QUOTE FOR FRIDAY:

“Polymorphous light eruption is a rash caused by sun exposure in people who are sensitive to sunlight. Polymorphous means that the rash can have many forms, such as tiny bumps, raised areas or blisters. The condition also is called sun allergy, sun poisoning and polymorphic light eruption.

Polymorphous light eruption is a rash that affects parts of the body that are exposed to more sunlight as daylight hours get longer, such as the front of the neck and chest. Polymorphous means that the rash can have many forms, such as tiny bumps, raised areas or blisters.”

MAYO CLINIC (https://www.mayoclinic.org/diseases-conditions/polymorphous-light-eruption/symptoms-causes/syc-20355868)

Part I July UV Safety Month – What’s Photosensitivity, Types of Ultra Rays, and PLE – Polymorphic Light Eruption and causes of PLE!

Polymorphic light eruption PLE 2       Polymorphic light eruption PLE

 

The Sun is by far the largest object in the solar system. It contains more than 99.8% of the total mass of the Solar System (Jupiter contains most of the rest).

It is often said that the Sun is an “ordinary” star. That’s true in the sense that there are many others similar to it. But there are many more smaller stars than larger ones; the Sun is in the top 10% by mass. The median size of stars in our galaxy is probably less than half the mass of the Sun. A process called fusion heats the Sun. Fusion happens in the core of the Sun. It is very hot there. In fact, the core’s average temperature is around 27,000,000 degrees Fahrenheit. The surface of the Sun is cool compared with the core, only 10,000 degrees Fahrenheit.

This fusion process not only heats the Sun, it makes the sunlight we see here on Earth. This sunlight travels the speed of light which is 186,282 miles per second or 299,792,458 meters per second. This means the light from the Sun takes 8.4 minutes to travel 93 million miles to Earth. If the world’s fastest land animal were to travel that same distance, it would take a cheetah over 151 years to reach the Earth running about 70 mph nonstop!

Ultraviolet radiation: Invisible rays that are part of the energy that comes from the sun. Ultraviolet radiation can burn the skin and cause skin cancer.

Ultraviolet radiation is made up of three types of rays — ultraviolet A, ultraviolet B, and ultraviolet C. Although ultraviolet C is the most dangerous type of ultraviolet light in terms of its potential to harm life on earth, it cannot penetrate earth’s protective ozone layer. Therefore, it poses no threat to human, animal or plant life on earth.

Ultraviolet A and ultraviolet B, on the other hand, do penetrate the ozone layer in attenuated form and reach the surface of the planet. Because ultraviolet A is weaker than ultraviolet B, scientists long blamed ultraviolet B as the sole culprit in causing skin cancer in persons with a history of sunburn and repeated overexposure to ultraviolet radiation. Recent research, however, has also implicated ultraviolet A as a possible cause of skin cancer.

Photosensitivity refers to various symptoms, diseases and conditions caused or aggravated by exposure to sunlight.

  • A rash due to photosensitivity is a photodermatosis (plural photodermatoses).
  • If the rash is eczematous, it is a photodermatitis.
  • A chemical or drug that causes photosensitivity is a photosensitiser.
  • A phototoxic reaction to a photosensitiser results in an exaggerated sunburn reaction and no immune reaction is involved.
  • A photoallergic reaction to a photosensitiser results in photodermatitis and is due to delayed hypersensitivity reaction.
  • A photoexacerbated condition describes a flare of an underlying skin disease on exposure to sunlight.

Photosensitivity is characterized into many groups:

Polymorphic light eruption (PLE):

PLE generally affects adult females aged 20–40, although it sometimes affects children and males (25%). It is particularly common in places where sun exposure is uncommon, such as Northern Europe, where it is said to affect 10–20% of women holidaying in the Mediterranean area. It is less common in Australasia. It has also been reported to be relatively common at higher altitudes compared to sea level.

PLE can occur in all races and skin phototypes and may be more prevalent in skin of colour than in white skin. There is a genetic tendency to PLE, and it is sometimes associated with or confused with photosensitivity due to lupus erythematosus (which generally is more persistent than PLE).

Some patients experience PLE during phototherapy, which is used to treat skin conditions such as psoriasis and dermatitis.

There are three types of UV rays:

Ultraviolet A (UVA): UVA rays penetrate deep into the layers of the skin and lead to premature signs of aging, which include fine lines and wrinkles. It is important to note that the amount of UVA stays relatively consistent throughout the year, and exceeds the amount of UVB in both summer and winter. Even on a cloudy day, 80% of the sun’s UVA rays pass through the clouds and reach our skin and eyes. It’s also important to know that tanning beds work by emitting primarily UVA rays.

Ultraviolet B (UVB): UVB rays are the primary cause of sunburn, thickening of the skin, and several types of skin cancers – including melanoma. UVB rays penetrate the outermost layer of skin and cause damage to skin cells. UVB also can cause damage to eyes and the immune system.

Ultraviolet C (UVC): UVC is the strongest of the UV spectrum radiation. Fortunately, UVC rays do not reach the earth’s surface because it is blocked by the ozone layer of the atmosphere. The only way we can be exposed to UVC radiation is from an artifical source, such as a lamp or laser. UVC rays can cause severe skin burns and eye injuries even when exposed for only a few seconds. Since the penetration depth of UVC rays is very low, these injuries usually resolve within a week with virtually no risk of long-term health risks (skin cancer, cateracts, vision loss).

Causes:

Genetic factors may be important with many affected individuals reporting a family history of PLE. Native Americans have a hereditary form of PLE (actinic prurigo).

PLE is caused by a delayed hypersensitivity reaction to a compound in the skin that is altered by exposure to ultraviolet radiation (UVR). UVR leads to impaired T cell function and altered production of cytokines in affected individuals. There is a reduction in the normal UV-induced immune suppression in the skin. This has been suggested to be either due to oestrogen or deficiency of vitamin D.

The rash is usually provoked by UVA (in 90%). This means the rash can occur when the sunlight is coming through window glass, and that standard sunscreens may not prevent it. Occasionally, UVB and/or visible light provoke PLE.

PLE may be a rare occurrence in the individual concerned or may occur every time the skin is exposed to sunlight. In most affected individuals, it occurs each spring, provoked by several hours outside on a sunny day. If further sun exposure is avoided, the rash settles in a few days and is gone without a trace within a couple of weeks. It can recur next time the sun shines on the skin. However, if the affected area is exposed to more sun before it has cleared up, the condition tends to get more severe and extensive with longer to heal.

Stay tune tomorrow for part II on Ultralight rays from the Sun to Sunburn and Types of photosensitivity for some!

QUOTE FOR THURSDAY:

“Your child’s medical care will require a variety of specialists, including neurologists, ophthalmologists, plastic surgeons, otolaryngologists and speech pathologists.

Moebius syndrome can impact cranial nerves responsible for control of muscles in the tongue, jaw, larynx and throat, as well as ones that produce speech. As a result, children with Moebius syndrome may struggle with proper articulation and resonance. Severe cases of Moebius syndrome may also require a special bottle or feeding tube to help with proper nutrition. However, feeding difficulties tend to improve with age as children develop proper motor control. Physical and speech therapy can help children gain greater control of their speaking and eating, as well as improve overall coordination and motor skills.”

John Hopkins Medicine (Moebius Syndrome | Johns Hopkins Medicine)

Part II Moebius Syndrome

Etiology of Moebius:

Most cases of Moebius syndrome occur randomly for unknown reasons (sporadically) in the absence of a family history of the disorder. The syndrome is listed as Online Mendelian Inheritance in Man (OMIM) Number 15700, with a gene map locus of 13q12.2-q13. Sporadic mutations in PLXND1 and REV3L genes have also been identified in a number of patients and confirmed to cause a constellation of findings consistent with Moebius syndrome when introduced in animal models.

In rare cases, familial patterns have been reported. Most likely, Moebius syndrome is multifactorial, which means that both genetic and environmental factors play some causative role. It is possible that in different cases there are different underlying causes (heterogeneity).
In familial cases, there is evidence that Moebius syndrome is inherited as an autosomal dominant trait. Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease. The abnormal gene can be inherited from either parent, or can be the result of a new mutation (gene change) in the affected individual. The risk of passing the abnormal gene from affected parent to offspring is 50% for each pregnancy regardless of the sex of the resulting child.

The spectrum of findings in Moebius syndrome suggests a developmental defect of the hindbrain. Several different theories have been proposed to explain the cause of Moebius syndrome. One hypothesis is the disorder is the result of diminished or interrupted blood flow (ischemia) to the developing fetus during pregnancy (in utero). Recent research suggests that the lack of blood affects certain areas of the lower brainstem that contain the cranial nerve nuclei. This lack of blood flow could result from an environmental, mechanical or genetic cause. Nevertheless, cause of the syndrome remains inconclusive and more basic and clinical research is necessary.

Affected Populations:

Moebius syndrome affects males and females in equal numbers. The disorder is present at birth (congenital). The exact incidence and prevalence rates of Moebius syndrome are unknown. One estimate places the incidence at 1 case per 50,000 live births in the United States.

Diagnosis:

A diagnosis of Moebius syndrome is based upon the characteristic signs/symptoms, a detailed patient history, and a thorough clinical evaluation. There are no diagnostic tests that confirm a diagnosis of Moebius syndrome. Some specialized tests may be performed to rule out other causes of facial palsy.

Treatments:

The treatment of Moebius syndrome is directed toward the specific abnormalities in each individual. Usually these children are managed by a multidisciplinary team, often in a craniofacial center. Involved specialists include: pediatricians; neurologists; plastic surgeons; ear, nose, and throat specialists (otolaryngologists); orthopedists; dental specialists; speech pathologists; specialists who assess and treat hearing problems (audiologists), specialists who treat eye abnormalities (ophthalmologists) and other healthcare professionals.

Corrective procedures for facial paralysis involve transfer of muscle and/or graft nerves from another area of the face or the body.

Splints, braces and prostheses may be necessary for individuals with congenital limb abnormalities. Genetic counseling may be of benefit for affected individuals and their families.

QUOTE FOR WEDNESDAY:

“Moebius syndrome is a congenital (present at birth) disease.

Moebius syndrome causes many symptoms, this it affects babies’ facial muscles, including their ability to smile or move their eyes, but may cause other symptoms. The most common and significant symptom is weakness in the facial muscles that control facial expression, eye movement, sucking and swallowing.

Moebius syndrome is a neurological disorder that’s present at birth (congenital). It happens when certain cranial nerves don’t develop as they should during pregnancy. It causes several medical conditions and issues, but the primary issues are weak or paralyzed facial muscles.

Babies born with this condition have issues feeding because they can’t nurse or take a bottle. They can’t smile, frown or raise their eyebrows. They can’t close their eyelids, even when they’re sleeping.

The condition is very rare. An estimated 2 to 20 in 1 million babies are born with this condition.”

Cleveland Clinic (https://my.clevelandclinic.org/…/6064-moebius-syndrome)

Part I Moebius Syndrome

 

Moebius syndrome is a rare neurological disorder characterized by weakness or paralysis (palsy) of multiple cranial nerves, most often the 6th (abducens) and 7th (facial) nerves. Other cranial nerves are sometimes affected. The disorder is present at birth (congenital). If the 7th nerve is involved, the individual with Moebius syndrome is unable to smile, frown, pucker the lips, raise the eyebrows, or close the eyelids. If the 6th nerve is affected, the eye cannot turn outward past the midline. Other abnormalities include underdevelopment of the pectoral muscles and defects of the limbs. Moebius syndrome is not progressive. The exact cause is unknown. It appears to occur randomly (sporadically) in most cases; however, some cases occur in families suggesting that there may be a genetic component.

Introduction:

Congenital facial and abducens palsy was first described by Von Graefe (1880) and Moebius (1888), a German neurologist after whom the syndrome was later named.

Signs and Symptoms:

The abnormalities and severity of Moebius syndrome vary greatly from one person-to-another. The classically accepted diagnostic criteria include: 1) facial paralysis or weakness affecting at least one but usually both sides of the face (7th cranial nerve), 2) paralysis of sideways (lateral) movement of the eyes (6th cranial nerve); and 3) preservation of vertical movements of the eyes. Less often, other cranial nerves, including the 5th, 8th, 9th, 10th, 11th, and 12th may be affected.

Infants with Moebius syndrome may drool excessively and exhibit crossed eyes (strabismus). Because the eyes do not move from side-to-side (laterally), the child is forced to turn the head to follow objects. Infants who lack facial expression often are described as having a “mask-like” face that is especially obvious when laughing or crying. Affected infants may also have difficulties feeding, including problems swallowing and poor sucking. Corneal ulceration may occur because the eyelids remain open during sleep.

There are a wide variety of additional abnormalities. Some children with Moebius syndrome have a short, malformed tongue and/or an abnormally small jaw (micrognathia). Cleft palate may also be present. These abnormalities contribute to feeding and breathing difficulties. Children with cleft palate are prone to ear infections (otitis media). There may be external ear anomalies including underdevelopment of the outer portion of the ear (microtia) or total absence of the outer portion of the ear (anotia). If the 8th cranial nerve is affected, there is likely hearing loss. Dental abnormalities are not uncommon. There is an increased risk for childhood cavities. Some affected children have difficulties with speech and delays in speech development.

Skeletal malformations of the limbs occur in over half of children with Moebius sydrome. Lower limb malformations include clubbed feet and underdevelopment of the lower legs; upper extremities may have webbing of the fingers (syndactyly), underdevelopment or absence of the fingers, and/or underdevelopment of the hand. In a few children there may be abnormal side-to-side curvature of the spine (scoliosis), and in approximately 15% of patients underdevelopment of the chest (pectoral) muscles and the breast on one side of the body also occur (see Poland-Moebius syndrome in the Related Disorder section below).

Some affected children exhibit delay in attaining certain milestones such as crawling or walking, most likely due to upper body weakness; however, most children eventually catch-up. Moebius syndrome rarely is associated with minor intellectual disability. Some children have been classified as being on the “autistic spectrum”. The exact relationship between Moebius syndrome and autism is unknown. Some studies have suggested that autism spectrum disorders occur with greater frequency in children with Moebius syndrome; other studies have not confirmed this and suggest that any such relationship is overstated. Moebius syndrome is often associated with a variety of social and psychological consequences. The lack of facial expressions and the inability to smile can cause observers to misinterpret what an affected individual is thinking or feeling or intends. Although clinical anxiety and depression are not more common in children and adolescents with Moebius syndrome, affected individuals may avoid social situations due to apprehension and frustration.

 

 

QUOTE FOR TUESDAY:

“AABB joins the health care community in recognizing July as National Cord Blood Awareness Month.

Despite its life-saving potential, most umbilical cord blood — and the hematopoietic stem cells it contains — is still discarded as medical waste in the United States. Cord Blood Awareness Month aims to raise awareness about the importance of saving and storing cord blood.

Throughout July, AABB will share educational resources and social media graphics to highlight cord blood’s life-changing role in stem cell transplants and cellular therapies.”

Association for the Advancement of Blood and Biotherapies – AABB (July is National Cord Blood Awareness Month)

 

Cord Blood Banking

 

Cord Blood Awareness Month  is July

The Office of Disease Prevention and Health Promotion designates specific National Health Observances (NHO) dedicated to raising awareness about important health topics. Towards this goal, July has been declared “Cord Blood Awareness Month.” Although people benefit from cord blood banking every day, this NHO provides a special opportunity to highlight the significant contributions made through stem cell research, trials, and treatment.

Treatments

Stem cells are at the forefront of one of the most fascinating and revolutionary areas of biology today. To date, there are more than 80 diseases being treated with umbilical cord blood and tissue. The list of diseases that are treatable with cord blood include stem cell disorders, acute and chronic forms of leukemia, myeloproliferative disorders, and many more.

Clinical Trials

With a very promising future, clinical trials using cord blood stem cells are being conducted for neurological, cardiovascular, and auto-immune disorders. Some of the common diseases that have clinical trials in Phase 1 and Phase 2 include autism, cerebral palsy, and diabetes. There are also promising clinical trials for acquired hearing loss, pre-/peri-natal stroke, and traumatic brain injury.

Cord Blood BankingCord Blood Awareness Month

Cord blood banking provides a simple process of safely and securely storing the blood within your child’s umbilical cord, as well as the tissue from the cord itself. Parents have the option of banking their baby’s cord blood with a public cord blood bank or a private cord blood bank.

Public cord blood banking is free and will provide life-saving benefits to a family in need. Once you donate your cord blood, however, you no longer have rights to those stem cells. If your child or family member is in need of cord blood stem cells, there is no guarantee that you or children can use their own cells. With private cord blood banking, there are fees but you own the cells. Your full rights to use it are preserved, and it is always immediately available to you.

A Perfect Match

One of the key benefits of using a private cord blood bank is the ability to provide a perfect match, since your child will be getting their own cells. Cord blood banking is especially vital for minorities and those of mixed race, where matching can be difficult using other stem cells from bone marrow or circulatory blood. Certain diseases also either depend upon, or greatly benefit from, using your own (autologous) cells. Luckily, it is easy to bank umbilical cord blood with New England Cord Blood Bank during Cord Blood Awareness Month and all year round.

QUOTE FOR MONDAY:

“Every July, Cord Blood Awareness Month shines a spotlight on one of the most powerful yet often overlooked medical resources: cord blood. Collected from a newborn’s umbilical cord shortly after birth, cord blood is rich in hematopoietic stem cells—the building blocks of blood and immune systems. These stem cells have the unique ability to develop into red and white blood cells and platelets, and they’re already being used to treat more than 80 serious and life-threatening conditions, including leukemia, lymphoma, sickle cell anemia, and certain metabolic and immune disorders.”

Fetal Health Foundation (Cord Blood Awareness Month: One Decision Could Save a Life)

 

 

Cord Blood Awareness Month-What it is and uses for it!

July is National Cord Blood Awareness Month, and it’s the perfect time to learn more about cord blood—a biological product regulated by the Food and Drug Administration. Found in the blood vessels of the placenta and the umbilical cord, cord blood is collected after a baby is born and after the umbilical cord is cut—an important point.

“Because cord blood is typically collected after the baby is delivered and the cord is cut, the procedure is generally safe for the mother and baby,” explains Keith Wonnacott, Ph.D., Chief of the Cellular Therapies Branch in FDA’s Office of Cellular, Tissue, and Gene Therapies.

Approved Uses By the Food and Drug Administration states the following:

Cord blood is approved only for use in “hematopoietic stem cell transplantation” procedures, which are done in patients with disorders affecting the hematopoietic (blood forming) system. Cord blood contains blood-forming stem cells that can be used in the treatment of patients with blood cancers such as leukemias and lymphomas, as well as certain disorders of the blood and immune systems, such as sickle cell disease and Wiskott-Aldrich syndrome.

“Cord blood is useful because it is a source of stem cells that form into blood cells. Cord blood can be used for transplantation in people who need regeneration, that is, ‘regrowth,’ of these blood-forming cells,” Wonnacott says.

For instance, in many cancer patients, the disease is found in the blood cells. Chemotherapy treatment of these patients kills both cancer cells and the healthy blood-forming stem cells. Transplanted stem cells from cord blood can help regrow the healthy blood cells after the chemotherapy.

However, cord blood is not a cure-all.

“Because cord blood contains stem cells, there have been stem cell fraud cases related to cord blood,” says Wonnacott. “Consumers may think that stem cells can cure any disease, but science doesn’t show this to be the case. Patients should be skeptical if cord blood is being promoted for uses other than blood stem cell regeneration.”

About Cord Blood Banking

After cord blood is collected, it is frozen and can be safely stored for many years. “The method of freezing, called ‘cryopreservation,’ is very important to maintain the integrity of the cells,” Wonnacott says. “Cord blood needs to be stored carefully.”

You may choose to store your baby’s cord blood in a private bank so it can be available if needed in the future by your child or first- or second-degree relatives. Private cord banks typically charge fees for blood collection and storage.

Or you may donate the cord blood to a public bank so that doctors can use for a patient who needs a hematopoietic stem cell transplant.

FDA regulates cord blood in different ways, depending on the source, level of processing and intended use.

Cord blood stored for personal use, for use in first- or second-degree relatives, and that also meets other criteria in FDA’s regulations, does not require the agency’s approval before use. Private cord banks must still comply with other FDA requirements, including establishment registration and listing, current good tissue practice regulations, and donor screening and testing for infectious diseases (except when cord blood is used for the original donor). These FDA requirements ensure safety of these products by minimizing the risk of contamination and transmission of infectious diseases.

Cord blood stored for use by a patient unrelated to the donor meets the legal definitions of both a “drug” and a “biological product.” Cord blood in this category must meet additional requirements and be licensed under a biologics license application, or be the subject of an investigational new drug application before use. The FDA requirements help to ensure that these products are safe and effective for their intended use.

Not every cord blood unit will meet requirements for public banking, adds Safa Karandish, M.T., an FDA consumer safety officer. If that happens, some of this donated cord blood may be used for non-clinical research.