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Spina bifida

Definition:
Spina bifida is a type of birth defect called a neural tube defect. It occurs when the bones of the spine (vertebrae) don’t form properly around part of the baby’s spinal cord. Spina bifida can be mild or severe….CLICK & SEE

Spina bifida malformations fall into three categories: spina bifida occulta, spina bifida cystica with meningocele, and spina bifida cystica with myelomeningocele. The most common location of the malformations is the lumbar and sacral areas. Myelomeningocele is the most significant and common form, and this leads to disability in most affected individuals. The terms spina bifida and myelomeningocele are usually used interchangeably.

Spina bifida meningocele and myelomeningocele are among the most common birth defects, with a worldwide incidence of about 1 in every 1000 births. The occulta form is much more common, but only rarely causes neurological symptoms.

Clasification:....CLICK & SEE
Spina bifida occulta:
Occulta is Latin for “hidden”. This is the mildest form of spina bifida. In occulta, the outer part of some of the vertebrae is not completely closed. The splits in the vertebrae are so small that the spinal cord does not protrude. The skin at the site of the lesion may be normal, or it may have some hair growing from it; there may be a dimple in the skin, or a birthmark.

Many people with this type of spina bifida do not even know they have it, as the condition is asymptomatic in most cases. The incidence of spina bifida occulta is approximately 10-20% of the population, and most people are diagnosed incidentally from spinal X-rays. A systematic review of radiographic research studies found no relationship between spina bifida occulta and back pain. More recent studies not included in the review support the negative findings.

However, other studies suggest spina bifida occulta is not always harmless. One study found that among patients with back pain, severity is worse if spina bifida occulta is present.

Incomplete posterior fusion is not a true spina bifida, and is very rarely of neurological significance.

Meningocele:
A posterior meningocele  or meningeal cyst  is the least common form of spina bifida. In this form, the vertebrae develop normally, but the meninges are forced into the gaps between the vertebrae. As the nervous system remains undamaged, individuals with meningocele are unlikely to suffer long-term health problems, although cases of tethered cord have been reported. Causes of meningocele include teratoma and other tumors of the sacrococcyx and of the presacral space, and Currarino syndrome.

A meningocele may also form through dehiscences in the base of the skull. These may be classified by their localisation to occipital, frontoethmoidal, or nasal. Endonasal meningoceles lie at the roof of the nasal cavity and may be mistaken for a nasal polyp. They are treated surgically. Encephalomeningoceles are classified in the same way and also contain brain tissue.

Myelomeningocele:
This type of spina bifida often results in the most severe complications. In individuals with myelomeningocele, the unfused portion of the spinal column allows the spinal cord to protrude through an opening. The meningeal membranes that cover the spinal cord form a sac enclosing the spinal elements. The term Meningomyelocele is also used interchangeably.

Myeloschisis:
Spina bifida with myeloschisis is the most severe form of myelomeningocele. In this type, the involved area is represented by a flattened, plate-like mass of nervous tissue with no overlying membrane. The exposure of these nerves and tissues make the baby more prone to life-threatening infections such as meningitis.

The protruding portion of the spinal cord and the nerves that originate at that level of the cord are damaged or not properly developed. As a result, there is usually some degree of paralysis and loss of sensation below the level of the spinal cord defect. Thus, the more cranial the level of the defect, the more severe the associated nerve dysfunction and resultant paralysis may be. People may have ambulatory problems, loss of sensation, deformities of the hips, knees or feet, and loss of muscle tone.

Signs and symptoms:
Physical complications:

*Leg weakness and paralysis
*Orthopedic abnormalities (i.e., club foot, hip dislocation, scoliosis)
*Bladder and bowel control problems, including incontinence, urinary tract infections, and poor renal function
*Pressure sores and skin irritations
*Abnormal eye movement

68% of children with spina bifida have an allergy to latex, ranging from mild to life-threatening. The common use of latex in medical facilities makes this a particularly serious concern. The most common approach to avoid developing an allergy is to avoid contact with latex-containing products such as examination gloves and condoms and catheters that do not specify they are latex free, and many other products, such as some commonly used by dentists.

The spinal cord lesion or the scarring due to surgery may result in a tethered spinal cord. In some individuals, this causes significant traction and stress on the spinal cord and can lead to a worsening of associated paralysis, scoliosis, back pain, and worsening bowel and/or bladder function

Neurological complications:
Many individuals with spina bifida have an associated abnormality of the cerebellum, called the Arnold Chiari II malformation. In affected individuals, the back portion of the brain is displaced from the back of the skull down into the upper neck. In about 90% of the people with myelomeningocele, hydrocephalus also occurs because the displaced cerebellum interferes with the normal flow of cerebrospinal fluid, causing an excess of the fluid to accumulate.  In fact, the cerebellum also tends to be smaller in individuals with spina bifida, especially for those with higher lesion levels.

The corpus callosum is abnormally developed in 70-90% of individuals with spina bifida myelomeningocele; this impacts the communication processes between the left and right brain hemispheres. Further, white matter tracts connecting posterior brain regions with anterior regions appear less organized. White matter tracts between frontal regions have also been found to be impaired.

Cortex abnormalities may also be present. For example, frontal regions of the brain tend to be thicker than expected, while posterior and parietal regions are thinner. Thinner sections of the brain are also associated with increased cortical folding. Neurons within the cortex may also be displaced.

Executive function:
Several studies have demonstrated difficulties with executive functions in youth with spina bifida, with greater deficits observed in youth with shunted hydrocephalus. Unlike typically developing children, youths with spina bifida do not tend to improve in their executive functioning as they grow older. Specific areas of difficulty in some individuals include planning, organizing, initiating, and working memory. Problem-solving, abstraction, and visual planning may also be impaired.  Further, children with spina bifida may have poor cognitive flexibility. Although executive functions are often attributed to the frontal lobes of the brain, individuals with spina bifida have intact frontal lobes; therefore, other areas of the brain may be implicated.

Individuals with spina bifida, especially those with shunted hydrocephalus, often have attention problems. Children with spina bifida and shunted hydrocephalus have higher rates of ADHD than typically developing children (31% vs. 17%). Deficits have been observed for selective attention and focused attention, although poor motor speed may contribute to poor scores on tests of attention.  Attention deficits may be evident at a very early age, as infants with spina bifida lag behind their peers in orienting to faces.

Academic skills:
Individuals with spina bifida may struggle academically, especially in the subjects of mathematics and reading. In one study, 60% of children with spina bifida were diagnosed with a learning disability.  In addition to brain abnormalities directly related to various academic skills, achievement is likely affected by impaired attentional control and executive functioning. Children with spina bifida may perform well in elementary school, but begin to struggle as academic demands increase.

Children with spina bifida are more likely than their typically developing peers to have dyscalculia. Individuals with spina bifida have demonstrated stable difficulties with arithmetic accuracy and speed, mathematical problem-solving, and general use and understanding of numbers in everyday life. Mathematics difficulties may be directly related to the thinning of the parietal lobes (regions implicated in mathematical functioning) and indirectly associated with deformities of the cerebellum and midbrain that affect other functions involved in mathematical skills. Further, higher numbers of shunt revisions are associated with poorer mathematics abilities. Working memory and inhibitory control deficiencies have been implicated for math difficulties, although visual-spatial difficulties are not likely involved. Early intervention to address mathematics difficulties and associated executive functions is crucial.

Individuals with spina bifida tend to have better reading skills than mathematics skills. Children and adults with spina bifida have stronger abilities in reading accuracy than in reading comprehension. Comprehension may be especially impaired for text that requires an abstract synthesis of information rather than a more literal understanding. Individuals with spina bifida may have difficulty with writing due to deficits in fine motor control and working memory.

Causes:
The exact cause of this birth defect isn’t known. Experts think that genes and the environment are part of the cause. For example, women who have had one child with spina bifida are more likely to have another child with the disease. Women who are obese or who have diabetes are also more likely to have a child with spina bifida.

Spina bifida is sometimes caused by the failure of the neural tube to close during the first month of embryonic development (often before the mother knows she is pregnant). Some forms are known to occur with primary conditions that cause raised central nervous system pressure, which raises the possibility of a dual pathogenesis.

In normal circumstances, the closure of the neural tube occurs around the 23rd (rostral closure) and 27th (caudal closure) day after fertilization. However, if something interferes and the tube fails to close properly, a neural tube defect will occur. Medications such as some anticonvulsants, diabetes, having a relative with spina bifida, obesity, and an increased body temperature from fever or external sources such as hot tubs and electric blankets may increase the chances of delivery of a baby with a spina bifida.

Extensive evidence from mouse strains with spina bifida indicates that there is sometimes a genetic basis for the condition. Human spina bifida, like other human diseases, such as cancer, hypertension and atherosclerosis (coronary artery disease), likely results from the interaction of multiple genes and environmental factors.

Research has shown the lack of folic acid (folate) is a contributing factor in the pathogenesis of neural tube defects, including spina bifida. Supplementation of the mother’s diet with folate can reduce the incidence of neural tube defects by about 70%, and can also decrease the severity of these defects when they occur. It is unknown how or why folic acid has this effect.

Spina bifida does not follow direct patterns of heredity like muscular dystrophy or haemophilia. Studies show a woman having had one child with a neural tube defect such as spina bifida has about a 3% risk of having another affected child. This risk can be reduced with folic acid supplementation before pregnancy. For the general population, low-dose folic acid supplements are advised (0.4 mg/day)

Treatment:
There is no known cure for nerve damage caused by spina bifida. To prevent further damage of the nervous tissue and to prevent infection, pediatric neurosurgeons operate to close the opening on the back. The spinal cord and its nerve roots are put back inside the spine and covered with meninges. In addition, a shunt may be surgically installed to provide a continuous drain for the excess cerebrospinal fluid produced in the brain, as happens with hydrocephalus. Shunts most commonly drain into the abdomen or chest wall. However, if spina bifida is detected during pregnancy, then open or minimally-invasive fetal surgery can be performed.

In childhood:
Most individuals with myelomeningocele will need periodic evaluations by a variety of specialists:

*Physiatrists coordinate the rehabilitation efforts of different therapists and prescribe specific therapies, adaptive equipment, or medications to encourage as high of a functional performance within the community as possible.

*Orthopedists monitor growth and development of bones, muscles, and joints.

*Neurosurgeons perform surgeries at birth and manage complications associated with tethered cord and hydrocephalus.

*Neurologists treat and evaluate nervous system issues, such as seizure disorders.

*Urologists to address kidney, bladder, and bowel dysfunction – many will need to manage their urinary systems with a program of catheterization. Bowel management programs aimed at improving elimination are also designed.

*Ophthalmologists evaluate and treat complications of the eyes.

*Orthotists design and customize various types of assistive technology, including braces, crutches, walkers, and wheelchairs to aid in mobility. As a general rule, the higher the level of the spina bifida defect, the more severe the paralysis, but paralysis does not always occur. Thus, those with low levels may need only short leg braces, whereas those with higher levels do best with a wheelchair, and some may be able to walk unaided.

*Physical therapists, occupational therapists, psychologists, and speech/language pathologists aid in rehabilitative therapies and increase independent living skills.

Transition to adulthood:
Although many children’s hospitals feature integrated multidisciplinary teams to coordinate healthcare of youth with spina bifida, the transition to adult healthcare can be difficult because the above healthcare professionals operate independently of each other, requiring separate appointments and communicate among each other much less frequently. Healthcare professionals working with adults may also be less knowledgeable about spina bifida because it is considered a childhood chronic health condition.  Due to the potential difficulties of the transition, adolescents with spina bifida and their families are encouraged to begin to prepare for the transition around ages 14–16, although this may vary depending on the adolescent’s cognitive and physical abilities and available family support. The transition itself should be gradual and flexible. The adolescent’s multidisciplinary treatment team may aid in the process by preparing comprehensive, up-to-date documents detailing the adolescent’s medical care, including information about medications, surgery, therapies, and recommendations. A transition plan and aid in identifying adult healthcare professionals are also helpful to include in the transition process.

Further complicating the transition process is the tendency for youths with spina bifida to be delayed in the development of autonomy, with boys particularly at risk for slower development of independence. An increased dependence on others (in particular family members) may interfere with the adolescent’s self-management of health-related tasks, such as catheterization, bowel management, and taking medications.  As part of the transition process, it is beneficial to begin discussions at an early age about educational and vocational goals, independent living, and community involvement.

Prevention:
There is neither a single cause of spina bifida nor any known way to prevent it entirely. However, dietary supplementation with folic acid has been shown to be helpful in reducing the incidence of spina bifida. Sources of folic acid include whole grains, fortified breakfast cereals, dried beans, leaf vegetables and fruits.

Folate fortification of enriched grain products has been mandatory in the United States since 1998. The U.S. Food and Drug Administration, Public Health Agency of Canada  and UK recommended amount of folic acid for women of childbearing age and women planning to become pregnant is at least 0.4 mg/day of folic acid from at least three months before conception, and continued for the first 12 weeks of pregnancy.  Women who have already had a baby with spina bifida or other type of neural tube defect, or are taking anticonvulsant medication should take a higher dose of 4–5 mg/day.

Certain mutations in the gene VANGL1 are implicated as a risk factor for spina bifida: These mutations have been linked with spina bifida in some families with a history of spina bifida.

Pregnancy screening:
Open spina bifida can usually be detected during pregnancy by fetal ultrasound. Increased levels of maternal serum alpha-fetoprotein (MSAFP) should be followed up by two tests – an ultrasound of the fetal spine and amniocentesis of the mother’s amniotic fluid (to test for alpha-fetoprotein and acetylcholinesterase). AFP tests are now mandated by some state laws (including California). and failure to provide them can have legal ramifications. In one case a man born with spina bifida was awarded a $2 million settlement after court found his mother’s OBGYN negligent for not performing these tests. Spina bifida may be associated with other malformations as in dysmorphic syndromes, often resulting in spontaneous miscarriage. In the majority of cases, though, spina bifida is an isolated malformation.

Genetic counseling and further genetic testing, such as amniocentesis, may be offered during the pregnancy, as some neural tube defects are associated with genetic disorders such as trisomy 18. Ultrasound screening for spina bifida is partly responsible for the decline in new cases, because many pregnancies are terminated out of fear that a newborn might have a poor future quality of life. With modern medical care, the quality of life of patients has greatly improved.

Resources:
http://en.wikipedia.org/wiki/Spina_bifida
http://www.webmd.com/parenting/baby/tc/spina-bifida-topic-overview

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Ohio Buckeye

Botanical Name :Aesculus glabra
Family : Hippocastanaceae / Sapindaceae
Genus: Aesculus
Kingdom: Plantae
Order: Sapindales
Species: A. glabra

Common Names: Ohio buckeye, American buckeye, or fetid buckeye(It derives its unflattering common name from the disagreeable odor generated from the flowers, crushed leaves, broken twigs, or bruised bark.)

Habitat :
Ohio buckeye grows mostly on mesophytic sites in western Pennsylvania to Nebraska, south to Tennessee and Oklahoma, Ohio, and southern Michigan west to Illinois and central Iowa. Its range extends south to eastern Kansas, southwestern Oklahoma, and central Texas; east to western Arkansas, Tennessee, and central Alabama with one location in eastern Mississippi . It has been planted in Europe and the eastern United States; in eastern Massachusetts, Minnesota, and western Kansas

Usually found in moist sites such as river bottoms and streambank soils, but it is sometimes also found on drier sites though does not grow so well there .

Description:
A deciduous Tree growing to 20m.  The leaves are palmately compound with five (rarely seven) leaflets, 8–16 cm (3-6 in.) long and broad. The flowers are produced in panicles in spring, yellow to yellow-green, each flower 2–3 cm (3/4 – 1 1/8 in.) long with the stamens longer than the petals (unlike the related Yellow Buckeye, where the stamens are shorter than the petals). The fruit is a round or oblong spiny capsule 4–5 cm (1 1/2 – 2 in.)diameter, containing 1-3 nut-like seeds, 2–3 cm (3/4 – 1 1/8 in.) diameter, brown with a whitish basal scar.

CLICK  &  SEE  THE  PICTURES
Flowering and Fruiting– Ohio buckeye is polygamo-monoecious, bearing both bisexual and male flowers. The pale greenish-yellow flowers appear after the leaves in the spring from March to May and are borne in upright branched clusters. Only those near the base of the branches of a cluster are perfect and fertile; the others are staminate . The fruit is a leathery capsule containing one, two, or three seeds. The ripe seed is dark chocolate to chestnut brown, smooth and shiny, with a large, light-colored hilum so that it resembles an eye. ‘The cotyledons are very thick and fleshy and contain no endosperm.

CLICK & SEE THE PICTURES

It is hardy to zone 5 and is frost tender. It is in flower from June to July, and the seeds ripen in October. The flowers are hermaphrodite (have both male and female organs)

The plant prefers light (sandy), medium (loamy) and heavy (clay) soils. The plant prefers acid, neutral and basic (alkaline) soils. It can grow in semi-shade (light woodland) or no shade. It requires moist soil.

Cultivation :-
Prefers a deep loamy well-drained soil but is not too fussy. This species is the state tree of Ohio. Its growth-rate is moderate in the wild, with trees living up to 100 years. In Britain, it grows best in eastern and south-eastern areas of England probably needing a continental climate in order to thrive. Although the trees are hardy when dormant, the new growth can be damaged by late spring frosts. The twigs, bark, flowers and leaves all produce a foetid odour if crushed. Most members of this genus transplant easily, even when fairly large.

Propagation:-
Seed – best sown outdoors or in a cold frame as soon as it is ripe. The seed germinates almost immediately and must be given protection from severe weather. The seed has a very limited viability and must not be allowed to dry out. Stored seed should be soaked for 24 hours prior to sowing and even after this may still not be viable. It is best to sow the seed with its ‘scar’ downwards. If sowing the seed in a cold frame, pot up the seedlings in early spring and plant them out into their permanent positions in the summer.

Constituents:-The fruits contain tannic acid, and are poisonous for cattle, and possibly humans, although they are often eaten by badgers. Native Americans would blanch them, extracting the tannic acid for use in leather.

Edible Uses:-
Edible Parts: Seed.
Seed – cooked. It can be dried, ground into a flour and used as a gruel. The seed is quite large, up to 35mm in diameter, and is easily harvested. It is quite rich in saponins and needs to be leached of these toxins before it becomes safe to eat – the Indians would do this by slow-roasting the nuts (which would have rendered the saponins harmless) and then cutting them into thin slices, putting them into a cloth bag and rinsing them in a stream for 2 – 5 days. By this time most of the minerals etc would also have been leached out.

The buckeye confection, made to resemble the tree’s nut, is made by dipping a spoonful of peanut butter fudge in milk chocolate, leaving a circle of the peanut butter exposed. These are a popular treat in Ohio, especially during the Christmas and NCAA college football seasons

Medicinal  Actions &  Uses
Antispasmodic; Miscellany.
Minute doses of the seed are used internally in the treatment of spasmodic coughs, asthma and internal irritations. It is used externally as a tea or an ointment in the treatment of rheumatism and piles. An extract of the bark has been used as an irritant of the cerebro-spinal system.

Disclaimer:The information presented herein is intended for educational purposes only. Individual results may vary, and before using any supplements, it is always advisable to consult with your own health care provider.

Other Uses:-
Soap; Wood.:The wood is light and soft and is used for pulpwood, woodenware, and occasionally for lumber….CLICK & SEE

Saponins in the seed are used as a soap substitute. The saponins can be easily obtained by chopping the seed into small pieces and infusing them in hot water. This water can then be used for washing the body, clothes etc. Its main drawback is a lingering odour of horse chestnuts. Wood – close-grained, light, soft, white, but often blemished by dark lines of decay. It weighs 28lb per cubic foot. It is easy to carve and resists splitting. Ideal to use in making artificial limbs, it is also used for woodenware, pulp etc and is occasionally sawn into lumber.

In addition to using the tannic acid for leatherworking, Native Americans would roast and peel the nut, and mash the contents into a nutritional meal they called “Hetuck”.

The buckeye nuts can also be dried, turning dark as they harden with exposure to the air, and strung onto necklaces. These are particularly popular among Ohio State fans.

Known Hazards : The seed is rich in saponins. Although poisonous, saponins are poorly absorbed by the human body and so most pass through without harm. Saponins are quite bitter and can be found in many common foods such as some beans. They can be removed by carefully leaching the seed or flour in running water. Thorough cooking, and perhaps changing the cooking water once, will also normally remove most of them. However, it is not advisable to eat large quantities of food that contain saponins. Saponins are much more toxic to some creatures, such as fish, and hunting tribes have traditionally put large quantities of them in streams, lakes etc in order to stupefy or kill the fish.

The seeds as well as the bark of Ohio buckeye are reported to be poisonous, and the Aesculus native to Illinois is known to contain a poisonous narcotic glucoside . The young shoots of buckeye are poisonous to cattle, and landowners in Indiana have exterminated buckeye in many areas because the seed is considered poisonous to livestock . On the other hand, some buckeye seed are apparently eaten by squirrels. In Ohio, it constitutes from 2 to 5 percent of the food of eastern fox squirrels during the fall, winter, and spring seasons. Other studies in Ohio list buckeye as an auxiliary food that was sampled by squirrels in September but not eaten in quantity . Thus, it seems probable that the use of buckeye seed for food by animals is not a limiting factor in its reproduction.

Fox squirrels in Illinois were observed eating the pith from terminal twigs . Buckeye pith contains 66 percent raffinose, a sweet-tasting 18-carbon sugar that is much sweeter and contains potentially more energy than sucrose.

Disclaimer : The information presented herein is intended for educational purposes only. Individual results may vary, and before using any supplement, it is always advisable to consult with your own health care provider.

Resources:
http://www.pfaf.org/database/plants.php?Aesculus+glabra
http://www.na.fs.fed.us/spfo/pubs/silvics_manual/volume_2/aesculus/glabra.htm
http://en.wikipedia.org/wiki/Aesculus_glabra

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