First European case of ehrlichiosis.
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Adult↗
Biomedical subjects
C Greene
Publications and source records attributed to C Greene.
Developmental profile of patients with maple syrup urine disease.
The progress of nine maple syrup urine disease patients (eight classical, one possible variant) was reviewed to look for similarities in developmental patterns. A consistent developmental profile of stronger Verbal than Performance IQ and lower than familially expected IQ was seen. Younger age at diagnosis was associated with a milder neonatal course. Those who were asymptomatic or suffered mild complications in the newborn period have higher IQs than children experiencing moderate or severe complications. Children with asymptomatic or mild neonatal course also required less special education services. Overall, a picture of ubiquitous motor, visual-analytic and learning deficits is seen.
Age Factors↗
Lovastatin, isoprenes, and myopathy.
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Creatine Kinase↗
Treatment of idiopathic hepatic lipidosis in cats: 11 cases (1986-1987).
Idiopathic hepatic lipidosis was diagnosed in 11 cats. Cats were treated by delivery of balanced nutrients supplemented with L-carnitine via a surgically placed gastrostomy tube. Feeding through the gastrostomy tube was initiated in the hospital and was continued at home in all cats. The mean duration of gastrostomy tube feeding was 48 days (range, 22 to 98 days). Vomiting associated with feeding (3 cats) and localized cellulitis at the gastrostomy site (2 cats) were the most frequent complications. Vomiting was controlled by reducing the volume of food administered at each feeding or by administration of metoclopramide. Cellulitis was treated successfully by parenteral administration of antibiotics and local wound cleansing. Seven of 11 cats (65%) survived and have remained clinically healthy for 15 to 29 months (mean, 20 months) since diagnosis. The other 4 cats died of peritonitis (n = 1), pneumonia (n = 1), hepatic encephalopathy (n = 1), or cardiopulmonary arrest (n = 1) between 0 and 10 days after surgery.
Animals↗
Accumulation of phosphorylated neurofilaments in anterior horn motoneurons of amyotrophic lateral sclerosis patients.
Perikaryal collections of intermediate filaments have been described in the anterior horn motoneurons of patients with amyotrophic lateral sclerosis (ALS), but these inclusions have generally been considered rare and mainly associated with the familial form of ALS. Using the monoclonal antibody NF2F11, which recognizes phosphorylated neurofilament epitopes, we showed that focal collections of neurofilaments in anterior horn motoneurons were a characteristic finding in sporadic as well as in familial ALS; they were present in seven of nine ALS patients, but in none of nine control spinal cords. These neurofilamentous collections are not cross-reactive with antibodies directed against paired helical filaments and the microtubule associated protein tau. In addition, diffuse staining for phosphorylated neurofilament epitopes in chromatolytic anterior horn perikarya was significantly more frequent in ALS patients than in controls.
Adult↗
Transplant aspiration cytology for diagnosis of liver allograft rejection.
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Biopsy, Needle↗
The association of neurofibromatosis, psammomatous ampullary carcinoid tumor, and extrahepatic biliary obstruction.
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Aged↗
Short-chain acyl-coenzyme A dehydrogenase deficiency. Clinical and biochemical studies in two patients.
We describe two patients with short-chain acyl-coenzyme A (CoA) dehydrogenase (SCADH) deficiency. Neonate I excreted large amounts of ethylmalonate and methylsuccinate; ethylmalonate excretion increased after a medium-chain triglyceride load. Neonate II died postnatally and excreted ethylmalonate, butyrate, 3-hydroxybutyrate, adipate, and lactate. Both neonates' fibroblasts catabolized [1-14C]butyrate poorly (29-64% of control). Neonate I had moderately decreased [1-14C]octanoate catabolism (43-60% of control), while neonate II oxidized this substrate normally; both catabolized radiolabeled palmitate, succinate, and/or leucine normally. Cell sonicates from neonates I and II dehydrogenated [2,3-3H]butyryl-CoA poorly (41 and 53% of control) and [2,3-3H]octanoyl-CoA more effectively (59 and 95% of control). Mitochondrial acyl-CoA dehydrogenase (ADH) activities with butyryl- and octanoyl-CoAs were 37 and 56% of control in neonate I, and 47 and 81% of control in neonate II, respectively. Monospecific medium-chain ADH (MCADH) antisera inhibited MCADH activity towards both butyryl- and octanoyl-CoAs, revealing SCADH activities to be 1 and 11% of control for neonates I and II, respectively. Fibroblast SCADH and MCADH activities were normal in an adult female with muscular SCADH deficiency.
3-Hydroxybutyric Acid↗
Lack of transmission of human immunodeficiency virus from infected to uninfected chimpanzees.
Four uninfected chimpanzees were each housed in separate cages with an HIV-infected chimpanzee for six to twenty-nine months. Despite close daily contact, all uninoculated chimpanzees remained seronegative for HIV, and virus was never isolated from peripheral blood mononuclear cells of the uninfected chimpanzees. These data indicate that the probability of transmission from infected animals to humans is extremely low and also provide supportive evidence for lack of transmission of HIV by casual contact.
Acquired Immunodeficiency Syndrome↗
Hypercalcemia associated with blastomycosis in dogs.
Hypercalcemia that could not be explained by previously recognized causes was detected in 4 dogs with systemic blastomycosis. A variety of malignancies and certain endocrine, metabolic, and renal disorders may cause hypercalcemia, but to our knowledge, no infectious diseases have been associated with hypercalcemia in dogs. Increased production of calcitriol by mononuclear cells was thought to be the mechanism of hypercalcemia in granulomatous disease.
Animals↗
Electroresection followed by neodymium-YAG laser photocoagulation of the dog prostate for establishment of safety parameters.
Electroresection of the prostate followed by Nd-YAG laser coagulation of residual prostate was carried out in eight dogs. All dogs voided spontaneously without significant bleeding in the immediate postoperative period. At the time of sacrifice in 6 to 8 weeks, no damage to adjacent tissue was seen on microscopic examination. Re-epithelization had occurred. This study suggests that the addition of neodymium-YAG coagulation to residual tissue after TURP in humans can be done safely.
Animals↗
Prune belly syndrome and heart defect in one of monozygotic twins, following exposure to Tigan and Bendectin.
One of twins was born with prune belly syndrome and congenital heart defect following exposure to Bendectin and Tigan. Red cell antigens and HLA typing were compatible with monozygosity. The possible associations of the prune belly syndrome to monozygotic twinning or to teratogenic agents is considered in light of this patient and review of the literature.
Benzamides↗
Alterations in the time of X chromosome replication induced by 5-azacytidine in a patient with 48,XXXY/47,XXY.
It has recently been shown that 5-azacytidine (5-azaC) can induce altered replication patterns of the late-replicating X chromosome in normal female cells. This has been demonstrated by bromodeoxyuridine labelling of cells late in the S phase. In the present study the same method was applied to the lymphocytes of a Klinefelter patient (48,XXXY/47,XXY). Significant 5-azaC-induced changes in the replication of the entire inactive X chromosome, from late to early, were found in the lymphocytes of this patient. These results indicate that hypomethylating agents can not only alter the replication of individual bands, but also change the gross replication schedule of multiple inactive X chromosomes in the presence of a Y chromosome.
Azacitidine↗
Smith-Lemli-Opitz syndrome in two 46,XY infants with female external genitalia.
Two infants with features of the Smith-Lemli-Opitz (SLO) syndrome were found to have a 46,XY karyotype and female external genitalia. Autopsies showed normal testes for age with normal Wolffian duct structures and without Müllerian duct derivatives. This failure of masculinization of the external genitalia is an unusual finding and may represent the extreme of a spectrum of the genital anomalies commonly seen in males with this autosomal recessive syndrome. An endocrine evaluation on one of these infants at 3 months suggested unusually low testosterone production and meagre response to stimulation with human chorionic gonadotropin (hCG). The failure of complete masculinization of external genitalia in some cases of SLO syndrome may be due to inadequate testosterone production in utero.
Abnormalities, Multiple↗
Subcutaneous emphysema after extraction of maxillary teeth: report of a case.
Inappropriate use of dental equipment that is powered by highly compressed air may directly lead to the formation of subcutaneous emphysema that involves the fascial planes of the head and neck. The high-speed dental handpiece and the air and water dental syringe are the instruments that are most commonly implicated in the reported cases. The treatment of such cases requires a reassured patient, prophylactic antibiotic coverage, and close postoperative follow-up care. Patients should avoid maneuvers that could increase intraoral pressure. Complete resolution should be expected within one week.
Dental Instruments↗
3-hydroxy-3-methylglutaric aciduria: a new assay of 3-hydroxy-3-methylglutaryl-coa lyase using high performance liquid chromatography.
A new assay has been developed for 3-hydroxy-3-methylglutaryl-CoA lyase, the final enzyme in the leucine degradative pathway. The assay was performed by incubating lysates of fibroblasts with [glutaryl-3-14C](D,L)-3-hydroxy-3-methyl-glutaryl coenzyme A. The products were analysed by high performance liquid chromatography with continuous liquid scintillation counting. This provided simultaneous identification and quantification of one of the enzymatic products, [3-14C]acetoacetic acid. The mean 3-hydroxy-3-methylglutaryl-CoA lyase activity in fibroblasts from five controls was 732 +/- 81 (SD) pmol/min X mg protein. Using this assay, we have studied skin fibroblasts cultured from a patient with 3-hydroxy-3-methylglutaric aciduria and found 3% of normal 3-hydroxy-3-methylglutaryl-CoA lyase activity. The activities in skin fibroblasts cultured from the parents were 46 and 53% of control activity which is consistent with heterozygocity. Kinetic studies of 3-hydroxy-3-methylglutaryl-CoA lyase in skin fibroblasts cultured from two normal subjects yielded Km values of 14.4 and 18.8 mumol/l for 3-hydroxy-3-methylglutaryl-CoA.
Acetoacetates↗
Biochemistry of Fern Spore Germination: GLYOXYLATE AND GLYCOLATE CYCLE ACTIVITY IN ONOCLEA SENSIBILIS L.
In chlorophyll-containing spores of Onoclea sensibilis, depletion of lipid reserves during germination is correlated with increases in the activity of the glyoxylate cycle enzymes isocitrate lyase and malate synthase. In Onoclea, the heterotrophic activity associated with lipid catabolism occurs at the same time that autotrophic activity is taking place. Increases in chlorophyll content and in the activity of glycolate oxidase were recorded during the earliest stages of spore germination. In this species, there is no temporal separation of heterotrophic and autotrophic reactions. Concurrent increases in glyoxylate and glycolate cycle activities appear to occur naturally.
Journal Article↗
Modified Steiner method for the demonstration of spirochetes in tissue.
The substitution of lead nitrate for uranium nitrate as used in the Steiner silver impregnation method for demonstrating spirochetes in tissue sections is described. The use of lead nitrate provides a chemical substitute free of any potential radiation hazard without loss of staining specificity. The use of lead nitrate in place of uranium nitrate in the Dieterle method for staining Legionnaires' disease organisms is proposed.
Histological Techniques↗