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91.
Williams syndrome (WS) usually occurs sporadically. Few familial cases of Williams syndrome have been described, and those reports have often lacked photographic documentation. We describe 3 families, including a 3-year-old boy and his 34-year-old father, a 2-year-old girl and her 30-year-old mother, and a 3-year-old girl and her 31-year-old mother. None of these patients has supravalvular aortic stenosis or chromosome abnormalities. In all 3 families, the parent with Williams syndrome was diagnosed after the identification of the syndrome in the affected child. © 1993 Wiley-Liss, Inc.  相似文献   
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Immunocytochemical staining after controlled proteolytic treatment of the sections with pronase revealed widespread distribution of neuronal cell bodies with somatostatin- like immunoreactivity (SLI) in the rat forebrain. SLI-positive neurons were found in regions of the neocortex, the pyriform cortex, the cingulate cortex, the striatum, the olfactory tract and tubercle, the nucleus accumbens, the septum, and the hypothalamus. These results are consistent with previous radioimmunoassay findings and suggest the presence of large somatostatin-like (possibly precursor) molecules in the neurons stained for SLI after pronase treatment.  相似文献   
95.
Diagnosis of Pneumocystis carinii pneumonia by bronchopulmonary lavage   总被引:5,自引:0,他引:5  
W L Drew  T N Finley  L Mintz  H Z Klein 《JAMA》1974,230(5):713-715
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96.
Plasma glucagon rises after major injury and could act to increase gluconeogenesis and ureagenesis in the post-traumatic state. This study documents the effect of prolonged glucagon infusion on ureagenesis and nitrogen excretion, as well as possible sources of the increased ureagenesis, in normal man. Four healthy men fasted for 6 days during intravenous infusion of glucose (750 gmday), establishing a steady state of minimal ureagenesis. Glucagon (1 mg/day) then was added to the infusion for 5 days. Glucose alone was given for the final 2 days. Forearm muscle flux of metabolites was determined by standard arterial-deep venous sampling and capacitance plethysmography. Glucagon concentration was suppressed during glucose infusion (11 +/- 13 pg/ml) and rose to levels seen in subjects with major trauma during glucagon infusion (669 +/- 138 pg/ml). Glucose infusion stabilized urine nitrogen excretion at 1.54 +/- 0.42 gm of N/sq m/day. Nitrogen excretion increased to 2.40 +/- 0.53 gm of N/sq m/day with glucagon infusion, with urea accounting for the increased excretion. Excretion of 3-methylhistidine was unchanged. Plasma amino acid concentration was strikingly reduced on the first day of glucagon infusion, where it stabilized. Forearm flux showed a slight net release of amino acid nitrogen during glucose infusion. Addition of glucagon to the glucose infusion resulted in a net uptake of nitrogen by forearm skeletal muscle. These evidences strong suggest that glucagon infusion in normal man increases ureagenesis, not only at the expense of the free amino acid pool, but by the hydrolysis of visceral protein as well, with muscle protein being maintained.  相似文献   
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Concentrations of mercury in passerine birds fed diets containing 40 ppm methylmercury were similar in tissues of birds that died from mercury poisoning and in those that were sacrificed after half the group had died. Residues were higher in tissues of birds that died, but the differences were not statistically significant. Residue levels were highest in livers, followed by kidneys and brains. Levels of mercury were similar in breast muscle, carcass, and whole body. Mercury levels were highest in redwinged blackbirds, lowest in grackles, and intermediate in starlings and cowbirds. Mercury concentrations exceeded 20 ppm in all tissues of all species and were similar to levels reported in wild birds known to have died of mercury poisoning.  相似文献   
99.
A 48 year-old woman with pyelonephritis was found to have bilateral ureteropelvic junction (UPJ) obstruction and a nonfunctioning right kidney. She initially underwent a laparoscopic left nondismembered pyeloplasty using absorbable polydioxanone Lapra-Ty suture clips (Ethicon Endosurgery, Cincinnati, OH) to secure the anastomosis. An antegrade endopyelotomy was later necessitated. Both procedures were complicated by postoperative bacteruria and funguria. She then underwent a laparoscopic dismembered pyeloplasty, again utilizing Lapra-Ty suture clips to secure the anastomosis. Postoperatively, her course was complicated by anastomotic extravasation, bacteriuria, and funguria. Subsequently, an antegrade nephrostogram revealed a 2.5-cm diverticulum just distal to the UPJ, which contained numerous 2- to 4-mm filling defects. Nephroscopic exploration of the pseudodiverticulum revealed numerous Lapra-Ty clips, which were basket extracted. The pseudodiverticulum was fulgurated with a holmium laser. She eventually had restricturing with recurrence of the pseudodiverticulum and was treated successfully by open ureterocalicostomy.  相似文献   
100.
Broccoli is a food often consumed for its potential health-promoting properties. The health benefits of broccoli are partly associated with secondary plant compounds that have bioactivity; glucosinolates and phenolic acids are two of the most abundant and important in broccoli. In an effort to determine how variety, stress, and production conditions affect the production of these bioactive components broccoli was grown in the greenhouse with and without selenium (Se) fertilization, and in the field under conventional or organic farming procedures and with or without water stress. High-performance liquid chromatography/mass spectrometry was used to separate and identify 12 primary phenolic compounds. Variety had a major effect: There was a preponderance of flavonoids in the Majestic variety, but hydroxycinnamic esters were relatively more abundant in the Legacy variety. Organic farming and water stress decreased the overall production of phenolics. Se fertilization increased glucosinolates in general, and sulforaphane in particular, up to a point; above that Se fertilization decreased glucosinolate production. Organic farming and water stress also decreased glucosinolate production. These data show environmental and genetic variation in phenolics and glucosinolates in broccoli, and warn that not all broccoli may contain all health-promoting bioactive components. They further show that selection for one bioactive component (Se) may decrease the content of other bioactive components such as phenolics and glucosinolates.  相似文献   
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