Kidney transplant patient with immunoglobulin A nephropathy subsequently diagnosed as concurrent autosomal dominant polycystic kidney disease during 17-year follow-up

AbstractA 14-year-old Japanese boy was diagnosed with immunoglobulin A nephropathy resulting in end-stage kidney disease (ESKD). He underwent ABO-compatible living kidney transplantation from his father at the age of 27. In the process of selecting a donor before the transplantation, it turned out that his mother had polycystic kidneys and that her family had a history of hypertension and cerebrovascular diseases. The patient himself also had bilateral multiple kidney cysts, with a normal-sized kidney, confusing us to make the diagnosis of acquired cystic kidney disease (ACKD) or ADPKD difficult at that point. Seventeen years later, his native kidneys showed bilateral swelling with multiple cysts. This, along with the histories of his mother and her relatives and with the existence of multiple liver cysts, led us to confirm the diagnosis of autosomal dominant polycystic kidney disease, not of ACKD. Contrary to previous studies that have suggested the size of cysts both in ADPKD and ACKD reduced with time, the present case showed an increase of 3.0% per year in total kidney volume (TKV) by computed tomography. It suggested the possibility that TKV, after decreasing in the relatively early stage after transplantation, may later increase in the long term after ESKD due to another kidney injury.
Source: CEN Case Reports - Category: Urology & Nephrology Source Type: research

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2017 ended as a banner year for my family, but things didn’t look great at the start. A death sentence met us in a boxing ring, and we had to school ourselves on fighting to live. I never thought much about the 37 million American adults who suffer from kidney disease until my husband Neil became one of them. Celebrating our first year of marriage in 2001, we learned by accident through an unrelated medical exam that my husband has polycystic kidney disease, an illness which causes the kidneys to fill with cysts over time, rendering the organs unable to function properly. There is no cure. There was nothing to do but...
Source: TIME: Health - Category: Consumer Health News Authors: Tags: Uncategorized health Healthcare medicine public health Source Type: news
CONCLUSIONS: This study suggests an important link between hypertension in pregnancy and ESKD. The patients were significantly younger, presented later and were more likely to have hypertensive nephropathy. Methamphetamine abuse appears to be a risk factor. The study suggests that all women with hypertensive disorders during pregnancy need further evaluation and follow-up postpartum. PMID: 31635591 [PubMed - in process]
Source: South African Medical Journal - Category: African Health Tags: S Afr Med J Source Type: research
AbstractBackgroundAlthough high-density lipoprotein (HDL) modulates many cell types in the cardiovascular system, little is known about HDL in the kidney. We assessed urinary excretion of apolipoprotein AI (apoAI), the main protein in HDL.MethodsWe enrolled 228 children with various kidney disorders and 40 controls. Urinary apoAI, albumin, and other markers of kidney damage were measured using ELISA, apoAI isoforms with Western blot, and renal biopsies stained for apoAI.ResultsPatients followed in nephrology clinic had elevated urinary apoAI vs. controls (median 0.074  μg/mg; interquartile range (IQR) 0.0160–...
Source: Pediatric Nephrology - Category: Urology & Nephrology Source Type: research
In conclusion, our study suggests that a large percentage did not have nephrology care before initiating dialysis. The ones who were seen by nephrology before dialysis were significantly more likely to initiate dialysis using an AVF. A national focus on improving nephrology referral in advanced CKD may allow better utilization of AVF as the method of access at dialysis initiation. PMID: 31696849 [PubMed - in process]
Source: Saudi Journal of Kidney Diseases and Transplantation - Category: Urology & Nephrology Authors: Tags: Saudi J Kidney Dis Transpl Source Type: research
A 55-year-old man with a history of end-stage renal disease due to autosomal dominant polycystic kidney disease and bilateral nephrectomy without adrenalectomy underwent his first kidney transplantation after 6 years of hemodialysis. The patient had no history of donor-specific antibodies. The donor was described as a 69-year-old man with a history of active smoking and  aortic valvulopathy who died of a stroke without cardiac arrest or collapse. Maintenance immunosuppressive therapy consisted of low-dose tacrolimus and everolimus.
Source: Kidney International - Category: Urology & Nephrology Authors: Tags: Make Your Diagnosis Source Type: research
We describe a rare case of a 29-year-old man with ARPKD presenting with subarachnoid hemorrhage secondary to a ruptured intracranial aneurysm. The diagnosis of ARPKD was at the age of eight years based on typical ultrasonography findings with polycystic kidneys and liver disease. Magnetic resonance cholangiography showed a nonobstructive dilatation of intrahepatic bile ducts. Liver biopsy showed hepatic fibrosis. None of the family members was affected. At the age of 15 years, he had progressed to end-stage kidney disease, and hemodialysis was started. The patient had always a severe arterial hypertension. At the age of 29...
Source: Saudi Journal of Kidney Diseases and Transplantation - Category: Urology & Nephrology Authors: Tags: Saudi J Kidney Dis Transpl Source Type: research
In conclusion, in this sample population from HD patients in Jordan, majority would recommend an AVF as mode of access. Perceived barriers include lack of timely referral for vascular surgical evaluation and poor understanding of disease. A systematic assessment of the process that precedes the creation of AVF, with focus on areas of reported barriers may allow for better utilization of AVF. PMID: 31464248 [PubMed - in process]
Source: Saudi Journal of Kidney Diseases and Transplantation - Category: Urology & Nephrology Authors: Tags: Saudi J Kidney Dis Transpl Source Type: research
Conclusion: These cases highlight the value of radiological imaging, pathological examination, and genetic evaluation for the diagnosis of CHF. When an individual with unexplained cirrhosis presents with bile duct dilation and malformation as well as polycystic kidneys, the possibility of CHF should be considered. For individuals found to have polycystic kidneys at a young age, the results of liver function tests and imaging examinations including Fibroscan imaging should be continuously and dynamically monitored to enable early diagnosis of CHF.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Discussion: Incomplete septal cirrhosis is an uncommon cause of non-cirrhotic portal hypertension. Its definition is not well known, morphological and pathophysiological. We have not found published cases of post-RT ascites secondary to this pathology, described as possibly associated with drugs, immune alterations, infections, hypercoagulability and genetic predisposition. PMID: 30897191 [PubMed - as supplied by publisher]
Source: Jornal Brasileiro de Nefrologia - Category: Urology & Nephrology Tags: J Bras Nefrol Source Type: research
The objective is to study the clinical profile of tuberculosis (TB) in chronic kidney disease (CKD). This is retrospective study of CKD patients who were diagnosed to have TB over a period of seven years at a tertiary care hospital. TB was diagnosed in 115 patients with an incidence of 4200/100,000. Mean age of the patients was 46.9 ± 16 years. Sixty-two patients (53.9%) were male. Causes of CKD were diabetic nephropathy and hypertension in 11.3% each, chronic glomerulonephritis in 31.3%, chronic tubulointerstitial nephritis in 39.1%, autosomal dominant polycystic kidney disease, and post-renal transplant CKD in 3.5...
Source: Saudi Journal of Kidney Diseases and Transplantation - Category: Urology & Nephrology Authors: Tags: Saudi J Kidney Dis Transpl Source Type: research
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