Smoking accelerates renal cystic disease and worsens cardiac phenotype in Pkd1-deficient mice.

Smoking has been associated with renal disease progression in ADPKD but the underlying deleterious mechanisms and whether it specifically worsens the cardiac phenotype remain unknown. To investigate these matters, Pkd1-deficient cystic mice and noncystic littermates were exposed to smoking from conception to 18 weeks of age and, along with nonexposed controls, were analyzed at 13-18 weeks. Renal cystic index and cyst-lining cell proliferation were higher in cystic mice exposed to smoking than nonexposed cystic animals.

Caffeine Accelerates Cystic Kidney Disease in a Pkd1-Deficient Mouse Model.

Background/aims: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive cyst formation and growth, leading to end-stage renal disease. A higher kidney volume is predictive of a more accelerated decline in renal function. This study aimed to examine the effects of caffeine, a phosphodiesterase inhibitor, on the progression of cystic kidney disease in a mouse model orthologous to human disease (Pkd1:Nestin).

Dialysis as a Treatment Option for a Patient With Normal Kidney Function and Familial Tumoral Calcinosis Due to a Compound Heterozygous FGF23 Mutation.

Primary tumoral calcinosis is a rare autosomal recessive disorder characterized by ectopic calcified tumoral masses. Mutations in 3 genes (GALNT3, FGF23, and KL) have been linked to this human disorder. We describe a case of a 28-year-old man with a history of painful firm masses over his right and left gluteal region, right clavicle region, knees, and left elbow.

Giant Renal Angiomyolipoma Following Ovarian Stimulation Therapy.

Renal angiomyolipomas (AMLs) are benign tumors with higher prevalence in women. Female hormones have been shown to induce AML enlargement. This case refers to a 40-year-old woman with 4 left kidney AMLs, the larger ones with 1.

Cardiac dysfunction in Pkd1-deficient mice with phenotype rescue by galectin-3 knockout.

Alterations in myocardial wall texture stand out among ADPKD cardiovascular manifestations in hypertensive and normotensive patients. To elucidate their pathogenesis, we analyzed the cardiac phenotype in Pkd1(cond/cond)Nestin(cre) (CYG+) cystic mice exposed to increased blood pressure, at 5 to 6 and 20 to 24 weeks of age, and Pkd1(+/-) (HTG+) noncystic mice at 5-6 and 10-13 weeks. Echocardiographic analyses revealed decreased myocardial deformation and systolic function in CYG+ and HTG+ mice, as well as diastolic dysfunction in older CYG+ mice, compared to their Pkd1(cond/cond) and Pkd1(+/+) controls.

Secondary hypertension caused by massive renal lymphangiomatosis.

Renal lymphangiomatosis is a rare disease characterized by lymphatic vessel proliferation. We present a case of an adult patient with chronic flank pain, hypertension, and a right kidney mass. The magnetic resonance imaging findings were consistent with unilateral renal lymphangiomatosis.

Hypercalcemia and suppressed PTH levels in a renal transplant patient infected with Pneumocystis carinii.

Pneumocystis carinii pneumonia is a serious and relatively common complication of immunosuppressive therapy. In immunocompromised patients, P. carinii pneumonia can cause significant morbidity and mortality.