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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The historical term "hemorrhagic disease of the newborn," which is used as a synonym for vitamin K deficiency bleeding (VKDB) in infancy, preferably should be abandoned, since neonatal bleeding is often not due to vitamin K (VK) deficiency and VKDB may occur after the neonatal period. VKDB is a form of bleeding that is caused by reduced activity of VK-dependent coagulation factors (II, VII, IX, X), has normal or even increased activity of VK-independent coagulation factors, and responds to VK. Acarboxy proteins are present. In a bleeding infant a prolonged one-stage prothrombin time (a decreased Quick value, which means prothrombin time expressed as percent of normal) in association with a normal (or increased) fibrinogen level and platelet count is almost diagnostic of VKDB. The diagnosis is proven, if administration of VK is followed by a shortening of the prothrombin time (after only 30 minutes) or cessation of bleeding. Classification is by age of onset as early, classic, and late form of VKDB. Rare cases of VKDB occur also after week 15; therefore the upper age limit should be 6 months and not 3 months. In idiopathic VKDB the cause (ther than breast-feeding) is unknown. In secondary VKDB additional factors can be demonstrated, such as poor intake or absorption of VK and increased consumption of VK. Most often cholestasis is present. Postnatal VK provides effective protection from the classic and late form of VKDB. The classic form can be prevented equally well by a single oral dose of 1 mg VK after birth as by parenteral VK. Parenteral prophylaxis appears to be more effective in preventing the late form, especially in patients with hepatobiliary disease. The protection achieved by single oral prophylaxis can be improved by repeated oral administration. In addition to reducing the incidence of VKDB, VK prophylaxis also reduced the proportion of intracranial hemorrhages and increases the age at onset of bleeding, parenteral prophylaxis being more effective than single oral prophylaxis in this respect. Because of the potential risks (cancer?) associated with extremely high levels of VK (20,000-fold the normal value for healthy newborns) and the possibility of injection injury, parenteral VK has been questioned as the first choice of prophylaxis for normal neonates. These disadvantages do not apply to oral prophylaxis. The major disadvantage of oral prophylaxis, namely, its lesser reliability in terms of intake and absorption, could be largely overcome by repeated administration. Although VK prophylaxis seems to be necessary only for breast-fed infants, breast-feeding should be promoted. As VK is involved not only in coagulation but also in carboxylation with multiple effects, care should be taken to avoid any excessive deviation from the physiologic conditions, that prevail in the fully breast-fed healthy mature infant, with low VK levels in the postnatal period. Repeated (daily or weekly) oral doses of VK are closer to physiologic conditions than single parenteral bolus doses, which expose neonates to excessively high VK levels. The incidence of intracranial VKDB can be reduced if the grave significance of warning signs is recognized (icterus, failure to thrive, feeding problems, minor, bleeding, diseases with cholestasis) or if a simple test for acarboxy proteins (similar to the Guthrie test) would be applicable. Whether or not the more reliable absorption of the new mixed micellar preparation of VK could reduce the protective oral dose of VK prophylaxis has to be evaluated.
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PMID:Vitamin K deficiency bleeding in infants and children. 858 59

A 7-week old, male infant died from an intracerebral haemorrhage due to vitamin K deficiency. He had been exclusively breast-fed. Directly after birth no vitamin K was administered. From day 10 a daily dose of 25 microgram vitamin K was given orally. At post mortem a mild cholestasis was found which may have been an additional factor contributing to late vitamin K deficiency.
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PMID:[An infant with a fatal cerebral hemorrhage due to vitamin K deficiency]. 867 75

A 76-year-old man under long term oral anticoagulant treatment showed unclottable prothrombin time (PT) without overt bleeding. After oral administration of vitamin K1, PT remained severely prolonged and the patient was hospitalized. INR was 8.0 and responded to parenteral vitamin K. Cholestasis resulting in poor intestinal vitamin K resorption was assumed to have caused "overanticoagulation". Quick test is a global clotting test for the extrinsic and common pathways of the coagulation system. Increased PT, i.e. decreased Quick percentage, may be due to different conditions and should--if unexplained--be further analyzed by assaying factors II, V, VII, X and fibrinogen. Preanalytical problems, plasma dilution with clotting factor-free volume replacement, decreased vitamin K-dependent clotting factors (oral anticoagulation, intoxication with certain rodenticides, vitamin K deficiency), impaired liver synthetic capacity, disseminated intravascular coagulation, or massive heparin contamination may cause prolonged PT. Newborns physiologically have longer PT and should receive vitamin K prophylaxis.
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PMID:[Derailed oral anticoagulation with very high INR values and poor response to oral vitamin K--cholestasis as a possible cause]. 1051 12

A 4-week-old boy had a fatal intracranial hemorrhage resulting from vitamin K deficiency. The infant had received no vitamin K prophylaxis and was exclusively breastfed. At autopsy, examination of the liver showed cholestasis and fibrosis. DNA was isolated from a blood spot on a Gutherie sample card obtained from the infant for routine metabolic screening. This DNA was used for alpha1-antitrypsin genotyping studies. Genotyping studies identified homozygosity for the point mutation 9989G-->A, confirming a diagnosis of alpha1-antitrypsin deficiency (ZZ phenotype), and resulted in appropriate screening of siblings born after this child's death. Alpha1-antitrypsin deficiency should be considered in the differential diagnosis of infants with late hemorrhagic disease of the newborn. Use of blood from the metabolic screening card as a source of DNA allowed confirmation of this diagnosis after the infant's death.
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PMID:Alpha1-antitrypsin deficiency with fatal intracranial hemorrhage in a newborn. 1262 Dec 52

The aim of this article is to describe the fat-soluble vitamin status in patients with hepatobiliary and pancreatic diseases, and the contribution of these vitamin deficiency or excess to hepatic injury. A considerable number of patients with advanced liver disease and cholestasis might actually be fat-soluble vitamin deficient, although clinical signs of deficiency are uncommonly seen in patients with vitamin A and E deficiency. Increased bone resorption may be the predominant cause of hepatic osteodystrophy. On the other hand, the possible causes of vitamin K deficiency seen in patients with hepatobiliary disease are the decrease of vitamin K absorption from intestine, the disturbance of vitamin K cycle and the decrease of pool area for vitamin K storage. The intake of vitamin A may be associated with the risk of liver cirrhosis in lifetime teetotalers, although the retinyl palmitate reduces hepatic fibrosis in rats.
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PMID:[Hepatobiliary and pancreatic disorders as risk factors for fat-soluble vitamin deficiencies]. 1054 Aug 89

We report an 8-month-old male presenting with gingival hemorrhages and nasal bleeding as the first symptom of a choledochal cyst (CC). On physical examination, there was a large cystic mass in the right upper abdominal quadrant. Laboratory studies on admission revealed moderate liver dysfunction and a bleeding tendency due to vitamin K deficiency. After administration of 5 mg vitamin K the bleeding tendency disappeared. At laparotomy, a large CC 5 cm in diameter was found and the liver showed moderate cholestasis. The sudden onset of a bleeding tendency in infants with congenital liver or biliary-tract disease may suggest not only biliary atresia, but also CC.
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PMID:A bleeding tendency as the first symptom of a choledochal cyst. 1066 55

Vitamin K deficiency in infants can cause life-threatening haemorrhages. To prevent this, neonates in the Netherlands receive an oral dose of 1 mg vitamin K directly after birth. In addition, because breast milk contains little vitamin K, breast-fed infants receive a daily dose of 25 micrograms the first three months. Of three female infants aged 4 weeks, 5 months and 3 months, respectively, two developed an intracranial haemorrhage, which caused death in one. In two cases there were signs of a bleeding tendency, but no tests were done because the patients appeared healthy otherwise. The underlying resorptive disorders, cholestasis and fat malabsorption, caused few symptoms and were discovered only after a vitamin K deficiency bleeding had occurred. In an infant with a bleeding tendency, one should consider the possibility of vitamin K deficiency, even if adequate prophylaxis has been given.
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PMID:[Vitamin K deficiency bleeding in an infant despite adequate prophylaxis]. 1460 58

A major complication of cholestasis is fat malabsorption related to decreased intestinal bile acids, which leads to malnutrition and fat-soluble vitamin deficiency. The impaired excretion of bile acids leads to a low intraluminal micellar concentration that causes long-chain triglyceride lipolysis and absorption to be ineffective. Medium-chain triglycerides (MCTs) are more readily absorbed when there are low concentrations of bile acids and therefore are a good source of fat calories; MCTs can be administered as MCT-containing formulas. In those children who are unable to take sufficient calories by mouth, it is important to start nocturnal enteral feeding to improve nutritional status. In infants with cholestasis, the absorption of fat-soluble vitamins (A, D, E and K) that require bile acids is also impaired, and supplementation is mandatory. Vitamin K deficiency may be responsible for hypoprothrombinaemia, which may lead to bleeding diathesis, Vitamin K (phytomenadione) should therefore be promptly administered intravenously, at a dose of 1 mg. Chronic vitamin E (alpha-tocopherol) deficiency is associated with a progressive neuromuscular syndrome that can cause cerebellar ataxia, areflexia and peripheral neuropathy. Supplements are given orally in doses of 3-5 times the normal requirement if cholestasis is incomplete. In complete cholestasis, supplements must be given intramuscularly at monthly intervals. In infants who fail to thrive, dietary supplements of carbohydrate polymers and MCTs are required.
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PMID:Hepatitis and cholestasis in infancy: clinical and nutritional aspects. 1459 52

The objective of this study was to study etiologies and outcome of neonatal cholestasis in Thai infants. The medical records of infants aged less than 3 months with the diagnosis of neonatal cholestasis in Department of Pediatrics, Siriraj Hospital from 1993 to 2004 were retrospectively reviewed. The etiologies were diagnosed by history, physical examination, and proper investigations. There were 252 infants, including 135 males (53.6%) and 117 females (46.4%). The etiologies of cholestasis were idiopathic neonatal hepatitis (INH) 23%, extrahepatic biliary atresia (EHBA) 22.2%, total parenteral nutrition (TPN)-related cholestasis 18.3%, infection 9.9%, endocrine causes 6%, choledochal cyst 5.6%, Down syndrome 4.4%, hemolytic anemia 1.6%, and miscellaneous causes 9.1%, respectively. TPN-related cholestasis was increasingly found due to advance management of critically ill premature infants. Inborn error of metabolism were suspected in 8 patients (3.21%). Seventeen cases (6.7%) developed cholestasis during the first week of life due to hemolytic anemia, intrauterine infection, hypoxia and others. During the 3 month follow-up period, 6 cases died of progressive dysfunction of liver and one case with idiopathic neonatal hepatitis died from intracranial bleeding from vitamin K deficiency. In conclusion, INH and EHBA are the most common causes of neonatal cholestasis. Due to advance management and nutritional support in critically ill premature infants, TPN-related cholestasis is found more often. Inborn error of metabolism related to neonatal cholestases is uncommon in Thai infants. Overall short-term prognosis of neonatal cholestases is good.
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PMID:Neonatal cholestasis in Thai infants. 1685 19

Vitamin K prophylaxis is recommended to prevent the hazard of haemorrhage caused by vitamin K deficiency in newborns. The present Dutch guideline recommends 1 mg of vitamin K(1) orally at birth, followed by a daily dose of 25 microg of vitamin K(1) from 1 to 13 weeks of age for breastfed infants. Since the introduction of this prophylaxis, the incidence of vitamin K deficiency bleeding (VKDB) has decreased; however, late VKDB is still reported. From 1 January to 31 December 2005, a nationwide active surveillance was performed by the Netherlands Paediatric Surveillance Unit (NSCK) to study the current incidence and aetiology of late VKDB in infants. Six cases could be validated as late VKDB: all were breastfed, one fatal idiopathic intracranial haemorrhage at the age of 5 weeks and five bleedings secondary to an underlying cholestatic liver disease between the age of 3 and 7 weeks. The total incidence of late VKDB and idiopathic late VKDB was calculated to be 3.2 (95% CI: 1.2-6.9) and 0.5 (95% CI: 0-2.9) per 100,000 live births, respectively. With the current Dutch guideline, idiopathic late VKDB is rare but late VKDB secondary to cholestasis still occurs in breastfed infants. Doubling the daily dose of vitamin K(1) to 50 microg, as is comparable to formula-feeding, may possibly prevent VKDB in this group. Further research, however, is needed to prove this hypothesis.
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PMID:Incidence of late vitamin K deficiency bleeding in newborns in the Netherlands in 2005: evaluation of the current guideline. 1733 71


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