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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Some infants with biliary atresia obtain dramatic improvement for prolonged periods after the performance of hepatic portoenterostomy. Such infants may have life styles not substantially different from those of normal children. In others, the benefit from this operation, if any, is short lived. These infants are very vulnerable to the debilitating effects of severe, prolonged malabsorption and ultimately require orthotopic liver transplantation to sustain life. The physician caring for infants awaiting liver transplantation can do much, not only to prolong survival but to maintain satisfactory growth and development. The key consideration is to provide adequate nitrogen and nonnitrogen calories, liberally utilizing modern methods of enteral alimentation when necessary. In addition, attention must be directed toward several vitamin and mineral deficiencies, particularly those of the fat-soluble vitamins, that inevitably accompany severe malabsorption in children. Management of extrahepatic biliary atresia in infants is difficult and requires meticulous attention to details. Nevertheless, the long-term cure of this disorder provided by liver transplantation makes their care a rewarding experience.
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PMID:Nutritional support for the infant with extrahepatic biliary atresia. 310 6

Four cases of extrahepatic biliary atresia are reported. Each of these infants presented with haemorrhagic phenomena rather than with prolonged jaundice. The increased bleeding tendency was due to a vitamin K deficiency, probably caused by cholestasis-induced malabsorption. Therefore extrahepatic biliary atresia should be considered in each infant with a bleeding diathesis.
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PMID:Bleeding as the first symptom of extrahepatic biliary atresia. 349 31

Mammalian reoviruses are connected with a variety of humans diseases, including gastroenteritis, malabsorption and hepatitis. Recently, reovirus-3 was found to be associated with neonatal biliary atresia. We describe a technique for the rapid isolation and identification of reovirus-3. Mouse fibroblasts (L 929 cells) were grown in monolayers in a RPMI 1640 medium containing 10% calf serum. The cytopathic effects were visualized by the rounding of the L 929 cells and the appearance of fine granulation in the cytoplasm 48 h after the infection. Hematoxylin-eosin staining showed swelling and rounding of the infected cells, diminished chromatin in the nuclei, and the absence of mitoses. The immunohistochemical staining by the avidin-biotin-peroxidase technique was positive in the infected monolayers of the L 929 cells. The positive staining was limited to cytoplasmic inclusions, which were surrounded by a halo and sometimes by vacuoles. We conclude that the described technique is useful for the rapid isolation and identification of reovirus-3.
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PMID:Rapid isolation and identification of reovirus-3. 368 Apr 64

The clinical, biochemical, and histological features of 27 children with syndromic paucity of the interlobular bile ducts are described. All presented in the first 5 months of life, 21 with jaundice, two with spontaneous bleeding due to vitamin K malabsorption in addition to jaundice, two with pruritus, and two with failure to thrive. Interlobular bile ducts were abundant in liver biopsies from five (18% of cases) in the first 6 months of life. The degree of portal fibrosis and cellular infiltrate was mild in all except three patients. Clinically significant heart lesions occurred in 52% but only 22% had peripheral pulmonary stenosis. Characteristic facial appearances were present in only 70%; embryotoxon and vertebral anomalies were present in 56 and 33%, respectively. Two infants died of cardiovascular complications, one of alimentary bleeding and one of progressive liver disease. Complications of vitamin K deficiency occurred in 15%, vitamin D deficiency in 30%, and vitamin E deficiency in 37%. Survivors at ages of 19 months to 16.5 years had considerable morbidity with pruritus occurring in 70%, jaundice in 48%, xanthomas in 30%, 74% having hepatomegaly and 63% splenomegaly. All had abnormal biochemical tests of liver function, 90% had growth retardation, and 50% developmental delay. We conclude that differentiation from extrahepatic biliary atresia can be difficult if biliary flow cannot be demonstrated. Prevention of fat-soluble vitamin deficiency is essential. Further research is required to decrease the morbidity associated with this syndrome in infancy.
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PMID:Syndromic paucity of the intrahepatic bile ducts: diagnostic difficulty; severe morbidity throughout early childhood. 368 72

Bone disease and low serum levels of 25-hydroxyvitamin D are prevalent in cholestatic syndromes such as primary biliary cirrhosis and biliary atresia. Defective hydroxylation, along with malabsorption of vitamin D, could be a factor in 25-hydroxyvitamin D depletion. To assess hepatic hydroxylation during experimental cholestasis, we studied vitamin D 25-hydroxylase activity in liver homogenates of rats after 7, 14, and 21 days of bile duct ligation. We have also studied the effects of bile acids on this enzyme in vitro. Hepatic 25-hydroxylation was depressed after 7 days ligation in only 1 of 4 animals, but by 14 days, all animals showed a marked reduction with a mean decrease of 64% in specific activity. Total liver enzyme activity was reduced by 43% at 14 days. In the ligated animals, liver histology showed progressive bile stasis, focal necrosis, bile ductular proliferation, periductular and periportal inflammation, and fibrosis. Addition of bile acids to the in vitro assay in concentrations approximating those found in cholestasis produced marked inhibition of vitamin D 25-hydroxylase activity.
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PMID:Hepatic vitamin D 25-hydroxylase: inhibition by bile duct ligation or bile salts. 697 44

Two adults are described who developed a progressive neurological disorder more than 20 years after the onset of chronic fat malabsorption. The clinical features included dysarthria, cerebellar ataxia, and prominent proprioceptive loss with depressed or absent tendon reflexes. Serum vitamin E was undetectable in both cases. One patient improved clinically and electrophysiologically after oral therapy with vitamin E. The findings in these patients were similar to those in others recently reported with vitamin E deficiency associated with biliary atresia. Electrophysiological observations suggested that the human deficiency state parallels that found neuropathologically in vitamin E-deficient animals.
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PMID:Spinocerebellar degeneration secondary to chronic intestinal malabsorption: a vitamin E deficiency syndrome. 718 49

In 656 patients aged from one to 39 years, the incidence of axonal dystrophy in the gracile nucleus (ADG) is correlated with underlying diseases. The age-related incidence of ADG (minimal to severe) in these patients, 13, 53, 76 and 97% in the first, second, third, and fourth decades, respectively, is comparable to that observed by other investigators. The incidence drops to 8, 16, 31, and 60% when more than five spheroids in each gracile nucleus are taken into account. Diseases with which ADG is frequently associated vary according to the patient's age. Congenital biliary atresia and cystic fibrosis account for 71% of cases of ADG (mild to severe) in the first decade, while cystic fibrosis and malignancies account for 38 and 35% in the second decade, respectively. In the third decade, malignancies, renal diseases, cystic fibrosis, and heart diseases account for 30, 14, 12, and 12% of ADG cases, respectively, while malignancies, renal diseases, heart diseases, and diseases of digestive organs account for 30, 24, 15, and 11% in the fourth decade, respectively. The incidence of ADG also increases progressively with age, duration of the clinical course in the individual disease, or both, regardless of types of the diseases. This fact, when viewed together with the precocious development of severe ADG in patients with the malabsorption syndromes and in those with grave illnesses with protracted course leading to cachexia, seems to indicate that malnutrition represents a common factor correlated with severe ADG in the young patients. It seems to be premature at this time to disregard the hypothetical question that ADG in man is in some way related to deficiency or altered metabolism of vitamin E.
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PMID:Axonal dystrophy in the gracile nucleus in children and young adults. Reappraisal of the incidence and associated diseases. 720 26

In 63 patients with malabsorption syndromes, 16 with congenital biliary atresia (BA) and 47 with cystic fibrosis (CF), axonal dystrophy in the gracile nucleus (ADG) was studied. Of the 16 patients with BA, ADG of considerable severity was observed in all 10 over one year of age. Of the 47 patients with CF, it was observed in 32, 61 and 80% of the cases in the first, second, and third decades, respectively. Evidence is presented that there has been a substantial decrease in the incidence of ADG in CF patients in recent years and that the decreased incidence is attributable to vitamin E (Aquasol E) therapy. The beneficial effect of vitamin E supplementation in CF patients is proffered as strong evidence that ADG in BA and CF is related to vitamin E deficiency. The present study indicates that BA and CF patients require vitamin E supplementation to maintain a normal integrity of axons related to the gracile and perhaps other sensory nuclei. Critical neurological evaluation for possible dysfunction of the sensory nuclei in these patients with malabsorption syndromes is advised.
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PMID:Axonal dystrophy in the gracile nucleus in congenital biliary atresia and cystic fibrosis (mucoviscidosis): beneficial effect of vitamin E therapy. 721

Docosahexaenoic acid (DHA) is believed to be an important long-chain polyunsaturated fatty acid (LCPUFA), which may be essential for neurofunction in infants. Patients with extrahepatic biliary atresia (EBA) may have DHA deficiency secondary to fat malabsorption. The authors investigated DHA and other LCPUFA levels in plasma and red blood cell (RBC) phospholipids of patients after the Kasai portoenterostomy and after supplementation with essential fatty acids. Ten children aged 8 to 17 months (mean, 12.6 months) comprised the study group. Five were jaundiced and five had a normal bilirubin level. The patients received 1 mL/kg of fat emulsions (10% Intralipid, containing 50% linoleic acid and 9% alpha-linolenic acid) in addition to an age-appropriate diet. Additional supplements were ursodeoxycholic acid (UDCA) (15 mg/kg/d) and taurine (100 mg/kg/d). The percentages of DHA in both plasma and RBC phospholipids of patients in the jaundiced group were significantly lower than those of normal children. Patients in the jaundice-free group had significantly lower levels of DHA and higher levels of linoleic acid in both plasma and RBC phospholipids in comparison to the normal group. This study shows that postoperative EBA patient become DHA-deficient even when supplemented with fat emulsions (largely composed of linoleic acid) that contain DHA's precursor, alpha-linolenic acid. This demonstrates a deficiency in the long-chain acid desaturase activity of these patients. It is recommended that excessive/linoleic acid intake be avoided and that all EBA patients have small amounts of DHA added to their lipid supplementation.
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PMID:Docosahexaenoic acid status of patients with extrahepatic biliary atresia. 784 20

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


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