Congenital and Genetic Abnormalities

Pancreas Division (Dominal Dorsal Duct Syndrome): - This problem occurs in up to 10% of the whole population. It results from dorsal and ventral pancreas to unite. Consequently most of the pancreas is drained through accessory papilla that is proximal to papilla of Vater. These types of patients are more prone to pancreatitis for condition appears to be associated with stenosis of opening of one or other papilla.
Diagnosis in made via endoscopic retrograde cholangiopancreatography (ERCP).

Annular Pancreas: - Ventral pancreas surrounds the second part of duodenum and it is frequently associated with other congenital defects such as mal-rotation of intestine, artesian and cardiac defects. Pancreas may constrict duodenum causing obstruction soon after the birth or in adult life. This may predispose to duodenal ulcer and acute pancreatitis. Diagnosis is made by barium studies and treatment is surgical bypass of constriction.

Ectopic Pancreatic Tissue: - It may occur in gastric antrum or duodenum and it takes the form of smooth nodule. It is normally asymptomatic and it does not require any treatment. If it is found coincidentally during surgery, the nodule is excised for it is subject to same conditions including carcinoma, as the pancreas itself.

Pathology: - The pathological findings and clinical features result from obstruction by abnormal viscid secretion of the ducts in salivary glands, digestive and biliary tracts and pancreas causing atrophy
Clinical Features: - The exocrine insufficiency leading to steatorrhoea and diarrhea is almost universal in childhood. If patient survives to adulthood, malabsortion tends to be less troublesome. The intestinal obstruction, commonly called “meconium ileus equivalent”, may occur in the children and in the adults, caused by inspissated food and secretions. Rectal prolapse is frequent in children because of the large bulky stools and frequent coughing. The recurrent episodes of the acute pancreatitis may also occur. The incidence of the diabetes mellitus increases with the age. Chronic pancreatitis may supervene. The disease of liver or biliary tract may occur.
Investigation: - The gastrointestinal problems are investigated according to suspected problems. There may be disorder in the gut epithelium and pancreas. 80% of the patients with cystic fibrosis have both pulmonary disease and pancreatic disease. But 15% patients have lung sepsis with clearly normal pancreatic function.

Management: - Optimal treatment in adolescent and adult depends on a team approach to the complicated respiratory, nutritional and hepatobiliary problems. Many treatment centers have established special clinics for such kind of patients with specialist care. Read more…

By: HealthGuy  :  Filed Under Diseases & Conditions, Health Tips

Cholestatic Jaundice – A Major Symptom of Liver Ailment

The symptom called ‘Jaundice’ refer to yellow appearance of the skin, sclera and the mucous membranes resulting from the increased bilirubin concentration in body fluids. It is detectable while the plasma bilirubin exceeds 50µm o1/1(3mg/dl). The internal tissues and the body fluids are colored yellow but not the brain since bilirubin does not cross blood-brain barrier other than in immediate neonatal period.
Cholestasis is the failure of bile flow, and the cause may lie anywhere between hepatocyte and duodenum. The jaundice becomes gradually severe in the unrelieved cholestasis because the conjugated bilirubin is unable to enter bile canaliculi and passes back into blood. It happens also because there is the failure of the clearance of un-conjugated bilirubin arriving in liver cells.
Aetiology: - Cholestasis may be because of failure of the hepatocytes to generate the bile flow, to the obstruction to bile flow in bile ducts in portal tracks, or to the obstruction to bile flow in extra-hepatic bile ducts between porta hepatic and papilla of Vater. The cause of cholestasis may operate at more than one of these levels. Those confined to extra-hepatic bile ducts can be amenable to the surgical correction.
Clinical Feature: - The clinical features in the cholestasic jaundice include those to the cholestasis itself and to the development of the cholangitis consequent on biliary obstruction. Jaundice is very variable. It may be static or may fluctuate. If it is prolonged and severer; it may give the skin a greenish look. Stools are pale or clay colored for the deficiency of pigment derived from bilirubin. The urine is dark from renal excretion of conjugated bilirubin. Many patients may have pruritus generated and the accessible parts of the body may also show scratch marks. The lipid deposits may develop in skin during prolonged cholestatic jaundice. Most of them are xanthelasmas on eyelids but sometimes xanthomas also occur. Prolonged cholestasis may also cause marked mal absorption leading to weight loss, severe steatorrhoea, and hemorrhagic tendency because of Vitamin K deficiency associated with the calcium and vitamin D deficiency. Clinical and biochemical evidence of the biliary cirrhosis and hepatocellular failure occur eventually. Cholangitis, due to the infection with or without rigors and the coincident pain and the hepatic tenderness may indicate the development of the hepatic abscess. None of the features are pathognomonic of any particular cause, but are more likely to be in some diseases than the others. Read more…

By: HealthGuy  :  Filed Under Disabilities, Uncategorized