Clinical Presentation and Complications of Autosomal Dominant Polycystic Kidney Disease: Experience from a Tertiary Care Center

Author(s): Islam SF, Hossain MK, Morshed SM, Mazumder MMA, Jahan F, Faroque MO, Hossain RM, Alam MR, Mondal MC, Karim MM, Salam MA, Rahman AKMS

Background: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most prevalent type of genetically inherited kidney diseases. It is one of the leading causes of end-stage renal disease (ESRD) and is responsible for a considerable portion of patients receiving hemodialysis globally. Treatment focuses on symptom management, blood pressure control and delaying the progression of the disease.

Objective: To determine the pattern of clinical presentation and complications of ADPKD at a Tertiary Care Hospital in Bangladesh.

Methods: A total of 100 patients with ADPKD were evaluated at a Tertiary Care Hospital, Dhaka, Bangladesh. Strict clinical criteria (Ravine’s criteria) were used to identify ADPKD patients. Their demographic profile, initial presenting complaints, co-morbidities, family history of ADPKD, any palpable mass on abdominal examination, cardiac assessment for any abnormal findings and usage of antihypertensive medications were all reviewed and documented. Relevant laboratory tests were done accordingly.

Results: Total 100 patients with ADPKD were enrolled, of them 60 were male and 40 were female, a male to female ratio was 1.5:1; their mean age was 36±12 years. We found that cyst burden was rises with increasing age. Hypertension (40%) and pain (45%) were the most common clinical presentation. Ballotable kidneys were found in 30% of ADPKD patients; while dysuria, asymptomatic microscopic hematuria and gross hematuria were present in 30%, 20% and 10% cases respectively. Regarding complications: acute pyelonephritis (12%), acute kidney injury (11%), cyst infection (10%) and salt loosing nephropathy (10%) were the most common. In this study; majority (55%) of ADPKD patients were in early stages of CKD (stage I and stage II); whereas 45% participants were in advanced stages of CKD (stage III to stage V). Among the study participants; diabetes mellitus was the most common (25%) co-morbidities, followed by hypothyroidism, chronic obstructive pulmonary disease, cholelithiasis, congestive heart failure and chronic liver disease. Acute pyelonephritis, cyst infection, septicemia and pyonephrosis were the most frequent infections observed in study cases. Escherichia coli (E-coli) was the major bacterial pathogen for these infections, other pathogens include Pseudomonas, Klebsiella, Enterobacter, Proteus, Acenetobacter and Candida.

Conclusion: This study highlights the clinical heterogeneity and systemic involvement of ADPKD. Hypertension, CKD progression, and cystrelated complications dominate the clinical spectrum. Blood pressure, renal function and relevant clinical evaluations should be performed regularly in ADPKD patients.