Sunday, May 17, 2015

Pain & Burden of PKD; Unique Treatment for PKD; Tattoos for PKD

Living with PKD

From MPR

Pain in Autosomal Dominant Polycystic Kidney Dz Often Complex

(HealthDay News) — The etiology of pain in autosomal dominant polycystic kidney disease is complex, and management of pain should be approached in a stepwise manner, according to a review published in the May issue ofThe Journal of Urology.
Matthew W. Tellman, from the Indiana University School of Medicine in Indianapolis, and colleagues conducted a systematic literature review of the etiology and management of pain in autosomal dominant polycystic kidney disease and anatomy of renal innervation.
The researchers found that for most patients with autosomal dominant polycystic kidney disease, pain occurs due to renal, hepatic, and mechanical origins. Patients may experience different types of pain complicating confirmation of etiology. Anatomical and histological assessment of renal innervation can help elucidate the mechanisms that can lead to renal pain. A stepwise approach is recommended for management of pain in autosomal dominant polycystic kidney disease. Due to the high incidence of acute causes of renal pain in autosomal dominant polycystic kidney disease, these must be ruled out first. Non-opioid analgesics and conservative interventions can be used first for chronic pain, before considering opioid analgesics. Surgical interventions such as renal cyst decortication, renal denervation, and nephrectomy can target pain from persistent renal or hepatic cysts.
"Chronic pain in patients with autosomal dominant polycystic kidney disease is often refractory to conservative, medical, and other noninvasive treatments," the authors write.  [Read more]



From AJMC

Burden of Autosomal Dominant Polycystic Kidney Disease: Systematic Literature Review

ABSTRACT 

Objectives: Autosomal dominant polycystic kidney disease (ADPKD), the most common hereditary kidney disorder, is a leading cause of end-stage renal disease. While there is no pharmacologic ADPKD therapy indicated, earlier supportive treatment may reduce disease burden, which may lead to the reduction or prevention of healthcare utilization and costs. To help US healthcare payers to understand this genetic disorder and the related healthcare utilization and costs, we performed a literature review on ADPKD. 

 Study Design: This literature review includes information on ADPKD incidence and prevalence, diagnostic criteria and risk factors, and the humanistic and economic burden. The information was summarized to characterize the impact of ADPKD on patients and healthcare systems. 

 Methods: PubMed and EMBASE databases from January 2003 to March 2013 were searched for articles containing relevant key terms, which were then screened for exclusion criteria to focus on data for ADPKD. For the selected publications, data were extracted and summarized. 

 Results: The results indicate that prevalence studies are outdated and have generally been on small populations. Additionally, diagnostic criteria are established, and a few possible disease progression prognostic factors have been identified. Pain is a commonly recognized element of humanistic burden, and a correlation between reduced kidney function and increased healthcare costs has been demonstrated [Read more]




From Business Wire

Unique Treatment for Polycystic Kidney Disease: Two Surgeries, One Anesthesia

FARMINGTON, CT--(BUSINESS WIRE)--Only a handful of transplant centers in the country treat polycystic kidney disease (PKD) with removal of both dysfunctional kidneys with a replacement kidney from a living donor in one operation.

BroadcastMed, Inc. and University of Maryland Medical Center present Terry J. Watnick, MD who works with the transplant team to help patients avoid multiple surgical operations, increased costs and potential prolonged dialysis by performing one procedure.

Dr. Watnick is an Associate Professor of Medicine at the University of Maryland School of Medicine and the Director of the Baltimore Polycystic Kidney Disease Research and Clinical Core Center.

Physicians can learn more at the UMMC Physician Learning Channel.

About University of Maryland Medical Center

The University of Maryland Medical Center (UMMC) performs at least 300 kidney transplants per year and has one of the nations largest kidney transplant programs. UMMC surgeons are also at the forefront of laparoscopic living donor nephrectomy, which has made kidney donation much easier, and allows for patients to get transplant faster. For more information visit umm.edu/transplant.





PKD Fundraising

From Northern Life, Canada, By: Sudbury Northern Life Staff

Sudburians urged to buy a temporary tattoo for Taylum

Two-year-old Taylum Lamoureux is expected to receive a kidney donation May 13, after spending his entire life living in Toronto's Ronald McDonald House due to his kidneys failing shortly after birth. Supplied photo.

Two-year-old Taylum Lamoureux is expected to receive a kidney donation May 13, after spending his entire life living in Toronto's Ronald McDonald House due to his kidneys failing shortly after birth. Supplied photo.

Fundraising event aims to help toddler, Ronald McDonald House

A fundraising event intended to help a Chelmsford toddler who is receiving a kidney transplant begins Monday and runs until Wednesday, when he's scheduled for surgery.

Taylum Lamoureux has Polycystic Kidney disease and has spent his entire life in Toronto living at Ronald McDonald House waiting to grow big enough to receive a kidney transplant. Now two years old, he will receive a transplanted kidney from a selfless stranger who will donate one of hers to him.

Starting this week, friends and supporters of the family will be selling temporary tattoos for $1 each and organizers are “encouraging all of our students, teachers, friends and family to wear them on May 13, the day of his transplant.

“The proceeds raised will be donated to Ronald McDonald House Toronto, and a portion of the proceeds will be used to purchase a play structure for Taylum when he is finally able to return home,” wrote Marymount Academy teacher Tammy Jutila, in an email. “It is the hope that each secondary school will be participating, and the campaign will only run for three days, May 11-13.”

Many local businesses will also be selling the tattoos to help the family. And anyone interested in buying a tattoo can contact one of the following organizers: Jenni Preen, 705-566-960; Jen Geddes, 705-969-2212; MacKenzie Crowther, 705-523-9235; and Tammy Jutila, 705-674-4231.

Since he was brought to Toronto, Taylum has lived at Ronald McDonald House, near the Hospital for Sick Children, with his mother Désirée Lamoureux. If all goes well, after the operation he'll be visiting the hospital every day for three months, for blood work and checkups, to make sure his new kidney is functioning properly.

After three months, if there are no complications, Taylum and his mother will be able to return home to Sudbury. But he will need to return to Toronto every two weeks for more checkups, over a three- to six-month period.




From CJME, Radio 980, Regina, Canada, Reported by Lasia Kretzel

'You have your life back,' transplant recipient, donor support annual walk


Deb Leisle and Bonnie Cockrum support annual Transplant Trot

Deb Leisle and Bonnie Cockrum share a unique bond: their kidneys.

Born with polycystic kidney disease, Deb Leisle started dialysis eight years ago. Four years later, while visiting a friend at St. Paul's hospital, she met nurse Connie Cockrum. Cockrum learned of Leisle's disease and need of a new kidney, and decided to donate one her own.

"It was just something from within that said, 'hey you should do this,'" Cockrum said.

The two were among the more than 100 people who came out Saturday to support and take part in the Transplant Trot. The annual run raises awareness for the need for organ donations and celebrates recipients and donors.

Provincial director of Canadian Transplant Association Phil Gleim said there are 100 people people on the Saskatchewan waiting list to receive a kidney. Many more are waiting on lungs, hearts, livers, bones and corneas.

As many as 50 per cent of all kidney transplants are through living donors while liver, bone and amniotic membranes can also come from live patients.

Deceased donors can save up to eight lives if their donate their organs, Gleim said.

"If people were to come out to events like these or meet someone who has had an organ transplant, I think they realize you're not just saving someone's life, you're giving somebody back a quality of life," he said, adding it is an individual decision.

Cockrum said on the day of the transplant, she wasn't nervous.

"That kind of thing really excites me, to be able to change someone's life like that," she said.

Leisle said the transplant gave back her life.

"You're not tied to a machine three days a week, you have your life back, you're able to eat (what you want)," she said. "Now I can go on a holiday."

Today, Leisle and Cockrum both live healthy lives and share a unique friendship.

"We're kind of hitched at the kidney," Leisle said with a laugh.

"I have visiting rights now," Cockrum added.




PKD Research

From JCI, Journal of Clinical Investigation

Macrophage migration inhibitory factor promotes cyst growth in polycystic kidney disease

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by renal cyst formation, inflammation, and fibrosis. Macrophages infiltrate cystic kidneys, but the role of these and other inflammatory factors in disease progression are poorly understood. Here, we identified macrophage migration inhibitory factor (MIF) as an important regulator of cyst growth in ADPKD. MIF was upregulated in cyst-lining epithelial cells in polycysitn-1–deficient murine kidneys and accumulated in cyst fluid of human ADPKD kidneys. MIF promoted cystic epithelial cell proliferation by activating ERK, mTOR, and Rb/E2F pathways and by increasing glucose uptake and ATP production, which inhibited AMP-activated protein kinase signaling. MIF also regulated cystic renal epithelial cell apoptosis through p53-dependent signaling. In polycystin-1–deficient mice, MIF was required for recruitment and retention of renal macrophages, which promoted cyst expansion, andMif deletion or pharmacologic inhibition delayed cyst growth in multiple murine ADPKD models. MIF-dependent macrophage recruitment was associated with upregulation of monocyte chemotactic protein 1 (MCP-1) and inflammatory cytokine TNF-α. TNF-α induced MIF expression, and MIF subsequently exacerbated TNF-α expression in renal epithelial cells, suggesting a positive feedback loop between TNF-α and MIF during cyst development. Our study indicates MIF is a central and upstream regulator of ADPKD pathogenesis and provides a rationale for further exploration of MIF as a therapeutic target for ADPKD. [Read more]

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