Journal of nephrology
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Journal of nephrology · May 2003
ReviewPortuguese-type amyloidosis (transthyretin amyloidosis, ATTR V30M).
Portuguese-type amyloidosis (transthyretin amyloidosis, ATTR V30M) is the most common form of systemic hereditary amyloidosis, inherited in autosomal dominant mode. The disease, also called familial amyloid polyneuropathy type I (FAP-I), is caused by a mutant transthyretin (TTR) protein, which is synthesized by the liver. A single amino acid substitution of methionine for valine at position 30 of the TTR molecule (TTR V30M) was found in Portuguese patients. ⋯ The progression towards end-stage renal disease (ESRD) affects 10% of the patients, and the survival after initiation of dialysis is a mean of 21 months. Patients who progress to ESRD have a late onset of neuropathy and lower prevalence of clinical disease in their families. Liver transplantation is a widely accepted treatment for FAP-I, and combined liver-kidney transplantation is also an option for selected patients with FAP-I and ESRD.
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Journal of nephrology · May 2003
Non-heart-beating donors: experience from the Hospital Clinico of Madrid.
Non-heart-beating donors (NHBD) have received attention in the last few years as an alternative source to increase the pool of kidney donors. The majority of reports focus on NHBD from controlled donors, i.e. patients who die in hospital. This report focuses on our experience using uncontrolled NHBD, i.e. patients who die outside of hospital and are transported to hospital for organ donation. ⋯ At 2 years renal function post-transplantation was better in NHBD organs, as evaluated by serum creatinine and creatinine clearance, while after 2 years it was similar. The incidence of acute renal rejection was lower in NHBD; however, delayed graft function, as expected, was more prevalent in NHBD, although interestingly it did not influence long-term survival. In conclusion NHBD from deaths outside the hospital may be a good source of donor kidneys and also a way to successfully increase the pool for organ transplantation.