Be it reciting a jingle heard on the television, running after everything that catches their fancy or bewildering adults with their endless questions, toddlers give us a glimpse of their infectious energy every day. Their energy and curiosity often leave parents astounded. This fascination about the world around them stems from the fact that the brains of young children are more active than adult brains.
Knowing more about brain development can help us make sense of their behavior and provide the best conditions for their growth. Up to 6 years of age, the brain is constantly learning, developing and forming memories. In fact, the brain develops up to 90% of its capacity by age 6¹. With the number of active brain connections, a toddler processes more information than an adult brain. Brain development in young children:
Overall, a toddler’s brain grows up to 25% of an adult brain size by age 3. We know that different areas of the brain serve different functions. Essential brain functions are active right from birth. After birth, sensory functions such as sight and smell are the first to develop followed by higher cognitive functions such as problem-solving. Language development occurs between the ages of two and four.
There are several parts of the brain that see phenomenal growth during the first few years of life. Synapses are connections between two different nerve cells in the brain. The development of synapses is the fastest among young children. This allows them to learn more than adults in the same amount of time.
The visual cortex, located at the hind side of the brain, aids in visual perception and the growth of this part improves the brain capacity to sense depth and color. The growth of the cerebellum is linked to motor skills that allow a child to crawl and later start walking.
There are quite a few factors that create ideal conditions for brain development. Mentally stimulating activities help exercise the developing brain while adequate nutrition provides the nutrients for its development.
Pre-eclampsia, eclampsia or toxemia of pregnancy Definition:
Pre-eclampsia or preeclampsia (PE) is a disorder of pregnancy characterized by high blood pressure and a large amount of protein in the urine. The disorder usually occurs in the third trimester of pregnancy and gets worse over time. In severe disease there may be red blood cell breakdown, a low blood platelet count, impaired liver function, kidney dysfunction, swelling, shortness of breath due to fluid in the lungs, or visual disturbances. PE increases the risk of poor outcomes for both the mother and the baby. If left untreated, it may result in seizures at which point it is known as eclampsia.
Toxemia of pregnancy is a severe condition that sometimes occurs in the latter weeks of pregnancy. It is characterized by high blood pressure; swelling of the hands, feet, and face; and an excessive amount of protein in the urine. If the condition is allowed to worsen, the mother may experience convulsions and coma, and the baby may be stillborn.
The term toxemia is actually a misnomer from the days when it was thought that the condition was caused by toxic (poisonous) substances in the blood. The illness is more accurately called preeclampsia before the convulsive stage and eclampsia afterward.
Preeclampsia affects between 2–8% of pregnancies worldwide. Hypertensive disorders of pregnancy are one of the most common causes of death due to pregnancy. They resulted in 29,000 deaths in 2013 – down from 37,000 deaths in 1990. Preeclampsia usually occurs after 32 weeks; however, if it occurs earlier it is associated with worse outcomes. Women who have had PE are at increased risk of heart disease later in life. The word eclampsia is from the Greek term for lightning. The first known description of the condition was by Hippocrates in the 5th century BCE
Swelling (especially in the hands and face) was originally considered an important sign for a diagnosis of preeclampsia. However, because swelling is a common occurrence in pregnancy, its utility as a distinguishing factor in preeclampsia is not great. Pitting edema (unusual swelling, particularly of the hands, feet, or face, notable by leaving an indentation when pressed on) can be significant, and should be reported to a health care provider.
In general, none of the signs of preeclampsia are specific, and even convulsions in pregnancy are more likely to have causes other than eclampsia in modern practice. Further, a symptom such as epigastric pain may be misinterpreted as heartburn. Diagnosis, therefore, depends on finding a coincidence of several preeclamptic features, the final proof being their regression after delivery.
The symptoms of toxemia of pregnancy (which may lead to death if not treated) are divided into three stages, each progressively more serious:
Mild preeclampsia symptoms include edema (puffiness under the skin due to fluid accumulation in the body tissues, often noted around the ankles), mild elevation of blood pressure, and the presence of small amounts of protein in the urine.
Severe preeclampsia symptoms include extreme edema, extreme elevation of blood pressure, the presence of large amounts of protein in the urine, headache, dizziness, double vision, nausea, vomiting, and severe pain in the right upper portion of the abdomen.
Eclampsia symptoms include convulsions and coma.
It is also more frequent in a women’s first pregnancy and if she is carrying twins. The underlying mechanism involves abnormal formation of blood vessels in the placenta amongst other factors. Most cases are diagnosed before delivery. Rarely, preeclampsia may begin in the period after delivery. While historically both high blood pressure and protein in the urine were required to make the diagnosis, some definitions also include those with hypertension and any associated organ dysfunction. Blood pressure is defined as high when it is greater than 140 mmHg systolic or 90 mmHg diastolic at two separate times, more than four hours apart in a women after twenty weeks of pregnancy. PE is routinely screened for during prenatal care. Causes:
There is no definitive known cause of preeclampsia, though it is likely related to a number of factors. Some of these factors include:
*Abnormal placentation (formation and development of the placenta)
*Prior or existing maternal pathology – preeclampsia is seen more at a higher incidence in individuals with preexisting hypertension, obesity, antiphospholipid antibody syndrome, and those with history of preeclampsia
*Dietary factors, e.g. calcium supplementation in areas where dietary calcium intake is low has been shown to reduce the risk of preeclampsia.
*Environmental factors, e.g. air pollution
*Those with long term high blood pressure have a risk 7 to 8 times higher than those without.
Physiologically, research has linked preeclampsia to the following physiologic changes: alterations in the interaction between the maternal immune response and the placenta, placental injury, endothelial cell injury, altered vascular reactivity, oxidative stress, imbalance among vasoactive substances, decreased intravascular volume, and disseminated intravascular coagulation.
While the exact cause of preeclampsia remains unclear, there is strong evidence that a major cause predisposing a susceptible woman to preeclampsia is an abnormally implanted placenta. This abnormally implanted placenta is thought to result in poor uterine and placental perfusion, yielding a state of hypoxia and increased oxidative stress and the release of anti-angiogenic proteins into the maternal plasma along with inflammatory mediators. A major consequence of this sequence of events is generalized endothelial dysfunction. The abnormal implantation is thought to stem from the maternal immune system’s response to the placenta and refers to evidence suggesting a lack of established immunological tolerance in pregnancy. Endothelial dysfunction results in hypertension and many of the other symptoms and complications associated with preclampsia.
One theory proposes that certain dietary deficiencies may be the cause of some cases. Also, there is the possibility that some forms of preeclampsia and eclampsia are the result of deficiency of blood flow in the uterus.
Diagnosis: Pre-eclampsia is diagnosed when a pregnant woman develops:
*Blood pressure >_ 140 mm Hg systolic or >_90 mm Hg diastolic on two separate readings taken at least four to six hours apart after 20 weeks gestation in an individual with previously normal blood pressure.
*In a woman with essential hypertension beginning before 20 weeks gestational age, the diagnostic criteria are: an increase in systolic blood pressure (SBP) of >_ 30mmHg or an increase in diastolic blood pressure (DBP) of >_15mmHg.
*Proteinuria >_ 0.3 grams (300 mg) or more of protein in a 24-hour urine sample or a SPOT urinary protein to creatinine ratio >_ 0.3 or a urine dipstick reading of 1+ or greater (dipstick reading should only be used if other quantitative methods are not available)
Suspicion for preeclampsia should be maintained in any pregnancy complicated by elevated blood pressure, even in the absence of proteinuria. Ten percent of individuals with other signs and symptoms of preeclampsia and 20% of individuals diagnosed with eclampsia show no evidence of proteinuria. In the absence of proteinuria, the presence of new-onset hypertension (elevated blood pressure) and the new onset of one or more of the following is suggestive of the diagnosis of preeclampsia:
*Evidence of kidney dysfunction (oliguria, elevated creatinine levels)
*Impaired liver function (impaired liver function tests)
*Thrombocytopenia (platelet count <100,000/microliter)
*Ankle edema pitting type
*Cerebral or visual disturbances
*Preeclampsia is a progressive disorder and these signs of organ dysfunction are indicative of severe preeclampsia. A systolic blood pressure ?160 or diastolic blood pressure ?110 and/or proteinuria >5g in a 24-hour period is also indicative of severe preeclampsia. Clinically, individuals with severe preeclampsia may also present epigastric/right upper quadrant abdominal pain, headaches, and vomiting. Severe preeclampsia is a significant risk factor for intrauterine fetal death.
Of note, a rise in baseline blood pressure (BP) of 30 mmHg systolic or 15 mmHg diastolic, while not meeting the absolute criteria of 140/90, is still considered important to note, but is not considered diagnostic.
There have been many assessments of tests aimed at predicting preeclampsia, though no single biomarker is likely to be sufficiently predictive of the disorder. Predictive tests that have been assessed include those related to placental perfusion, vascular resistance, kidney dysfunction, endothelial dysfunction, and oxidative stress. Examples of notable tests include:
*Doppler ultrasonography of the uterine arteries to investigate for signs of inadequate placental perfusion. This test has a high negative predictive value among those individuals with a history of prior preeclampsia.
*Elevations in serum uric acid (hyperuricemia) is used by some to “define” preeclampsia, though it has been found to be a poor predictor of the disorder. Elevated levels in the blood (hyperuricemia) are likely due to reduced uric acid clearance secondary to impaired kidney function.
*Angiogenic proteins such as vascular endothelial growth factor (VEGF) and placental growth factor (PIGF) and anti-angiogenic proteins such as soluble fms-like tyrosine kinase-1 (sFlt-1) have shown promise for potential clinical use in diagnosing preeclampsia, though evidence is sufficient to recommend a clinical use for these markers.
*Recent studies have shown that looking for podocytes, specialized cells of the kidney, in the urine has the potential to aid in the prediction of preeclampsia. Studies have demonstrated that finding podocytes in the urine may serve as an early marker of and diagnostic test for preeclampsia. Research is ongoing.
Pre-eclampsia can mimic and be confused with many other diseases, including chronic hypertension, chronic renal disease, primary seizure disorders, gallbladder and pancreatic disease, immune or thrombotic thrombocytopenic purpura, antiphospholipid syndrome and hemolytic-uremic syndrome. It must be considered a possibility in any pregnant woman beyond 20 weeks of gestation. It is particularly difficult to diagnose when preexisting disease such as hypertension is present. Women with acute fatty liver of pregnancy may also present with elevated blood pressure and protein in the urine, but differs by the extent of liver damage. Other disorders that can cause high blood pressure include thyrotoxicosis, pheochromocytoma, and drug misuse Treatment:
Preeclampsia and eclampsia cannot be completely cured until the pregnancy is over. Until that time, treatment includes the control of high blood pressure and the intravenous administration of drugs to prevent convulsions. Drugs may also be given to stimulate the production of urine. In some severe cases, early delivery of the baby is needed to ensure the survival of the mother.
Recommendations for prevention include: aspirin in those at high risk, calcium supplementation in areas with low intake, and treatment of prior hypertension with medications. In those with PE delivery of the fetus and placenta is an effective treatment. When delivery becomes recommended depends on how severe the PE and how far along in pregnancy a person is. Blood pressure medication, such as labetalol and methyldopa, may be used to improve the mother’s condition before delivery. Magnesium sulfate may be used to prevent eclampsia in those with severe disease. Bedrest and salt intake have not been found to be useful for either treatment or prevention.
Protein or calorie supplementation have no effect on preeclampsia rates, and dietary protein restriction does not appear to increase preeclampsia rates. Further, there is no evidence that changing salt intake has an effect.
Supplementation with antioxidants such as vitamin C and E has no effect on preeclampsia incidence, nor does supplementation with vitamin D. Therefore, supplementation with vitamins C, E, and D is not recommended for reducing the risk of pre-eclampsia.
Calcium supplementation of at least 1 gram per day is recommended during pregnancy as it prevents preeclampsia where dietary calcium intake is low, especially for those at high risk. Low selenium status is associated with higher incidence of preeclampsia.
Taking aspirin is associated with a 1% to 5% reduction in preeclampsia and a 1% to 5% reduction in premature births in women at high risk. The WHO recommends low-dose aspirin for the prevention of preeclampsia in women at high risk and recommend it be started before 20 weeks of pregnancy. The United States Preventive Services Task Force recommends a low-dose regimen for women at high risk beginning in the 12th week.
There is insufficient evidence to recommend either exercise or strict bedrest as preventative measures of pre-eclampsia.
In low-risk pregnancies the association between cigarette smoking and a reduced risk of preeclampsia has been consistent and reproducible across epidemiologic studies. High-risk pregnancies (those with pregestational diabetes, chronic hypertension, history of preeclampsia in a previous pregnancy, or multifetal gestation) showed no significant protective effect. The reason for this discrepancy is not definitively known; research supports speculation that the underlying pathology increases the risk of preeclampsia to such a degree that any measurable reduction of risk due to smoking is masked. However, the damaging effects of smoking on overall health and pregnancy outcomes outweighs the benefits in decreasing the incidence of preeclampsia. It is recommended that smoking be stopped prior to, during and after pregnancy
Restriction of salt in the diet may help reduce swelling, it does not prevent the onset of high blood pressure or the appearance of protein in the urine. During prenatal visits, the doctor routinely checks the woman’s weight, blood pressure, and urine. If toxemia is detected early, complications may be reduced.
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.
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.
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.
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
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.
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.
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.
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)
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.
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.
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.
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.
Roseola is a generally mild infection that usually affects children by age 2. It occasionally affects adults. Roseola is extremely common — so common that most children have been infected with roseola by the time they enter kindergarten. CLICK & SEE THE PICTURES
Two common strains of herpes viruses cause roseola. The condition typically causes several days of fever, followed by a rash.
Some children develop only a very mild case of roseola and never show any clear indication of illness, while others experience the full range of symptoms.
Roseola typically isn’t serious. Rarely, complications from a very high fever can result. Treatment of roseola includes bed rest, fluids and medications to reduce fever.
It is frequently called roseola, although this term could be applied to any rose-colored rash.
The child may have a runny nose, sore throat, and eye redness.
A fever usually occurs before the rash appears. It lasts for 3 (sometimes up to 7) days. The fever may be as high as 105° Fahrenheit, and it generally responds well to acetaminophen (Tylenol).
Between the second and fourth day of the illness, the fever drops and a rash appears (often as the fever falls).
•The rash starts on the trunk and spreads to the limbs, neck, and face. The rash is pink or rose-colored, and has fairly small sores that are slightly raised.
•The rash lasts from a few hours to 2 – 3 days. It usually does not itch.
Other symptoms include: CLICK & SEE
•High fever that comes on quickly
Until recently, its origin was unknown, but it is now known to be caused by two human herpesviruses, HHV-6 (Human herpesvirus 6) and HHV-7, which are sometimes referred to collectively as Roseolovirus. There are two variants of HHV-6 and studies in the US, Europe and Japan have shown that exanthema subitum is caused by HHV-6B which infects over 90% of infants by age 2. Current research indicates that babies congenitally infected with the HHV-6A virus can have inherited the virus on a chromosome
The virus is spread through the faecal-oral route (poor hygiene after using the toilet) or by airborne droplets. Careful handwashing can help prevent its spread.
Occasionally other viruses cause an illness very similar to roseola.
Like other viral illnesses, such as a common cold, roseola spreads from person to person through contact with an infected person’s respiratory secretions or saliva. For example, a healthy child who shares a cup with a child who has roseola could contract the virus. CLICK & SEE
Roseola is contagious even if no rash is present. That means the condition can spread while an infected child has only a fever, even before it’s clear that the child has roseola. Watch for signs of roseola if your child has interacted with another child who has the illness.
Unlike chickenpox and other childhood viral illnesses that spread rapidly, roseola rarely results in a communitywide outbreak. The infection can occur at any time of the year.
Roseola occurs throughout the year. The time between becoming infected and the beginning of symptoms (incubation period) is 5 to 15 days.
Older infants are at greatest risk of acquiring roseola because they haven’t had time yet to develop their own antibodies against many viruses. While in the uterus, babies receive antibodies from their mothers that protect them as newborns from contracting infections, such as roseola. But this immunity fades with time. The most common age for a child to contract roseola is between 6 and 15 months.
Seizures in children
Occasionally a child with roseola experiences a seizure brought on by a rapid rise in body temperature. If this happens, your child might briefly lose consciousness and jerk his or her arms, legs or head for several seconds to minutes. He or she may also lose bladder or bowel control temporarily.
If your child has a seizure, seek emergency care. Although frightening, fever-related seizures in otherwise healthy young children are generally short-lived and are rarely harmful.
Complications from roseola are rare. The vast majority of otherwise healthy children and adults with roseola recover quickly and completely.
Concerns for people with weak immune systems
Roseola is of greater concern in people whose immune system is compromised, such as those who have recently received a bone marrow or organ transplant. They may contract a new case of roseola — or a previous infection may come back while their immune system is weakened. Because they have less resistance to viruses in general, immune-compromised people tend to develop more severe cases of infection and have a harder time fighting off illness.
People with weak immune systems who contract roseola may experience potentially serious complications from the infection, such as pneumonia or encephalitis — a potentially life-threatening inflammation of the brain.
Roseola is usually diagnosed from the history and symptoms, especially if the infection has recently been reported in the community.
•Physical exam of rash
•Swollen lymph nodes on the neck (cervical nodes) or back of the scalp (occipital nodes)
Typically the disease affects a child between six months and two years of age, and begins with a sudden high fever (39–40 °C; 102.2-104 °F). This can cause, in rare cases, febrile convulsions (also known as febrile seizures or “fever fits”) due to the sudden rise in body temperature, but in many cases the child appears normal. After a few days the fever subsides, and just as the child appears to be recovering, a red rash appears. This usually begins on the trunk, spreading to the legs and neck. The rash is not itchy and may last 1 to 2 days. In contrast, a child suffering from measles would usually appear more infirm, with symptoms of conjunctivitis and a cough, and their rash would affect the face and last for several days. Liver dysfunction can occur in rare cases.
The rare adult reactivates with HHV-6 and can show signs of mononucleosis.
The disease usually gets better without complications.
Most children recover fully from roseola within a week of the onset of the fever. With your doctor’s advice, you can give your child over-the-counter medications to reduce fever, such as acetaminophen (Tylenol, others) or ibuprofen (Advil, Motrin, others). However, don’t give aspirin to a child who has a viral illness because aspirin has been associated with the development of Reye’s syndrome, which can be serious.
There’s no specific treatment for roseola, although some doctors may prescribe the antiviral medication ganciclovir (Cytovene) to treat the infection in people with weakened immunity. Antibiotics aren’t effective in treating viral illnesses, such as roseola.
Like most viruses, roseola just needs to run its course. Once the fever subsides, your child should feel better soon. However, a fever can make your child uncomfortable. To treat your child’s fever at home, your doctor may recommend:
*Plenty of rest. Let your child rest in bed until the fever disappears.
*Plenty of fluids. Encourage your child to drink clear fluids, such as water, ginger ale, lemon-lime soda, clear broth or an electrolyte solution (such as Pedialyte) or sports drinks (such as Gatorade and Powerade) to prevent dehydration. Remove the gas bubbles from carbonated fluids. You can do this by letting the carbonated beverage stand or by shaking, pouring or stirring the beverage. Removing the carbonation will mean having your child avoid the added discomfort of excess burping or intestinal gas that carbonated beverages may cause.
*Sponge baths. A lukewarm sponge bath or a cool washcloth applied to your child’s head can soothe the discomfort of a fever. However, avoid using ice, cold water, fans or cold baths. These may give the child unwanted chills.There’s no specific treatment for the rash of roseola, which fades on its own in a short time
Because there’s no vaccine to prevent roseola, the best you can do to prevent the spread of roseola is to avoid exposing your child to an infected child. If your child is sick with roseola, keep him or her home and away from other children until the fever has broken. Once the rash appears, the virus is much less contagious.
Most people have antibodies to roseola by the time they’re of school age, making them immune to a second infection. Even so, if one household member contracts the virus, make sure that all family members wash their hands frequently to prevent spread of the virus to anyone who isn’t immune.
Adults who never contracted roseola as children can become infected later in life, though the disease tends to be mild in healthy adults. The main concern is that infected adults can pass the virus on to children.
Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.
Pyloric stenosis is a condition that causes severe vomiting in the first few months of life. There is narrowing (stenosis) of the opening from the stomach to the intestines, due to enlargement (hypertrophy) of the muscle surrounding this opening (the pylorus, meaning “gate”), which spasms when the stomach empties. It is uncertain whether there is a real congenital narrowing or whether there is a functional hypertrophy of the muscle which develops in the first few weeks of life. Babies with this condition may seem to always be hungry click to see the pictures……..(01).…..…(1)..….…(2).……..(3)....……
Pyloric stenosis also occurs in adults where the cause is usually a narrowed pylorus due to scarring from chronic peptic ulceration. This is a different condition from the infantile form.
Prompt treatment of pyloric stenosis is important for preventing complications. Pyloric stenosis can be corrected with surgery.
Males are more commonly affected than females, with firstborn males affected about four times as often, and there is a genetic predisposition for the disease. It is commonly associated with people of Jewish ancestry, and has multifactorial inheritance patterns. Pyloric stenosis is more common in Caucasians than Hispanics, Blacks, or Asians. The incidence is 2.4 per 1000 live births in Caucasians , 1.8 in Hispanics, 0.7 in Blacks, and 0.6 in Asians. It is also less common amongst children of mixed race parents. Caucasian babies with blood type B or O are more likely than other types to be affected
Signs of pyloric stenosis usually appear within three to five weeks after birth. Pyloric stenosis is rare in babies older than age 3 months.
Signs and symptoms are: *Frequent projectile vomiting. Pyloric stenosis often causes projectile vomiting — the forceful ejection of milk or formula up to several feet away — within 30 minutes after your baby eats. Vomiting may be mild at first and gradually become more severe. The vomit may sometimes contain blood.
*Persistent hunger. Babies who have pyloric stenosis often want to eat soon after vomiting.
*Stomach contractions. You may notice wave-like contractions that move across your baby’s upper abdomen (peristalsis) soon after feeding but before vomiting. This is caused by stomach muscles trying to force food past the outlet of the pylorus.
*Dehydration. Your baby may cry without tears or become lethargic. You may find yourself changing fewer wet diapers or diapers that aren’t as wet as you expect.
*Changes in bowel movements. Since pyloric stenosis prevents food from reaching the intestines, babies with this condition may be constipated.
*Weight problems. Pyloric stenosis can prevent a baby from gaining weight, and can sometimes even cause weight loss.
*Less active or seems unusually irritable
*Urinating much less frequently or is having noticeably fewer bowel movements
The cause of the thickening is unknown, although genetic factors may play a role. Children of parents who had pyloric stenosis are more likely to have this problem.
Normally, food passes easily from the stomach into the duodenum (the first part of the small intestine) through a valve called the pylorus. In pyloric stenosis, the muscles of the pylorus are thickened. This thickening prevents the stomach from emptying into the small intestine.
*Sex. Pyloric stenosis occurs more often in males than in females.
*Birth order. About one-third of babies affected by pyloric stenosis are firstborns.
*Family history. More than 1 in 10 babies with pyloric stenosis has a family member who had the disorder.
*Early antibiotic use. Babies given certain antibiotics, such as erythromycin, in the first weeks of life for whooping cough (pertussis) have an increased risk of pyloric stenosis. In addition, babies born to mothers who were given certain antibiotics in late pregnancy also may have an increased risk of pyloric stenosis.
Complications: Pyloric stenosis can lead to:
*An electrolyte imbalance. Electrolytes are minerals, such as chloride and potassium, that circulate in the body’s fluids to help regulate many vital functions, such as heartbeat. When a baby vomits every time he or she eats, dehydration and an imbalance of electrolytes eventually occurs
*Stomach irritation. Repeated vomiting can irritate your baby’s stomach. This irritation may even cause mild bleeding.
*Jaundice. Rarely, infants who have pyloric stenosis develop jaundice — a yellowish discoloration of the skin and eyes caused by a buildup of a substance secreted by the liver called bilirubin.
Diagnosis is via a careful history and physical examination, often supplemented by radiographic studies. There should be suspicion for pyloric stenosis in any young infant with severe vomiting. On exam, palpation of the abdomen may reveal a mass in the epigastrium. This mass, which consists of the enlarged pylorus, is referred to as the ‘olive,’ and is sometimes evident after the infant is given formula to drink. It is an elusive diagnostic skill requiring much patience and experience. There are often palpable (or even visible) peristaltic waves due to the stomach trying to force its contents past the narrowed pyloric outlet.
At this point, most cases of pyloric stenosis are diagnosed/confirmed with ultrasound, if available, showing the thickened pylorus. Although somewhat less useful, an upper GI series (x-rays taken after the baby drinks a special contrast agent) can be diagnostic by showing the narrowed pyloric outlet filled with a thin stream of contrast material; a “string sign” or the “railroad track sign”. For either type of study, there are specific measurement criteria used to identify the abnormal results. Plain x-rays of the abdomen are not useful, except when needed to rule out other problems.
Blood tests will reveal hypokalemic, hypochloremic metabolic alkalosis due to loss of gastric acid (which contain hydrochloric acid and potassium) via persistent vomiting; these findings can be seen with severe vomiting from any cause. The potassium is decreased further by the body’s release of aldosterone, in an attempt to compensate for the hypovolaemia due to the severe vomiting.
The gastric outlet obstruction due to the hypertrophic pylorus impairs emptying of gastric contents into the duodenum. As a consequence, all ingested food and gastric secretions can only exit via vomiting, which can be of a projectile nature. The vomited material does not contain bile because the pyloric obstruction prevents entry of duodenal contents (containing bile) into the stomach.
This results in loss of gastric acid (hydrochloric acid). The chloride loss results in hypochloremia which impairs the kidney’s ability to excrete bicarbonate. This is the significant factor that prevents correction of the alkalosis.
A secondary hyperaldosteronism develops due to the hypovolemia. The high aldosterone levels causes the kidneys to:
*avidly retain Na+ (to correct the intravascular volume depletion)
*excrete increased amounts of K+ into the urine (resulting in hypokalaemia).
The body’s compensatory response to the metabolic alkalosis is hypoventilation resulting in an elevated arterial pCO2.=[pp\][[\=0808i[po9il;
Infantile pyloric stenosis is typically managed with surgery; very few cases are mild enough to be treated medically.
Prior to surgery and surgery alternatives:
The danger of pyloric stenosis comes from the dehydration and electrolyte disturbance rather than the underlying problem itself. Therefore, the baby must be initially stabilized by correcting the dehydration and hypochloremic alkalosis with IV fluids. This can usually be accomplished in about 24–48 hours.
Intravenous and oral atropine may be used to treat pyloric stenosis. It has a success rate of 85-89% compared to nearly 100% for pyloromyotomy, however it requires prolonged hospitalization, skilled nursing and careful follow up during treatment. It might be an alternative to surgery in children who have contraindications for anesthesia or surgery.
The definitive treatment of pyloric stenosis is with surgical pyloromyotomy known as Ramstedt’s procedure (dividing the muscle of the pylorus to open up the gastric outlet). This is a relatively straightforward surgery that can possibly be done through a single incision (usually 3–4 cm long) or laparoscopically (through several tiny incisions), depending on the surgeon’s experience and preference. CLICK & SEE THE PICTURES
Today, the laparoscopic technique has largely supplanted the traditional open repairs which involved either a tiny circular incision around the navel or the Ramstedt procedure. Compared to the older open techniques, the complication rate is equivalent, except for a markedly lower risk of wound infection. This is now considered the standard of care at the majority of Children Hospitals across the US, although some surgeons still perform the open technique. Following repair, the small 3mm incisions are hard to see.
The vertical incision, pictured and listed above, is no longer usually required. Though many incisions have been horizontal in the past years.
Once the stomach can empty into the duodenum, feeding can commence. Some vomiting may be expected during the first days after surgery as the gastro-intestinal tract settles. Very occasionally the myotomy was incomplete and projectile vomiting continues, requiring repeat surgery. But the condition generally has no long term side-effects or impact on the child’s future.
Surgery usually provides complete relief of symptoms. The infant can usually tolerate small, frequent feedings several hours after surgery.
There are no known ways of preventing pyloric stenosis, although it is possible that breastfeeding might reduce the risk.
Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.