Why do children suffer severe recurrent infections?

The research has just been published in the prestigious journal Blood and has already drawn much interest worldwide.

By JUDY SIEGEL-ITZKOVICH
Children receiving medical treatment in hospital 370 (R) (photo credit: Jorge Lopez / Reuters)
Children receiving medical treatment in hospital 370 (R)
(photo credit: Jorge Lopez / Reuters)
Agroup of Hadassah Medical Organization researchers have discovered the reason for recurrent life-threatening infections and bone marrow failure in children. Following six months of intensive research, they found a gene connected to intracellular storage and transport in select white blood cells of the immune system and bone marrow.
The research has just been published in the prestigious journal Blood and has already drawn much interest worldwide.
The researchers – Dr. Polina Stepensky, a pediatric hematology/ oncology and bone marrow transplant specialist; Prof. Orly Elpeleg, head of the genetics and metabolic diseases department; and Prof.
Dror Mevorach, head of the internal medicine B department and director of the rheumatology research center, began their interdisciplinary study when five children from different families were admitted to Hadassah with recurrent, serious infections with the subsequent bone marrow failure.
Using cutting-edge Hadassah technology, they focused on a specific gene found to be defective in Arab families of Saudi Arabian descent.
It was then that the researchers attempted to decipher the mechanism – how the defective gene led to such a catastrophic illness.
They learned that the mutation caused increased programmed cell death in select white blood cells and bone marrow. Additionally, the researchers were able to show that the intracellular transport system in the blood cells was collapsing due to the absence of intracellular storage vesicles (lysosomes and alpha-granules).
An immediately obvious advantage of these findings is the doctors’ ability to offer mutation testing for the families of the sick children, as well as in the general population. As of today, two of the children have recovered from this debilitating condition thanks to successful bone marrow transplants performed at Hadassah.
KUDOS FOR ORGAN RECIPIENT CHOICE The Israeli technique for setting priorities for allocating donated organs has been praised by Stanford University Prof. Alvin Roth, recipient of the 2012 Nobel Prize for Economics. At a conference held in march on “Organ Donation and Incentives” – which was attended by Prof. Jacob Lavee, head of the heart transplant unit at Sheba Medical Center at Tel Hashomer – Roth praised how Israelis do it.
The local mechanism objectively hands out points to potential organ recipients according to the severity of their condition, their blood and tissue types and other criteria, and higher priority to people who are registered as potential organ donors with the ADI organization. The idea of providing a “jump in the queue” for organs to people who have been registered for a specific time with ADI was proposed by Lavee, and has proven very successful in bringing in many more donors.
Roth said that after studying the Israeli technique, he was persuaded that it is the best and fairest available and can significantly increases the number of potential donors.
TOBACCO CONTROL WORKS Over a span of nearly 20 years, California’s tobacco control program cost $2.4 billion and reduced health care costs by $134b., according to a new study at the University of California at San Francisco. Additionally, the study – published in PLoS One, covering the beginning of the program in 1989 through 2008 – found that the state program helped lead to some 6.8 billion fewer packs of cigarettes being sold that would have been worth $28.5b. in sales to cigarette companies.
The study was designed to calculate the fiscal impact of California’s large public health program on smoking prevalence and cigarette consumption. The new research shows that tobacco control funding is directly tied to reductions in both the prevalence of smoking and cigarette consumption per smoker – and generates significant savings in overall health care expenditures. “These health care cost savings began to appear almost immediately after the program started and have grown over time, reaching more than $25b. a year in 2008,” said UCSF clinical pharmacy Prof. James Lightwood.
The study was designed to calculate the fiscal impact of California’s large public health program on smoking prevalence and cigarette consumption. The new research shows that tobacco control funding is directly tied to reductions in both the prevalence of smoking and cigarette consumption per smoker – and generates significant savings in overall health care expenditures.
NEW CHAIRMAN OF MED SCHOOL DEANS
Prof. Yoseph Mekori, dean of Tel Aviv University’s Sackler Faculty of Medicine, has been named head of the Israel Medical School Deans’ Association. He replaces Prof. Eran Leitersdorf, dean of the Hebrew University Medical Faculty in Jerusalem.
The association coordinates the positions of the country’s five medical schools on medical education and research, and represents the deans when they voice their positions before state authorities in Israel and abroad.
MDA’S BRITISH FRIENDS HELP OUT In 2012, Magen David Adom UK launched an appeal for Israeli Holocaust survivors. The organization set out, modestly, to raise $150,000. The appeal turned out to be one of the most successful ever run, raising $250,000 for one of Israel’s neediest populations.
Now, a year later, MDA UK is working directly with Israel’s largest national survivors’ foundation.
With 35 survivors dying each day, MDA UK is working without middlemen; the funds raised are going directly to those who need it most, the organization’s leaders say. The effort is functioning in conjunction with MDA in Israel, the Welfare and Social Services Ministry, and UK Ambassador to Israel Matthew Gould, who launched the initiative over two years ago and is still actively involved today.
The funds have been used to pay for survivors’ ambulance rides, medicines and emergency medical treatment not covered by either the state or the public health funds. While before the effort, many survivors in their 80s and 90s were reluctant to dial 101 for fear of getting a hefty MDA bill, they can now do so knowing that any costs they incur will be covered, according to the friends’ organization’s chief executive, Daniel Burger. At the end of May, Sir Ian Gainsford – life president of Magen David Adom UK – will join Gould at the Holocaust Survivors’ Club to see firsthand the people they are supporting.


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