Learn about Autosomal Dominant Polycystic Kidney Disease (ADPKD), also known as Polycystic Kidney Disease Type 1 and Type 2. Find information on diagnosis, clinical documentation, and medical coding for ADPKD, including ICD-10 codes and SNOMED CT terms relevant for healthcare professionals. Explore resources for managing and treating ADPKD and understand the importance of accurate medical coding for PKD.
Also known as
Autosomal dominant polycystic kidney
Inherited disorder with multiple kidney cysts.
Autosomal recessive polycystic kidney
Inherited disorder with cysts in kidneys and liver.
Polycystic kidney, unspecified
Kidney cysts without specified genetic cause.
Other specified disorders of kidney and ureter
Includes other specified kidney conditions like acquired cysts.
Follow this step-by-step guide to choose the correct ICD-10 code.
Is the polycystic kidney disease confirmed as Autosomal Dominant?
Yes
Is it specified as Type 1?
No
Do NOT code as ADPKD. Evaluate for other forms of PKD (e.g., ARPKD)
When to use each related code
| Description |
|---|
| Inherited kidney disease forming cysts |
| Acquired kidney cysts, not inherited |
| Genetic disorder with cysts in kidneys and other organs |
Incorrect coding for laterality (unilateral/bilateral) can impact reimbursement and data accuracy for ADPKD.
Miscoding PKD type (Type 1 vs. Type 2) affects clinical documentation integrity and research data validity.
Failure to code associated manifestations like hypertension or liver cysts can underrepresent ADPKD complexity.
Q: How can I differentiate between Autosomal Dominant Polycystic Kidney Disease (ADPKD) Type 1 and Type 2 in my clinical practice?
A: Differentiating between ADPKD Type 1 and Type 2 can be challenging due to overlapping symptoms. While both are inherited forms of polycystic kidney disease characterized by cyst development, they differ in genetic basis, disease progression, and extrarenal manifestations. PKD1 gene mutations cause Type 1, typically leading to earlier onset and more aggressive disease progression with larger cysts predominantly in the kidneys. PKD2 mutations cause Type 2, generally resulting in later onset, slower progression, and smaller, more evenly distributed cysts. Liver cysts are more common in Type 1. Accurate diagnosis relies on genetic testing alongside clinical evaluation, including family history, imaging studies (ultrasonography, CT, MRI), and assessment of renal function. Consider implementing genetic counseling for patients and their families to understand inheritance patterns and risks. Explore how advances in genetic testing can enhance diagnostic accuracy and inform personalized management strategies for ADPKD.
Q: What are the best evidence-based management strategies for patients with rapidly progressing ADPKD and chronic kidney disease?
A: Managing rapidly progressing ADPKD with chronic kidney disease requires a multifaceted approach. Firstly, rigorously control blood pressure, ideally with an angiotensin-converting enzyme inhibitor (ACEi) or angiotensin receptor blocker (ARB). Tolvaptan, a vasopressin V2 receptor antagonist, is approved for slowing cyst growth and decline in kidney function in adults at risk of rapidly progressing ADPKD. Closely monitor renal function and electrolyte levels. Address pain management with appropriate analgesics, avoiding nephrotoxic medications. Nutritional counseling plays a vital role in managing dietary restrictions and fluid intake. Consider implementing a multidisciplinary approach involving nephrologists, geneticists, and dietitians to optimize patient outcomes. Explore how clinical trials are investigating novel therapeutic targets for ADPKD to stay abreast of emerging treatment options.
Patient presents with concerns regarding Autosomal Dominant Polycystic Kidney Disease (ADPKD), also known as Polycystic Kidney Disease Type 1 or PKD1. Family history is positive for ADPKD, confirming the genetic predisposition. The patient reports experiencing symptoms consistent with ADPKD, including flank pain, hypertension, and palpable abdominal masses. Renal ultrasound reveals multiple bilateral renal cysts, meeting the diagnostic criteria for ADPKD. Laboratory findings indicate a decline in glomerular filtration rate (GFR) and elevated creatinine levels, suggesting chronic kidney disease (CKD) progression. Assessment includes evaluation for intracranial aneurysms, hepatic cysts, and cardiac valvular abnormalities, common extrarenal manifestations of ADPKD. The current treatment plan focuses on blood pressure management with ACE inhibitors or angiotensin receptor blockers (ARBs), pain control, and management of CKD complications. Patient education emphasizes the importance of regular monitoring of renal function, blood pressure control, and genetic counseling for family members. Follow-up appointments are scheduled to track disease progression and adjust treatment as needed. ICD-10 code Q61.2 is used for documentation and billing purposes. The patient demonstrates understanding of the chronic nature of ADPKD and the importance of adherence to the prescribed treatment plan.