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At least 631 records · Page 35Linked to original sources
PubMedDate not supplied

Microdissection study of the myenteric plexus in acardia, ataxia-telangiectasia, cystic fibrosis, extrahepatic biliary atresia, pediatric AIDS and Werdnig-Hoffmann disease.

Microdissection-point count morphometric study of the myenteric (Auerbach) plexus or esophagus, small intestine, and colon was done for infants and children with acardia (2), ataxia-telangiectasia (5), cystic fibrosis of the pancreas (CFP) (25), extrahepatic biliary atresia (EBA) (17), pediatric AIDS (10), and Werdnig-Hoffmann disease (WHD) (8). Values for fractional area of neural tissue in the plane of the plexus were compared to those of control patients in same age range as those in each disease category by t-test. Statistically abnormal values included low values for small intestine and colon in Werdnig-Hoffmann disease, high values for small intestine and colon in biliary atresia, and high value for colon but a low value for small intestine in cystic fibrosis. Values for all three loci were within the normal range for ataxia telangiectasia and pediatric AIDS. The mechanisms of the low value for small and large intestines in WHD, which causes chronic constipation as a result of skeletal muscle weakness, and of the high values for colon in CFP and EBA, both causing malabsorption with bulky stools, are unclear. The value for small intestine in acardia was normal for term but lower than expected for fetal bowel of the same size, possibly because of reduced neural crest inflow to the fetal bowel.

Acquired Immunodeficiency Syndrome↗
PubMed1981

Determination of protease-cleaved p-aminobenzoic acid (PABA) in serum after oral administration of N-benzoyl-L-tyrosyl-p-aminobenzoic acid (PABA-peptide) in children.

A modification of the urine PABA tet published by Imondi et al. is described. Ninety minutes after oral administration of PABA peptide, PABA was determined in serum. The average concentration in healthy children was 0.42 +/- 0.055 mg per 100 ml. Up to that time we recovered in the urine 66.1 +/- 6.1% of the substance previously administered. In the children with cystic fibrosis of the pancreas (CF) serum PABA concentrations were less than 0.1 mg per 100 ml. The infants under 2 months old also had clearly low serum concentrations, on average 0.29 +/- 0.06 mg per 100 ml, whereas the PABA concentrations in the older babies lay within the range for the older children (0.40 +/- 0.07 mg%).

4-Aminobenzoic Acid↗
PubMed1987

Vitamin E deficiency and neurologic disease in adults with cystic fibrosis.

We report the cases of two adult patients with cystic fibrosis affecting the pancreas and liver, who also had severe vitamin E deficiency and neurologic disease. The most prominent clinical features were abnormal eye movements, diminished reflexes, decreased vibratory and position sense, ataxia, and muscle weakness. Treatment with intramuscular injections of vitamin E partially corrected the neurologic deficits. Vitamin E absorption tests documented severe malabsorption, which was later alleviated by the addition of dessicated ox bile to the regimen of alpha-tocopheryl acetate. These studies suggest that a decreased intraluminal concentration of bile salts is an important factor in the development of severe vitamin E deficiency and in the poor response to oral replacement therapy that is seen in some patients with cystic fibrosis.

Administration, Oral↗
PubMed1992

The promoter of the human cystic fibrosis transmembrane conductance regulator gene directing SV40 T antigen expression induces malignant proliferation of ependymal cells in transgenic mice.

Transgenic mice bearing a human cystic fibrosis transmembrane conductance regulator (CFTR) promoter-SV40 T antigen fusion transgene were generated in order to localize in vivo the potential oncogenesis linked to the tissue-specific activity of the promoter for the CFTR gene. Surprisingly, the only site of tumors resulting from expression of the reporter onc gene was ependymal cells lining the brain ventricles. SV40 T antigen expression in these cells led to a consistent pathology in the first weeks of age: ependymoma and consequent hydrocephaly. Tumor-derived cell lines were established, characterized and shown to originate from SV40 T antigen-induced ependymoma. No pathological alterations were found in other organs, such as lungs and pancreas, in which cystic fibrosis is pathologically manifest in humans. Such transgenic mice and derived cell lines may represent valid models for analysing (1) the role of SV40 T antigen in ependymoma formation and (2) CFTR function in ependymal cells.

Animals↗
PubMed1983

[Andrologic anomalies in various gastrointestinal diseases].

In several gastroenterological diseases andrological anomalies have become known. From this point of view up to now the liver cirrhosis has been examined most frequently. In patients with cirrhosis apparently the two functions of the testicles are disturbed. On the one hand, a decreased or missing fertility is to be assumed, on the other hand, a cirrhosis does not always exclude the procreative capacity. The hormone analyses plead for the fact that the hypogonadism might rather be conditioned testicularly, in which case the direct toxicity of alcohol may be of importance. In haemochromatosis the hypogonadism develops by a combined mechanism. The cystic fibrosis of the pancreas is practically always associated with a male infertility. In Crohn's disease a disturbance of the spermatogenesis is observed. Even the salazo-sulphapyridine therapy is accompanied by unfavourable influences on the spermiogramme. Cimetidine used in the ulcer therapy shows a certain antiandrogenic effect and after a longer time may evoke impotence and other undesirable andrological side-effects which we, however, did not realize in 4 weeks treatment periods.

Cimetidine↗
PubMed2004

Additional disruption of the ClC-2 Cl(-) channel does not exacerbate the cystic fibrosis phenotype of cystic fibrosis transmembrane conductance regulator mouse models.

Cystic fibrosis is a fatal inherited disease that is caused by mutations in the gene encoding a cAMP-activated chloride channel, the cystic fibrosis transmembrane conductance regulator (CFTR). It has been suggested that the cystic fibrosis phenotype might be modulated by the presence of other Cl(-) channels that are coexpressed with CFTR in some epithelial cells. Because the broadly expressed plasma membrane Cl(-) channel, ClC-2, is present in the tissues whose function is compromised in cystic fibrosis, we generated mice with a disruption of both Cl(-) channel genes. No morphological changes in their intestine, lung, or pancreas, tissues affected by cystic fibrosis, were observed in these mice. The mortality was not increased over that observed with a complete lack of functional CFTR. Surprisingly, mice expressing mutant CFTR (deletion of phenylalanine 508), survived longer when ClC-2 was disrupted additionally. Currents across colonic epithelia were investigated in Ussing chamber experiments. The disruption of ClC-2, in addition to CFTR, did not decrease Cl(-) secretion. Colon expressing wild-type CFTR even secreted more Cl(-) when ClC-2 was disrupted, although CFTR transcript levels were unchanged. It is concluded that ClC-2 is unlikely to be a candidate rescue channel in cystic fibrosis. Our data are consistent with a model in which ClC-2 is located in the basolateral membrane.

Animals↗
PubMed1977

Minocycline in the antibiotic regimen of cystic fibrosis patients: weight gain and clinical improvement.

Minocycline hydrochloride was given to 100 patients with cystic fibrosis of the pancreas for periods of 3 months over a broad-spectrum antibiotic drug rotation which lasted 2 years. Increased weight gain and some clinical improvement was seen during treatment with minocycline. Those patients with severe disease gained more weight than those with mild or moderate disease, and males gained more than females. When minocycline hydrochloride was not the drug in use, patients lost weight and their health declined.

Adolescent↗
PubMed1975

Conjunctival goblet cells in patients with cystic fibrosis.

In five patients with cystic fibrosis of the pancreas the mucous glandular system of the conjunctiva was studied, as changes, if any, in the conjunctival goblet cells might be applicable as a diagnostic test in questionable cases. A whole-mount technique was used, specially developed for studying conjunctival goblet cells. In all five cases the qualitative as well as quantitative goblet-cell findings were in accordance with a previously reported normal material. In particular, there were no signs of stagnated secretion.

Child↗
PubMed1977

Influence of lyophilized total pancreas on plasma lipids and on fat absorption in cystic fibrosis.

31 children with cystic fibrosis (CF) subdivided into three groups of age were studied before and after treatment with lyophilized pancreas and compared to 27 controls. CF was characterized by lower phospholipid, cholesterol and polyunsaturated fatty acid plasma concentrations and reduced coefficient of fat absorption. Treatment tended towards normalization of these values. With age polyunsaturated fatty acids increased in normals but not in CF. The significant correlations existing between fat absorption coefficient and polyunsaturated fatty acid percentage in neutral lipids of CF children suggests the use of the latter percentage as index of the stage of malabsorption.

Adolescent↗
PubMed1982

Comparisons of eccrine sweat gland anatomy in genetic, chromosomal, and other diseases, and a suggested procedure for use of sweat gland measurements in differential diagnosis.

Statistical analysis of the dimensions of microdissected eccrine sweat glands (duct length, coil volume, ratio of coil volume to duct length, and axis ratio of coil) was performed for several diseases (cystic fibrosis of the pancreas, Werdnig-Hoffmann disease, tetralogy of Fallot, chronic renal disease, and trisomies 13, 18, and 21) using both individual and grouped age-matched control patients. Duct length, coil volume, and the ratio of the two all rise with age. Eccrine gland duct length was found to be significantly large in tetralogy of Fallot and Werdnig-Hoffmann disease and small in chronic renal disease (less so in males than in females, trisomy 13 and trisomy 18). Secretory coil volume was significantly smaller than normal in trisomy 21 (Down syndrome) and in chronic renal disease, and the ratio of coil volume to duct length was low in trisomy 21 and chronic renal disease. The shape of the secretory coil (axis ratio) was possibly abnormal in trisomy 13. Gland dimensions were normal for cystic fibrosis. Using the multivariate procedure of discriminant analysis, it was found that sweat gland measures significantly contributed to the differentiation of diseases, after adjustments were made for variations in age-at-death. This suggested the possibility that criteria for distinction of clinically similar genetic, metabolic, or chromosomal diseases by study of the anatomic properties of eccrine glands obtained by skin biopsy could be developed. A procedure of analysis comparing the "percentage of normal" of gland dimensions for each disease to control values, and thereby differentiating disease categories on the basis of the "percentage of normal" values, is presented.

Age Factors↗
PubMedDate not supplied

Multifocal myocardial necrosis: a distinctive cardiac lesion in cystic fibrosis, lipomatous pancreatic atrophy, and Keshan disease.

Multifocal myocardial necrosis (MMN) is an unusual cardiomyopathy of childhood, characterized by multiple patchy areas of myocardial fiber necrosis/fibrosis involving mainly the middle part of the left ventricle, but also, to a lesser extent, the right ventricle and the atria. These necrotic lesions are isolated and are not accompanied by an inflammatory reaction or vascular alterations. They are responsible for acute cardiac failure. MMN lesions are observed in various pathologic conditions including cystic fibrosis of the pancreas, pancreatic lipomatous hypoplasia/atrophy, malnutrition due to extensive intestinal resection with subsequent total parenteral feeding, and in Keshan disease. MMN is the main and the most characteristic feature of Keshan disease, an endemic and idiopathic condition affecting Chinese rural children. The causes and mechanisms of MMN presently are unknown. However, the presence of similar cardiac lesions in such different pathological conditions suggests the role of a selective deficiency of a hypothetical extrinsic factor (selenium, molybdenum iodide, other), probably crucial for the metabolism of the myocardial fiber.

Atrophy↗