Table 2 Determinants and recommendations in severe PCLD and ADPKD
From: Polycystic liver disease: an overview of pathogenesis, clinical manifestations and management
Polycystic liver disease (PCLD) | ||
|---|---|---|
Organ | Determinant | Recommendations |
Liver | Female sex | Stop exogenous estrogen use in female patients [78] |
Advise alternative contraceptive strategies | ||
Environmental factors associated with PLD disease course [21, 22]: | ||
-Prolonged oral/exogenous female steroid use: estrogens, contraceptive pill or (post-menopausal) hormonal replacement therapy | ||
-Multiple pregnancies | ||
Brain | Similar recommendations seem appropriate for patients with isolated ADPLD, but more studies are required [19, 79] | Indiscriminate screening is not recommended at present [44] |
Heart | Similar as in the general population [47] | No recommendations |
Autosomal dominant polycystic kidney disease (ADPKD) | ||
Organ | Determinant | Recommendations |
Kidney | Environmental factors associated with renal cyst growth [83]: | Avoid (excessive) caffeine administration and nephrotoxic agents |
-caffeine | Smoking cessation | |
-smoking | ||
-hypertension (≤35 years) - renal infection | -Routinely standardized blood pressure measurement | |
-proteinuria - total kidney volume | ||
-hematuria (<30Â years) - male sex | -Elektrocardiogram in hypertensive patients for LVH assessment | |
-urinary tract infection - low birth weight | ||
-kidney stones - aging | -Plasma LDL cholesterol control; urinary albumin excretion; left ventricular mass index calculation | |
-Angiotensin converting enzyme inhibitors and/or angiotensin receptor blockers | ||
Dietary protein and salt restriction | ||
Sufficient daily fluid intake | ||
PKD1 gene mutation have a more severe disease course and earlier onset of end-stage renal disease compared to PKD2 carriers [41] | Molecular diagnostics [24] | |
Liver | Female sex | Stop exogenous estrogen use in female patients [78] |
Advise alternative contraceptive strategies | ||
Environmental factors associated with PLD disease course [24, 25]: | ||
-prolonged oral/exogenous female steroid use: estrogens, contraceptive pill or (post-menopausal) hormonal replacement therapy | ||
-multiple pregnancies | ||
Renal function/glomular filtration rate [23]; in particular females [43] | ||
Brain | Patients at risk: | Patients with reasonable estimated life expectancy: periodic 3–5 years MR/CT-angiography screening [51] |
-positive family history of (ruptured) ICA or stroke <50Â years old | ||
-previously ruptured ICA | Surveillance/rescreening after negative results in patients with a positive family history: 5–10 years (high-to low-risk) [13, 81] | |
-warning symptoms: unusual headaches | ||
-high-risk occupation (for example: airline pilot) | ||
preparation for major elective surgery (for example: kidney transplantation) [13, 51] | ||
Smoking cessation | ||
Blood pressure control | ||
The position of the mutation in PKD1 is predictive for development of intracranial aneurysms [80] | ||
Hyperlipidemia control [51] | ||
Molecular diagnostics [80] | ||
Heart | ||
-a murmur or systolic clicks are detected on examination | ||
-positive family history of thoracic aorta dissection | ||
Aorta | ADPKD patients receiving hemodialysis [49] | AAA: routine screening of the aortic size, using CT or abdominal ultrasonography [49] |
Similar as in general population for AAA [81]: | 1-time screening with abdominal ultrasonography [81] | |
-Male between the ages of 65–75 and smoked >100 cigarettes in a lifetime | ||
| Â | -Male >60Â years and a family history of AAA | Â |