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At least 1,045 records · Page 58Linked to original sources
PubMed1985

Effect of stanozolol on factors VIII and IX and serum aminotransferases in haemophilia.

The treatment of haemophilia has been dramatically improved since the introduction of factor VIII and IX concentrates, however these concentrates have brought new problems such as hepatitis and A.I.D.S. An oral agent which could raise endogenous levels of factor VIII and IX would be of great benefit. Danazol, an anabolic steroid, has recently been shown to increase levels of factors VIII and IX in haemophilia. We therefore studied the effect of stanozolol, a closely related anabolic steroid, in 15 patients with haemophilia A or Christmas disease over a 2-4 week period. There was no consistent change in factor VIIIc or factor IX, and fibrinolysis was significantly enhanced. No effect was apparent on the incidence of spontaneous bleeds. However serum aminotransferases which were abnormal in 11 of the 15 patients at the start of the study fell significantly with stanozolol therapy. This raises the interesting possibility that anabolic steroids may be beneficial in patients with chronic liver diseases.

Blood Viscosity↗
PubMed1985

Effect of danazol on coagulation parameters and bleeding in hemophilia.

Danazol was given orally at a dose of 600 mg/day to six hemophiliacs for eight to 14 weeks. All patients showed a significant decrease in activated partial thromboplastin time (APTT) beginning with the first measurement (two weeks) and persisting until use of the drug was discontinued. However, a corresponding increase in the deficient factor activity could not be consistently demonstrated. Despite the shortened APTT, bleeding episodes continued in the severe hemophiliacs and the patient with Christmas disease. In four patients, bleeding appeared to increase in severity or change in pattern, and in two cases the bleeding manifestations did not respond to usual factor infusions but responded to discontinuation of the drug therapy and further factor replacement. Euglobulin lysis times were measured in five patients (one hemophiliac and four with nonhemophilic conditions) who were receiving danazol. The lysis times were markedly shortened. Increased fibrinolytic activity may be responsible for the increased bleeding manifestations in danazol-treated hemophiliacs.

Adult↗
PubMed1977

[Transcription complexes in subnuclear fractions isolated from mammalian cells: ultrastructural study].

Miller Beatty's technique was adapted to the study of definite chromatin fractions (nucleolar and nonnucleolar chromatin) isolated from Mammalian cells. The ultrastructural organization of the transcriptional complexes obtained depended on the nuclear compartment studied. In isolated nucleoli, there were "Christmas-tree"-like figures. In nonnucleolar chromatin, the figures were different from the former by the internal structure of the RNP fibrils being synthesized.

Animals↗
PubMed1985

Genetic disorders on the island of St Helena.

The island of St Helena lies in the South Atlantic ocean and is isolated by distance from any land mass and by lack of a harbour or airfield. In these circumstances there could be an unusual pattern of inherited disease, and for this reason we have attempted to document the presence and frequency of genetic conditions in the population, which numbers approximately 5 500. Genetic disorders identified included autosomal dominant genu valgum, hereditary angioneurotic oedema, Christmas disease and pseudo-achondroplasia. The presence of the autosomal recessive form of retinitis pigmentosa provides a possible explanation for the occurrence of that condition in the islanders of Tristan da Cunha, since they have ancestral links with the St Helena community. The recognition of these genetic disorders in this isolated population has practical implications in terms of clinical management and the provision of medical services.

Achondroplasia↗
PubMed1978

[Visualization of transcription complexes in spread chromatin from mammalian cells: high resolution autoradiographic study].

RNA transcription processes were visualized within chromatin from cultured Mouse cells, spread according to Miller, and Bakken (3), by high resolution autoradiography. The cells were labelled for a short time with 3H-uridine and lysed using the detergent Nonidet P 40. Transcription complexes of both ribosomal ("Christmas tree"-like forms) and non-ribosomal types were revealed and their structure is described.

Autoradiography↗
PubMed1971

The activated coagulation time of whole blood as a routine pre-operative sceening test.

Patients with disorders of hemostasis who undergo surgical procedures are in danger of hemorrhage. While the careful medical history remains the most sensitive test of a bleeding tendency, some such patients can give no suggestive history. In three patients with coagulopathy-one with mild classical hemophilia, one with Christmas disease, and one with warfarin toxicity-the abnormality was missed by routine preoperative history but promptly detected by the routine preoperative use of the activated coagulation time (act). Either this test or the activated partial thromboplastin time should be included in the routine preoperative work-up, along with appropriate additional tests of the hemostatic mechanism.

Adult↗
PubMed1979

[Prophylaxis of suicide in old age (author's transl)].

The author reports on sociopsychiatric care of old alone living peoples during Christmas-time and the end of year. This help of living was given under the primary aspects of suicide prevention. Nevertheless the conception of the arrangement is important for geropsychiatric prevention at all. The author gives informations about effects of this type of care. After the two weeks living in the hospital and getting special care the most of the old people looks from an active point of view to their biological and social conditions of aging, and better than before they can manage their life.

Aged↗
PubMed1977

Influenza. The Birmingham Research Unit of the Royal College of General Practitioners.

The ;weekly returns' system for the reporting of infectious and communicable diseases to the Birmingham Research Unit of the Royal College of General Practitioners is described. A detailed analysis of the influenza returns for the winter epidemic of 1975/76 is presented and compared with similar data from the previous ten-year period.This analysis allows the following generalizations to be made which can, to a limited extent, be used as broad guidelines for predictions.In any week in which a rate of 20 or more reports per 100,000 population is followed by a week in which there is a trebling of the rate, a major epidemic is imminent in which a peak rate of 500 cases per 100,000 population can be expected within three to four weeks.In any week other than a week referred to previously in which a rate of 30 cases or more per 100,000 population is followed by a doubling of the rate, a moderate epidemic is imminent and peak rates in the range 150 to 500 per 100,000 population will be reached within three to four weeks.The earlier in the critical period just before and just after Christmas that either of these changes are noted, the earlier and larger the peak is likely to be. Where neither of these thresholds is crossed, the peak rate for reported influenza is unlikely to exceed 150 cases per 100,000 people.

Adolescent↗
PubMed1984

Congenital hemorrhagic disorders in Jordan.

The results of a three year prospective study of inherited bleeding syndromes in Jordan is presented. There were 112 patients from 64 families. Of these there were 42 patients with hemophilia A, 23 with Glanzmann's thrombasthenia, 22 with von Willebrand's disease, 11 with Christmas disease, 6 with hypofibrinogenemia, 3 with afibrinogenemia, 2 with factor XIII deficiency, 2 with storage pool disease and 1 with factor XI deficiency. The pattern of inherited bleeding syndromes in Jordan is different from that seen in Europe and U.S.A. in that Glanzmann's thrombasthenia is very common. High proportion of hemophiliacs were severe. Arthropathy was common. A significant number of bleeders had fatal hemorrhage. In a high proportion of patients, no family history of bleeding was found.

Adolescent↗
PubMed1984

Mild bleeding disorders: review of 120 patients.

Of 120 patients presenting with mild bleeding disorders, 63 were found to have a definite coagulopathy. The commonest disorders were haemophilia, Christmas disease and von Willebrand's disease (vWd), the latter being predominant. Diagnosis led to prophylactic treatment prior to surgery in 18 patients with prevention of excessive haemorrhage. Three patients who had received blood products developed hepatitis. DDAVP (desamino-cys-1-8-D-arginine vasopressin) is the treatment of choice in suitable mildly affected patients with haemophilia A and vWd. Examination of blood group distribution suggests an excess of group O among patients with bleeding disorders, especially those with vWd.

Adult↗
PubMed1983

[Treatment of nasal saddle-back deformities using a median naso-frontal five-point star flap].

Severe saddle-back deformities of the nose require reconstructive surgery using bone grafts, often in association with tegmental tissue when the point of the nose is retracted. To diminish the inconveniences of the multiple techniques proposed, the procedure employed involves the use of a mesiofrontal, inter-eyebrow sliding graft with its superior point in VY, and with a pedicle consisting of the remaining tissue of the nasal ridge. Four triangular flaps arise from the edges of this flap, their apices being external and supra- or infra-eyebrow in position. They are transposed downwards for one stage, into two infra-eyebrow and infra-canthal zones. These will fill the raw surfaces to allow the bone graft to be applied. Loss of frontal substance is compensated by a VY above and by apposition of the edges laterally. The lateral flaps replace the two peri-orbital losses of substance, and give a sufficient amount of tissue on either side of the nasal ridge to enable the bone graft to be covered without excessive tension. This technique presents the advantages of being performed as a one-stage operation and of producing less scarring. The flap has the shape of a star with five branches and is termed a christmas tree flap; the pedicle is situated below and forms the roots of the tree.

Humans↗
PubMed1984

A unique precipitating autoantibody against plasma thromboplastin antecedent associated with multiple apparent plasma clotting factor deficiencies in a patient with systemic lupus erythematosus.

A 42-yr-old woman with systemic lupus erythematosus without bleeding diathesis developed a prolonged activated partial thromboplastin time that was not corrected by normal plasma. An inhibitor that acted rapidly and inactivated 0.5 U/ml plasma thromboplastin antecedent (PTA, factor XI) at a 1:200 plasma dilution was demonstrated. In addition to a low titer of PTA (less than 0.01 U/ml), plasma assayed at 20-fold dilution also showed low titers of Hageman (factor XII, 0.02 U/ml), Fletcher (plasma prekallikrein, 0.02 U/ml), and Fitzgerald (high molecular weight kininogen, less than 0.01 U/ml) factors. The titer of these factors, except PTA, returned to normal upon further plasma dilution or upon removal of the inhibitor by protein A adsorption. Thus, the inhibitor appeared to interfere with these clotting factor assays, possibly by inactivating PTA in the substrate plasmas in the test system. Its specificity was further confirmed. The inhibitor did not interfere with surface-induced proteolytic cleavage of Hageman factor. Surface-induced generation of plasma kallikrein activity (amidolysis of H-D-pro-phe-arg-pNa and cold-promoted factor VII activity enhancement) requires only Hageman, Fletcher, and Fitzgerald factors and was normal. Reactions requiring all 4 contact phase factors, including PTA, such as surface-induced generation of plasmin activity (amidolysis of H-D-val-leu-lys-pNa) and activated Christmas factor (factor IXa) activity, were defective. Furthermore, the inhibitor bound to agarose-protein A inactivated and removed PTA selectively from normal plasma. The inhibitor was an IgG-lambda autoantibody that precipitated PTA. The inactivated activated PTA (factor XIa) without the requirement for an additional cofactor. Furthermore, it inhibited surface-induced activation of PTA by interfering with its proteolytic cleavage upon glass surface exposure and with its binding onto the reactive surfaces.

Adult↗
PubMed1983

The management of musculoskeletal problems in hemophilia. Part I. Principles of medical management of hemophilia.

Musculoskeletal bleeding in general and arthropathy in particular are the central problems among many in the two major forms of hemophilia: classic hemophilia, caused by factor VIII deficiency, and Christmas disease, caused by factor IX deficiency. Currently available replacement therapy, if properly used in a multidisciplinary setting, should provide significant benefit to hemophilic patients despite its cost and occasional complications.

Anemia, Hemolytic↗
PubMed1984

Trichinosis in southern Lebanon.

An outbreak of trichinosis in two villages in southern Lebanon affected over 100 patients, aged 3 to 70 years, who celebrated Christmas and New Year's of 1981 by feasting on rare pork. Six were hospitalized in Israel. The diagnosis was made on clinical and histological grounds. The clinical picture included the classical features, but was unusual in that rarely reported pedal or pretibial edema, pruritus and vertigo, were prominent. There was one case with suspected myocarditis. Two women in the first trimester of pregnancy had miscarriages. All patients recovered. With the unstable political situation in Lebanon in recent years, veterinary and sanitary supervision has deteriorated, and most pigpens are infected with trichinosis.

Abortion, Spontaneous↗
PubMed1980

The Darwin outbreak of oyster-associated viral gastroenteritis.

Approximately 60 persons attended a Christmas dinner, at a Darwin hotel, where oysters were served au natural as part of the menu. Twenty-five of the 28 persons who ate oysters developed symptoms of food poisoning--an attack rate of 89%. Of the 60 persons attending the dinner 44 were investigated. The incubation period and duration of illness were about 36 hours. Diarrhoea occurred in 100% of patients, with colic and nausea in 88% and 80% respectively. Half the patients complained of vomiting and headache. The storage temperature at which the oysters were kept was satisfactory and no bacterial pathogens were grown from the oyster and stool specimens. Electron microscopy revealed two distinct parvovirus-like particles in stool specimens, one of which was identified as Norwalk virus. Serological studies by immune electron microscopy showed the development of antibodies to the Norwalk-like particle by seven out of 10 patients. Confirmatory studies by radioimmunoassay showed a significant rise in antibody titre to Norwalk virus in seven patients.

Australia↗
PubMed1980

Management of common congenital disorders of haemostasis.

The management of haemophilia, Christmas disease and von Willebrand's syndrome are reviewed. The principal advances in diagnosis are the measurement of procoagulant activity, the ristocetin cofactor and the levels of the protein associated with procoagulant activity by immunologic methods. The mainstay of treatment is replacement of the coagulation factors by partially or highly purified blood products.

Antibodies↗
PubMed1981

Organization of transcribed and nontranscribed chromatin in isolated nuclei of Zea mays root cells.

A slightly modified technique of Miller was applied to nuclei isolated from roots of germinating corn embryos. Under the conditions used, the extranucleolar chromatin was easily spread but the nucleolar chromatin often remained aggregated. The nontranscribed chromatin regions consisted of long deoxyribonucleoprotein (DNP) fibrils with the typical "beads-on-a-string" appearance of nucleosomal arrangement. When the EDTA concentration of the spreading medium was increased, linear arrangement of nucleosomes, 20 nm and 40-50 nm DNP granules were observed on the same DNP fibril. The supranucleosomal granules were also closely packed without any apparent order. Independently of the EDTA concentration, numerous non-nucleolar ribonucleoprotein (RNP) fibrils with a knobby structure were seen laterally bound to DNP fibrils. These knobby RNP fibrils (transcripts) were highly folded and appeared in "bunch-of-grapes" configurations. Each RNP knob measured 23 nm in diameter and the longest of these RNP fibrils were estimated to reach 1.5 micron. The RNP fibrils were often solitary and never exceeded 8 per 10 micron of the DNP axis. Distribution, morphology, and size of these transcripts distinguished them from the nucleolar "Christmas-tree"-like figures, which were rarely observed with our material. These non-nucleolar transcriptional units may correspond to genes coding for the heterodisperse nuclear RNA (hnRNA) and the knobs of 23 nm could be related to informofers or hnRNA particles. Up to now, the visualization of transcriptional units for higher plant tissues have never been reported.

Chromatin↗
PubMed1981

Blood components for oral surgery.

World-wide blood demands are increasing at 18% per year. Around 50% of collected blood is used whole -- for surgery, accident victims, anaemias, malignancies, and haemoglobinopathies. Blood component therapy is placing extraordinary demands upon Blood Collection Services. This therapy uses multiple or single cell types, and a variety of plasma-derived components. Malignant over-growth depresses marrow function; and, active treatment, temporarily, similarly depresses cell production. Specific transfusion cells are available for the patient's protective mechanism. Platelets are required, fairly fresh, for depletion and for their primary clotting ability. Similarly, infused platelets are used to counter local or systemic drug sensitive platelet depression. From plasma components, specific coagulation products are available for health and during surgery for haemophilia, Christmas, and Von Willebrand's diseases. Prior to oral surgery, all possible coagulation abnormality problems need investigation and specific factors. Anti-coagulation therapy must be reduced or stopped as indicated by the need for therapy and the availability of the appropriate, previously depressed, Vitamin K dependent-clotting factors (II, VII, IX, X). Local measures, formerly the only treatment, are now of less importance. Some countries do not have these blood factors available.

Anticoagulants↗