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Bile puberty

Miia H Vapola, Aare Rokka, Raija T Sormunen, Leena Alhonen, Werner Schmitz, Ernst Conzelmann, Anni Wärri, Silke Grunau, Vasily D Antonenkov, J Kalervo Hiltunen
To understand the functional role of the peroxisomal membrane channel Pxmp2, mice with a targeted disruption of the Pxmp2 gene were generated. These mice were viable, grew and bred normally. However, Pxmp2(-/-) female mice were unable to nurse their pups. Lactating mammary gland epithelium displayed secretory lipid droplets and milk proteins, but the size of the ductal system was greatly reduced. Examination of mammary gland development revealed that retarded mammary ductal outgrowth was due to reduced proliferation of epithelial cells during puberty...
July 1, 2014: Developmental Biology
Helen H Wang, Min Liu, Deborah J Clegg, Piero Portincasa, David Q-H Wang
Epidemiological and clinical studies have found that at all ages women are twice as likely as men to form cholesterol gallstones, and this gender difference begins since puberty and continues through the childbearing years, which highlight the importance of female sex hormones. Estrogen is a crucial hormone in human physiology and regulates a multitude of biological processes. The actions of estrogen have traditionally been ascribed to two closely related classical nuclear hormone receptors, estrogen receptor 1 (ESR1) and ESR2...
November 2009: Biochimica et Biophysica Acta
Avnish Kumar Seth, Michael N Badminton, Darius Mirza, Scott Russell, Elwyn Elias
Porphyrias are a heterogenous group of diseases that may result in disabling or life threatening neurovisceral symptoms and/or cutaneous photosensitivity. In acute intermittent porphyria, the clinical features, particularly neurological symptoms, may be life-threatening and disabling. Conventional treatment with human hemin, though effective in reducing symptoms, does not reverse neuropathy when structural nerve damage has occurred and may cause intense phlebitis. Liver transplantation (LT) may be considered as treatment for those with repeated life-threatening acute attacks resulting in poor quality of life, requirement of ventilatory support, and progressive loss of venous access due to hemin infusion...
September 2007: Liver Transplantation
Serena Tonstad, Gilbert R. Thompson
Heterozygous familial hypercholesterolemia (FH) affects one in every 500 persons and is the most common cause of markedly elevated cholesterol levels in children. Other causes of primary hyperlipidemia include familial combined hyperlipidemia, which is also common (approximately 1%) but not usually manifest until after puberty, and very rare genetic disorders that may lead to severe hypertriglyceridemia and chylomicronemia syndrome. In children with heterozygous FH, the short-term risk of clinical events is low; therefore, management starts with stratification of risk, followed by dietary modification, and in high-risk cases, pharmacologic treatment initiated after puberty...
October 2004: Current Treatment Options in Cardiovascular Medicine
V Diwakar, L Pearson, S Beath
Identification, evaluation and treatment of liver disease are increasingly important challenges in children with cystic fibrosis (CF). Liver disease usually presents at puberty and is receiving more attention with improved life expectancy. The abnormal CF transmembrane regulator protein in the apical surface of the biliary epithelium causes the disease. Hyperviscous bile accumulates in the biliary tree causing cholangiocyte and hepatocyte injury, stimulating focal fibrosis. Fibrosis is thought to lead on to cirrhosis over a period of years, a process which is usually asymptomatic...
December 2001: Paediatric Respiratory Reviews
L Starck, I Björkhem, E M Ritzén, B Y Nilsson, U von Döbeln
In 1993 the Smith-Lemli-Opitz (SLO) syndrome, known as a malformation syndrome characterized by certain stigma, turned out to be a metabolic disease with a defect in the last step of cholesterol biosynthesis. This led to the possibility of identifying affected individuals by biochemical methods and of increasing understanding of pathogenic mechanisms. Hopes of influencing the effects of the metabolic defect by dietary supplementation were raised and reports with some benefits of treatment have been published...
July 1999: Acta Paediatrica
C H Hurst, B D Abbott, M J DeVito, L S Birnbaum
Prenatal exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) interferes with fetal development at doses lower than those causing overt toxicity in adult animals. In a multigeneration study (Murray et al., 1979), female rats that were administered 0.01 microgram TCDD/kg/day in their diet did not experience reduced fertility; however, reduced fertility was seen in the F1 and F2 generations. Exposure to TCDD during development produces alterations in the reproductive system of the developing pups, such as delayed puberty and reduced sperm counts in males (Mably et al...
October 1998: Toxicological Sciences: An Official Journal of the Society of Toxicology
L Liu, C D Klaassen
Female-dominant sulfotransferases (ST) are a group of enzymes catalyzing sulfation of steroid hormones, bile acids and exogenous carcinogens. To understand the regulation of these enzymes, developmental and hormonal regulation of three female-dominant ST was studied. The three ST, ST-20/21, ST-40/41 and ST-60, were differentiated by specific oligonucleotide probes, and their gene expression was examined by Northern-blot analyses. The three ST have different developmental patterns, as ST-20/21 and ST-60 were increased after puberty in female rats, although ST-40/41 was higher in immature rats...
October 1996: Journal of Pharmacology and Experimental Therapeutics
R Santer, A Claviez, H D Oldigs, J Schaub, R B Schutgens, R J Wanders
We describe a 16-year-old boy suffering from psychomotor retardation, sensorineuronal hearing impairment, peripheral neuropathy, hepatosplenomegaly, short stature and delayed puberty. Postnatally, muscular hypotonia, mild facial dysmorphism and delayed fontanelle closure had been noticed. At the time of our examination, adrenal cortical function was normal. Biochemical analysis revealed accumulation of very long (> C22) chain fatty acids in plasma and fibroblasts. Furthermore, elevated levels of intermediates of bile acid synthesis and phytanic acid were detectable...
April 1993: European Journal of Pediatrics
L L Hughes, M J Baruzzi, R C Ribeiro, G D Ayers, B Rao, D M Parham, C B Pratt, L E Kun
BACKGROUND: The combined modalities of surgery, chemotherapy, and radiation therapy have greatly improved the survival rate in childhood paratesticular rhabdomyosarcoma, but the incidence of complications and late side effects is a cause for concern. METHODS: We reviewed the records of 18 patients treated for paratesticular rhabdomyosarcoma at St. Jude Children's Research Hospital between 1962 and 1989. Patients with Group I disease were treated with orchiectomy, retroperitoneal lymph node dissection, and multi-agent chemotherapy; more advanced cases also received radiation therapy with concurrent chemotherapy...
January 15, 1994: Cancer
L J Chen, B Kane, Y Bujanover, M M Thaler
Hepatic bile salt sulfotransferase activity was extremely limited in fetus, gradually increasing after birth. At puberty, enzyme activity declined in males but not in females, suggesting the influence of gonadal hormones associated with sexual maturation. Extremely high enzyme activity was found in pregnant rats at term. The neonatal bile salt sulfotransferase activity could be stimulated by bile acid feeding during pregnancy or maternal bile duct ligation. In contrast, a decrease in enzyme activity was detected in the treated pregnant females...
November 12, 1982: Biochimica et Biophysica Acta
A I Morris, J M Little, R Lester
The bile acid pools of developing rats were measured by gas-liquid chromatography. From shortly after birth the bile acid pools fell to a nadir on the 10th day of life (0.295 +/- 0.031 mg X g-1 body weight at 2 days to 0.144 +/- 0.012 mg X g-1 body weight at 10 days, p less than 0.001). The pool re-expanded rapidly between the 12th and 15th day. After weaning and during puberty there was a further temporary increase in pool size, during which females had larger pools than males. By adulthood the pool size had returned to the 2-day-old and weanling (15- and 18-day-old) levels, expressed per gramme body weight, and there was no longer a significant sex difference...
1983: Digestion
D H Van Thiel, J S Gavaler, A B Zajko, C F Cobb
Prepubertal male rats underwent bile-duct ligation or a sham operation. Sham-operated animals were divided into two groups: isocalorically-fed (matched to the bile-duct-ligated animals) and ad-libitum-fed animals. At 60 days of age (after puberty in a male rat) all animals were killed. Bile-duct-ligated animals had larger livers, greater bilirubin, greater bile acid, greater aspartate transaminase, and greater alkaline phosphatase levels and lower testosterone and luteinizing hormone levels in their serum than did the controls...
August 1985: Journal of Pediatric Gastroenterology and Nutrition
D Alagille, A Estrada, M Hadchouel, M Gautier, M Odièvre, J P Dommergues
We have observed two types of paucity of interlobular bile ducts (PILBD) in children with chronic cholestasis: the syndromic type, which is more frequent (80 cases), and the nonsyndromic type (31 cases). Study of patients with syndromic PILBD has enabled us to recognize five major features: peculiar facies (95%), chronic cholestasis (91%), posterior embryotoxon (88%), butterfly-like vertebral arch defects (87%), and peripheral pulmonary artery hypoplasia or stenosis, either isolated or associated with complex cardiovascular abnormalities (85%)...
February 1987: Journal of Pediatrics
N M Wolfish, A Shanon
A 15-year-old girl evaluated for short stature and delayed puberty was found to have features of syndromatic arteriohepatic dysplasia or Alagille's syndrome. The characteristic triangular face, stubby nose, peripheral pulmonic stenosis, a history of prolonged neonatal jaundice and evidence of hepatic parenchymal disease were present as well as bilateral small kidneys and delayed puberty. The recognition of kidney involvement in this syndrome and the characterization of the frequency and nature of the renal disorder are of great prognostic significance and could influence the morbidity and mortality of these patients...
1988: Child Nephrology and Urology
R E Kane, L J Chen, J J Herbst, M M Thaler
The mature female rat has three times the hepatic bile salt sulfotransferase (BSS) activity compared with male rats. This study examined the changes in two hepatic BSS isoenzyme activities during sexual maturation, and the role of estrogen in development of sex differences in BSS activities in mature rats. DEAE-Sephadex A-50 chromatography of hepatic cytosol from prepubescent pups revealed that more than 90% of total BSS activity was due to BSS I activity relative to BSS II, similar to postpubertal females...
August 1988: Pediatric Research
K Von Bergmann, M Becker, O Leiss
The lipid composition of gallbladder bile was determined in forty-seven normal, non-obese subjects (twenty females and twenty-seven males) without gallstones ranging in age from seven months to twenty-nine years. Before puberty, bile was undersaturated with cholesterol in both sexes to the same extent. After puberty a marked increase in cholesterol saturation was observed in females but not in males (138% vs 88%; P less than 0.01). A significant correlation between age and cholesterol saturation could be observed in females (r = 0...
December 1986: European Journal of Clinical Investigation
M Becker, D Staab, O Leiss, K von Bergmann
Lipid composition of gallbladder bile was determined in 20 patients with cystic fibrosis (CF) (9 females and 11 males, ranging in age from 3 to 18 years). The results were compared with 47 normal subjects matched for age, sex, and pubertal stage. In patients with CF, bile was undersaturated with cholesterol before puberty in both sexes and no differences with normal controls could be observed. After puberty, a similar increase in cholesterol saturation was noted in females with CF (85 +/- 15% vs. 130 +/- 38%, p less than 0...
April 1989: Journal of Pediatric Gastroenterology and Nutrition
J E Heubi, N C O'Connell, K D Setchell
Children with ileal resection/dysfunction since infancy have bile that is not supersaturated with cholesterol. Five sexually mature subjects (age 16-19 years) who had been previously investigated in childhood (age 4-9 years) were studied. Gallstones were found in one. Bile rich duodenal aspirates were analyzed for lipid content (molar fraction) and the cholesterol saturation index was calculated. In the postpubertal subjects, a significantly higher proportion of biliary cholesterol (22.0% +/- 4.8% vs. 3.2% +/- 0...
August 1992: Gastroenterology
M W Scobey, M S Wolfe, L L Rudel
The effects of age and dietary fat on bile cholesterol saturation index (CSI) and cholesterol gallstone formation were studied in three age groups of male African green monkeys. Animals were fed semipurified diets containing cholesterol (0.34 g/100 g diet) and 42% of energy as saturated or polyunsaturated fat. Animals were killed at 16 (pre-pubertal), 32 (peri-pubertal) or 60 (young adult) mo of age; the presence of gallstones was determined and gallbladder bile was analyzed for CSI and bile acid composition...
April 1992: Journal of Nutrition
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