Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During the last 9 years, two factor IX concentrates produced in Scotland, PPSB and DEFIX, have been used for the treatment of haemophilia B and acquired coagulation disorders, including those due to liver disease, coumarin therapy, neonatal immaturity and post-operative bleeding. During the period of study 112 batches of DEFIX and 40 batches of PPSB were used in the Edinburgh and South-East Scotland Region. Data were analysed from 575 non-haemophilic patients, receiving 968 treatment episodes, as well as 24 haemophiliacs. Serial coagulation studies and analysis of retrospective data showed that both concentrates corrected the coagulation deficiencies in all the above patient groups; in no case was there any evidence of intravascular coagulation resulting from concentrate infusion.
 ...
PMID:The use of factor IX concentrates in man: a 9-year experience of Scottish concentrates in the South-East of Scotland. 743 48

The relationships between various hepatobiliary disorders and the administration of total parenteral nutrition (TPN) were reviewed and, in particular, the role of TPN in their pathogenesis was critically evaluated. Several clinical and pathological entities including steatosis, steatohepatitis, cholestasis, and cholelithiasis have been commonly linked to TPN, and instances of chronic decompensated liver disease have been reported. However, it is concluded that it is often difficult to extricate the effects of TPN on hepatobiliary function from many other hepatotoxic factors that may be operative in these patients. Thus, whereas considerable evidence exists to support a role fro carbohydrate or calorie excess in TPN solutions in the pathogenesis of steatosis, a loss of enteric stimulation and not TPN per se may be the primary factor in the development of cholestasis, biliary sludge, and gallstones. The apparent predilection of infants to TPN-related cholestasis may be based on the relative immaturity of the neonatal biliary excretory system.
 ...
PMID:Hepatobiliary complications of total parenteral nutrition. 834 99

Measurement of energy balance represents a basic theoretical concept in the determination of nutritional and fluid requirements in humans in health and disease. Infants have special nutrient requirements, more limited reserves and relative immaturity of organ function. Energy requirements of infants have been based either retrospectively on intakes required to achieve normal growth or on equations derived from energy expenditure studies performed early this century. Recently, improved techniques for studying resting energy expenditure (REE), total energy expenditure (TEE) and metabolically active body compartments in infants have facilitated more accurate estimates of energy requirements. Such studies indicated that current reference values for energy requirements are overestimates, and that compared with measured values, predicted values vary markedly between the various predictive equations with wide co-efficients of variation. In disease states with altered body composition, such as cystic fibrosis and end-stage liver disease, predictive equations markedly underestimate both energy and fluid requirements. In cystic fibrosis, both TEE and REE are 25% higher than values in healthy infants. In extrahepatic biliary atresia, energy expenditure per unit body cell mass is markedly elevated, suggesting that this is a catabolic condition in infants. Current estimates of energy and fluid requirements in both health and disease in infants need reappraisal. Bedside and free living energy expenditure methodology should be used to define accurately components of energy requirement in individual infants.
 ...
PMID:Energy expenditure in infants in health and disease. 911 8

Bile acid synthetic defects are uncommon disorders that cause progressive cholestatic liver disease that is often lethal in infancy or early childhood. Five specific primary defects have been described. Diagnosis is based on mass spectrometry of urine and serum. Pathogenesis of liver injury is related to persistent reduction in levels of normal bile acids and accumulation of abnormal, potentially hepatotoxic, intermediaries. Sites of injury are the liver cell, the bile canaliculus, and the smallest bile ductules. The interlobular bile ducts are normal. The liver lesion is progressive chronic hepatitis with an especially high incidence of GCT in patients who present in infancy. Bile acid replacement therapy is usually effective in arresting the liver injury. Regression of liver damage has been documented during treatment of patients who were diagnosed early in life. Because bile acid synthetic disorders are the only cholestatic diseases of infancy in which GCT of hepatocytes is consistently present, the author suggest that the injury responsible for GCT may be specific for toxic bile acids. Accordingly, immaturity of the bile acid synthetic pathway may render many otherwise normal infants vulnerable to transient "neonatal hepatitis" with GCT in a broad range of cholestatic disorders.
 ...
PMID:Liver disease caused by disorders of bile acid synthesis. 1123 60

We carried out an in-depth evaluation of psychosocial status in a sample of 18 children (mean age 6.8 yr, range 4.4-10.8 yr) who had suffered from severe liver disease and undergone orthotopic liver transplantation (OLT). Mean age at OLT was 3.4 yr. The assessment was psychoanalytically oriented and included individual sessions and testing procedures for children--the Children Apperception Test (CAT), the Weschsler Intelligence Scale for Children (WISC-R), the Weschsler Preschool and Primary Scale of Intelligence (WIPPSI), and the Human Figure Test--and a semi-structured interview with a separate questionnaire for parents. Patients were compared with an age- and gender-matched control group. The main findings in patients compared with controls were: IQ 91.6 (range 70-117) vs. 118 (range 94-135) (p<0.0001); immaturity of ego and drives (72.2% vs. 27.7%; p=0.018), fear of death (61.1% vs. 11.1%; p=0.04), anxiety of loss (50%, vs. 27.7%; p=NS), and depressive feelings (61.1% vs. 22.2%; p=0.04); a mild defect of body image (44.4% vs. 33.3%; p=NS) associated with recurrent representations of motionless (72.2% vs. 38.8%; p=NS) and inexpressive (88.8% vs. 16.6%; p<0.0001) human figures. Fantasies about OLT as a 'magic rebirth' or a 'body transformation' were detected in few patients (30%). Although a recurrent set of feelings, conflicts, and fantasies about OLT were expressed by children, individual specific psychological responses to this experience were often detected. In spite of the fact that approximately 50% of the parents mentioned emotional or behavioral disturbances of their child, only three parents were seriously concerned about this problem. The theme of transplantation was most often absent from communication between the child and their parents. Our results suggest that psychic 'working through' of the chronic liver disease and OLT experience is difficult for children. Further studies are necessary to verify whether changes of parental attitude to OLT as a 'family secret' may facilitate integration of the OLT experience in the child's personality development.
 ...
PMID:Psychological impact of liver transplantation on children's inner worlds. 1126 Apr 87

Biotransformation of drugs is one of the major functions of liver. Hepatic drug metabolism develops early in organogenesis and continues in postnatal life through puberty. Genetic and developmental studies on hepatic drug metabolism show that immaturity, polymorphisms, and altered balance of different hepatic enzymatic activities affect pharmacologic inactivation and alter the risk of toxic effects of drugs on the hepatic parenchyma. Although drug-induced liver disease is less common in children, several reports of hepatotoxicity are published every year. Furthermore, the increasing use of nonregulated remedies (eg, herbal preparations or recreational drugs) increases the risk of unpredictable and potentially severe reactions. Many significant advances in the treatment of hepatic diseases have been achieved recently. However, differences in clinical features, natural history, and response to treatment between children and adults require evaluation of new therapeutic options in focused pediatric clinical trials.
 ...
PMID:Drugs and the liver: advances in metabolism, toxicity, and therapeutics. 1235 55

Human umbilical cord blood (CB) cells have many advantages as a source for stem cell transplantation because of immaturity and availability. It has been reported that CB cells transplanted into an injured liver displayed hepatocyte-like phenotypes. However, there have been few studies to characterize CB-derived hepatocyte-like cells (HLCs). In this study, CB cells were transplanted into mice with 2 types of liver damage: transient and chronic damage. We analyzed the expression of hepatic differentiation markers in CB-derived HLCs. In the liver of NOD/SCID mice with transient damage, CB-derived HLCs were detected infrequently at 3 weeks after transplantation. In contrast, in the liver of SCID mice damaged chronically by a urokinase-type plasminogen activator transgene under the control of albumin promotor/enhancer (ALB-uPA/SCID mice), more human HLCs colonized the host liver compared with hosts with transiently damaged livers. The CB-derived HLCs in both the transiently and the chronically damaged liver expressed a few markers of human hepatocytes, whereas the transcripts related to mature hepatic functions, including cytochrome P450s, were detected only in the ALB-uPA/SCID mice. These data indicated that CB cells were able to display a similar phenotype to functional hepatocytes in the recipient liver with chronic damage. CB cells may represent a transplantable source for chronic decompensated liver disease.
 ...
PMID:Human cord blood cells transplanted into chronically damaged liver exhibit similar characteristics to functional hepatocytes. 1727 13

Parenteral nutrition (PN) is necessary for infants unable to receive adequate calories enterally due to prematurity, decreased bowel length, or functional intestinal disorders. While PN can be life saving, its use is associated with significant risks of sepsis from catheter-associated infections and progressive liver dysfunction from prolonged use. The preterm infant population is at highest risk for these complications due to the presence of multiple comorbidities and immaturity of the biliary system. Strong data has implicated parenteral lipids in the multifactorial pathogenesis of PN-associated liver disease (PNALD). However, lipids are essential in early infant development, particularly in the neurocognitive development of preterm infants. Substitution of the lipid source from soybean oil to fish oil has emerged as a safe and efficacious treatment of PNALD, with marked improvements in morbidity and mortality. Knowledge of the developmental needs and physiologic limitations of preterm infants is crucial to optimizing parenteral lipid administration to nurture growth, and minimize and treat associated complications. The purpose of this review is to provide an overview of lipid requirements of the preterm infant and discuss the role of parenteral lipid emulsions in the management of PNALD and other diseases of prematurity.
...
PMID:Role of parenteral lipid emulsions in the preterm infant. 2399 51

Parenteral nutrition (PN) is frequently required by extremely preterm infants due to gastrointestinal immaturity and complications of prematurity. Parenteral nutrition-associated cholestasis (PNAC) and intestinal failure-associated liver disease (IFALD) are common complications of prolonged PN. Plant-based intravenous lipid emulsions, containing proinflammatory omega-6 fatty acids and phytosterols, may contribute to these conditions as well as other comorbidities such as bronchopulmonary dysplasia and retinopathy of prematurity. Intravenous lipid emulsions containing animal-based fats, such as fish oil, contain fewer proinflammatory omega-6 fatty acids and more anti-inflammatory omega-3 fatty acids and antioxidants. SMOFlipid, recently Food and Drug Administration (FDA)-approved for adult use, is a blend of plant- and animal-based lipid emulsions with a favorable omega-6:omega-3 ratio that may prevent the development and progression of PNAC/IFALD in infants. Careful review of data supporting this alternative intravenous lipid emulsion is required prior to widespread use in neonatal intensive care.
 ...
PMID:Intravenous Lipid Emulsions in Infants: Is Balanced Better? 3067 55

The use of nutraceuticals is considerably increasing worldwide with a demand for organic and clean foods in the last two decades, which is probably incomparable with other periods of our civilization. The consistent application of nutraceuticals and so-called "superfood" may have remarkable effects on the prevention of several chronic diseases, including cancer. Moreover, the increased rate of overweight and obesity in Western countries does not spare childhood and youth, and the number of parents using natural remedies for preventing pediatric illness is vastly increasing worldwide. However, the overwhelming effects on diseases often overshadow the side effects of such nutrition, particularly in societies without millennial experience with botanicals and natural elements. Thus, the final result may be disastrous for some individuals. The liver is the most important and conspicuous target organ of numerous molecular compounds, and the cell damage is particularly striking on the infantile and pediatric liver due to the immaturity of the hepatocytes. Here, we target some generic data on fulminant hepatic failure, the benefits, and toxicity of epigallocatechin-3-gallate, which is one of the major components of green tea, and the histopathology of the "green-tea"-associated liver disease.
 ...
PMID:Epigallocatechin-3-Gallate Toxicity in Children: A Potential and Current Toxicological Event in the Differential Diagnosis With Virus-Triggered Fulminant Hepatic Failure. 3206 42


1 2 Next >>