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Mortality from external causes in South African adolescents, 1984-1986.

The external causes of death in South African adolescents are described. Nationally registered mortality data for 1984-1986 were used to calculate proportional mortality. Mortality rates were also calculated, except in the case of black deaths, since these deaths are known to be under-registered and the estimated population figures are known to be inaccurate. Of the 16,348 adolescent deaths registered in 1984-1986, external causes accounted for 56.8% and symptoms, signs and ill-defined conditions for 10.0%. A greater proportion of girls died from symptoms, signs and ill-defined conditions whereas a greater proportion of boys died from external causes. A larger proportion of black adolescent deaths were categorised as symptoms, signs and ill-defined conditions. The risk of death by external cause for coloureds aged 15-19 years was 1.7 that of whites, while in the 10-14 year age group it was the same as that of whites. In the 15-19-year age group assault was the most common external cause of death in blacks and coloureds, compared with road accidents for whites. The highest number of deaths by external cause per day occurred over the Christmas period. The analysis indicated that mortality rates in South African adolescents are high and that many deaths may be the result of risk-taking behaviour. With the increasing urbanisation of blacks, the impact of external causes of death can be expected to increase further.

Accidents↗

Stealing and dealing: cocaine and property crimes.

A common thread in all studies of this nature is the level of use of cocaine and/or the concomitant use of other drugs, suggesting that economic necessity plays a role in the decision to commit crimes to help defray the costs of use. While a truly causal link between use and crime activity in marginal income populations is apparent. Whether that association is driven primarily by economics or lifestyle considerations is not answered by simple examination of the numbers. Statistically, the use of cocaine is related to criminal activity as a function of the income level and prior criminal experience of the user. This relationship is better defined by looking at the threshold effect in marginal income groups, where use that goes beyond what the pocket can bear produces a significantly greater chance that illegal sources will be found. However, many occasional users or even regular users with resources are able to fund their use through routine sources and never resort to criminal activity or to unconventional financial resources. A large number of cocaine users probably fall into this middle ground: they are neither the "high rollers" that often make the media nor the traditional heroin/cocaine addicts. For them, criminal activity may surface when use exceeds funds or not at all. For still others, cocaine is part of a criminal lifestyle rather than a motivation for it. Statistically, all these cocaine users look the same, though the relationship between their use and their crime may be quite varied. The descriptions of three cases discussed in an earlier paper (Hunt et al. 1985) clarify this point. The first case was a 32-year-old white male former heroin addict and former drug dealer who reported cocaine use intravenously three to four times a month, smoked marijuana weekly, and used no other drugs. He was married, working, and had a small child. He also reported dealing in stolen merchandise and clothing that he got from someone else to sell. This pattern had been his custom for several years, observed at close hand by the author, and he had not been arrested for many years, though he had a prior history of arrests dating back more than 15 years. This individual did not link his selling stolen merchandise with his cocaine use. He linked it with the need for supplementary funds and was as likely to deal goods for Christmas money as for cocaine.(ABSTRACT TRUNCATED AT 400 WORDS)

Cocaine↗

Haemophilia in Zimbabwe.

Of the 120 haemophiliacs diagnosed in Zimbabwe in 8 years, haemophilia A and Christmas disease accounted for 90% and 10% respectively (i.e. a ratio of 9:1). Although the clinical and laboratory parameters were essentially similar to those previously described in Caucasian, African and other populations in the World, sub-haemophiliac cases are probably still being missed particularly in very busy health centres where the index of suspicion is low and malnutrition and infectious disease predominate and therefore readily attract the attention of most health workers. However, with the steadily improving socio-economic status and decentralization of health care facilities, more of these cases are likely to be diagnosed. Major constraints in the diagnosis and management of haemophilia in an African setting are succinctly discussed; including home therapy; and the implications of recent findings of HIV sero-positivity. The study serves as evidence that haemophilia is common in Zimbabwe contrary to earlier published literature.

Adolescent↗

Notes on clotting in a Burmese python (Python molurus bivittatus).

Studies of the clotting mechanisms in the plasma of a Burmese python (Python molurus bivittatus) confirm earlier information that both extrinsic and intrinsic pathways of thrombin formation participate in reptilian hemostasis. Plasma fibrinogen was present at a concentration comparable to that in human plasma. Other assays were hampered by the need to use nonreptilian reagents. The activated partial thromboplastin time was shorter than was that of human plasma, thus implying the presence of prothrombin in python plasma; however, this protein could be demonstrated only in trace amounts. Similarly, only small amounts of Hageman factor (factor XII) and antihemophilic factor (factor VIII) were detected, and none of plasma prekallikrein, high-molecular-weight kininogen, and Christmas factor (factor IX). The prothrombin time was slower than that of human plasma. Factor VII was not detected, but both proaccelerin (factor V) and Stuart factor (factor X) were present. Python plasma inhibited bovine thrombin and human plasmin, but it was deficient in fibrinolytic capacity.

Animals↗

Surveillance for injuries: cluster of finger amputations from snowblowers.

In an investigation of the health effects of a Christmas eve snowstorm in 1982, a review of emergency room records in the Denver area identified a cluster of 17 cases of finger amputations. Fifteen (88) percent of these amputations were associated with snowblower use. An additional 12 persons with hand or finger injuries without amputations from snowblowers were identified. A case-control study was performed comparing these patients with a control group who had used snowblowers. Patients were more likely than controls to have had their machines become clogged with snow (odds ratio [OR], 3.4, 95 percent confidence limits [CL], 0.74-15.4). Using a hand to dislodge trapped snow was the only risk factor identified for the patients (OR, 116; 95 percent CL, 16-820). No differences were found for other variables such as type of snowblower, instruction for use, or previous experience using a snowblower. The findings suggest that the most feasible measure to prevent such injuries is a change in snowblower design to preclude entry of a hand while the machine is running. This investigation illustrates the importance of surveillance in detecting and controlling injuries. Without such surveillance, the similarity among injuries reported on this paper would not have been recognized. Ongoing surveillance for injuries might identify other clusters of injuries.

Accidents, Home↗

In vitro amplification techniques for the detection of nucleic acids: new tools for the diagnostic laboratory.

The acceptance of nucleic acid probes as diagnostic tools for the clinical laboratory has been hampered by a number of factors, including laborious techniques and limited sensitivity. The focus of this review is on the recent development of amplification techniques to enhance the signal generated by nucleic acid-based detection systems. Three general areas are discussed: (1) amplification of target sequences using the polymerase chain reaction or the transcript amplification system, (2) amplification of the probe sequences using Q beta replicase, and (3) amplification of probe-generated signals with compound or "Christmas tree" probes. The hope of these new technologies is to simplify yet improve on the sensitivity of nucleic acid-based tests to enable them to attain a more prominent place in the diagnostic repertoire of the clinical laboratory.

Animals↗

Factor IX molecular defects in diagnosing hemophilia B: a review.

The past several years have seen an explosive growth in the application of recombinant DNA methods to study the molecular pathology of various inherited disorders. As a consequence, molecular defects responsible for the disease have been identified at the sequence level. In this review, I briefly describe the recent progress in the uses of factor IX gene probes in clinical diagnosis of hemophilia B (Christmas disease) carriers, as well as their use for analyzing the structural gene abnormalities that are responsible for the disease.

Chromosome Deletion↗

In vitro carboxylation of a blood coagulation factor IX precursor produced by recombinant-DNA technology.

Blood coagulation factor IX (Christmas factor) is a plasma protein which is required for normal haemostasis. A functional deficiency of factor IX results in haemophilia B, a bleeding disorder which is generally treated by infusions of factor IX concentrates prepared from pooled human plasma. The use of human blood products is connected with the risk of transmitting viral agents responsible for diseases such as hepatitis B and AIDS. Recombinant DNA techniques may provide the means to produce the required proteins without exposing the patients to these risks and at lower costs. One of the problems which has to be overcome before recombinant factor IX can be used for therapeutical purposes is related to the vitamin K-dependent carboxylation of its 12 NH2-terminal glutamate residues. In cell cultures this carboxylation, which is required to render the protein its procoagulant activity, is far from complete, especially at high expression levels. In this paper we describe the in vitro carboxylation of non and/or partly carboxylated recombinant factor IX produced by transformed Chinese hamster ovary cells. The identity of the newly formed Gla residues was verified and it could be demonstrated that all carboxyl groups had been incorporated into the recombinant factor IX.

Animals↗

Evaluation of a mass measles immunisation campaign in a rapidly growing peri-urban area.

A mass measles immunisation campaign, with a target coverage rate of 85-90%, was launched in Khayelitsha, a rapidly growing urban township in the Cape Town area. Cross-sectional surveys of the measles immunisation status of resident 6-23-month-old infants were conducted immediately before, immediately after, and approximately 2 months after the campaign in order to determine the effectiveness of a mass campaign in boosting coverage in an area with a high influx rate. Immunisation coverage rates were 55% in the first survey, 76% in the second, and 72% in the third. In the first survey, coverage rates for children born in Cape Town were 1.4 times higher than those born elsewhere. This trend continued throughout the survey. Duration of stay in Cape Town also influenced coverage, with rates for the recently arrived children being considerably lower than those for the more established Cape Town residents. The influx rate between the second and third survey (over the Christmas period) was 9.1% per month. Failure to reach the targeted rate is attributed to the influx rate, campaign design and implementation, and factors related to child and career mobility. Alternative immunisation strategies, with social awareness playing a key role, are being urgently investigated.

Cross-Sectional Studies↗

The molecular pathology of haemophilia.

The great success of recombinant DNA technology in unravelling the pathology of the thalassaemias at a molecular level has encouraged the application of these methods to other single gene disorders of man in the hope of gaining a deeper insight into the biochemical defects underlying them. An example of this approach is provided by the sex-linked recessive disorders of blood clotting: haemophilia and Christmas disease. These clinically indistinguishable, life-long disorders result from the deficiency or abnormality of the clotting proteins factor VIII and factor IX, respectively, which both participate in the activation of factor X in the intrinsic pathway of blood coagulation. This paper looks at the information concerning the molecular biology and pathology of the haemophilias which has recently been forthcoming. The genes for factor VIII and factor IX have both been successfully cloned within the past five years, with that of factor VIII, achieved in 1984, being a particular tour de force. It encompasses 0.1 per cent of the human X chromosome and is the largest gene yet characterised. Gene cloning is the starting point from which gene probes can be designed to elucidate the molecular pathology of the haemophilias. The implications of these discoveries for the practice of clinical medicine are reviewed, with special emphasis on prenatal diagnosis and carrier detection by means of restriction fragment length polymorphisms, and replacement therapy with recombinant factor VIII.

Genetic Engineering↗

[Hemophilic patients. Treatment protocol in the dental office].

Hemophilia is an inherited hemorrhagic disease which is due to the insufficiency of Factor VIII, or Factor IX, or Factor XI. Hemophilia patients are regarded as special patients with increased dental problems. The present paper consists of two parts. In the first part the types of hemophilia, ways of transmission, severity forms, and clinical characteristics are described. In the second part a protocol concerning the dental treatment of hemophilia patients is presented. There are four basic types of hemophilia: hemophilia A or classical hemophilia or Factor VIII deficiency, hemophilia B or Christmas disease, hemophilia C and von Willebrand's disease. Hemophilia is transmitted either as a sex-linked recessive or as an autosomal dominant trait, depending on the type of the disease. The severity of hemophilia depends on the amount of the coagulation factor present. According to this amount, there are four scales of severity. The clinical characteristics of the disease also depend on the amount of the factor present and vary, from occasional bleedings to serious and even life-threatening bleeding episodes. In the second part of the paper the special psychological and physiological problems of the hemophiliacs are discussed. In addition, there is reference to the hematologic coverage these patients need, as well as to the protection measures for the dental personnel against hepatitis and AIDS. The dental treatment plan at the office is presented in detail, including a discussion of the advantages and disadvantages of the treatment of hemophilia patients in the operating room under general anesthesia.

Dental Care for Persons with Disabilities↗

AIDS and haemophilia.

Approximately 1% of all AIDS cases are haemophiliacs. LAV/HTLV-III is transmitted by blood and in factor VIII concentrates. Since 1981, increasing numbers of haemophiliacs have been infected, as indicated by detection of antibodies to LAV/HTLV-III. Up to 90% of haemophiliacs in some populations are now seropositive, but to date less than 1% have progressed to clinical AIDS. Immunological abnormalities, in particular reduced T-lymphocyte helper/suppressor ratios, are common in haemophiliacs treated with factor VIII. Such abnormalities do not necessarily indicate past infection by the AIDS virus, but they may predispose to infection following exposure to the virus. Blood Transfusion agencies are introducing screening tests for antibodies to LAV/HTLV-III to help prevent the spread of AIDS by blood products. Heat treated factor VIII concentrate is now available and appears not to transmit AIDS. Factor IX concentrate may also transmit LAV/HTLV-III, but less frequently. A few cases of AIDS have occurred in Haemophilia B (Christmas Disease) patients.

Acquired Immunodeficiency Syndrome↗

Inherited bleeding syndromes in Iraq.

This paper presents data on the occurence and pattern of inherited bleeding syndromes (IBS) in Iraq, a hitherto unexplored problem. During the first fourteen months of a prospective on-going study at a major university center, 116 patients from 62 families were diagnosed as having IBS. All patients were referred because of moderate to severe bleeding diatheses. They included 62 haemophiliacs 32 patients with von Willebrand's disease (VWD), 9 with Christmas disease (CD), 6 with afibrinogenemia, 1 with prothrombin deficiency, and 6 were thought to have platelet dysfunction. 32 other bleeders (16 hemophiliacs, 14 VWD, and 2 CD) were also recognized among the pedigrees studied but were not available for full investigations. The clinical and laboratory features of the patients observed in Iraq do not seem to be significantly different from those of patients in Western Europe or North America. Although the absolute incidence and relative distribution of these disorders in the entire population cannot yet be determined, the rate of occurence per segment population is likely to be high, most likely due to the high rate of consanguinity and large number of births per family, phenomena still prevalent in this country.

Adolescent↗

Drug therapy reviews: clinical use of hemostatic agents.

Systemic hemostatic agents are reviewed. Among the agents discussed are vitamin K preparations (phytonadione, menadione, menadione sodium bisulfite, menadiol sodium diphosphate); and blood products (whole blood, plasma, cryoprecipitate, factor VIII concentrates, factor IX concentrates and fibrinogen concentrates). Normal and abnormal hemostasis and fibrinolysis are discussed, as is the general management of systemic hemostatic defects. Specific disorders covered are clotting factor deficiencies, hemophilia A, factor VIII inhibitors, von Willebrand disease, hemophilia B (Christmas disease), other congenital coagulation disorders, acquired deficiency of factors II, VII, IX and X, and defibrination syndrome.

Antibodies↗

Epidemiological trends of suicide in Puerto Rico: 1931 to 1985.

This work fulfills the need for an historic-developmental study of suicide in Puerto Rico. Mortality trends, risk groups and other demographical data with preventive and comparative value is presented. The data for this descriptive study was mainly taken from the Puerto Rico Vital Statistics and the Forensic Institute files. Analysis was done correlating incidence, age, sex and method. Seasonal tendencies were explored. Graphs were designed to illustrate the tendencies and interrelationship of the given variables. The results show that after a decline in incidence in the first 30 years, suicide mortality has been stable for the past 25 years (9.2 x 100,000). Age, sex and method showed certain interrelationships; 1) aging is directly proportional to an increase in suicidal risk. 2) Youth suicide rates have consistently diminished. 3) Adolescents tend to choose the traditional methods in Puerto Rico, with a recent increase in firearms use. 4) At age 16, males differ from females by increasing their suicide rates, particularly by hanging. 5) Adult females show unpredictability as to their methods. Seasonal risk of suicide was found statistically significant (ANOVA) for the Christmas and summer periods and major events affecting the general public.

Age Factors↗

Historical vignette #8. General Ulysses S. Grant's hip fracture.

Many biographers have discussed the throat cancer that plagued General Ulysses S. Grant toward the end of his life. However, little attention has been focused on his orthopaedic problems. On Christmas Eve in 1883, Grant slipped on an icy walk and fell. Following this accident he was bedridden for weeks and orthopaedically disabled for the remainder of his life. Although biographers have documented this incident, the outcome of the accident has been variously attributed to a sprain, muscle rupture, or simply a lack of exercise. The history of the injury and the nature of the symptoms and disability, which suggest that the General may have sustained a hip fracture, are reviewed with a discussion of the state of hip fracture diagnosis, treatment, and prognosis taken from the textbooks of the period.

Famous Persons↗

Danazol therapy in haemophilia.

Three patients with severe haemophilia A and one patient with moderately severe Christmas disease were given danazol and placebo in a double blind cross over trial to study the effects on haemostatic variables and bleeding tendency. In each case there was a shortening of the activated partial thromboplastin time (APTT) and euglobulin clot lysis time (ECLT) during danazol therapy but factor VIII and IX levels did not show any consistent change and there was no improvement in the bleeding tendency.

Adult↗

Haemophilic arthritis.

A detailed clinical and radiological examination of the joints and laboratory studies were carried out on 139 haemophiliacs attending a single centre. The group included more patients with mild and moderate haemophilia (factor levels 6 to 60 per cent) than in previous studies. Haemarthrosis, the most common bleeding manifestation, had affected more than two-thirds of patients including many with mild or moderate disease. Restriction and contracture of the knees and elbows were the most common clinical features and, with the ankles, these joints were most frequently affected both clinically and radiologically. Using a combination of clinical and radiological features, 42 per cent of the patients could be classed as having 'definite' and a further 14 per cent 'possible' haemophilic arthritis. Although haemarthroses were equally prevalent in patients with classical haemophilia and Christmas disease, arthritis was more frequently present in the former. Haemarthrosis and joint disease were exceptional in von Willebrand's disease. The prevalence of arthritis generally related to disease severity as measured by factor level but, in contrast to earlier studies, definite arthritis was seen in some patients with factor levels up to 20 per cent of normal although the number of affected joints was less in these patients with milder disease. Laboratory test abnormalities including circulating immune complexes and hypocomplementaemia were noted in some patients but the abnormalities correlated poorly with clinical features. The present results suggest a recent slight reduction in the prevalence or severity of haemophilic arthritis, possibly attributable to recent improvements in factor replacement treatment. Longer-term studies are required to show whether arthritis is indeed lessening or whether the onset is merely being delayed.

Adolescent↗