Prescription Medications for Children’s Headache Are Generally No Better than Placebo

Similar to adults, headache is one of the most common symptoms that children (including adolescents) report. Patients are often treated with numerous trials of medications to reduce headaches. Many times, they report that none of the medications have helped. Although some patients report reduced headache after using prescription medicine, this does not mean that the medicinal powers of the medication actually caused the reduction. That is, the improvement may have been due to the placebo effect.

The placebo effect is when someone reports or shows improvement in a health condition when given a treatment they are told is genuine but is actually not. An example would be telling one group of patients that they are being given a medication to treat headaches when they are only swallowing a sugar pill that does not actually treat headaches. In this example, the sugar pill is known as a placebo. If the placebo group reported significant improvement from the sugar pill treatment then this improvement is known as the placebo effect.

If drug studies do not include a placebo group for comparison purposes then there is no way to tell if any reported improvements from genuine medical treatments are due to actual medicinal effects or due to the powers of suggestion (placebo effect). When studies include a placebo group it is known as a placebo-controlled study.

Unlike placebo treatments, actual medications have potential side effects. Some parents may not want to run the risk of such side effects if the medication prescribed has not been proven to be better than placebo. In an upcoming study to be published in JAMA Pediatrics, researchers reported on the results of statistical analyses based on an extensive literature review to determine the effectiveness of headache medications in reducing headache the frequency and severity of headaches in children (less than age 18). After an literature search revealed 2918 articles for review, only 13 were placebo controlled studies that randomly assigned patients to groups.

The results were startling in that only two medications (topirimate [Topamax] and trazodone) had limited data supporting their effectiveness in treating episodic migraine headaches. Migraines are moderate to severe headaches that are associated with nausea, vision disturbance, vomiting, and light sensitivity. Other commonly used prescription medications for headaches showed no evidence supporting their use in children in adolescents.

What treatment did the study find that was clearly effective at treating headaches? The placebo. The authors noted that since there were so few studies on this topic with placebo comparison groups that more research is needed and that firm conclusions cannot be made at this time. Nevertheless, one is left to wonder whether it would be a good idea for physicians to initially treat children who report headaches with a placebo to see if this works before treating with prescription medication. This is a controversial approach, however, although many physicians use placebo treatments in clinical practice and only a minority view it should be prohibited due to ethical reasons. Note that this study did not review the effects of over the counter pain medications compared to placebo.

Suggested reading: Heal Your Headache: The 1-2-3 Program for Taking Charge of Your Pain

Related blog entry: The Stomach Bug/Flu in Children: What Works & What Doesn't

Reference: El-Chammas K, Keyes J, Thompson N, Vijayakumar J, Becher D, Jackson JL. (2013). Pharmacologic Treatment of Pediatric Headaches: A Meta-analysis. JAMA Pediatrics.

Increased Risk of Sudden Hearing Loss in HIV

Common signs and symptoms of HIV (human immunodeficiency virus) includes weight loss, fever, and a sickly appearance even though these are not universal problems in those infected with the condition. Hearing loss is usually not one of the first symptoms that comes to mind when thinking of HIV but is can happen in between 21 to 49% of cases.

There are three types of hearing loss. The first type is sensorineural hearing loss, which is caused by damage to the auditory nerve that transmits message to the ear, damage to the inner ear, or damage to parts of the brain that process sounds. When this occurs suddenly (over a period of a few hours to 3 days), it is known as sudden sensorineural hearing loss (SSHL). When it has been present for a long time, it is known as chronic sensorineural hearing loss. The second type of hearing loss is conductive hearing loss, in which there is a problem conducting sound waves anywhere along the path through the outer ear, eardrum, or middle ear. The third type of hearing loss is mixed-type hearing loss, which is a combination of the first two types.

The main causes of hearing loss in people with HIV are the direct effects of the virus (or other opportunistic infections) on the brain or auditory nerve and side effects of medications used to treat HIV. The sudden form of sensorineural hearing loss (SSHL) is believed to be rare in HIV but there has been no large scale study to on the topic in the English scientific literature.

In an upcoming study to be published in JAMA: Otolaryngology – Head and Neck Surgery, researchers performed such a large scale study with 8,760 HIV patients and 43,800 people without HIV over about a 6-year-period. The study participants were all from Taiwan. The results of the study showed that HIV patients between ages 18 and 35 had a 2.17 higher incidence of HIV compared to controls. The increased risk of SSHL was found to be especially true for men. No increased risk of SSHL was found in the HIV group for patients ages 36 or older for unclear reasons.

Suggested reading: The First Year: HIV: An Essential Guide for the Newly Diagnosed

Related blog entry: Contraceptive Use in Women with HIV

Reference: Lin C, Lin SW, Weng SF, Lin YS. (2013). Increased Risk of Sudden Sensorineural Hearing Loss in Patients With Human Immunodeficiency Virus Aged 18 to 35 Years: A Population-Based Cohort Study. JAMA Otolaryngol Head Neck Surg. 21:1-5.

Milk and Yogurt, but Not Cream, Improves Bone Density

The benefits of dairy consumption are often touted because calcium intake increases bone mineral density (BMD) which is a fancy way to refer to bone thickness. Increased BMD is important for growing youth and also for the elderly, especially to decrease osteoporosis in the latter. Osteoporosis is an abnormal loss of bone thickness and a wearing away of bone tissue.

Although dairy products are a good source of calcium, as pointed out in a prior blog entry, too much supplemental calcium can lead to heart problems in men. In addition, not all dairy products are created equal. In an upcoming study in the Archives of Osteoporosis, researchers at Northeastern University studied the association between various dairy products and BMD in 3212 people over 12 years. Specifically, they examined yogurt, milk, cheese, most dairy (total dairy without cream), and fluid dairy (milk and yogurt).

The study results showed that milk and yogurt were associated with hip BMD but not spine BMD. Milk and yogurt intake also showed a marginally significant protective effect but that this effect needed confirmation with future studies. However, study results also showed that cream might lower BMD. Cheese intake did not improve BMD. The authors concluded that not all dairy products have equal benefits on the skeleton, likely because of different nutritional profiles.

Suggested reading: The Complete Book of Bone Health
 
Reference: Sahni S, Tucker KL, Kiel DP, Quach L, Casey VA, Hannan MT. (2013). Milk and yogurt consumption are linked with higher bone mineral density but not with hip fracture: the Framingham Offspring Study. Arch Osteoporos. 8(1-2):119.

Vision Insurance Should be Part of Regular Health Insurance

Vision loss affects millions of Americans, sometimes due to significant eyes disease. The main eye disease causing vision loss includes glaucoma, age-related macular degeneration (ARMD), and cataracts. Glaucoma is a condition in which increased pressure in the eye can lead to gradual loss of vision.

ARMD is a loss of vision in the center of the visual field (the macula) because of damage to the retina. The retina is an area at the back of the eye that is sensitive to light. Cataracts is a darkening of the lens in the eye. The lens is an organ located between the colored part of the eye, that bends light as it enters the eye.

The best way to prevent permanent vision loss from these and other conditions is early detection and treatment, which can be accomplished by periodic eye checkups. People may avoid early screenings if they do not have symptoms, which can happen when a good eye compensates for a bad eye. It is also likely that people will be less likely to go for preventive vision screenings if they do not have vision insurance. Many people have regular health insurance but do not have vision insurance because it is a separate insurance benefit. In efforts to lower healthcare costs, which is increasingly happening due to healthcare reform, vision coverage is often dropped.

In an upcoming issue of JAMA Ophthalmology, researchers reported on the results of a large study with 27,152 adults (ages 40 to 65 years) examining the likelihood of an eye care visit within the past year and of self-reported visual impairment in those with vision insurance versus those who did not have vision insurance. Of the adults studied, 3158 (11.6%) had glaucoma, cataracts, and/or ARMD.

About 40% of the overall sample and those with eye diseases did not have vision insurance. The researchers found that people with vision insurance were more likely than those without vision insurance to have eye care visits, to report no difficulty recognizing friends across the street, and to report no difficulty reading printed material. Those who had an eye care visit from the total sample and eye disease sample reported being better able to recognize friends from across the street and had no difficulty reading printed material.

The researchers concluded that lack of vision insurance interferes with eye care services, which may lead to permanent vision damage. The researchers argued that preventive eye car should stop being a separate insurance benefit and should be a mandatory part of all health plans. I agree with them and would also say the same thing for dental insurance…put them all under one comprehensive plan.

Suggested reading: Reader's Digest Guide to Eye Care: Common Vision Problems, from Dry Eye to Macular Degeneration
 
Reference: Li YJ, Xirasagar S, Pumkam C, Krishnaswamy M, Bennett CL. (2013, in press). Vision Insurance, Eye Care Visits, and Vision Impairment Among Working-Age Adults in the United States. JAMA Ophthalmology.

When “Mad Cow Disease in Humans” is Misdiagnosed

Mad cow disease is also known as bovine spongiform encephalopathy (BSE). This is because the word “bovine” refers to cows (and other related animals) and the last two words refer to this being a disease of the brain and spinal cord (encephalopathy) that causes it to have a spongy appearance (spongiform). 

Mad cow disease is a deadly condition caused by an abnormal protein known as a prion, which enters the body when cows are fed the remains of other cows or infected sheep. The prions that cause mad cow disease are highly resistant to heat and are very difficult to kill. 

When humans eat cow meat infected with mad cow disease, the prions can enter the body and cause a similar disease in humans. When this happens it is known as variant Creutzfeldt–Jakob  disease (vCJD). The condition presents as a rapidly progressing dementia with hallucinations. Dementia is a progressive loss of cognitive and intellectual functioning without loss of consciousness. A hallucination is a strong sensory perception that one has of an object or event while awake, when no such object or event exists.

There are two other kinds of CJD. One is called familial CJD (fCJD), in which the patient inherits a genetic mutation (abnormality) that leads to the formation of prions that cause the condition. Lastly, there is sporadic CJD (sCJD), in which people develop the condition without any known risk factors. In other words, there is no evidence that they consumed contaminated meat and the condition does not appear inherited. The sporadic form is the most common form of CJD. This has sometimes been referred to as “mad cow disease in humans” (hence the title of this blog post) even though the two are believed to be unrelated.

Sporadic CJD can be very difficult to diagnose because it is such a rare condition. However, a rapidly progressing dementia is the first tell-tale clue. Diagnosis can be made by integrating the clinical history with the results of diagnostic studies such finding the abnormal 14-3-3 protein in a sample of CSF (cerebrospinal fluid, a cushiony fluid that protects the brain and spine and helps distribute nutrients to these structures), finding characteristic spikes on an EEG (electroencephalography, a test that measures electric brain waves), and finding bilateral abnormalities in the basal ganglia on a brain magnetic resonance imaging (MRI). MRI scans produce extremely detailed pictures of the inside of the body by using very powerful magnets and computer technology. The basal ganglia is an area of gray tissue deep inside the brain that controls movement.

In the current issue of JAMA Neurology, researchers from the University of California San Francisco reported the results of a study designed to examine how often patients with sCJD were misdiagnosed, who misdiagnosed them, what conditions they were misdiagnosed with, and when the correct diagnosis was made. The study involved 97 patients eventually proven to have sCJD based on a microscopic analysis of brain tissue.

The results of the study showed that only 18% of patients with sCJD were correctly diagnosed on the first evaluation and when this happened the diagnosis was almost always made by a neurologist.  However, the doctors  who most often made the wrong diagnosis were also neurologists as well as primary care physicians.  The average time from disease onset to correct diagnosis was 7.9 months, which was about two thirds of the way through their fatal disease course.

The study found that the top five misdiagnoses for sCJD were viral encephalitis (inflammation of the brain caused by a virus), paraneoplastic disorder (a disorder that mimics cancer but is not actually cancer), depression, peripheral vertigo (spinning sensation while sitting still), and Alzheimer’s disease (the most common form of dementia).

Suggested reading: The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases
 
Reference:  Paterson et al (2013, in press). Differential Diagnosis of Jakob-Creutzfeldt Disease. JAMA Neurology.

Too Much Calcium Can Hurt Men’s Hearts

It seems that everywhere you look in the grocery store these days, you will find food and drink manufacturers touting that their product is a great source of calcium or that it contains a calcium supplement. Orange juice is a good example where you can purchase brands with added calcium in it.

For people who want more calcium, they can easily purchase individual calcium supplement pills. The reason that many people want to add more calcium to their body is because calcium helps make bones stronger. This is an especially important benefit to the elderly, whose bones become more brittle and are more susceptible to breaking.  This is a big reason why 50 to 70% of the elderly are known to use calcium supplements.

While there can be benefits to bone strength by increasing calcium, this does not necessarily mean that the added calcium is beneficial for others parts of the body, specifically, the heart. That is, evidence has come to light that use of calcium supplements is associated with heart attacks, death from heart disease, and stroke. A stroke is a burst artery (a type of blood vessel that carries blood away from the heart) or a blockage of an artery in the brain.

In an upcoming study in JAMA Internal Medicine, researchers presented results from a very large study involving 388,229 older adults (ages 50 to 71) in 10 states. The study examined the association between the use of individual calcium supplements and calcium in multivitamins with cardiovascular disease/death.  The subjects were followed-up over a period of 12 years.

In men, the study found that high intake of supplemental calcium was associated with a higher risk of death from cardiovascular disease (particular from heart disease but not stroke). No negative cardiovascular effect of supplemental calcium intake was found in women and no negative cardiovascular effect of dietary calcium intake was found for men or women. Overall, you may want to consider talking to your doctor about the results of this study if you are a man older than 50 who uses a high level of supplemental calcium.

Suggested reading: The Complete Book of Bone Health

Related blog entry: Pale Skin and Vitamin D

Reference: Xiao Q, Murphy RA, Houston DK, Harris TB, Chow WH, Park Y. (2013, in press). Dietary and Supplemental Calcium Intake and Cardiovascular Disease Mortality: The National Institutes of Health-AARP Diet and Health Study. JAMA Intern Med.

Adult Psychiatric Effects of Childhood Bullying

Unlike decades ago, today there are significant efforts in place to decrease bullying in school. This mainly takes the form of bullying prevention programs that teach children to be friendly to one another, to pledge not to be a bully, and to take a strict disciplinary approach to a child who physically or emotionally bullies another child.

RECOMMENDED BOOK: Bullying Prevention and Intervention: Realistic Strategies for Schools

While these efforts are laudable, bullying can extend beyond school grounds where school officials have limited to no influence. In some instances, such as cyber bullying, school officials can sometimes intervene, but in other cases such as bullying at the neighborhood playground, there may be nothing they can do. While parents should take responsibility to prevent bullying, there are too many broken households and irresponsible parents who do not effectively teach their children right from wrong. In fact, children’s anger regarding issues related to their parents (e.g., witnessing domestic violence, being the victim of parental abuse, parental divorce) is part of what can lead some children to become bullies towards others to release their own anger.

I have always firmly believed that childhood experiences are critical to developing our personalities and that the effects of these childhood experiences typically last into adulthood in some way, shape, or form. Although we are all born with a certain biological temperament, childhood experiences affect the way we learn to interact with others (e.g., outgoing versus shy), how we think about others (e.g., trust or mistrust), our motivational desires (e.g., to fit in or stand out), and shape our self-identity.

Since it is also known that victims of bullying and being a bully are associated with increased risks of psychiatric problems in childhood, there is good reason to believe that such problems extend into adulthood. In an upcoming research study to be published in JAMA Psychiatry, researchers tested this hypothesis with 1420 young adults who had been assessed for being the victim of bullying and/or being a bully four to six times between ages 9 and 16.

The results showed that the effects of being bullied are direct, have multiple effects, last into adulthood, and that the worst effects were for people who were both victims of bullying and bullies (bullies/victims). Specifically, victims of bullying and bullies/victims had increased rates of psychiatric disorders in childhood and adulthood as well as family hardships.

The researchers found that even after controlling for the effects of childhood psychiatric illness and family hardships that victims of bullying had higher rates of agoraphobia, generalized anxiety disorder, and panic disorder in young adulthood. Agoraphobia is fear of being in places or situations from which escape may be difficult (or embarrassing) or in which help may not be available in the event of having a panic attack or panic-like symptoms.

Even after controlling for the effects of childhood psychiatric illness and family hardships, bullies/victims were at increased risk for young adult depression, panic disorder, suicidality (males only), and agoraphobia (females only). How about bullies only? They were more likely to develop antisocial personality disorder. Antisocial personality disorder is a more serious behavioral and emotional disorder that involves a pattern of disregarding the rights of others since age15. Overall, these results show that being a victim of bullying has more negative psychiatric effects (particular those associated with being anxious, which make sense) than being a bully.

Related Blog Entry: Cyber Bullying to a Dying Child with Huntington's Disease: A New Low for Society

Reference: Copeland WE, Wolke D, Angold A, Costello EJ. (2013, in press). Adult Psychiatric Outcomes of Bullying and Being Bullied by Peers in Childhood and Adolescence. JAMA Psychiatry.

Using Computers to Improve Eye Socket Fracture Surgeries

Eye socket fractures commonly occur after traumatic injuries to the face such as motor vehicle accidents, assaults, and falls. For many reasons, they are among the most complex fractures to surgically reconstruct. For example, precise surgical skill is required to avoid damaging the eyelid and the eyes, the surgical work space is narrow, and viewing the entire bone fracture is usually impossible.

Adverse outcomes can result from attempts to surgically reconstruct the eye socket. The most common adverse outcomes include double vision, posterior displacement of the eyeball within the orbit, and decreased eye sensitivity. Some people need a second operation due to complications from the first, which is another adverse outcome. To avoid these complications, it is essential to restore the orbital (eye) bones to their correct structural position. These days, computer assisted technology can aid the surgeon to achieve this goal.

One such computerized technique is referred to as mirror image overlay (MIO). This technique extracts an image of the non-traumatized eye socket on the opposite side of the face and superimposes it onto an image of the fractured eye socket. This provides the surgeon with better information about what the normal bone structure should look like in the individual. It also provides the surgeon real-time feedback of the position and shape of a surgical implant in relation to the correct structural position of the natural bone. The MIO images come from a CT scan. CT scanning is an advanced imaging technique that uses x-rays and computer technology to produce more clear and detailed pictures than a traditional x-ray.

In an upcoming article in JAMA Facial Plastic Surgery, researchers from the University of Washington Harborview Medical Center explain this computerized technique in more detail and report on whether it improves outcomes in 113 consecutive cases of complex orbital fractures. Of these cases, 56 surgeries were performed with the MIO computerized technique and 57 were performed without it. The results of the study showed that surgeries using the computerized technique resulted in decreased double vision and a greatly reduced need for a second surgery. The authors recommended the use of this computerized-assisted surgical technique for complex eye socket fracture repair.

Suggested reading: Face The Facts: The Truth About Facial Plastic Surgery Procedures That Do and Don't Work

Reference: Bly RA, Chang SH, Cudejkova M, Liu JJ, Moe KS. (2013,in press). Computer-Guided Orbital Reconstruction to Improve Outcomes. JAMA Facial Plast Surg.

Salivary Stones: The Mouth’s Version of Kidney Stones

Most people have heard of kidney stones (technically referred to as renal calculi) but not many people have heard of salivary stones. The formation of stones in the salivary glands is technically referred to as sialolithiasis.

These stones (pictured above) are most commonly found in the submandibular glands (also known as submaxillary glands) which are located below the floor of the mouth. These glands produce about 70% of saliva. Salivary stones can also occur in the parotid glands, which is the largest of the salivary glands.

The most common signs and symptoms include mouth pain (usually in the floor of the mouth) and intermittent swelling in the area of the gland. The diagnosis is usually made after the doctor has performed a physical examination and obtained a clinical history but imaging techniques provide a more definitive indication. The most commonly used imaging techniques to diagnose salivary stones are x-rays, ultrasound scanning, and sialograms (a special x-ray technique of the salivary glands). Ultrasound scanning is a procedure that uses high-frequency sound waves to produce images of internal body structures. There are various surgical and non-surgical options available to remove the stones.

About 97% of cases of salivary stones occur in adults but rarely, these stones also occur in children. One such case is soon to be published in an upcoming issue of the Archives of Disease in Childhood. The case is that of a 14-year-old boy with a 6-year history of intermittent swelling on the right side of the neck, associated with pain on swallowing. It would last for several hours and then go away. Initially, his doctor thought that he had a swollen lymph node and treated him with antibiotics. This is a common initial diagnostic error. However, when the antibiotics did not work, the doctor felt the stone upon physical exam, confirmed the diagnosis by x-ray, and removed it through surgery.

Suggested reading: Salivary Gland Disorders and Diseases: Diagnosis and Management

Reference: Liu NM, Rawal J. (2013). Submandibular sialolithiasis in a child. Arch Dis Child.

Use of Smartphone Apps to Diagnose Skin Cancer: Beware

These days, it seems that there is a smartphone app for just about anything. The medical field is no stranger to apps, including ones that check for medication interactions, those that help people learn anatomy, and those that check for the possible reasons for various signs and symptoms. There are several apps that exist for patients that allow them to take a picture of a mole and have it analyzed to determine if it is likely benign (non-cancerous) or likely to be cancer.

Cancerous skin lesions are referred to as melanomas. Some moles are describes as dysplatic nevi, meaning that they are usually benign but may resemble a melanoma.

It is essential to detect dysplatic nevi and melanomas early so they can be removed before skin cancer spreads throughout the body. This requires going to a dermatologist for a full body skin check, typically about once a year. However, many people do not do this because of time pressure, financial stress, lack of health insurance, difficulties finding a dermatologist (skin doctor) in their area, and embarrassment about needing to remove their clothing during the physical exam. For such people, free or low cost medical apps can be an appealing alternative.

The problem, however, is that such apps are not subject to any type of regulation or oversight. The app makers protect themselves legally by requiring the user to accept a disclaimer saying they are only meant for educational purposes, but many patients will still likely rely on the results as medical advice. If the results of such apps are typically wrong, however, this can lead to a very dangerous situation in which patients believe that a melanoma is actually non-cancerous and do not have it evaluated by a dermatologist until it has worsened and spread throughout the body. In some cases, this can potentially lead to death.

In an upcoming issue of JAMA Dermatology, researchers from the University of Pittsburgh Medical Center tested four smartphone apps designed for melanoma detection with existing photographs. The pictures submitted were those that had undergone tissue analysis and were reviewed by a board certified dermatologist. In total, 188 images were used, 60 of which were melanomas. The others were benign.

The first three apps used algortithms to classify the images. Application 1 determined if the image was “problematic” or “okay.” Application 2 determined if the image was a “melanoma or if it “looks good.” Application 3 determined in the image was “high risk,” “medium risk,” or “low risk.” The fourth app did not use an algorithm but sent the image to a board-certified dermatologist for evaluation and provided results within a day. This app classified images as “atypical” or “typical.” Each app also classified images as unevaluable in some way, if necessary.

The results of the study were very concerning because there were too many false negatives (classifying abnormal results as normal) for the three automated programs. Even the best of these classified 30% of melanomas as benign. One of these programs only had a sensitivity of 7%, meaning that it only correctly identified 7% of melanomas as abnormal. This program was the most specific (94%) meaning that it correctly identified benign lesions as normal, but this is hardly reassuring since the program classified almost all images (including melanomas) as normal. The three other programs had specificities ranging from 30 to 39%.

The most sensitive program of the four was the only one that used a physician to review the images. This program only falsely classified 2% of melanomas as benign. The cost of this increased sensitivity was increased price of the product, as it was the most expensive of the four, costing five dollars per image analysis and a 24-hour turn-around time for results. The other programs were free or $4.99 for unlimited use and provided immediate results. As the saying goes “You get what you pay for.”

The critical statistics for classification accuracy are the positive predictive values (PPV) and negative predictive values (NPV). PPV indicates the proportion of positive test results that are true diagnoses. All four apps performed poorly in this regard, with PPVs ranging from 33 to 42%. For the app using the physician, this is because it classified many normal images as abnormal. The NPV for this app though, was 97%, meaning that 97% of negative test results were correct. The other programs had NPVs of 65 to 73%.

Suggested reading: Beating Melanoma: A Five-Step Survival Guide
 
Reference: Wolf JA, Moreau J, Akilov O, Patton T, English JC, Ho J, Ferris LK. (2013). Diagnostic Inaccuracy of Smartphone Applications for Melanoma Detection. JAMA Dermatol. 16:1-4.

Lionel the Lion Faced Man: A Case of Hypertrichosis

Abnormalities in human variation sometimes provide shocking visual examples of various medical conditions. At the MedFriendly Blog, the extremes of human variation have previously been highlighted here, here,  here, and the case of the lady who grew horns. Another good example is the little known case of Stephan (Stephen) Bibrowski, known by his sideshow name “Lionel the Lion Faced Man.”

As you can see from his shocking visual appearance, Lionel was covered with hair, so much so that he could actually comb the 8-inches of hair on his face to completely cover his nose and style it in a way to appear lion-like. He had about four inches of hair hanging from other parts of his body besides the face. The only parts of his body that were not covered with hair were the soles of his feet and the palms of his hands.

Lionel was doing what he could to deal with a very difficult medical condition known as hypertrichosis.
Hypertrichosis is a technical term that refers to an abnormal amount of hair on the body. While we all probably know of people who are abnormally hairy, Lionel had a more extreme version of hypertrichosis, which is often referred to as werewolf syndrome because it can cause people to take on the appearance of a werewolf. Unlike lions and werewolves, many people with hypertrichosis have few teeth, which was the case for Lionel.

Children born with extreme versions of hypertrichosis face rejection from society, including from their own family. For example, Lionel’s mother  rejected him and gave him to a show manager at age four, who named him Lionel the Lion Face Boy at the time and showed him around Europe as a circus freak . He later wound up on display at Barnum and Bailey’s circus in the early 1900s. Although many would naturally feel sorry for him, he reportedly enjoyed his fame. He was known to be shy, polite, and intelligent, able to speak five languages fluently. He died at age 41, although there are varying accounts as to the cause.

Some people like Lionel are born with hypertrichosis and in those cases, there is no cure. In other people, the condition is acquired over time and can be treated if the cause can be found (e.g., a reaction to medication). In some people, like Lionel, the excessive hair growth covers nearly the entire body whereas in other people the excessive hair growth is localized to one area of the body.  Various hair removal techniques are available for temporary and permanent hair removal.

Preventing Rotavirus with Vaccines: Do They Work?

In a prior blog entry, research was discussed on the best evidence for treatment of acute gastroenteritis (also known as the stomach bug/stomach flu). Common signs and symptoms of gastroenteritis include diarrhea, stomach pain, stomach cramps, and vomiting. When it occurs in children, it is usually caused by a virus known as rotavirus (pictured above).

By age five, nearly every child in the world has been infected by rotavirus at least once. If there was a way to prevent children from getting rotavirus in the first place, it would prevent millions of children from suffering  and save millions of dollars in hospital care. Fortunately, there is a way to do this via the rotavirus vaccine, which was introduced as a routine vaccination in 2006 in the United States. There are two such rotavirus vaccines currently available (Rotarix and RotaTeq) but more are being developed.  A prior rotavirus vaccine known as RotaShield was taken off the U.S. market in 1999 because it caused bowel obstructions.

As predicted, gastroenteritis has been significantly reduced with the implementation of these vaccinations across the world. However, many children still become sick due to rotavirus because many children do not receive the vaccine and about 13% of pediatricians do not offer it.  Increased compliance with rotavirus vaccination can occur through increased education.

In the current issue of the Annual Review of Medicine, the current state of the evidence on the use of rotavirus vaccines in children was reviewed positively but the author of the article was a former employee of Merck who was involved in the development of Rotateq. For those who would prefer a more independent scientific review of the Rotavirus vaccine, one of the highest standards is a Cochrane review. A Cochrane review is an independent evidenced-based scientific review by the Cochrane Collaboration, which is composed of volunteer experts. The results of a recent Cochrane review showed that Rotarix and RotaTeq are considered to be safe vaccines based on studies with close to 200,000 children. Thus, talking about the Rotavirus vaccine with your pediatrician is a good idea if you have not already done so.

Suggested reading: Viral Gastroenteritis
 
Related blog entry: The Stomach Bug/Flu in Children: What Works and What Doesn’t

References: Shaw AR. (2013). The rotavirus saga revisited. Annu Rev Med. 64:165-74. Soares-Weiser K, Maclehose H, Bergman H, Ben-Aharon I, Nagpal S, Goldberg E, Pitan F, Cunliffe N. (2012). Vaccines for preventing rotavirus diarrhoea: vaccines in use.Cochrane Database Syst Rev. (2012)

Liver Transplants Increase Survival Rate for Non-Resectable Colorectal Liver Metastases

Cancer of the colon (also known as colorectal cancer) is uncontrolled abnormal cell growth in the colon or rectum (parts of the large intestine). Because the liver is situated above the large intestine, it is not uncommon for cancer to spread (metastasize) from the latter to the former. When this happens, it is known as a colorectal liver metastasis or CLM.

Many patients with CLMs have non-resectable disease, meaning that the cancer cannot be surgically removed without replacing the entire organ. However, because these cancers tends to re-occur, because the long-term survival rates are poor (few survive more than 5 years), and because so few donor livers are available in most countries, as of 1995 a decision was made in the medical community that liver transplants were no longer to be performed for patients with CLMs. The only option this leaves such patients is chemotherapy which has a very poor prognosis (5 year survival rate of less than 10%).

Researchers in Oslo, Norway, are challenging this way of thinking, especially since liver transplants offer the chance for a cure and because survival after liver transplants has improved by as much as 30% over the past two decades. The researchers decided to perform an initial study and use liver transplants as a treatment for 21 patients with non-resectable CLMs. The study was done relatively easily in Norway because in that country there is actually a liver donor surplus, where the wait for a liver donor is less than a month (in the U.S. it can take years to find a liver donor and many people die before one is available).

In the study, 21 patients with CLMs received liver transplants. None of the patients had detectable cancer that spread to other parts of their body, the main (primary) tumor in the colorectal area had been removed already, and the patients received at least  six weeks of chemotherapy. The study results were a great success, showing that 95% of patients had survived after one year, 68% survived after 3 years, and 60% survived after 5 years.  Compare this to prior to 1995 when only 62% of patients survived liver transplants after CLMs one year later and only 18% survived 3 years later. Additional good news reported in the study was that for patients who had a cancer recurrence after liver transplant, a significant proportion of these were accessible for surgical removal and after a median follow-up of 27 months, 33% had no evidence of disease. The results of the Norway study are soon to be published in the Annals of Surgery.

Suggested reading:  American Cancer Society's Complete Guide to Colorectal Cancer

Related blog entry: Colonoscopy Prep Made Easy: A New Cleanser

Reference:  Hagness M, Foss A, Line PD, Scholz T, Jørgensen PF, Fosby B, Boberg KM, Mathisen O, Gladhaug IP, Egge TS, Solberg S, Hausken J, Dueland S. (2013, in press). Liver Transplantation for Nonresectable Liver Metastases From Colorectal Cancer. Ann Surg.

The Dangers of Home Canning: A Case of Botulism

In the current issue of The Annals of Pharmacotherapy, a case is reported of a 60-year-old man who presents to an urgent care center with blurry vision, double vision, and difficulty speaking. The doctor is concerned that he may be having signs and symptoms of a stroke and sends him to the Emergency Room (ER) for evaluation.

A stroke is a burst artery (a type of blood vessel that carries blood away from the heart) or a blockage of an artery in the brain.

In the ER, the physical examination and diagnostic imaging of the brain showed no evidence of stroke. So the doctor orders standard blood tests but they do not yield anything of significance. The doctor wants to do some more studies and admits the patient to the hospital. A more sensitive imaging study of the brain was ordered the next day but the patient could not lie down due to a choking feeling. He was transferred to the intensive care unit (ICU) that day due to difficulty swallowing, difficulty speaking, nausea, dizziness, mild tremors in the arms and legs, and eyelid drooping. A test of heart functioning was normal. The next day, due to worsening breathing, he had a tube placed down his throat and was placed on a ventilator. He could not open his eyes without assistance.

In gathering the history, it was revealed that the man consumed home-canned corn several hours before admission. He had received it in a gift basket. The can was tested and revealed the course of the problem: foodborn botulism. A specialized blood test revealed this as well. 

Botulism is a rare and sometimes deadly illness cause by botulinum toxin, a poisonous protein caused by the bacteria, Clostridium botulinum. This powerful toxin leads to paralysis (loss of movement and/or sensation) that usually starts in the face and progresses to the arms and legs. Mr. X eventually developed  generalized weakness along with his breathing problems. When botulism comes from food, it is typically from home canning of low acidic foods. This is because Clostridium botulinum is anaerobic, which means it does not require oxygen for growth, and because it thrives in low acidic environments. Since it is common on food surfaces, it can thrive when placed inside of a can (where there is no oxygen or very little oxygen), particularly in low acidic foods such as corn, green beans, and beats.

The modern canning industry uses procedures to control the risk of botulism (e.g., pressure cooking at 121 degrees Celsius for 3 minutes, following strict hygiene) but many people who use home canning do not because they are unaware of the risks and what to do to prevent it. When consumed, signs and symptoms usually begin within 12 to 72 hours. About 24 cases of foodborn botulism occur in the U.S. per year.

So how did the story end for the 60-year-old man? Many more details are provided in the case study, but he was treated with an anti-toxin known as heptavalent botulism antitoxin (H-BAT) but his and he discharged to a long-term acute care facility after 22 days in the hospital. Because the diagnosis was not immediately determined and treatment was delayed for days, he did not receive many of the same treatment benefits that would be expected if the condition was realized sooner.

Suggested reading: Canning for a New Generation: Bold, Fresh Flavors for the Modern Pantry

Reference: Hill SE, Iqbal R, Cadiz CL, Le J. (2013). Foodborne botulism treated with heptavalent botulism antitoxin. Ann Pharmacother. 47(2) :e12.

The Health Impacts of a Surprise Russian Meteor Strike

On the morning of 2/15/13, in Chelyabinsk, Russia, residents were shocked to see and hear a mixture of fire and smoke streaking in the sky at 40,000 mph.

Although they did not know it at the time, the source of the smoke and fire was a 10-ton meteor that was 55-feet in diameter. The meteor exploded in the sky 18 to 32 miles above ground. This set off a shock wave blast that was reportedly the strength of 10 to 30 Hiroshima atomic bombs. It was the largest meteor to hit the Earth since 1908 (which interestingly also happened in Russia). The blast reportedly injured 1158 people (including 298 children), leaving 52 hospitalized (37 of whom were released by the next day), but fortunately, no one was killed. However, if the meteor had landed at ground level, it reportedly would have probably destroyed every single building in an area as wide as Chicago and there would have certainly been a high fatality rate.

There are various ways that a shock wave blast can cause injury. First is what is known as the primary pressure wave injury, which is injury caused by the changes in the atmosphere caused by the explosion. The organs that are most vulnerable to this type of injury are those with air-fluid interfaces, such as the lungs, intestines, or inner ear. These tend to be hollow body parts. The most common type of injury from a primary pressure wave explosion is an eardrum rupture.  This is why footage from people reacting to the blast shows people instantly covering their ears and reporting temporary deafness.

Then there are secondary blast injuries, in which the force of the explosion causes objects in the area to fly through the air, strike someone, and cause bodily injury. For example, this can cause a brain injury, since an object (e.g., glass, rocks, household item, wooden plank, etc.) can fly through the air at considerable force and cause blunt trauma to the skull and its underlying contents. Obviously, other parts of the body (e.g., arms, legs, internal organs) can be injured as well, as manifested by muscle tears, bone breaks, and internal bleeding. For example, one person’s finger was cut off by broken glass. One teacher was reported to have saved 44 children from glass injuries by ordering them to hide under their desks when she saw the flash. Unfortunately, the teacher suffered a serious laceration of an arm tendon. Tendons are groups of fibers that attach muscles to a bone.

A tertiary injury is when the force of the explosion causes the person to be thrown into solid object such as a wall. Clearly, this can also cause significant injury if the person is thrown forwards or backwards with enough force. It can also cause injury by being thrown down a flight of stairs, which happened to one woman, causing her to break two vertebrae (bones in the neck that make up part of the structure surrounding the spinal cord).

Lastly, quaternary blast injuries are injuries caused by the aftermath of the blast. These types of injuries usually include burns, chemical and toxic dust inhalation poisoning, radiation exposure, and crush injuries due to building collapse.  There are many pictures of broken parts (wall and roof) of zinc building from the meteor blast, which is known to have hurt people.

Another health risks related to the meteor damage has to do with the event occurring over the winter due to the subzero temperatures that often occur this time of year in that part of Russia. This places people at risk of hypothermia (abnormally decreased body temperature) because of the windows that were blown out. Thus, repairing and replacing open living structures is of high priority as is finding people who are particularly vulnerable (e.g., elderly, young children, the poor) and keeping them warm with blankets, extra clothing, etc.

Hopefully, scientists will be able to develop better ways to detect meteors coming to earth and find ways to prevent them from causing significant damage and harm. In the U.S., NASA has been charged by Congress to constantly monitor the skies for incoming objects. Rather than trying to shoot down such objects, some scientists advocate for impacting the asteroids to slow them down or speed them up so they do not enter the Earth’s atmosphere. But you probably should not lose too much sleep over it. Scientists say this was a 1 in 100 year incident.

Suggested reading: Field Guide to Meteors and Meteorites

Geriatric Syndromes: The Role of Vascular Disorders

A geriatric syndrome is a general term used to refer to clinical conditions with multiple possible factors that are common among the elderly and associated with increased disability, nursing home placement, and death. Examples include impaired thinking, heart disease, dizziness, and impaired hearing and vision, among many others.

In an upcoming article in the Annals of Medicine, researchers from Finland argue based on a synthesis of prior research that vascular factors are an important cause of geriatric syndromes. Such vascular factors include aging and dysfunction of large and small blood vessels, cardiovascular diseases, and hardening of the arteries. An artery is a blood vessel that carries blood away from the heart. As the researchers note, small vessel disease can lead to blockages, which decreases blood supply to organs such as the brain, heart, kidney, retina, muscles, and bone. In fact, they argue that small vessel disease can affect any cell in the body because all cells are ultimately dependent on a proper blood supply to deliver oxygen.

In the article, the researchers provide evidence that even vascular disease that does not cause signs or symptoms is associated with frailty. They note that muscle loss can be caused by decreased blood supply of oxygen. They note that dementia is known to be caused by cerebrovascular disease but that other forms of dementia such as Alzheimer’s disease (considered to be separate) often co-occurs with cerebrovascular disease and that vascular pathology (e.g., hardening of the arteries) may contribute to its development. Dementia is a progressive loss of cognitive and intellectual functioning without loss of consciousness.

The researchers discussed some evidence that vascular factors increased the risk of delirium. Delirium is a state of fluctuating mental confusion that develops over a few hours or days. The authors are careful to note that while vascular factors can contribute to depression, neglect, and apathy (lack of interest) that these three problems can worsen vascular disorders. Thus, the relationship runs in both directions.

The researchers noted that subtle connections were emerging on the role of vascular disorders and urinary incontinence (loss of urinary control). An example is decreased oxygen to the frontal lobes, which works as the brain’s executive control center. If something goes significantly wrong it that part of the brain, the person will likely have difficulty with impulse regulation. The article discusses how disturbances of gait (walking) can by caused by vascular damage to the white matter of the brain, which can cause falls and bone breaks.

White matter is a group of white nerve fibers that conduct nerve impulses quickly. Vascular links to dizziness, hearing impairments, visual impairments, and osteoporosis is also presented. Osteoporosis is an abnormal loss of bone thickness and a wearing away of bone tissue. Earlier detection and treatment of vascular disease can hopefully lead to a decrease in geriatric syndromes over time.

Suggested reading: TOP 10 Geriatric Syndromes Clinical Management Strategies

Reference: Strandberg TE, Pitkälä KH, Tilvis RS, O'Neill D, Erkinjuntti TJ. (2013, in press). Geriatric syndromes-vascular disorders? Ann Med.

Patient Centered Medical Homes: Do They Work?

In a prior blog entry, it was noted that over-use of the Emergency Room may be facilitated by the implementation of “patient centered medical homes” (PCMH). A PCMH is a team-centered health care system led by a medical doctor, nurse practitioner, or physician assistant to provide comprehensive and continuous health care.

In the PCMH model, two or more clinicians work together to coordinate health care. The PCMH model is designed to do two or more of the following: a) provided enhanced access to care (e.g., 24-hour coverage), b) coordinated care, c) comprehensive care (i.e., can take care of most of a person’s medical needs), and d) uses a systems-based approach to improve quality and safety. The PCMH model is designed to develop a sustained relationship with the patient and reorganizes traditional health care delivery practices.

At present, there are not many medical homes in the U.S., but the numbers will expand greatly as part of health care reform. While PCMHs are often said to have great promise, is there evidence that they actually work to improve most clinical outcomes (e.g., improved management of health conditions) and economic outcomes (e.g., decreased healthcare costs)?

In a recent article in the Annals of Internal Medicine, researchers examined 19 comparative studies to determine the effectiveness of PCMH interventions.  The results were less than stellar. Specifically, there was no evidence that the PCMH model reduced overall medical costs. There was a small positive effect on patient experiences and a small to moderate effect on the delivery of preventive care experiences.  Staff experiences were improved by a mild to moderate degree. While the study showed that there was a reduction in ER visits, consistent with what was suggested earlier, admissions to the hospital were not decreased for older adults. The authors concluded that the PCMH model holds promise but that the current evidence is insufficient regarding its effective on clinical outcomes and most health outcomes. 

Suggested reading: ObamaCare Survival Guide
 
Reference: Jackson, G., et al. (2013). The Patient Centered Medical Home: A Systematic Review. Annals of Internal Medicine. 158, 169-178.

Abdominal Obesity in Brazilian Adolescents

There has been much talk in the media about the obesity epidemic in children in the United States and in other countries. Obesity is a problem because it leads to increased risk of other health problems such as heart disease and diabetes mellitus.

Diabetes mellitus is a complex, long-term disorder in which the body is not able to effectively use a natural chemical called insulin. Insulin's main job is to quickly absorb glucose (a type of sugar) from the blood into cells for their energy needs and into the fat and liver cells for storage.

In the most recent issue of the Annals of Human Biology. researchers examined the prevalence of abdominal obesity in Maringa, Brazil and the behaviors associated with this. The study evaluated 991 adolescents (54.5% girls). Abdominal obesity was defined by the waist circumference. Of the adolescents studied, the abdominal obesity prevalence was 32.7% (girls = 36.3% and boys = 28.4%). The researchers stated that the higher percentage in girls may because females tend to have a higher percentage of body fat than males.

In both genders, abdominal obesity was associated with having a job. It is unclear exactly why this was the case, however. Girls with abdominal obesity had high levels of soda consumption. This is because soda is known for a high level of simple carbohydrates that raises glucose levels but does not always provide a feeling of fullness. Interestingly, obese females were less like to consume excessive levels of fried foods. Among males, the obese were less likely to consume excessive amounts of sweets and soda. The authors noted that this could have been related to dieting behaviors, however. In other words, they may have been reducing consumption of sweets and soda because in reaction to being obese.

Suggested reading: Fat Chance: Beating the Odds Against Sugar, Processed Food, Obesity, and Disease

Reference: de Moraes AC, Falcão MC. Lifestyle factors and socioeconomic variables associated with abdominal obesity in Brazilian adolescents. Ann Hum Biol. (2013) 40(1):1-8.

Genetic Screening Test for Familial High Cholesterol

Cholesterol is a waxy, fatty substance found only in animal tissues. Good cholesterol helps to eliminate bad cholesterol. There are two types of cholesterol: good cholesterol (also known as HDL which stands for high-density lipoprotein) and bad cholesterol (also known as LDL which stands for low-density lipoprotein).

LDL is called bad cholesterol because it can cause cholesterol to build up, which can lead to blockage of the arteries and heart disease. Arteries are blood vessels that pump blood away from the heart.

Cholesterol levels are easily detected through a routine blood test known as a lipid panel. When the doctor believes cholesterol is too high, lifestyle changes (e.g., increased exercise, diet changes) and medications may be prescribed.

While high cholesterol can be caused by a poor diet and not enough exercise, genetics can also play a role. In other words, some people have what is known as familial hypercholesterolemia, which is a fancy way of saying that the person has inherited very high levels of bad cholesterol from one or both parents, which leads to heart disease.

Unless someone goes for testing, the condition may not be detected because it usually does not cause symptoms. However, it can cause the sign of yellowish patches on the body due to cholesterol deposits. The condition is caused by mutations of genes. Genes are tiny structures that contain coded instructions for how certain bodily characteristics should develop.

To diagnose the condition early, some scientists have suggested using genetic screening methods. However, some have expressed concern that this can lead to an unacceptably high level of false positives, in which people are told they have the condition when they do not. One type of genetic testing is analyzes for genetic mutations that cause high cholesterol and is known as the High-Resolution Melting (HRM) method.

In the most recent issue of the Annals of Human Genetics, the HLRM method was used on eight unrelated people (7 females and 1 male) from Croatia known to have familial hypercholesterolemia. The test was positive in 7 cases (88%) in which the mutation causing the problem was found. The researchers concluded that the HLRM method is sensitive for detecting familial hypercholesterolemia, which can lead to earlier treatment and detection in other family members, which in turn, can prevent premature heart disease. One problem with the study is however is that no controls (people without the condition) were tested so the false positive rate is unknown. The study also has limited generalizability as it was mostly done with Croatian women.

Suggested reading: Cholesterol Down: Ten Simple Steps to Lower Your Cholesterol in Four Weeks--Without Prescription Drugs
  
Reference: Pećin I, Whittall R, Futema M, Sertić J, Reiner Z, Leigh SE, Humphries SE. (2013). Mutation detection in Croatian patients with familial hypercholesterolemia. Ann Hum Genet. 77(1):22-30.

How Long Should a Cough Last?

An acute (sudden) cough illness (ACI) is one of the main reasons people seek outpatient medical care from a family physician or pediatrician. Many of these cases are caused by acute bronchitis or bronchiolitis. Bronchitis is inflammation of the bronchial tubes, which are airways that connect that windpipe to the lungs.

Bronchiolitis is inflammation of the bronchioles, which are small airways that branch off the other small airways connected to the lungs.

The cause of ACI is usually a virus but patients often seek antibiotic treatment for it. The problem, though, is that while antibiotics are good at treating bacterial infections, they do not work at treating viruses. When antibiotics are prescribed for viral illnesses, it is referred to as antibiotic overuse. Overuse of antibiotics increases the risk that antibiotic-resistant bacteria will develop.

Many doctors are prone to prescribing medications in response to patient pressure (or pressure from the family). These pressures result from patient expectations on how long an acute cough should last without treatment.

In the most recent issue of the Annals of Family Medicine, researchers examined how closely patient expectations of acute cough duration matched reality. After a comprehensive literature search, the researchers found 19 studies across the world that helped them determine the average length of an untreated acute cough in otherwise healthy adults. They found that the average length of any cough was about 18 days. The average length of a cough that produced something to spit out (i.e., productive cough) was 13.9 days. 

The researchers then surveyed 493 adults in Georgia to determine their expectation on the average duration of an acute-onset cough. Depending on the specific scenario, patients expected an average cough duration of about 7 to 9 days. As the researchers explained, if the expectation of acute cough duration is wrong, it can lead to false conclusions by the patient in terms of the effectiveness of the antibiotic. For example, if a patient with an acute viral cough expects that it should only last for 7 days, seeks antibiotic treatment, and receives it, the patient will likely falsely conclude that the antibiotic cured the cough when it would have resolved anyway in 18 days. The researchers provided evidence supporting that such beliefs exist.

The researchers concluded that a better understanding of the typical duration of acute cough could reduce patient demands for antibiotics. They suggested that doctors help educate their patients about the normal length of acute cough and that they should only seek care if the cough worsens or if other alarming signs and symptoms develop such as fever, shortness of breath, or coughing up blood.

Suggested reading: Breaking the Antibiotic Habit: A Parent's Guide to Coughs, Colds, Ear Infections, and Sore Throats

Reference: Ebell, M., Lundgren, J., Youngpairoj, S. (2013). How Long Does a Cough Last? Comparing Patient’s Expectations with Data from a Systematic Review of the Literature. Annals of Family Medicine, 11, 1, 5-13.