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At least 667 records · Page 37Linked to original sources
PubMed2005

Ascites after orthotopic liver transplantation in children.

Ascites is a poorly understood postoperative complication of orthotopic liver transplantation (OLT). It is associated with additional morbidity and can prolong hospitalization considerably. The incidence, the factors predictive of occurrence and the etiology of this complication are not known. The charts of 118 patients with 138 OLT were analyzed according to the following criteria: ascites lasting longer than the first 10 postoperative days, assessed by loss of ascitic fluid through drainage tubes, surgical wounds or paracentesis, with a peak volume of > or =10 mL/kg/day. Patients were divided into three groups: Group 1, no ascites; Group 2, ascites associated with postoperative complications, including chylus ascites; and Group 3, ascites not associated with postoperative complications. Postoperative ascites occurred in 43 of 138 OLT (31.2%). Patients with biliary atresia, preoperative portal hypertension, postoperative pleural effusion or at retransplantation had ascites significantly more often. In 32 of 138 (23.2%) OLT, ascites was associated with postoperative complications, including thrombosis, abdominal infections, intestinal perforation, biliary leak, pancreatitis, and chylus ascites. In 11 of 138 (7.9%) OLT, ascites was the only postoperative complication (group 3). Group three patients were significantly older, and had lower preoperative platelet counts and preoperative ascites more often than group 1 patients. The primary liver diseases were mainly cystic fibrosis of the pancreas, congenital hepatic fibrosis, and North American Indian childhood cirrhosis. The serum-ascites albumin gradient suggested a hepatic origin of ascites. Postoperative ascites is associated with the duration and degree of preoperative portal hypertension. We speculate that the mechanism involved includes a disproportion between venous blood volume and liver uptake capacity of the donor organ.

Ascites↗
PubMed2006

Caerulein-induced acute pancreatitis in mice that constitutively overexpress Reg/PAP genes.

BACKGROUND: The cystic fibrosis (CF) mouse pancreas has constitutively elevated expression of the Reg/PAP cell stress genes (60-fold greater Reg3alpha, and 10-fold greater PAP/Reg3beta and Reg3gamma). These genes are suggested to be involved in protection or recovery from pancreatic injury. METHODS: To test this idea the supramaximal caerulein model was used to induce acute pancreatitis in wild type and CF mice. Serum amylase, pancreatic water content (as a measure of edema), pancreatic myeloperoxidase activity, and Reg/PAP expression were quantified. RESULTS: In both wild type and CF mice caerulein induced similar elevations in serum amylase (maximal at 12 h), pancreatic edema (maximal at 7 h), and pancreatic myeloperoxidase activity (MPO, a marker of neutrophil infiltration; maximal at 7 h). By immunohistochemistry, Reg3alpha was strongly expressed in the untreated CF pancreas but not in wild type. During pancreatitis, Reg3alpha was intensely expressed in foci of inflamed tissue in both wild type and CF. CONCLUSION: These data demonstrate that the severity of caerulein-induced pancreatitis is not ameliorated in the CF mouse even though the Reg/PAP stress genes are already highly upregulated. While Reg/PAP may be protective they may also have a negative effect during pancreatitis due to their anti-apoptotic activity, which has been shown to increase the severity of pancreatitis.

Animals↗
PubMed1980

The radioimmunoassay of human urinary kallikrein and comparisons with kallikrein activity measurements.

Human urinary kallikrein was purified to homogeneity, and an antiserum to it was raised in rabbits. A RIA was devised which uses this rabbit antiserum (Keq = 2.75 x 10(11) M-1) in a final dilution of 1:2,500,000 and the purified kallikrein labeled with 125I using a lactoperoxidase method. Assay sensitivity is 8 pg kallikrein. Thus far, the assay is specific for human and perhaps monkey urinary kallikrein. Correlations between this assay of immunoreactive kallikrein and the alpha-N-Tosyl-L-arginine-[3H]methylester (Tos-Arg-OMe) activity method or a kininogenase assay were highly significant (r = 0.94 and 0.96, respectively) and show that each assay measures human urinary kallikrein comparably. Low or high dietary sodium intakes, maneuvers known to change human urinary Tos-Arg-OMe esterase excretion, change immunoreactive kallikrein to an equivalent degree. Normal black children, already known to excrete significantly less Tos-Arg-OMe esterase than white children, excrete similarly reduced amounts of immunoreactive kallikrein. Kallikrein excretion in children with cystic fibrosis of the pancreas was not different from that in normal children. The data show that a specific and sensitive direct RIA for human urinary kallikrein has been developed and that both the Tos-Arg-OMe esterase and kininogenase assays measure human urinary kallikrein activity specifically, at least in the described circumstances.

Animals↗
PubMed1983

Genetic screening of newborn in Australia. Results for 1981.

Since screening of newborn infants for phenylketonuria (PKU) by the Guthrie bacterial inhibition assay was established in the 1960s, 3 017 703 infants have been tested in Australia. Two hundred and fifty-one cases of PKU (0.83/10 000) and six cases of the variant forms of malignant hyperphenylalaninaemia (MHPA) (0.02/10 000) have been detected. In 1981, 11 infants with PKU were detected. Screening for congenital hypothyroidism was carried out in seven States, and 66 new cases were detected in 1981 (2.13/10 000). In Adelaide, 154 310 infants have been tested for galactosaemia and a total of seven cases have now been detected (0.45/10 000). In New South Wales, 35 955 infants have been tested for cystic fibrosis of the pancreas, and 17 cases were found (4.73/10 000).

Australia↗
PubMed1987

Nakahara memorial lecture. Rare events and cancer epidemiology.

Physicians who think epidemiologically are rare. A method is suggested for detecting their aptitude early in their career when help may be offered to make the most of their special talent. Clusters geographically or in families may provide clues to cancer etiology. Clusters have been systematically thought by mapping cancer mortality in the US and independently in China. Case-control studies have revealed environmental exposure responsible for some of the clusters. Clusters noted by alert clinicians or other astute observers have revealed most of the known environmental causes of human cancers. Genetic influence in carcinogenesis has been identified by studies of peculiar cancer occurrence, such as familial aggregation, multiple primary cancer or the occurrence of cancer with other diseases as, for example, congenital malformations and immunodeficiency disorders. Ethnic differences in cancer occurrence may be revealing. Thus, in Japan there is low frequency of B-cell lymphoma but high frequency of certain autoimmune diseases, as if inherent protection against one predisposes the other. As a rule of thumb, the occurrence of three rare observations is not likely to be due to chance. Examples include ideal carcinoma in three persons with cystic fibrosis of the pancreas who survived to about 30 years of age, and the occurrence in Klinefelter's syndrome of germ cell tumor of the pineal--a neoplasm that has an unusually high frequency in Japan. Finally, the history of discoveries concerning cancer etiology, an aspect of what Comroe has called "research on research", can point the way to new discoveries in the future.

Demography↗
PubMed1982

[Clinical studies of cefsulodin in the pediatric field].

Pharmacokinetic and clinical evaluations of cefsulodin (CFS) were made and the following results were obtained. 1. Pharmacokinetic study Three hundred fifty grams of CFS (20 mg/kg) was administered by 30 minutes intravenous drip infusion to 7 years old child (17.5 kg in weight). Serum concentrations of CFS at the end of the infusion and 1,1.5,2.5,6.5 hours thereafter were 46.0,44.9,23.0,11.9 mcg/ml and 0.6 mcg/ml respectively. Urinary recovery rate until 6 hours from the start of infusion was 66.2%. 2. Clinical study CFS was administered to the case of bronchitis with cystic fibrosis of the pancreas and bronchiectasia (treatment was made 2 times), and each 1 case of pyelonephritis with renal calculus and measles pneumonia with infantile spasm. All infections were caused by P. aeruginosa and administration and dosage of CFS was 47 to 86 mg/kg/day, 2 to 4 times daily by intravenous injection or intravenous drip infusion for 5 to 11 days. Result was good in 3 infections (2 cases) and fair in 1 case, i.e. measles pneumonia. Effectiveness rate was 75.0%. Side effect as well as abnormal change of laboratory findings were not observed. Thus, CFS is considered to be the useful drug for the treatment of pediatric infection caused by P. aeruginosa.

Age Factors↗
PubMed1994

[Liver cirrhosis in metabolic disorders].

The most early cirrhosis is observed in newborns with neonatal hemachromatosis. Early cirrhosis occurs in hereditary tyrosinemia type I, peroxisomal diseases and glycogen storage disease (type IV). In Wilson's disease, a case complicated with cirrhosis was reported in a 4-year-old patient. Slowly progressive cirrhosis is seen in patients with familial progressive intrahepatic cholestasis. Focal biliary cirrhosis is found in cystic fibrosis of the pancreas. Moreover, many other metabolic disorders, except for urea cycle disorders, are occasionally or rarely complicated with cirrhosis. Early diagnosis and proper management could prevent the development of cirrhosis in patients with galactosemia, hereditary fructose intolerance, etc. The occurrence of hepatoma must be monitored in these patients. Liver transplantation is indicated in a part of the patients with cirrhosis.

Carcinoma, Hepatocellular↗
PubMed1994

Association of pancreatic adenocarcinoma, mild lung disease, and delta F508 mutation in a cystic fibrosis patient.

A case of adenocarcinoma of the pancreas and mild lung disease in a 39-year-old man homozygous for the delta F508 cystic fibrosis mutation is presented. Cystic fibrosis is the most common lethal genetic disease in Caucasians, and is most commonly associated with severe obstructive lung disease. To our knowledge, this is only the fifth case of adenocarcinoma of the pancreas in a CF patient to be reported and the first case for which molecular data are available. The rare incidence of this type of malignancy in the general population suggests a possible association of CF with this malignant disease.

Adenocarcinoma↗
PubMed1980

[Diagnosis of pancreatic insufficiency with fluorescein dilaurate in patients with cystic fibrosis (author's transl)].

The tubeless pancreas-function test with fluorescein dilaurate, which is based on the saponification of the test substance by pancreas-specific arylesterases, and which has proved itself in adults, was given to children for the first time. After reduction of the size of the test dose and the amount of liquid to be drunk, there were no difficulties in carrying out the test. Elimination of the fluorescein released from the flourescein dilaurate and excreted via the kidneys was clearly less in patients with cystic fibrosis than in normal subjects. Thus, the test in its modified form for children can provide adequate differentiation between normal and pathological pancreas function.

Adolescent↗