Renal disease autosomal dominant polycystic kidney

This patient, who had autosomal dominant polycystic kidney disease (ADPKD), almost drowned and then developed ALPE. On July 20, 1990, he nearly drowned in the sea at 1500 hours, and was brought to our hospital by ambulance for dyspnea and severe loin pain at 1620 hours. On admission, metabolic acidosis was observed. His CRP, serum creatinine, CPK, amylase, and urinary protein levels were 1+, 1.5mg/dl, 116 U/l, 592IU/1 (derived from the salivary gland), and 2+, respectively. His body temperature was 37.7°C, and his blood pressure was 110/60 mmHg. His pulse and respiratory rate were 120/min and 22/min, respectively. Delayed CT 6h after the administration of contrast medium showed wedge-shaped contrast enhancement in the noncystic renal parenchyma (Fig. 34). On July 24, a bone scan with MDP revealed patchy lesions (Fig. 35). His serum creatinine level was 1.3 mg/dl, which had decreased to 1.0 mg/dl on July 27. The patient was then discharged. 

Fig. 34. Delayed CT 6h after the administration of contrast medium to an autosomal dominant polycystic kidney disease (ADPKD) patient with ALPE. Top. Wedge-shaped contrast enhancement can be seen in the noncystic renal parenchyma region at onset. Bottom. CT under the same conditions in the recovery phase did not show wedge-shaped contrast enhancement...

Cowley et al. (1996) described the Han SRPD rat strain which develops autosomal dominant polycystic kidney disease with chronic renal failure that resembles human autosomal dominant polycystic kidney disease. 

Gabow PA. Autosomal dominant polycystic kidney disease— more than a renal disease. Am J Kidney Dis 1990 16 403-13. 

Gabow PA, Johnson AM, Kaehny WD, et al. Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease. Kidney Int 1992 41 1311-1319. 

Estroff JA, Mandell J, Benacerraf BR (1991) Increased renal parenchymal echogenicity in the fetus Importance and clinical outcome. Radiology 181 135-139 Fanconi G, Hanhart E, Albertini A et al (1951) Die familiare juvenile Nephronophthise. Hel Pediatr Axta 6 1-49 Fetterman GH, Habib R (1969) Congenital bilateral oligo-nephronic renal hypoplasia. AM J Clin Path 52 199-201 Fick GM, Johnson AM, Strain JD et al (1993) Characteristics of very early onset autosomal dominant polycystic kidney disease. J Am Soc Nephrol 3 1863-1870... 

Sigmund G, StOver B, Zimmerhackl LB et al (1991) RARE MR urography in the diagnosis of upper urinary tract abnormalities in children. Pediatr Radiol 21 416-420 Strand WR, Rushton HG, Markle BM et al (1989) Autosomal dominant polycystic kidney disease in infants asymmetric disease mimicking a unilateral renal mass. J Urol 141 1151-1153... 

Adult polycystic kidney disease (APKD) is one of the most common autosomal dominant diseases, affecting about 1/1,000 whites. The key feature of this disease is the progressive accumulation of renal cysts, which ultimately culminate in kidney failure. APKD is responsible for approximately 10% of end-stage renal disease in North America. Patients may also have hypertension, cerebral aneurysms, liver cysts, and cardiac valvular defects. 

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