Decisions 2003:

In 2003, we approved renewal of Drs. M. Freedman (Haematology/Oncology, HSC), E. Leung and Y. Dror’s project titled "Angiogenesis in Shwachman-Diamond Syndrome Bone Marrow Stroma: The Interplay Between Angiogenic Characteristics, Apoptosis and Leukemogenic Potential." A further $23,220/year for 2 years has been granted.

Decisions 2002:

In 2002, we awarded three grants totaling $80,220 as follows: 1) Drs. M. Glogauer (Dentistry, University of Toronto) and Y. Dror (Haematology/Oncology, HSC) have been awarded a $30,000 "Griffiths-McBurney&Partners Research Grant" to support a project titled "Oral Disease Prevalence and Quality of Life in Patients with SDS". 2) Drs. Glogauer and Dror have been awarded a $27,000 grant for a project titled "Relationship of Oral Disease and Oral Neutrophil Function in Patients with SDS". 3) Drs. M. Freedman (Haematology/Oncology, HSC), E. Leung and Y. Dror have been awarded $23,220 for a project titled "Angiogenesis in Shwachman-Diamond Syndrome Bone Marrow Stroma: The Interplay Between Angiogenic Characteristics, Apoptosis and Leukemogenic Potential."

Decisions 2001:

Following successful peer review, a three year grant for $79,200 was awarded to Dr. Yigal Dror and his colleagues at The Hospital for Sick Children in Toronto for a project titled "Abnormal Cellular Processes causing Premature Cell Death (Apoptosis) of Marrow Cells of Patients with Shwachman-Diamond Syndrome: Implications for Bone Marrow Failure." Dr. Dror and his colleagues have previously shown that, as well as proliferating poorly, SDS bone marrow cells undergo premature cell death (apoptosis). Dr. Dror and his collaborators, Drs. Freedman & Abdelhaleem, will be investigating further this process. This may ultimately lead to improved treatments prolonging the blood cell life span, and improve bone marrow transplantation outcomes.

Aside from these research competitions, in 2001 SDS Canada awarded $10,000 over two years to Dr. Dror et al. to help fund a Canadian Inherited Marrow Failure Registry and $10,000 to researchers from The Hospital For Sick Children for travel to the 1st International Scientific Congress on SDS in Italy.

Decisions 2000:

A grant of $35,550 was awarded to Dr. Johanna Rommens and Dr. Melvin Feedman for research to determine which genes play a role in the deficiencies in the bone marrow stroma of SDS patients and to determine what the consequences of the stroma deficiencies might be. Previous studies by this group have shown that there are two abnormal conditions in the marrow of SDS patients: 1) there are inadequate numbers of "precursor" white blood cells; and 2) the "stroma" (i.e., the marrow environment) is abnormal and cannot adequately support the growth of the precursor cells. The researchers hypothesize "that these stromal deficiencies lead to the reduced numbers of precursor cells and that this disturbance causes the susceptibility to leukemia." The research plan is to use "gene array" technology to analyze over 400 of the genes known to be involved in the blood system to see which, if any, are abnormal in the stroma of SDS patients. The stroma samples will be taken at the same time as the routine bone marrow aspirates of SDS patients. Potential benefits include: 1) by understanding the mechanism behind how the production of cells is altered in SDS patients, it may be possible to identify which growth factors can be safely used to increase blood cell counts in these patients; and 2) the information from these studies may also lead to therapies to prevent leukemia in people with SDS.

A grant of $25,311 was awarded to Dr. Peter Durie and his colleagues for their study on acinar cell function in SDS-patients. Acinar cells, which produce enzymes to digest food, can be found in the pancreas and in the parotid gland. The parotid gland produces saliva in the mouth. It is known that people with SDS have defective pancreatic acinar cells. The researchers hypothesize that people with SDS may also have defective acinar cell function in the parotid gland. The parotid gland produces amylase (to digest starch) in a form that is different from that produced by the pancreas. The researchers' preliminary data to date suggests that people with SDS have deficient blood levels of both the pancreatic and the parotid forms of amylase. Their work aims to: 1) compare levels of blood concentrations of pancreatic and parotid amylase in 3 groups of people: people with SDS, people with CF and healthy people; 2) compare secretion of parotid fluid electrolytes and enzymes in people with SDS, people with CF and healthy people; 3) assessment of starch digestion in people with SDS. This research could lead to diagnostic tests for SDS and, as well, could result in improved treatments for starch maldigestion in SDS patients.