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Showing posts from May, 2022

Chronic Intestinal Pseudo-Obstruction (CIP)

What is Chronic Intestinal Pseudo-Obstruction (CIP)? Chronic intestinal pseudo-obstruction (CIP) is a rare disorder of gastrointestinal motility where coordinated contractions (peristalsis) in the intestinal tract become altered and inefficient. When this happens, nutritional requirements cannot be adequately met. "Motility" is the term used to describe the contraction of muscles in the gastrointestinal (GI) tract. To facilitate normal digestion and the absorption of nutrients, food content is propelled through the GI tract by synchronized or coordinated contractions in a process called "peristalsis." Peristalsis is dependent upon the coordination between the muscles, nerves, and hormones in the digestive tract. In CIP the intestines react as if there is a true mechanical obstruction or blockage. However, when tests are performed, no physical evidence of blockage is found. There is no unique sign or symptom that allows a positive diagnosis of the pseudo-obstruction....

Churg-Strauss syndrome

Churg-Strauss syndrome is a disorder marked by blood vessel inflammation. This inflammation can restrict blood flow to organs and tissues, sometimes permanently damaging them. This condition is also known as eosinophilic granulomatosis with polyangiitis (EGPA). Adult-onset asthma is the most common sign of Churg-Strauss syndrome. The disorder can also cause other problems, such as nasal allergies, sinus problems, rash, gastrointestinal bleeding, and pain and numbness in your hands and feet. Churg-Strauss syndrome is rare and has no cure. Symptoms can usually be controlled with steroids and other powerful immunosuppressant drugs. Symptoms Churg-Strauss syndrome varies greatly from person to person. Some people have only mild symptoms. Others have severe or life-threatening complications. Also known as EGPA, the syndrome tends to occur in three stages and gets progressively worse. Almost everyone with the condition has asthma, chronic sinusitis and elevated counts of white blood cells ca...

Aortic dissection

Overview An aortic dissection is a serious condition in which a tear occurs in the inner layer of the body's main artery (aorta). Blood rushes through the tear, causing the inner and middle layers of the aorta to split (dissect). If the blood goes through the outside aortic wall, aortic dissection is often deadly. Aortic dissection is relatively uncommon. It usually occurs in men in their 60s and 70s. Symptoms of aortic dissection may mimic those of other diseases, often leading to delays in diagnosis. However, when an aortic dissection is detected early and treated promptly, the chance of survival greatly improves. different types of aortic dissection There are two main types: Stanford Type A Aortic Dissection : This type of dissection occurs in the first part of the aorta, closer to the heart, and can be immediately life-threatening. It usually requires emergency open chest surgery to repair or replace the first segment of the aorta where the tear started (ascending aorta +/- the...

Charge Syndrome

Overview CHARGE syndrome is a recognizable genetic syndrome with known pattern of features. It is an extremely complex syndrome, involving extensive medical and physical difficulties that differ from child to child. CHARGE syndrome is correlated with genetic mutation to CHD7 and the prevalence of CHARGE syndrome is 1:10,000-1:15,000 live births. Babies with CHARGE syndrome are often born with life-threatening birth defects. They spend many months in the hospital and undergo many surgeries and other treatments. Swallowing and breathing problems make life difficult even when they come home. Most have hearing two little girls sitting on a carpet, one girl has a trach and is biting her finger.loss, vision loss, and balance problems that delay their development and communication. Despite these seemingly insurmountable obstacles, children with CHARGE syndrome often far surpass their medical, physical, educational, and social expectations. One of the hidden features of CHARGE syndrome is the ...

Chronic Bronchitis

Overview Coughing is a way for your body to get rid of harmful things in your lungs. But coughing too much can be bad, too. If you’ve had a cough that’s gone on for what feels like forever, you might have a serious condition called chronic bronchitis. That’s when the air tubes in your lungs called bronchi get irritated and inflamed, and you have coughs for at least 3 months a year for 2 years in a row. It’s a long-term illness that keeps coming back or never fully goes away. It’s a type of chronic obstructive pulmonary disease (COPD). The other type is emphysema. Symptoms Chronic bronchitis fills your airways with thick mucus. The small hairs that normally move phlegm out of your lungs are damaged. That makes you cough. As the disease goes on, it’s harder for you to breathe. Other signs of chronic bronchitis may include: Cough, often with mucus Wheezing Tight chest Shortness of breath Feeling tired Your symptoms may be worst in the winter, when humidity and temperatures drop. Causes Ci...

Tricho-hepato-enteric Syndrome

Description Trichohepatoenteric syndrome is a condition that affects the hair (tricho-), liver (hepato-), and intestines (enteric), as well as other tissues and organs in the body. This condition is also known as syndromic diarrhea because chronic, difficult-to-treat diarrhea is one of its major features. Within the first few weeks of life, affected infants develop watery diarrhea that occurs multiple times per day. Even with nutritional support through intravenous feedings (parenteral nutrition), many of these children experience failure to thrive, which means they do not gain weight or grow at the expected rate. Most children with trichohepatoenteric syndrome are small at birth, and they remain shorter than their peers throughout life. Abnormal hair is another feature of trichohepatoenteric syndrome. Hair in affected individuals is described as wooly, brittle, patchy, and easily pulled out. Under a microscope, some strands of hair can be seen to vary in diameter, with thicker and thi...