidney disease (ADPKD). This article (mainly B-mode), found a higher prevalence of e m p h a s i z e s t h e m u l t i s y s t e m nature of A D P K D hepatic cysts with a linear increase in preva and demonstrates that complications arising in lence from the third (25%) to sixth (80%) decades extrarenal sites m a y lead to substantial morbid of life. T h o m s e n and Thaysen* used computed ity and even mortality. The goals of this editorial tomography and obtained age-related prevalence are twofold: to familiarize t h e reader with n e w figures almost identical to those of Griinfeld and information about risk factors a n d function of associates.^ Although all studies demonstrated hepatic cysts a n d to evaluate t h e clinical ap t h a t t h e prevalence of hepatic cystic disease increased with advancing age, none compared proach to hepatic cyst infection. The primary extrarenal manifestations of the age-related prevalence of hepatic and renal A D P K D are hepatic cysts, mitral valve prolapse, cysts, a n d no study w a s designed to determine cerebral a n e u r y s m s , and, possibly, diverticular w h e t h e r a g e w a s a n independent predictor of disease of t h e colon.'"'' Although t w o genetic hepatic cyst formation. My colleagues a n d I recently conducted a variants of A D P K D exist,^-'"' t h e frequency of each extrarenal manifestation, except perhaps prospective study of risk factors for hepatic cysts'* intracranial aneurysm, is similar within all af in a large number of subjects enrolled in an fected kindreds. Hepatic cysts, t h e most com investigation of the natural history of ADPKD.' mon extrarenal manifestation of A D P K D , are Overall, 2 3 9 patients with A D P K D a n d 189 similar to renal cysts in that t h e y are probably unaffected family members underwent a formal derived from epithelia engaged in transport of history and physical examination, routine blood solutes. For t h e s e reasons, t h e study of hepatic chemistry testing, a n d real-time ultrasonogra cysts in A D P K D m a y yield insight about t h e phy of the liver a n d kidneys. The age-related pathogenesis of not only hepatic cysts b u t also prevalence of hepatic a n d renal cysts differed A D P K D in general. considerably (Fig. 1). Forty-six percent of pa Risk Factors for Hepatic Cystic Disease. tients younger t h a n a g e 3 0 years who were at A g e . — T h e prevalence of hepatic cysts in pa risk for A D P K D had renal cysts, but only 18% of tients with A D P K D increases with advancing the s a m e patients had hepatic cysts. Renal cysts age. Unlike renal cysts, hepatic cysts are not were commonly detected in childhood, b u t no detected in utero and rarely are detected before patient younger t h a n age 16 years h a d detect puberty. In the most extensive autopsy study of able hepatic cysts. B y logistic regression analy A D P K D to date, 17% of patients 2 9 to 3 9 years sis, a g e w a s a n independent predictor of both prevalence and number of hepatic cysts. The increase in hepatic cystic disease with advanc Address reprint requests to Dr. G. T. Everson, Gastroen ing age s u g g e s t s that genetic or environmental terology Division, Box B-158, University of Colorado Health factors m a y somehow exert a cumulative effect Sciences Center, 4200 East Ninth Avenue, Denver, CO over time on t h e growth of hepatic cysts. 80262. Mayo Clin Proc 65:1020-1025, 1990
1020
EDITORIAL 1021
Mayo Clin Proc, J u l y 1990, Vol 65
100%
E3 RENAL CYSTS HEPATIC CYSTS 80
δ ζ sg^O
60
20
1 0-10
10-20
20-30
16
28
80
30-40
40-50
50-60
60+
45
42
AGE (years) ( Ν
137
80
)
Fig. 1. Frequency of renal and hepatic cysts stratified by age in a population at risk for autosomal dominant polycystic kidney disease (ADPKD), as detected by real-time ultra sonography. Population at risk included 239 patients with ADPKD and 189 unaiTected family members. Number of subjects in each decade is indicated at bottom. (From Gabow and associates.^ By permission of the American Association for the Study of Liver Diseases.)
Gender, Pregnancy, and Female Steroid H o r m o n e s . — S e v e r a l authors have suggested that m a s s i v e hepatic cysts are more likely to develop in w o m e n t h a n in men. Griinfeld and colleagues^ found that the prevalence of hepatic cysts in w o m e n w a s almost twice t h a t in m e n in relatively young, nondialyzed patients. In con trast, they found no difference in prevalence of hepatic cysts between m e n and w o m e n a m o n g older patients on long-term hemodialysis. A recent study also found t h a t w o m e n were more likely t h a n m e n to have m a s s i v e hepatic cystic d i s e a s e . I n our study of risk factors,^ we found t h a t the overall prevalence of hepatic cysts in nonazotemic patients w a s similar b e t w e e n m e n (46%) and w o m e n (50%). Women, however, were more likely to have more and larger hepatic cysts. Women with hepatic cysts had more pregnancies t h a n did those without hepatic cysts (2.9 ± 0.3 versus 1.6 ± 0.2; P
