News and analysis · Nouvelles et analyses

CMAJ 2000;162:243-56

• Can Quebec afford dialysis for every 80-year-old patient?
• Renowned cardiac surgeon resigns post
• London MDs provide window to surgery's future
• Pediatric ethical conflicts increasingly confusing
• Reform Party rejects physician's reforms
• E. coli outbreak linked to fall fair
• Are faculty members facing more harassment?
• Dracula wants your blood. Now !
• Newfoundland boosts reentry positions
• Dal medical school is open for business
• Colleges can do little to regulate telemedicine: registrar
• Pulse: Are Canadians aware of the risks of smoking
• Want to quit smoking? Call your surgeon
• Research Update:
  • The unravelling of chromosome 22
  • Socioeconomic status at the heart of health care inequality

After the Quebec Hospital Association began stoking the province's health care fires this fall, separatism was no longer the only issue creating heat in the province. The 135-member organization stated publicly that difficult and unpopular decisions affecting patients' lives have to be made in a climate of restricted government spending. Long waiting lists and reduced access to health care led the association to pose some tough questions. Now President Marie-Claire Daigneault-Bourdeau and Vice-President Daniel Adam want a public debate involving both federal and provincial governments to supply the answers. Should we develop a role for the private sector in Canada? Can we afford to offer all services to everyone on an equal basis? "Should we put a pacemaker in an 82-year-old patient?" asked Adam. "Should we accept giving dialysis treatment to an 80-year-old patient? This is the type of debate we should carry out."

Many people find this kind of thinking unethical and unacceptable. "They're trying to prepare the public for less treatment or no treatment at all for certain vulnerable groups like senior citizens," warned Dr. Paul Saba, president of the Coalition of Physicians for Social Justice. "This is a very dangerous precedent." He called this type of triage a slippery slope and stated emphatically that "all life is of value."

But some clinicians and ethicists stated that there are simply not enough ICU beds, dialysis machines and ventilators to accommodate an increasing number of critically ill patients, not all of whom would be well served by such sophisticated and costly interventions. Dr. Michael Dworkind, director of the Palliative Care Unit at Montreal's Jewish General Hospital, stressed that when it comes to making difficult decisions there are limitations to technology. "You have to be selective about the goals you have at the end of life. Many of the technologies I see are just prolonging the dying process." He added the caveat that each case has to be decided upon its own merits.

There is a paradox between Canadian society's commitment to provide care for all and the reality of scarce resources and competing demands, argues Dr. Eugene Bereza, a clinical ethicist in the Biomedical Ethics Unit at McGill. Bereza, a member of the CMA's Committee on Ethics, said decisions about allocating resources need to be discussed by society as a whole and not by isolated physicians caught in crisis situations, or by hospital administrators. "Sometimes doctors are placed in situations where this [extreme intervention] is doing more harm than good for the patient. An extremely distant consideration is that it's an imprudent use of resources. But it shouldn't be doctors on the front line taking the heat for that. This should be society's decision."

Members of the Quebec Hospital Association would agree. Their real beef is that hospital administrators are being held responsible or being made scapegoats for cuts in services that result from inadequate public funding. "Quebec hospitals no longer accept the burden of imposed deficits and cuts in service," said Daigneault-Bourdeau. "The government should spell out its responsibilities [to the population]." — Susan Pinker, Montreal

[Contents]

Dr. Wilbert Keon, who performed Canada's first transplant involving an artificial heart, has resigned as head of the University of Ottawa Heart Institute following an incident involving an undercover police officer posing as a prostitute.

During a mid-December news conference, an emotional Keon, 64, said he stopped his car on a downtown Ottawa street at about 10 p.m. on Nov. 25 to talk to a woman who had approached his vehicle. Although Keon said his "intent was a conversation," he was picked up by police as part of the sting operation.

"This [talking to the undercover officer] placed me in a compromising situation for which I must take full responsibility," Keon said. He participated in a program for first-time offenders and was not charged with solicitation.

In 1985 Keon became the first Canadian to implant the Jarvik artificial heart in a patient and in 1989 he performed Canada's first heart transplant involving an infant. Then Prime Minister Brian Mulroney named Keon to the Senate in 1990. He has not yet decided whether he will resign his Senate seat, but he has stepped down from his administrative duties at the heart institute. Many Ottawans stated vehemently that Keon should not have resigned from the institute, which he helped found in 1976. Dr. Donald Beanlands, the institute's former deputy director general, is the acting director.— Steven Wharry, CMAJ

[Contents]

It's fitting that the world's first closed-chest, robot-assisted, beating-heart coronary artery bypass graft (CABG) took place 3 months before the end of the century, because the surgeon who performed it says the procedure represents "a preview into cardiac surgery for the [next] millennium."

Boyd: "enabling technology"

A "new era of cardiac surgery is upon us," says London, Ont., cardiac surgeon Douglas Boyd, who performed the procedure at the University Campus of the London Health Sciences Centre (LHSC) in October. That operation attracted national and international attention, but since then Boyd's team has quietly performed another 5 procedures. He believes robotic and computer-enhanced surgery will revolutionize surgery in all disciplines. "We just don't know what the limits are yet. Right now, we're rethinking the way we do every procedure."

With minimally invasive computer-enhanced surgery, the surgeon can work more easily in a confined space. In closed-chest surgery, Boyd sits at a console several feet from the patient. He activates the robot and controls instruments with his voice. The entire procedure, including sewing of the graft, is performed with the robot through 3 pencil-sized incisions and 1 incision 2 to 3 cm long. "This enabling technology gives us the tools and dexterity that hitherto were just impossible," said Boyd, the director of minimally invasive and robotic cardiac surgery.

When Boyd joined LHSC 3 years ago, he thought this achievement was a decade away. Then the team "mapped out a plan of what the utopia of cardiac surgery would be." They defined "utopia" as CABG without opening the chest or stopping the heart.

The group established 5 or 6 critical tasks and divided them into 110 steps. "At each step we did the laboratory experimentation, the clinical experimentation and the outcomes evaluation," explains Boyd. The group then published the results.

These organizational details not only led to a world first, but also helped secure funding for the Zeus Robotic Surgical System from London philanthropists Richard and Beryl Ivey. LHSC is the only centre in Canada and 1 of 4 in North America to use the Zeus system clinically. The technology, initially developed by NASA for a mission to Mars, is expected to contribute significantly to patients' well-being and to reduce costs and improve access to treatment.

Although data are not yet available for the latest procedure, findings from 58 mini-thoracotomy, robot-assisted, video-enhanced coronary bypasses at LHSC — these involve a 7- to 8-cm incision and hand-sewn graft — indicate significant improvements over conventional surgery. The average length of hospital stay was 3 days compared with 6.5, and the incidence of atrial fibrillation was 4% compared with 25%. There were 20% fewer complications and no patients required blood. "The world's most advanced technology is not worth a thing if it doesn't benefit patients," says Boyd. "That's why we think it's important to evaluate what we do to ensure it's better for patients. What we've found with our systematic approach is that this is a better way to do coronary revascularization." — Lynne Swanson, London, Ont.

[Contents]

If patients are not competent enough to make health care decisions, what principles should govern the decision-making of their health care professionals, especially when life-and-death issues are involved?

Anyone working in pediatrics is acutely aware of the conflicts involved in these cases. Dr. Christine Harrison, director of bioethics at Toronto's Hospital for Sick Children, told the recent meeting of the Canadian Association of Paediatric Hospitals that "such cases are not unusual in our system. But they are receiving a lot of media attention because of particular pressures in contemporary Canada."

In Canada's pluralistic society, cultural practices are often in conflict with the imperatives of evidence-based medicine. Moreover, as pediatric institutions shift to an emphasis on "family-centred care," professionals and parents must establish a partner­partner relationship rather than an expert­client one.

As well, more and more Canadians are turning to alternative therapies: 75% of the families bringing children to Sick Kids are also using alternative treatments such as herbal medicines. In the case of Tyrell Dueck, a 13-year-old Alberta cancer patient, health authorities had to back off and watch Dueck's parents waste $65 000 (of which they have raised only $45 000) on a trip to a Mexican clinic for an unlicensed, unproven therapy.

"What mechanisms are required within institutions to turn conflicts into consensus?" asked Harrison, who acknowledged that these cases never involve "win­win" solutions. When it appeared possible that the courts would order the health care team to impose treatment on Tyrell Dueck, hospital staff faced the horrifying prospect of forcing the child to undergo surgery. (His cancer proved too far advanced for surgery, and he died last summer.)

In Canada, the Bioethics Committee of the Canadian Paediatric Society has already articulated the principles on which decisions about the withdrawal of treatment for critically ill children should be based (CMAJ 1986;135[5]:447-8). — Charlotte Gray, Ottawa

[Contents]

Dr. Keith Martin is a disappointed man. The British Columbia MP, who serves as the Reform Party's health critic, recently presented his proposals for reform of the health care system to the party's shadow cabinet. He shouldn't have bothered, because his colleagues were not interested: "I lost round one," he told CMAJ ruefully.

Dr. Keith Martin: lost first round

Martin's presentation distilled the ideas contained in speeches and articles that he has penned during his 6 years in the House of Commons. He is convinced that the only way to save medicare is to permit the development of private clinics that operate entirely outside the public system. He says this would allow the wealthy to choose to spend disposable income on health care. By making that choice they would free up public facilities for everybody else and make more money available on a per capita basis. The rich, he suggests, would thus subsidize the not-so-rich.

"Is it unequal?" he asked in a recent newspaper article. "Yes, but I would argue that it is better to have an unequal system that provides for better access for everyone than the system we have now, which ensures continuing declining access to treatment for all but the rich, who can get better health care by flocking south of the border."

Why didn't his party buy his arguments? "They didn't want to go there because they share the belief that public opinion should lead the debate. They thought Reform would get burnt if we got too far ahead."

For a young man who entered politics because he wanted to effect change, this was a dispiriting lesson. "I thought we were here to lead and to fix problems. I think it is unfortunate that we must wait for public opinion, even when we can see that our current system is failing Canadians." Even if people don't agree with his ideas, he says, Canadians should at least be having a debate. At present, politicians are too cautious. "Defend the status quo and you look like the proverbial white knight. Advocate change and you are labelled an enemy of the health of Canadians. This strategy may be politically advantageous for some, but it stops a useful debate on health care dead in its tracks." — Charlotte Gray, Ottawa

[Contents]

It appears that autumn poses special risks for infection by verotoxin-producing Escherichia coli (VTEC), the Canadian Food Inspection Agency (CFIA) and a health unit in London, Ont., report.

Last fall the Middlesex-London Health Unit reported 159 suspected cases of E. coli O157:H7 infection that were linked to an agriculture pavilion at the Western Fair. The investigators found "very strong evidence" linking the outbreak to sheep and goats at the fair's petting zoo. VTEC, commonly found in the intestines of healthy cattle, is transmitted to humans by fecal-oral contact. One 9-year-old London boy was hospitalized with hemolytic uremic syndrome (HUS). Another infected child attended a day-care centre, prompting the health unit to remove temporarily all children who had diarrhea. Children and elderly persons are especially vulnerable to VTEC infection.

Southwestern Ontario is no stranger to the bacterium. A 1985 outbreak in London, which was traced to sandwiches containing uncooked meat or poultry, killed 19 nursing-home residents. Soon after, another outbreak in nearby Sarnia affected children who had consumed unpasteurized milk during a school visit to a dairy farm. Last fall, at least 116 people attending a fall fair near Albany, NY, were also affected. Eleven of the children involved developed HUS, and a 3-year-old girl and 79-year-old man died. The problem was traced to high levels of E. coli O157:H7 found in unchlorinated water from a shallow well that had been used by some of the fair's food vendors.

In October, the CFIA warned about the risk of VTEC infection from unpasteurized fruit juice or cider. The problem may arise when cider is made from dropped apples contaminated by grazing cattle. In 1998 at least 6 people in Ontario's Perth County were infected with VTEC after consuming cider from a 264-L batch produced by 2 farm families and shared with friends and relatives. —David Helwig, London, Ont.

[Contents]

Faculty members at some medical schools report that they face harassment and intimidation from their students, colleagues and administrators. "We need to look at intimidation of faculty by residents," Dr. William Wrixon, associate dean of postgraduate medical education at Dalhousie told physicians attending a plenary session during the Fifth Annual Conference on Residency Education in Montreal last fall.

Most complaints received at the University of British Columbia's Equity Office used to come from students, but for the last 2 years the majority have come from male faculty members. "The pendulum has shifted," said Dr. Peggy Ross, associate dean of equity at UBC.

Some faculty complain of personal harassment when they don't receive the same resources as colleagues. "Sometimes more junior people get the resources and there's no rhyme nor reason to it," said Ross. Her solution is to have departments draw up resource guidelines.

Ross says most faculty harassment involves other faculty members and administrators, but Wrixon thinks there has been an increase in harassment of faculty members by residents. One of the more common ploys is to threaten to call the Royal College if faculty don't fix some aspect of a training program the resident doesn't like, such as the evaluation protocol.

In one case a faculty member was ill and could not complete this mid-rotation evaluation. When the faculty member refused to pass the resident, he protested because the evaluation had not been done. The faculty member stood by his decision but many don't, says Wrixon. "A lot will say, 'let's see if the next rotation fails him.' " This is partially because the appeal process can take several years and countless meetings.

Dr. Chris de Gara, division director for general surgery at the University of Alberta, told of one student who had threatened to take action each time he disagreed with an evaluation, and repeatedly used every appeal process. During a workshop on intimidation in surgical training, de Gara told of a staff member who tried to commit suicide after a graduate student threatened his career.

Wrixon said there is little research into intimidation of teachers by students. "We shouldn't focus only on the interests of the residents. It has got to work both ways because we're all capable of misbehaving." — Barbara Sibbald, CMAJ

[Contents]

Canada's new national blood agency might wish to examine a blood donor campaign launched in Berlin last year: it featured a blue blood said to be Count Dracula's last surviving descendant.

The count, whose full name is Ottomar Rudolphe Vlad Dracul Prince Kretzulesco, lives in a crumbling castle on the southern outskirts of the German capital. He agreed to help the Red Cross after Germany's health minister appealed for more donors in an attempt to replenish dwindling national stocks.

Noting that supplies were so low that all nonurgent operations had been put on hold, the minister appealed to all Germans between 18 and 68 to donate blood or plasma. The Count helped the campaign by attending special showings of vampire movies for blood donors. All those who gave blood received his autograph.

The Count is the last descendant of the bloodthirsty Romanian prince named Vlad the Impaler, who some consider the inspiration for Irish novelist Bram Stoker's 1897 gothic horror fantasy, Dracula. — Gil Kezwer, Toronto

[Contents]

Some good news finally seems to be emerging for practising physicians who wish to train in a new specialty. Newfoundland is funding 13 entry-level residency positions that begin July 1, up from the 5 positions it sponsored last year. "This is an avenue of approach that didn't exist before," Melody Marshall of the Department of Postgraduate Medical Studies at Memorial University said of the expanded number of re-entry positions. She said Newfoundland has been offering the positions for about 5 years, but this is the largest number available so far.

These positions have all but disappeared in Canada since the residency system was reorganized 7 years ago. Today there are only enough training slots for new graduates of Canadian medical schools. Slots that remain unfilled tend to be in unpopular specialties such as laboratory medicine.

The openings in Newfoundland, which will be spread across different specialties, contain no return-of-service provision, but Marshall said priority will be given to in-province applicants. "Emphasis will be placed on length of service to the province, with value placed on the remoteness of the area served." Information on the program is available from Postgraduate Medical Studies at Memorial University, 709 737-6680. The application deadline is Mar. 31, 2000. — Patrick Sullivan, CMAJ

[Contents]

Although the Business Development Office at Dalhousie medical school is only a few months old, it has already met its goal of forming 3 companies a year. Since last April, the office has helped 5 faculty members at the medical school turn biotechnology research in cancer diagnostics, amyotrophic lateral sclerosis treatment and pain management into commercial enterprises.

The office has also raised $2 million in seed money from venture capital corporations to kick start the companies — Liposome Pain Management, NovaNeuron and Onco-Dynamics. In about a year, each company will require an additional $2 million to $5 million in investment capital. "The growth is exponential," says Neil Ritchie, whose firm, BioMed Management, runs the Business Development Office for the school. "They have the potential to become significant companies."

Neil Ritchie: "The growth is exponential."

Ritchie, who doubles as president of NovaNeuron, says his office earmarks research discoveries at the medical school that have commercial potential, helps the scientists form companies, finds investors and defines markets. The office also assists with basics, such as filing patents and drafting business plans. "Our objective is to try to create some infrastructure for building these companies," says Ritchie. He says that health and life-sciences businesses in the Maritimes have thus far amounted to little more than a cottage industry.

Ritchie's office has identified 30 of about 400 research projects at the medical school that have commercial possibilities. "About 10% of basic medical research projects have commercial potential. Of that, about 10% will form the basis of a company."

Ritchie says the medical school is not driven by a desire to reap profits from any companies founded by its faculty, although it may hold up to 10% ownership in some future ventures and intends to charge for the use of its facilities. "All of the intellectual property is owned by the scientist/entrepreneur. The school is interested more in the spin-off benefits of an enriched academic environment."

Have faculty been receptive to commercialization? "Initially I had the impression that scientists would be resistant," Ritchie says. "But the reality is that they're starting to understand the importance of having commercial partners. We're seeing a new mindset emerging, and it's not a sellout at all. Scientists want to make a difference in people's lives." — Nancy Robb, Halifax

[Contents]

Licensing in telemedicine can be a complex issue because of jurisdictional and geographic factors, says Dr. Tom Handley, registrar of the College of Physicians and Surgeons of BC.

The college's Board of Directors recently discussed 4 resolutions on telemedicine developed by the Federation of Medical Licensing Authorities of Canada. They call for licensing bodies to establish requirements for doctors wanting to practise telemedicine "through whatever regulatory or legislative mechanisms are appropriate." Handley said there are limits to what regulatory bodies can do because there is often no prosecution involved and the mandate of "protecting the public" is interpreted differently across the country.

The federation's second recommendation was that telemedicine services should be provided where the patient lived, a notion that Handley said is unrealistic. As an example, he cited a complaint to the college regarding a doctor who was involved in an incident with a flight attendant during a flight. "In that case, where is the jurisdiction?" he asked. Similarly, Handley said the federation's recommendation that physicians practising telemedicine satisfy the licensing requirements "of the jurisdiction in which their intended patients reside," is impossible to apply in all circumstances. Moreover, the federation suggests that professional misconduct in a jurisdiction includes telemedicine practices in which the physician has not obtained the necessary approval to provide the medical services.

Handley responded that the college could only monitor its members' standards of conduct within the province and could not control illegal practitioners, including those who practise across the province's or the country's borders by electronic means. "We have to tell patients that if they choose to get medical care by phone or any other method [that may involve a doctor outside the province], the college can't deal with any complaints." However, it will help a member of the public to complain to another jurisdiction, especially within Canada or the US. — Heather Kent, Vancouver

[Contents]

   | Other Pulse articles / Autres chroniques Médicogramme |

Statistics Canada's 1996/97 National Population Health Survey found that 96% of Canadians older than 12 are aware of smoking-related health risks.

Younger people are more likely to know about the risks — 98% of those aged 20-24 knew of them, versus 93% of those aged 75 or older — but awareness was the same among males and females. Quebec residents are somewhat less likely than other Canadians to know that smoking is linked to health risks (94% versus the national average of 96%). Residents of Manitoba and BC (98%) are most likely to know about these dangers.

Of those who recognize that smoking poses risks, 97% are aware of the specific risk of lung cancer, but only 85% of respondents named stroke as a risk associated with smoking.

There is almost no gender variation in awareness of specific health risks. However, there is a modest variation by age, with younger Canadians being somewhat more likely to be aware of the risks of lung cancer, heart disease, respiratory ailments and stroke. For example, 99% of those aged 12-14 are aware of the association between lung cancer and smoking, compared with 93% of those aged 75 or older. Residents of Quebec and Saskatchewan are least likely to know that a risk of stroke is associated with smoking (79% and 81%, respectively), while Ontarians and New Brunswick residents (90%) are most aware of the link between stroke and smoking.

Surprisingly, Canadians with lower levels of education tended to be more aware of the association between smoking and lung cancer, heart disease, stroke and respiratory ailments. — Shelley Martin, CMA

[Contents]

Researchers at Vancouver's St. Paul's Hospital are betting that smokers who are admitted for surgery may decide it's time to quit. They've developed the Facilitating Abstinence in Smokers of Tobacco (FAST) project, funded by the National Cancer Institute of Canada. Some 400 patients will participate in the randomized trial, which differs from other smoking-cessation programs in that patients in the experimental group will be encouraged to fast from tobacco for 28 hours prior to surgery.

Studies have shown that fasting for even 24 hours offers postoperative benefits such as improved cardiac function and healing in orthopedic patients. "Most of these patients anticipate being told to stop smoking [by hospital staff] and are surprised when they are not," says Pamela Rutner, assistant professor of nursing at UBC and the study's principal investigator.

The patients are also counselled on stress-management techniques and told about postoperative benefits such as increased blood oxygenation. They receive stop-smoking kits containing nicotine gum and written material about dealing with cravings, plus a toll-free phone number. Patients are seen again before leaving hospital and are then followed up for 4 months. The control group of patients receives normal treatment, without intervention. "Some surgeons will advise patients to quit, but not give them any assistance," says Rutner.

Although most surgeons support the project, she says some think that discussing smoking before surgery is paternalistic and judgemental. "We are saying that people welcome having a discussion with a health professional because they are often looking for support. As long as it is done in a supportive way, there is nothing inappropriate about broaching the subject." — Heather Kent, Vancouver

[Contents]

The news that researchers had sequenced the first human chromosome (22) marks the first milestone in the Human Genome Project (HGP), which is now racing to map the entire genome (Nature 1999;402:489-95). The announcement, made Dec. 2, may seem esoteric to physicians in everyday clinical practice, but it is a portent of the genetic knowledge that is going to transform medicine. Ultimately, human genome sequencing will allow physicians to concentrate on prevention instead of focusing on treatment.

The whole chromosome 22 picture (generated dynamically from database)

"The 21st century will be the era of genetic medicine," says Dr. Richard Bruskiewich, a Canadian medical geneticist working at the Sanger Centre in Cambridge, UK, where a third of the international HGP sequencing research is being done. "Sequencing now allows us to 'look under the hood' to identify all the components of the biological system, and hence their interactions with each other and the environment." Bruskiewich, who specializes in bioinformatics, the computational analysis of biological systems, is a coauthor of the Nature paper.

Chromosome 22, the second smallest human chromosome, is thought to be associated with at least 27 human disorders (see sidebar); causative genes in 8 of them — including schizophrenia — remain to be discovered.

By summer, the draft sequence of about 90% of the entire human genome is slated to be finished; refining the draft sequences into a finished reference sequence and sequencing the remaining 10% — the most difficult — will take another 2 to 3 years. The full sequence will identify the 200 000 to 300 000 proteins that direct the formation of a human being.

The 5-year-old HGP involves hundreds of researchers, mainly in 5 sequencing centres in the US and England but also in smaller centres in Europe, Japan and China. Canada will soon play a larger role with the start-up of the BC Cancer Agency's Vancouver Millennium Genome Sequence Centre.

In the HGP's clone-by-clone sequencing strategy, each DNA fragment is cloned and propagated by inserting it into the genome of a bacterial artificial chromosome (BAC) or a bacteriophage P1 artificial chromosome (PAC). Each BAC or PAC is 40 000 to 400 000 base pairs long, and these base pairs are then sequenced — or rearranged in the order in which they occur on the chromosome. During the mapping of chromosome 22, researchers sifted through 33.4 million base pairs of DNA and identified 545 genes, which contain instructions on creating specific proteins. The sequence is not quite complete; for technical reasons there are 11 gaps.

Although the number and location of the vast majority of genes are the same, sequence variation — for example, single nucleotide polymorphisms (SNPs) — give humans individual characteristics and genetic disorders, including predisposition to various diseases. Once researchers know which genetic variations of the estimated 3 million SNPs are involved in a particular disorder such as diabetes, heart disease or stroke, they can warn people who are at risk to avoid environmental triggers. Prevention, rather than treatment, will become the cornerstone of modern medicine. Information from the human genome sequence may also eventually allow researchers to predict and correct some developmental disorders.

"A complete patient history will eventually include a characterization of the patient's genotype that contributes to disease susceptibility and modulates the patient's response to therapies," says Bruskiewich. "The biggest challenge lying ahead for physicians is how to integrate this overwhelming body of new genetic knowledge effectively into daily practice. I would like to know what physicians think they need to achieve this task."

Geneticist Heather McDermid, who led the only Canadian research team contributing to the Nature paper, believes that "this is the start of a big boom in genetic research." McDermid, along with a technician and graduate student at the University of Alberta, mapped the cat-eye syndrome region, associated with a genetic duplication, and the 22q13 deletion syndrome region, associated with a genetic deletion. Cat-eye syndrome can lead to heart, eye, kidney and facial defects, anal atresia and mild mental retardation. The deletion syndrome causes mental retardation and loss of expressive speech.

The HGP is 1 of 2 research groups racing to complete the sequencing of the human genome. Celera Genomics Systems, a private company in Rockville, Md., started sequencing in September using the "whole genome shotgun" method. With this approach, researchers shatter the entire genome into fragments and read them simultaneously by feeding them into a supercomputer. Aside from methodology, the other major difference between the 2 groups is that Celera sells its information, while the HGP presents all its findings free to the public www.ncbi.nlm.nih.gov/genemap99/). "The human genome is the common property of all humankind, not just of those who can afford to pay for the information," emphasizes Bruskiewich. "The imposition of any embargo upon that free exchange stifles the progress of scientific understanding." — Barbara Sibbald, CMAJ

Chromosome 22 disease list
At least 27 human disorders are known to involve chromosome 22. Other genes may also be associated with some of these disorders.
Amyotrophic lateral schlerosis,susceptibility to Breast cancer, t(11:22) associated Cat-eye syndrome Cataract, cerulean, type 2 Bernard-Soulier syndrome, type B Breakpoint cluster region (CML) Colon cancer (deletions) Deafness, autosomal dominant 17 Dermatofibrosarcoma protuberans DiGeorge syndrome Ewing's sarcoma breakpoint region 1 Glioma of brain (deletions)	Glucose-galactose malabsorption Glutathionuria Heme oxygenase-1 def. Hirschsprung disease (dominant megacolon) Hyperprolinemia type 1 Lysosomal a-N-acetylgalactosaminidase deficiency Malignant rhabdoid tumour Meningioma Mental retardation, chr. 22-associated Metachromatic leukodystrophy Myoneurogastrointestinal encephalomyopathy Neurofibromatosis, type 2 Opitz G/BBB syndrome, autosomal dominant	Ovarian cancer (deletions) Pheochromocytoma Pulmonary alveolar proteinosis (rare cases) Schizophrenia 4 Schwannomatosis Sorsby's fundus dystrophy Spinocerebellar ataxia 10 Succinylpurinemic autism Thrombofilia due to heparin cofactor 2 def. Transcobalamin 2 deficiency 22q13 deletion syndrome

[Contents]

A recently published Canadian study suggests that our health care system may not be doing enough to direct cardiac care and promotional strategies to poor patients — the people who generally need these services the most (N Engl J Med 1999;341:1359-67).

Researchers with Ontario's Institute for Clinical Evaluative Sciences found that patients living in neighbourhoods with the highest average income received coronary angiography 23% more often and had 45% shorter waiting times for treatment than patients living in the lowest-income neighbourhoods. As well, each $10 000 step up in neighbourhood median income brought with it a 10% drop in the risk that a person would die within 1 year because of acute myocardial infarction (AMI). "Our findings raise the question: Could we as a system be doing a better job in reaching patients of lower socioeconomic status with health care and preventive strategies?" says Dr. David Alter, a cardiologist with Toronto's Sunnybrook and Women's College Health Sciences Centre.

The study followed 51 591 Ontario patients admitted to hospital for an AMI between April 1994 and March 1997. Researchers defined patients' socioeconomic status according to the average incomes of the communities where they lived. All data were adjusted for age, sex, severity of illness, specialty of the attending physician and hospital characteristics.

Alter says the results are evidence of real differences in the health status of patients that appear to be related to socioeconomic status. As such, they lend weight to findings of previous studies that there are disparities between classes in the prevalence of cardiac risk factors. "Why is that? Is it genetic? Or is it related to the way we deliver services and educate the public?" Psychosocial factors such as depression and job stress are also believed to cause worse outcomes for poorer or less educated people with coronary disease, although the precise mechanisms are not fully understood. "What's needed now is a study to address and disentangle all the different factors at play here," says Alter. "I suspect that when we finally do answer the 'why' question, we'll find it's not just diet, or just lifestyle, but rather a whole multitude of factors and how they interplay." —  Greg Basky, Saskatoon

Send a letter to the editor Envoyez une lettre à la rédaction

© 2000 Canadian Medical Association or its licensors