Plague and Hemochromatosis

I tweeted this a few days back, but today everyone decided to tell me about it. So here's the latest story about the scientist with undiagnosed hemochromatosis who died of the attenuated plague virus he was working with, a year and a half ago now.

Casadaban was conducting laboratory research on the bacterium that causes the plague when he became sick. The germ was genetically weakened and considered harmless to humans. It was considered so safe, Casadaban’s work with the live plague bacteria wasn’t noted when he fell ill, according to the CDC. A professor at the university for 30 years, by all accounts he had followed the proper safety protocols, the report said.
[...] An autopsy found the researcher had a medical condition called hemochromatosis, which causes an excessive buildup of iron in the body, according to the CDC report. The disorder affects about 1 in 400 people and goes unnoticed in about half of patients.
Casadaban’s illness is important because of the way the plague bacterium had been weakened. Yersinia pestis needs iron to survive. Normally it gets this iron by stealing it from a host’s body with proteins that bind to it and help break it down. To make the bacterium harmless, scientists genetically stripped it of the proteins needed to consume iron.

It seems hemochromatosis may not be the protection against Yersinia pestis its been made out to be.

Mmm-mmm Melamine, Part MII

In the wake of the latest incident of melamine-contaminated milk in China, the Epoch Times reports that wealthy Chinese are now growing their own vegetables to ensure food safety.

In recent years, Chinese people have fought hard but ineffectively against poor food safety standards. There are simply too many tainted foods, from raw food products to cooking oil and food utensils. Waste oil, toxic chopsticks, and toxic lunch boxes can still be cleaned up, but there are three sources of poisoning which are most difficult to handle.

The three areas are: pesticides, food "additives" like melamine, and pollution.

The End of Rinderpest

Via twitter: The Food and Agricultural Organization of the UN reports the likely eradication of rinderpest, a disease of cattle.

"The disease has affected Europe, Asia and Africa for centuries and has caused widespread famine and decimated millions of animals, both domestic and wild. In the 1880s, rinderpest caused losses of up to one million head of cattle in Russia and central Europe," said Diouf.
When it entered Africa in the nineteenth century, it decimated millions of heads of livestock and wildlife and triggered widespread famine. It is estimated that in that pandemic alone, up to one-third of the human population of Ethiopia died of starvation as a result. The last known outbreak of rinderpest occurred in 2001 in Kenya.
A joint FAO/OIE announcement of global rinderpest eradication is expected in mid-2011, pending a review of final official disease status reports from a handful of countries to the World Organisation for Animal Health (OIE).

Wikipedia is already referring to rinderpest in the past tense. PlagueBlog, however, cannot help wondering whether there's still some stashed in a freezer somewhere...

New Prion Disease Discovered

Via ProMED-mail: last month ScienceDaily reported the publication of a journal article on a new spontaneous prion disease:

In 2008, Pierluigi Gambetti, MD, and Wen-Quan Zou, MD, PhD, with collaborators, reported the discovery of this novel disease, which affected patients who exhibit only one of the three types of the prion protein gene. In this follow-up study, they discovered that all three genetic groups can be affected also by this novel disease which now joins sCJD in displaying this feature. However, VPSPr is associated with an abnormal prion protein that exhibits characteristics very different from those of sCJD, as well as other prion diseases, suggesting that it may be caused by a different mechanism, perhaps more akin to other neurodegenerative diseases, such as Alzheimer's disease. This finding may exemplify, for the first time, the possibility that the prion protein affects the brain with different mechanisms.

Some claim there's a smoking cow behind this new variant.

Childhood Schizophrenia

Via twitter: the LA Times reports on a tragic case of childhood schizophrenia.

Doctors and other mental health experts don't fully understand the disease, which has no cure. Jani's extreme early onset has left them almost helpless. The rate of onset in children 13 and under is about one in 30,000 to 50,000. In a national study of 110 children, only one was diagnosed as young as age 6.
"Child-onset schizophrenia is 20 to 30 times more severe than adult-onset schizophrenia," says Dr. Nitin Gogtay, a neurologist at the National Institute of Mental Health who helps direct the children's study, the largest such study in the world on the illness.
"Ninety-five percent of the time they are awake these kids are actively hallucinating," Gogtay says. "I don't think I've seen anything more devastating in all of medicine."
For Jani's parents, the most pressing issue is where Jani should live. She has been on the UCLA psych ward -- where she was placed during an emergency -- since Jan. 16. The ward is not designed for long-term care.

Abortive Rabies

Via ProMED-mail: the CDC reports on a rabies case from last year in which the victim was intermittently symptomatic ("severe frontal headache, photophobia, emesis, neck pain, dizziness, and paresthesia of face and forearms") for two and a half weeks before rabies vaccine and immune globulin were administered. She soon appeared to recover without intensive care.

This is the first reported case in which certain clinical and serologic findings indicate abortive human rabies and in which, despite an extensive medical investigation, no alternate etiology for the illness was determined. The patient's positive serologic results offer evidence of rabies virus infection; IFA and Western blot assays indicated the presence of antibodies capable of binding to rabies virus antigens before the patient received rabies PEP. Rabies virus can crossreact serologically with other members of the Lyssavirus genus, distributed in Australia, Eurasia, and Africa (5) or, theoretically, with as yet uncharacterized rhabdoviruses. However, this patient had no history of foreign travel and no evidence of infection with KCV, the only other rhabdovirus associated with bats in North America.
[...]
Certain other clinical and laboratory findings also support a diagnosis of abortive rabies in the patient described in this report. First, the onset of acute encephalopathy approximately 2 months after exposure to bats is compatible with documented incubation periods after rabies virus exposure. Second, central nervous system (CNS) findings (e.g., fever, photophobia, emesis, neck pain, dizziness, paresthesia, limitation of visual field, and altered behavior with agitation and combativeness) are compatible with clinical aspects of rabies. Although this patient did not have classic symptoms such as laryngeal spasms (manifested as hydrophobia) or autonomic instability, the lack of such symptoms has been documented in other rabies patients (1,2,6). Finally, despite an extensive medical workup, no alternate infectious etiology was identified for the patient's neurologic symptoms, increased intracranial pressure, and CSF pleocytosis.

I should note that, after her apparent recovery, the patient returned to the hospital two more times complaining of headaches, and afterwards went missing. ("The current clinical status of the patient or [sic] her boyfriend is unknown.") Judging from the reporting state, the patient was probably in the country illegally. PlagueBlog would not jump to the conclusion that she is still here, or still alive.

Mumps in NYC Update

Via ProMED-mail: the CDC reports that the mumps outbreak in New York/New Jersey has reached 1,521 reported cases, with no apparent end in sight. They also speculate about possible demographic causes of the outbreak in a relatively well-vaccinated community:

Like the mumps outbreaks that occurred in 2006 (2), much of the current outbreak is occurring in congregate settings, where prolonged, close contact among persons might be facilitating transmission. Within the affected religious community, cases have occurred predominantly among school-aged boys, who attend separate schools from girls. The higher rate among boys might be a result of the additional hours that boys in this community spend in school compared with girls, including long periods in large study halls, often face-to-face with a study partner.
In addition, transmission in the community overall might be facilitated by relatively large household sizes. According to the 2000 U.S. Census, the mean household size in one of the affected communities was 5.7, compared with a mean U.S. household size of 2.6. The limited transmission to persons outside the community might be a result of the relatively less interpersonal contact between persons inside and outside the community.
Although the school settings and large household sizes might be promoting transmission, the high vaccination coverage in the affected community likely is limiting the size of the outbreak. In addition, high vaccination coverage in surrounding communities is the most plausible reason that the few cases outside of the affected community have not caused other outbreaks.

PlagueBlog recommends letting the boys out for recess more frequently and sending them home earlier, poor things.