Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The mechanisms and consequences of Vitamin E deficiency were studied in 12 children presenting with chronic cholestasis. Preliminary results indicate that: (1) Vitamin E serum levels are lowest in children with the long-lasting cholestasis and in children in whom fat malabsorption is deepest. (2) Signs of neurologic dysfunction involving peripheral nerves, cerebellum, eye movements and retina were present in 7 children. (3) In vitro study of RBC showed increased hemolysis with oxidating agents and in physiological saline. Degree of hemolysis was inversely related to the serum level of vitamin E. (4) Increased platelet aggregation was observed in 8 patients; 6 of these also had low levels of serum Vitamin E.
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PMID:[Vitamin E and cholestasis in children]. 714 9

External biliary drainage is often required to relieve bile duct obstruction. Most often this is a temporary situation and internal drainage is soon established by means of surgery or by transhepatic biliary stents placed through the obstruction into the duodenum. When internal drainage cannot be established and external biliary losses are prolonged, malabsorption, dehydration, and electrolyte imbalance may ensue. In this report we present two such cases in which an external biliary-gastric fistula was created by connecting the biliary drainage catheter to a percutaneous endoscopic gastrostomy. In both cases internal diversion was effectively achieved. We believe this technique warrants consideration in those patients otherwise committed to prolonged external biliary drainage.
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PMID:External biliary-gastric fistula: a simple method for recycling bile. 714 97

Cholestasis associated with TPN is now diminishing in frequency. One factor is likely to be the early initiation of oral feeding. One of the effects of oral intake is to restore the enterohepatic circulation of bile acids. This in turn promotes lipid absorption, which leads to improved nutrition and a decreased in mucosal atrophy. The role of the enterohepatic cycling of bile acids has been explored in this review. In infants or children on TPN who have bile acid malabsorption, UDCA can be used to correct decreased secretion of endogenous bile acids. However, controlled studies showing that UDCA actually decreases moribidity and mortality in infants on prolonged TPN have not yet been performed and they are sorely needed. UDCA is nontoxic and acts as a natural bile acid after conjugation. An improved formulation of UDCA with rapid and efficient intestinal absorption has been developed, but the optimal formulation of UDCA is still not available. Cholylsarcosine, a new conjugated bile acid analogue that has been developed as a bile acid replacement agent, improves lipid absorption in animals with steatorrhea caused by bile acid malabsorption and intestinal resection. However, this compound has not been tested clinically.
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PMID:Defective biliary secretion during total parenteral nutrition: probable mechanisms and possible solutions. 763 79

A substantial effort has been made over the past decade to characterize the metabolism of the fat-soluble vitamins in chronic cholestasis to both improve the clinical care of affected patients and to understand the pathophysiology of the vitamin deficiency states. Cholestatic liver disease is a unique cause of fat malabsorption in which standard indices to evaluate vitamin status may be inaccurate. Thus, specific approaches to define vitamin status are being developed. Using the treatment modalities outlined in this review, fat-soluble vitamin deficiency should be a manageable problem and not lead to significant morbidity in patients with chronic cholestasis. The most subtle consequences of deficiency of each vitamin remains to be discovered.
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PMID:Fat-soluble vitamins and their importance in patients with cholestatic liver diseases. 769 27

Primary biliary cirrhosis is a chronic, usually progressive, cholestatic liver disease of presumed autoimmune etiology that affects predominantly young and middle-aged women. It is nearly always associated with an antibody directed against a component of the pyruvate dehydrogenase complex located on the inner wall of the mitochondria. The disease is associated with a number of other associated autoimmune disorders. No totally effective medical treatment has been established for the disease, although urosdeoxycholic acid appears promising. Complications of cholestasis such as fat malabsorption and fat-soluble vitamin deficiency should be excluded or corrected when found. Individual patient prognosis varies. Several models for estimating individual patient survival are available. Liver transplantation is recognized as a procedure to extend and improve the quality of life for patients with advanced disease.
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PMID:Primary biliary cirrhosis. 771 16

Idiopathic neonatal hepatitis is one of the more important causes of neonatal cholestasis. It is regarded one of the clinical presentations of 'idiopathic obstructive cholangiopathy', just like extrahepatic biliary atresia. Is it not possible to discriminate between intrahepatic and extrahepatic causes of neonatal cholestasis, or between idiopathic neonatal hepatitis and metabolic, infectious, or toxic causes, by using clinical or laboratory parameters. Liver histology is slightly more helpful: giant cell formation, focal liver necrosis, and lymphocytic and neutrophilic infiltration may be found in idiopathic neonatal hepatitis. In infectious hepatitis liver pathology mostly is only a lesser part of the symptomatology. Sporadic idiopathic neonatal hepatitis has a better prognosis than familial; about 75% of children with sporadic hepatitis experience complete recovery as compared to less than 25% of children with familial hepatitis. Therapy is confined to the prevention and treatment of complications such as itching, portal hypertension and variceal bleeding, and (fat) malabsorption.
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PMID:[Idiopathic neonatal hepatitis]. 812 25

A 4-week-old male infant was admitted to the hospital with acute gastrointestinal bleeding and marked coagulopathy secondary to vitamin K malabsorption in the presence of cholestasis. Physical examination revealed hepatomegaly and cutaneous haemangiomas. Ultrasonography, CT, and MRI demonstrated a multifocal vascular process and allowed the diagnosis of infantile hepatic haemangioendothelioma to be made without the use of more invasive diagnostic procedures. To avoid high-output congestive heart failure, the patient was treated with oral corticosteroids. After 5 months, rapid involution of the vascular malformations ensued. At age 2 years, a magnetic resonance scan confirmed complete resolution of the hepatic haemangioendothelioma.
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PMID:Imaging diagnosis and follow-up of infantile hepatic haemangioendothelioma: a case report. 815 13

Fat-soluble vitamin deficiency is known to result in various complications that may be prevented if the problem is recognized and managed appropriately. In infants and children with chronic cholestasis, replacement therapy of the fat-soluble vitamins, vitamins A, D, E, and K, may prove extremely difficult because low concentrations of intraluminal bile acids lead to malabsorption of these compounds and other fat-soluble substances. Recent progress in the use of a water-soluble form of vitamin E, d-alpha-tocopheryl polyethylene glycol-1000 succinate, has enabled correction of vitamin E-deficiency states in these patients. It has also allowed for the admixture and coadministration of other fat-soluble vitamins and compounds in d-alpha-tocopheryl polyethylene glycol-1000 succinate to enhance their absorption. For managing vitamin K deficiency, similar success has been achieved using a vitamin K compound solubilized in glycocholate and lecithin. Vitamin A deficiency has been implicated in the higher incidence of childhood mortality and morbidity in Third World countries. Increased risk of childhood cancer has recently been associated with intramuscular injection of vitamin K to newborns. Finally, it is worth noting that among the pediatric population, exclusively breastfed infants, in general, are at risk for hypovitaminosis D, and at even greater risk in the absence of adequate exposure to sunlight or when the maternal diet is not sufficient to provide for vitamin D requirements.
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PMID:Fat-soluble vitamin deficiency in infants and children. 828 79

The submitted paper is a contribution to the problem of cholestasis from the aspect of contemporary knowledge recorded in the literature as well as the author's experience assembled in a hepatological department. The author expands on individual steps of the diagnostic procedure--is it a case of cholestasis? What type of cholestasis? The most frequent causes of intrahepatic cholestasis are discussed. The main point is a summary of therapeutic possibilities, in particular in chronic forms of cholestasis. The author emphasizes that dietary provisions are always essential, a as well as treatment of itching, malabsorption and prevention of the development of osseous changes. Drugs which are considered specific therapy are mentioned and their effect and side-effects are briefly evaluated. It appears that ursodeoxycholic acid is most promising. In the terminal stage under local conditions treatment is symptomatic in future we may be able to ensure liver transplantations which are indicated in chronic cholestasis.
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PMID:[Diagnosis and therapy of cholestasis]. 830 80

Four infants with conjugated hyperbilirubinemia who were brought for treatment primarily because of a hemorrhage are reported. Underlying disorders included extrahepatic biliary atresia, choledochal cysts, and alpha 1-antitrypsin deficiency. Prodromal signs of disturbed coagulation and diminished bile excretion were not recognized. The increased bleeding tendency was probably caused by vitamin K deficiency, resulting from a combination of cholestasis-induced fat malabsorption, absence of vitamin K supplementation after birth, and low vitamin K intake as a result of breast feeding.
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PMID:Bleeding as presenting symptom of cholestasis. 841 Mar 91


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