Hirschsprungs disease, Biology

Hirschsprungs Disease (Congenital Megacolon):

This  is also known as congenital megacolon. There is absence of ganglion cells, submucus plexus  and intramural plexus  (parasympathetic ganglion cells)  in distal segment usually rectosegmoid  junction. Incidence  is one in 5000  live birth.  

It is more common in males  than in females. 

In Hirschsprungs Diseasefthere is an arrest in embroylogic development affecting the migratioon  of parasympathetic nerves (innervation) of  the intestine occuring before 12 weeks of gestation due to genetic cause. This leads to the functional obstruction of the colon due  to  lack of peristalsis  in this area  leading  to  the accumulation of feces and gas proximal to the area of defected part. Due to accumulation of feces the colon becomes distended  so  the condition  is called as megacolon.

Nursing Assessment:

Neonate will present with failure to pass meconium and abdominal distention within a day or two after birth and if  rectal examination is done by  little finger there will be an explosive leakages of accumulated meconium and gas. There may be weight loss and dehydration due to bile-stained vomiting as a result of obstruction and failure to take fluid. Neonate may develop shock. Older childern will present with obstinate constipation with abdominal distention undernutrition and anorexia. There may be diarrhoea alternating with constipation due to bacterial action which liquefies the stool. The fecal mass can be palpated in left lower portion of  abdomen and rectal examination shows empty rectum. Diagnosis is supported by barium enema which shows constricted portion of aganglionic bowel distal to the dilated part of colon and diagnosis is confirmed by rectal biopsy which shows absent ganglion cells in submucosa and intermusular plexus with or without increased number of  nerve fibres.     

Posted Date: 10/26/2012 7:46:21 AM | Location : United States







Related Discussions:- Hirschsprungs disease, Assignment Help, Ask Question on Hirschsprungs disease, Get Answer, Expert's Help, Hirschsprungs disease Discussions

Write discussion on Hirschsprungs disease
Your posts are moderated
Related Questions
Define Use of Soy Protein Concentrates in Meat products? This area probably represents the most important application of soy protein concentrates (SPC) in the food industry. SP

DISORDER OF SKELETON AND JOINTS - 1 .      Sprain - It involves a stretching or tearing of tendons or ligaments. 2.      Arthritis - It is infalmation of joints.

What are the main conventional symbols and signs used in genetic family trees? In genetic family trees the male sex is usually shown by a square and the female by a circle. Cro

In a plasma membrane, which of the below provides a general barrier to the movement of molecules? a) Lipids b) Proteins c) Carbohydrates d) all of these Answer: a

Chest Drainage Tubes (24-72 hours) Continue milking of chest tube hourly.  Monitor the amount, colour and record on the flow chart.  Assess dressing for any soak

Phototherapy Unit Principle Photoisomerisation and photo-oxidation changes indirect bilirubin into water soluble substance. Operational Instruction Adjust

Explain Micafungin It is an echinocandin indicated for treatment of esophageal candidiasis and prophylaxis of invasive Candida infections in patients undergoing hematopoietic s

Explain about the Infantile beriberi? Infantile beriberi is commonly seen in many South-East Asian countries where the diets consist mostly of "polished rice" and are deficient

Rapid Crawling and Swimming Rapid Crawling This movement depends upon mainly on the contralateral waves of contraction of the longitudinal muscles of the body wall that

Structure and Fate of Endosperm The cells of endosperm are usually thin-walled, large isodiametric and devoid of pits and store large amount of food materials. The starch alo