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The Sixth Element

Quadriplegia

Quadriplegia is a neurological condition which affects the upper and lower body of the affected individual, including all 4 limbs. The condition is also commonly called Tetraplegia. The name comes from the Latin word “quattuor” (or Greek word “tetra”) meaning 4 and the Greek word “plege” meaning stroke. Full body paralysis is another name for the quadriplegic condition.

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Filed under: Medical World, Quadriplegia , , ,

Gestational Diabetes

Introduction

Gestational diabetes (or gestational diabetes mellitus, GDM) is a condition in which women without previously diagnosed diabetes exhibit high blood glucose levels during pregnancy.

Gestational diabetes generally has few symptoms and it is most commonly diagnosed by screening during pregnancy. Diagnostic tests detect inappropriately high levels of glucose in blood samples. Gestational diabetes affects 3-10% of pregnancies, depending on the population studied.No specific cause has been identified, but it is believed that the hormones produced during pregnancy increase a woman’s resistance to insulin, resulting in impaired glucose tolerance.

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Situs Inverus – A Rare medical challenge

Definition190px-situs_inversus_-_mirrored_heart_and_lungs

Situs inversus is a condition in which the organs of the chest and abdomen are arranged in a perfect mirror image reversal of the normal positioning.

Description

Normal human development results in an asymmetrical arrangement of the organs within the chest and abdomen. Typically, the heart lies on the left side of the body (levocardia), the liver and spleen lie on the right, and the lung on the left has two lobes while the lung on the right has three lobes. This normal arrangement is known as situs solitus.
However, in about 1 in 8,500 people, the organs of the chest and abdomen are arranged in the exact opposite position: the heart is on the right (dextrocardia), as is the two-lobed lung, and the liver, spleen, and three-lobed lung are on the left. Yet because this arrangement, called situs inversus, is a perfect mirror image, the relationship between the organs is not changed, so functional problems rarely occur. Read the rest of this entry »

Filed under: Medical World, Situs Inverus - A Rare medical challenge

SMART MEDICINE

Intelligent pill’ knows where to release drug

Dutch group Philips has developed an “intelligent pill” that contains a microprocessor, battery, wireless radio, pump and a drug reservoir to release medication in a specific area in the body. Philips, one of the world’s biggest hospital equipment makers, said on Tuesday that the “iPill” capsule, measures acidity with a sensor to determine its location in the gut, and can then release drugs where they are needed. Delivering drugs to treat digestive tract disorders such as Crohn’s disease directly to the location of the disease means doses can be lower, reducing side effects, Philips said. While capsules containing miniature cameras are already used as diagnostic tools, those lack the ability to deliver drugs, Philips said. The “iPill” can also measure the local temperature and report it wirelessly to an external receiver

Filed under: Medical World, SMART MEDICINE

Primary Pulmonary hypertension

What is Pulmonary Hypertension?

The human body has two major areas of blood vessels that distribute from and return blood to the left and right heart. The portion of the circulation that distributes the blood from the left side of the heart, throughout the body, is referred to as systemic circulation. The portion of the circulation that distributes the blood from the right side of the heart, to the lungs, is referred to as the pulmonary (lung) circulation. When the doctor or a nurse measures the blood pressure on a person’s arm, it is the systemic blood pressure that is being measured. When these pressures are abnormally high, the person is diagnosed as having high blood pressure or hypertension.

The right ventricle pumps venous blood returning from the body into the arteries of the lungs to receive oxygen. The pressures in the lung arteries (pulmonary arteries) are normally lower than the pressures in the systemic circulation. When pressure in the pulmonary circulation becomes abnormally elevated, it is referred to as pulmonary hypertension.
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Filed under: Medical World, Primary Pulmonary hypertension ,

Long QT Syndrome

Recent report in Mumbai Mirror prompted me to post this. Click here to read the full story

Definition

Long QT syndrome or LQTS is a heart rhythm disorder that can potentially causes fast, chaotic heartbeats. The rapid heartbeats, caused by changes in the part of the heart that causes it to beat, may lead to fainting. In some cases the hearts rhythm may beat so erratically that it can cause sudden death. Read the rest of this entry »

Filed under: Long QT Syndrome, Medical World , , , , , , , , , ,

Parkinsons Disease

What is Parkinson’s Disease?

Parkinsons Disease (PD) is a progressive, neurodegenartive, movement disorder.

Progressive:- PD gets worse over time

Neurodegenerative:- It is caused by the degeneration of the cells in brain.

Movement Disorder:- The most prominent symptoms of PD affect movement, although many other symptoms may also occur, some of which can be even more disabling than the movement symptoms. Read the rest of this entry »

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Arnold Chiari Malformation

Arnold Chiari Malformation is a malformation of the brain. It consists of a downward displacement of the cerebellar tonsils and the medulla through the foramen magnum, sometimes causing hydrocephalus as a result of obstruction of CSF (cerebrospinal fluid) outflow.

Terminology

Some sources use the term “Chiari Malformation” to describe four specific grades of the condition reserving the term “Arnold-Chiari” for type II only. Other sources use “Arnold Chiari” for all four types. This article uses the latter convention. One advantage of using the term “Arnold Chiari” is that the term “Chiari’s Syndrome” can refer to Budd-Chiari syndrome a hepatic conditon also named after Hans Chiari

What is Chiari Malformation?

An image showing a brain affected the chiari malformation image courtesy braincatalognucleusinc.com

An image showing a brain affected the chiari malformation image courtesy braincatalognucleusinc.com

Chiari Malformations (CMs) are structural defects in the cerebellum, the part of the brain that controls balance. When indented bony space at the lower rear of the skull is smaller than normal, the cerebellum and brainstem can be pushed downward. The resulting pressure on the cerebellum can block the flow cerebrospinal fluid (the liquid that surrounds and protects the brain and spinal cord) and can cause a range of symptoms including dizziness, muscle weakness, numbness, vision problems, headache and problems with balance and coordination. There are three primary types of CMs:- Type I :- Most common. It may not cause symptoms and is often found by accident during an examination for another condition. Type II :-Also called Arnold Chiari Malformation is usually accompanied by a myelomeningocele- a form of spina bifida that occurs when the spinal canal and backbone donot close before birth, causing the spinal cord to protrude through an opening in the back. This can cause either partial or complete paralysis below the spinal opening in the back. Type III :- This is the most serious form of CM, and causes severe neurological defects. Other conditons sometimes associated with CM include hydrocephalus, syringomyelia and spinal curvature.

History

Hans chiari, an austrian pathologist first described these hindbrain malformations in the 1890s Image courtesy weborionpharma.se

An Austiran pathologist, Hans Chiari, first described these hindbrain malformations in the 1890s. A colleague of Professor Chiari, Dr. Julius Arnold, later contributed to the definiton of the condition, and students of Dr. Arnold (Schwalbe and Gredig) suggested the term “Arnold – Chiari Malformation” to henceforth refer to the condition. Between 1891 and 1896, German pathologist Hans Chiari described a series of anomalies of the caudal cerebellum and brainstem on the basis of autopsy observations. In 1891, he described an anomaly consisting of elongated peglike cerebellar tonsils that are displaced into the upper cervical canal through the foramen magnum. This is now designated as the Chiari Type I malformation. Five years later, he published a further report on a hindbrain anomaly, now known as the Chiari type II malformation. He also reported a single case of cervical spina bifida associated with herniation of the cerebellum through the foramen magnum, which has since been called Chairi III malformation. Some authors have added a form of severe cerebellar hypoplasia without displacement of brain through the foramen magnum, the so-called Chiari IV malformation. The Chiari II is a complex congenital malformation of the brain, nearly always associated with myelomeningocele. This condition includes downward displacement of the medulla, fourth ventricle and cerebellum into the cervical spinal canal as well as elongation of the pons and fourth ventricle probably due to a relatively small posterior fossa

Mortality / Morbidity

Neonatal Chiari II malformation continue to result in significant morbidity and mortality. Hindbrain dysfunction is the major cause of the mortality. The mortality rate is 15% in first years of life among the patients with Chiari II malformation. Symptomic Chiari II malformation is the leading cause of mortality in the myelodysplastic population. One third of the patients with myelomeningocele develop brainstem dysfunction by the age of 5 years. Of these one third die in infancy. Cranial nerve and brainstem dysfunction are the most serious and potentially life threatening problems. Respiratory difficulties occur in about 29-76 percent of patients; which are the most common and leathal manifestation of the conditon.

Preferred Examination

The Chiari II malformation is a complex anomaly with skull, dural, brain, spinal and spinal cord maifestations. Traditionally, when suggestions and symptoms were suggestive of a Chiari II malformation, plain radiography of the head or spine was performed, followed by myelography. Because myelography is a invasive procedure, clinicians were reluctant to perform the test until the severity of the symptoms warranted it. The introduction of modern imaging techniques, specifically, MRI, has radically changed the evaluation of symptoms referable to the brain and spinal cord. MRI is usually used for the detailed evaluation of lesions and complications due to Chiari II malformations. Chiari II malformations are also diagnosed with the help of CT scan and UltraSonography. CT is especially useful after the neonatal period in following up obstructive hydrocephalus in infants who have undergone a ventriculoperitoneal shunt procedure. Ultrasonography or US, is routinely used during gestation for screening purposes and in the neonatal period for diagnosis and follow up of hydrocephalus

Treatment

Once symptomatic onset occurs, a common treatment is decompression surgery in which a nuerosurgeon usually removes the first and part of the second and sometimes third crevical vertebrae and part of the occipital bone of the skull to allow the flow of spinal fluid and may be accompanied by a shunt. This treatment is well recognised and accepted. Medications may ease certain symptoms, such as pain but surgery is the only treatment.

Source:-

Filed under: Arnold Chiari Malformation, Medical World

Alzheimer’s Disease – An Unsolved Challenge

Alzheimer’s disease (AD), also called Alzheimer Disease or simply Alzheimer’s is the most common cause of dementia. It is a degenerative and terminal disease for which there is no known cure. In its most common form, it afflicts indviduals over 65 years old, although a less prevalent early onset form also exists. Read the rest of this entry »

Filed under: Alzheirmers Disease, Medical World , , , ,

Ectopia Cordis – A rare congenital malformation

- TheFetus.Net

A Baby affected with ectopia cordis Image Courtesy:- TheFetus.Net

Ectopia cordis is a rare congenital malformation in which the heart is located partially or totally outside the thoracic cavity. It may occur as an isolated malformation or it may be associated with a larger category of verntral body wall defects that affect the thorax, abdomen or both. This is a very rare anomaly with an estimated prevalance of 0.079/10000 births and may occur more frequently in females. The prenatal diagnosis is easliy made with ultrasound by visualizing the heart outside the thoracic cavity. There are four types of ectopia cordis according to the position of the heart:-

  1. Anterior to the sternum (thorcacic)
  2. Between thorax and abdomen (thoracoabdominal)
  3. Within abdomen (Abdominal)
  4. In the neck (Crevical)

The thoracic and thoracoabdominal types are most common followed by the abdominal and crevical types. The combination of thoracoabdominal ectopia cordis, lower sternal defect, anterior diaphragmatic hernia, midline supraumblical defect along with pericardial and intracardiac defects constitue the Pentalogy of Cantrell

Pathogenesis

- PubMedCentral

Thoracic Ectopia Cordis Image Courtesy:- PubMedCentral

Embryologically, the development of the ventral body wall begins after the eigth day of embryonic life with differentiation and proliferation of a mesodermal layer between the trophoblast and the endoderm. Lateral migration of the paired mesoderm then occurs. At about 16 days, the mesoderm splits laterally into an outer somatic layer and an inner splachnic layer with the coelomic cavity interspaced between. Ventromedial folding of the paired mesodermal layers gives septum transversum of the diaphragm, pericardium and epimyocardium (splanchnic mesoderm). The heart originally develops in a very cephalad location and reaches its definitive position by the lateral folding and ventral flexing of the embryo at about 16- 17 days of development. Midline fusion and formation of the thoracic and abdominal cavities is complete by the 9th embryonic weeks. Failure, either complete or partial, of midline fusion at this embroynic stage can result ina variety of disorders ranging from isolated ectopia cordis to complete ventral evisceration. Earlyl rupture of the chorion and /or yolk sac causing compression of the thorax preventing midline fusion has been proposed as mechinal cause of these defects as has early rupture of the amnion with formation of fibrous amniotic bands. Ectopia cordis has been attributed to intrauterine drug exposure in animal models, a finding which has not been confirmed in human beings.

Associated Anomalies

Ectopia cordis is frequently associated with other congential defects involving multiple organ systems. Ventricular Septal Defect and Tetralogy of Fallot are the most common intracardiac defects while omphalocele is the most common abdominal wall defect associated with ectopia cordis. There is some slim evidence that ectopia cordis may be related to Turner Syndrome. Chromosomal Anomalies like Trisomy 18 have also been observed in some children with ectopia cordis. In general, however, there is no recognizable cause for this conditon.

Treatment

Sadly, most cases of ectopia cordis result in stillbirth or death shortly after birth. A few case of ectopia cordis have been treated successfully, but this defect still poses the biggest challenge and rate of failure for pediatric cardiothoracic surgeons. Most cases are identified through routine prenatal ultrasound. When a case is suspected, the mother will be referred to a pediatric or fetal echocardiologist who will confirm the diagnosis. Attempts of surgical correction have been largely unsuccessful because of the extent of the associated anomalies. Surgical management includes immediate covering of the exposed heart and abdominal contents with a silastic prosthesis, as well as full evaluation and correction of any intracardiac defects prior to replacement of abdominal contents. The procedure often fails secondary to kinking of the great vessels or mechanical compression of the heart resulting in decreased cardiac output. Advances in the fetal ultrasound techniques have aided in early recogniton of such anomalies and termination of pregnancy prior to viablility should be considered and discussed with parents.

Sources:-

  1. Wikipedia
  2. TheFetus. Net (Images)
  3. WiseGeek
  4. PUBMed Central (Images)
  5. Interactive Cardio Vascular and Thorcacic Surgery (Images)
  6. Journal of Indian Association of Pediatric Surgeons
  7. Humpath.com (Images)
  8. BioPortifolio.com (Images)
  9. Home and Garden WebShots.com (Images)
  10. Childrens hospital of Wiscosin
  11. Medical Dictionary (For definitions of medical terms)

Filed under: Ectopia Cordis - A rare congenital malformation, Medical World ,

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