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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]
Aging [21, 22] 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
Influencing factors for renal cystogenesis [43, 45]: Hypertension [43, 45, 46, 81]:
-hypertension (≤35 years) - renal infection -Routinely standardized blood pressure measurement
-proteinuria - total kidney volume
-hematuria (<30 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]
Aging [21, 22] 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 <50 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 Screening is indicated [13, 45]: Echocardiography [13, 45]
-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
  -Male >60 years and a family history of AAA  
  1. This scheme indicates distinct factors that are related to physical health and disease progression in PCLD and ADPKD patients. These clinical elements listed per organ may be valuable for consideration in management. Patients’ mental condition and health-related quality of life should be assessed by standardized evaluation of symptoms in all PLD patients [82].