Clinical facts and curios

Clinical facts and curios

Clinical Facts and Curios ~ You and your family are off to a well-deserved Caribbean vacation. As you cruise the Internet to find an update on the is...

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Clinical Facts and Curios ~

You and your family are off to a well-deserved Caribbean vacation. As you cruise the Internet to find an update on the islands, you notice a forecast that there will be a considerable increase in the number of jellyfish stings this year. Should you bone up on what the best treatment is? What will you learn ?

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Of the true jellyfish, only Pelagia noctiluca (nightlight jellyfish), Chrysaora (compass jellyfish), and Cyanea species usually sting. Of course, we all worry about the hydrozoan Physalia physalis, better known as the Portuguese man- of-war. True jellyfish have round bells, whereas the Portuguese man-of-war is recognized by its boat-shaped, blue or pinkish float and its stings, which are characterized by interrupted wheals. So what is the harm of jellyfish? The toxins are complex, species-specific mixtures of peptides. Pain is the most frequent symptom, one that may cause a swimmer to get into significant difficulty. Wheals occur early and occasionally progress to vesicles or ulcers. Severe local neuropathies and vascular effects may occur. What do you do? Immediate management is to get out of the water. Unless immediate resuscitation is needed, it is important to prevent further stings from adherent but undischarged nematocysts. Vinegar inactivates the nematocysts from many species. Remaining tentacles may be picked off, but rubbing should be avoided. Cold packs may give local relief, and oral analgesia is appropriate. Some victims may require epinephrine. If you get bitten by a jellyfish, think of yourself as a salad. Find the biggest bathtub you can, fill it with vinegar, and jump in. (Levine DF. B MJ 1996;313:1061).

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What virus is found in 60% of mesotheliomas, in 50% of osteosarcomas, in some brain cancers, and was a contaminant in some batches of polio vaccine in the late 1950s?

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The simian virus 40 (SV40). Although there is much worry about a link between SV40 and human disease, there is no proof. After 2 days of heated discussions about new data, investigators at the National Institutes of Health reached a conclusion that was reached almost 30 years ago: SV40, particularly that found in the original polio vaccines, is not a public health threat. Nonetheless, it would be fair to say that monkey viruses are for monkeys, not for humans, and I will keep an eye out for data about this interesting, several-decades-long story. (Editorial comment. Science 1997;275:748.)

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Just how many genes are there ?

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More than a century after Austrian monk Gregor Mendel first detailed how genetic material was passed from one generation to the next, researchers still do not know how many genes we walk around with. The essential problem is that our genes are hidden in a haystack of apparently meaningless genetic information. Only about 3 % of the 3 billion individual units known as bases that make up DNA actually code for proteins, which is the simplest definition of a gene. Until the

Curr Probl Pediatr 1997;27:285-90 Copyright 9 1997 by Mosby-Year Book, Inc. 0045-9380/97/$6.00 + .10 5 3 / 1 / 8 2 8 4 3

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international Human Genome Project is completed in 2003, we will not know for sure the total number of genes in human chromosomes. This does not lessen the certainty of many scientists who say they already know the answer. At the low end is Sydney Brenner, who launched the field of nematode genetics. Brenner says we have exactly 60,000 genes. The reason he predicts that we will find that many genes is that he has studied the genome of the Japanese puffer fish and found one specific gene for every 6000 bases. The puffer fish has 360 million bases, yielding 60,000 total genes. Brenner assumes that this number would be true across almost all species. Others say that this number is too low, because already we have identified more than 43,000 unique genes, we are still counting, and the curve of acquisition of new genes has not yet flattened. Your bet is as good as anybody else' s about how many human genes there are. If you have invested in the genome project, chances are that you hope there are a lot more than 60,000. When the genome project is done, genes will merely be one more commodity. The more of them there are, the more the commodity. If you do not believe this, see the interesting editorial on this topic in the journal Science (Editorial comment. Science 1997;275:769).

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A couple comes to see you for a prenatal visit. Everything seems fine, but the father asks you a question. As soon as his Wife became pregnant, he decided to stop smoking to prevent any deleterious effects on the pregnancy or on the health of the child to be born. His wife has never smoked. Does paternal cigarette smoking before conception increase the risk of childhood cancer among offspring of nonsmoking mothers? Where would you have to go to sort out such a possibility?

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As you may suspect, the relation of paternal cigarette smoking with childhood cancer has not been extensively evaluated. Most studies attempting to relate the cause of childhood cancer to cigarette smoking have focused primarily on the effect of maternal smoking. One of the few places you can seek information of this sort is the People's Republic of China. Investigators in Cheng Hi, where the prevalence of smoking is extremely high among men but almost unheard of among women, were able to test the hypothesis that paternal preconception smoking might be related to an elevated risk of childhood cancers. They found that there is a link, particularly for acute leukemia and lymphoma. A father who smokes before conceiving has a child with a fourfold increased risk of acute lymphatic leukemia, a 4.5-fold increased risk of lymphoma, a threefold increased risk of a brain tumor, and a twofold increased risk for a variety of other cancers combined. These risks appeared to be independent of the effect of the passive smoking by pregnant women. To say this differently, cigarette smoking does something to sperm cells. Presumably, it produces an oxidative damage to DNA. All of this proves that it is never too early to expect smoke to do damage and never too late to think about stopping.

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What is the prevalence of mental retardation among persons between 60 and 65 years of age ? Are there pockets of high and low prevalence ?

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In 1993, when the last estimates were done, approximately 1.5 million persons between the ages of 60 and 64 in the United States had mental retardation, for an overall rate of 7.6 cases per 1000 persons. Strangely, state-specific rates vary approximately fivefold. The lowest prevalence of mental retardation is found in Alaska (3 cases per 100,000 persons). The state with the highest prevalence of mental retardation is West Virginia (16.9 cases per 1000 persons). The 10 states with the highest overall rates of mental retardation are contiguous and are located in east south central (Alabama, Kentucky, Mississippi, and Tennessee), south Atlantic (West Virginia, North Carolina, and South Carolina), west south central (Arkansas and Louisiana), and east north central (Ohio) regions. The states with the lowest rates are found in the Pacific and Mountain regions of the United States.

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Although Jeff Foxworthy probably would be willing to venture a guess about why certain parts of the United States vary from other parts of the United States with respect to the prevalence of this disability, the Centers for Disease Control and others have yet to have pinpoint why mental retardation varies so widely across the land (Centers for Disease Control. MMWR Morb Mortal Wkly Rep 1995;45:61).

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During World War II, a large number of U.S. military recruits were rejected because of a problem that is not nearly as common today as it was 50 years ago. Name the problem.

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Missing teeth. During World War II, almost 9% of U.S. military recruits were rejected because they did not meet the standard of six opposing teeth in each jaw. There were a lot of missing teeth in the average young adult a generation or two ago. Today, the average young person has only one or two cavities by the time he or she graduates from high school. This does not mean that there is not a problem with dental health among our children, because many children have a high concentration of caries, with others having relatively few or none. To learn more about how dental sealants can protect kids, see the terrific review on this topic by Siegal (Siegal MD, et al. Public Health Rep 1997;112:9). Do readers of "Clinical Facts and Curios" know why the government thought it was important to have teeth in World War II?

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A 5-year-old child comes to your office with crampy abdominal pain and diarrhea. Fecal leukocytes are detected, and you suspect a bacterial gastroenteritis. No one in the family is ill other than this child. The child has not been in day care. The only thing he eats that is different from the rest of the family is beef jerky, which the youngster has recently taken a liking to. Is it possible that beef jerky can transmit a bacterial organism which causes gastroenteritis ?

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You bet. Over the years, there have been a number of reports of outbreaks of salmonellosis associated with beef jerky. One of the most recent of these was in New Mexico in 1995 (Centers for Disease Control. MMWR Morb Mortal Wkly Rep 1995;44:785). Although beef jerky and other processed meats are generally ready to eat and pathogen free, if beef jerky is not prepared properly, it can readily transmit Salmonella organisms. Conditions recommended for the prevention of bacterial growth during jerky production include rapid drying at high temperatures (i.e., initial drying temperature >155~ [68.3~ for 4 hours and then >140~ [60~ for an additional 4 hours) and decreased atmospheric water content. If you do not want to be left high and dry, make sure your beef jerky has been dried at high!

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You get an emergency call from your local water treatment plant. An employee there brought his 4-year-old along to work with him, and he just found the youngster sitting in a pile of dry fluoride, used to fluoridate the town- -s local water supply. It appears that some of the fluoride has been ingested, but the amount is unknown. What would you recommend as the initial treatment ?

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This scenario has actually happened. If less than 5.0 mg of fluoride ion has been ingested per kilogram of body weight, calcium (i.e., milk) should be given orally to relieve gastrointestinal symptoms. At this low fluoride exposure, observation is all that is needed. Inducement of vomiting is unnecessary. If the amount ingested is unknown or is more than 5.0 mg of fluoride ion per kilogram of body weight and if the child is conscious, vomiting should be induced. A full glass of milk or any other source of soluble calcium, such as a 5% calcium gluconate or calcium lactate solution, must be given immediately. The child should then be taken to the hospital as quickly as possible. Accidental ingestion of dry fluoride is common around places that have large quantities of fluoride. Such places should keep cans of evaporated milk available at all times for emergency treatment.

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Water treatment plants know this, and must have this antidote available, Milk is not good for too terribly much, but when it comes to fluoride overdoses, milk of any kind is the tincture needed.

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You are a big barbecue person. Why is it always good to drink a beer with your barbecue?

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Starting in the 1970s, a number of research groups identified substances in charred meats and fish as carcinogens. Trp-P-2 is one of a class of potent carcinogens known as heterocyclic amines that are produced by burning fish, meat, and tobacco leaves, among other things. As little as 0.1 ml, about two drops, of beer completely ablates the carcinogenic effects of Trp-P-2 in the laboratory (Editorial comment. Science 1996;274:1309). Aahhh, nothing like a good char-broiled steak and a draft of beer! Maybe soon somebody will figure out a way of counteracting the effects of the after dinner cigar. Perhaps a little sherry?

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Three teenage boys present simultaneously to your hospital's emergency room. They have similar findings. The blood pressure is moderately low, the heart rate is not elevated, and the electrocardiogram in one shows a right bundle branch block pattern. All three develop bradycardia and then a series of life-threatening arrhythmias. Toxicologic screening shows evidence of digoxin in their blood, well above therapeutic levels. The only thing these youngsters had in common was the fact that, approximately 15 hours before, all had visited a trendy New York club in which aphrodisiacs were freely sold. What do you suspect is going on?

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You do not have to go to a trendy club to get aphrodisiacs. In New York City, for example, the product that caused these three patients' problems is sold as an aphrodisiac in grocery stores, in smoke shops, and by street Vendors. The "trade" name for commonly sold aphrodisiacs in the Big Apple goes by the name "stone," "love stone," "black stone," and "hard rock." These aphrodisiacs contain bufadienolides, which are cardioactive steroids. These steroids adversely affect the myocardium, and the most life-threatening manifestations of toxicity include arrhythmias, ventricular ectopy, sinus bradycardia, atrial arrhythmias, and hyperkalemia. Similar cardiac steroids are found in other nontraditional therapies such as Chan su and teas made from oleander. The three patients described are real. Two of the three died. All three had swallowed aphrodisiacs that were intended for topical use. The reasons why these patients all had detectible digoxin in their blood is because bufadienolides act as a digitalis compound and is measurable as such. Treatment of such an aphrodisiac overdose is with 10 vials of Digibind (Centers for Disease Control. MMWR Morb Mortal Wkly Rep 1995;44:853).

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A 3-week-old boy is admitted to the hospital because of a febrile episode. The examination initially shows no focus of infection. Initial laboratory results were unremarkable. Because of his age, broad-spectrum antibiotics are given. The next day, this infant is spiking even higher temperatures. He now has splenomegaly. His hemoglobin concentration has fallen precipitously. The family history is negative. The infant's mother, a native of Honduras, has been in the United States for approximately 11 months. What diagnosis should you consider? ~

Malaria. The case of this infant is real. This baby' s mother had been hospitalized at 37 weeks' gestation because of anemia and thrombocytopenia, at which time a diagnosis of Plasmodium vivax infection was made. The infant was born before the mother could receive treatment. The baby was not treated because the infant's blood smear was negative for malarial parasites. Some weeks later, when the baby presented with fever, the peripheral smear was positive. Cases of neonatal malaria are reported in the United States each year. Although the total number of cases of malaria in this country have decreased in recent years, as many as 1000 still occur annually,

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a small percentage of which manifest in very early infancy. (Centers for Disease Control. M M W R Morb Mortal Wkly Rep 1995;44:11).

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A 15-year-old pregnant girl comes to your emergency room with a complaint of "heavy eyelids." She is a known drug addict who reports skin popping black tar heroin. An upper respiratory tract infection is diagnosed, and she is released, only to return 2 days later with difficulty swallowing and speaking. She develops ophthalmoplegia and a profound, symmetric, proximal paralysis of her extremities, affecting her arms more than her legs. The Tensilon test result is negative. Findings of a lumbar puncture are unremarkable. Nonetheless, Guillain-Barrd syndrome is suspected, and treatment is begun with intravenous gamma globulin. No improvement occurs. Electrolyte levels are normal. What have you missed?

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What you have missed is the possibility of wound botulism, first described in association with traumatic injury, but now increasingly being reported among drug addicts, first in New York City and now elsewhere (Weber JT, et al. Clin Infect Dis 1993;16:635). Many of these cases are associated with the use of black tar heroin. If you are not familiar with black tar heroin, it is dark and gummy. Its use is increasing and recently has supplanted traditional forms of heroin in certain parts of the country, particularly the West Coast. Its use seems to be associated with cases of wound botulism. Skin popping of heroin is common among chronic users who are unable or reluctant to inject the drug intravenously. Unlike the toxin, which is inactivated by heat, spores of Clostridium botulinum--which could be in the heroin or in the liquid used to dissolve heroin--are not destroyed by heating the heroin and liquid mixture. Spores injected into subcutaneous tissue can germinate and produce toxin. Botulinum infection should be included in the differential diagnosis of anyone who develops profound weakness. Wound botulism requires a meticulous physical examination to detect the source of the problem. Treatment is accomplished with botulinal antitoxin, which can be given during pregnancy. The next time you see a weak teenager, consider the possibility of wound botulism related to substance abuse.

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Which state among the contiguous states had the highest number of cases of malaria during 1992 ?

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That year is nothing special, but it is one for which the Centers for Disease Control reported complete malaria prevalence and case data. Curiously, the state with the highest number of reported cases was not one along the Gulf Coast, but a southern state a bit farther north. It was North Carolina, with 85 confirmed cases of malaria. Maine, Rhode Island, North Dakota, Wyoming, Idaho, and Alaska had just one case each. No cases were reported in just one state: Montana. Of the total of almost 1000 malaria cases that were reported during 1992, only seven cases were acquired within the United States. Four of these had been acquired congenitally, and three had been acquired through blood transfusions. Approximately equal numbers of malaria cases were seen in U.S. civilians and foreign civilians. If y' all want to see a case of malaria, come visit down this way here in Chapel Hill. Chances are you will run across a case or two (Centers for Disease Control. M M W R Morb Mortal Wkly Rep 1995;44:1).

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You and your family have never taken time off to explore the southern half of this Western Hemisphere. On a hunch, you decide to look up the statistics related to patterns of homicide in major cities south of the bordel; just to be certain your family will be safe while they are touring. What city in the Western Hemisphere has experienced a fivefold increase in homicide rates in the last decade ?

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There is one city south of us where your chance of being murdered is about 1 in 10,000 yearly. It is Call, Columbia (1995 population of 1.8 million), the city with the highest murder rate in the Western Hemisphere. It is not difficult to figure out why the homicide rate in Cali is so high. In the 15-month period from January 1993 through May 1994, there were 2700 homicides. For 2500 of these homicides, no suspect was charged with murder. All too often we complain about the streets of Philadelphia or San Francisco or New York because of the urban violence, including drive-by shootings and gang "get-me-backs," but such violence does not come close to what occurs in certain parts of South America. Although we have little to brag about, the crude homicide rate in our largest cities (>1 million) is one third that of Cali and seems to be dropping (Centers for Disease Control, MMWR Morb Mortal Wkly Rep 1995;44:734). That backyard swing is starting to look pretty good, isn't it? James A. Stockman III, MD President, American Board of Pediatrics Professor of Clinical Pediatrics, University of North Carolina School of Medicine Consultant Professor, Duke University Medical Center

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