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Judging from your response to our September feature on cholesterol testing providing dietary advice has become of paramount importance to OHNs. The Flora Project for Heart Disease Prevention offers information on the risk factors of high cholesterol and has become a major noninstitutional authority on coronary heart disease. With Yuletide in sight The Flora Project offers advice on a cholesterol-clear Christmas.
A 40-year-old patient with moderate factor IX deficiency (Christmas disease) underwent quadruple saphenous vein coronary bypass grafts for angina and severe coronary atherosclerosis involving the left and right main, left anterior descending, and circumflex coronary arteries. Excessive bleeding was prevented by infusion of factor IX concentrates during and after the operation. The surgical procedure and total body perfusion were carried out in the same manner as in patients without a hemorrhagic disorder. The patient was discharged after 13 days of hospitalization. He is doing well at the time of this publication and has returned to work.
A double-blind controlled study was carried out to investigate the effectiveness of treatment with factor VIII or factor IX concentrate and a reducing dose of prednisolone in contolling haematuria in patients with haemophilia and Christmas disease. 41 episodes of haematuria were studied in 30 different patients. No appreciable benefit was observed in the treated, as compared with the control group and this is at variance with the results of the few studies reported elsewhere.
Evidence has been sought of activation of the coagulation system in two groups of patients following the infusion of two heparinised clotting factor concentrates. No changes were detected in 13 patients with mild hepatic dysfunction. In six studies on patients with Christmas disease induced abnormalities occurred in only one. Activation of the coagulation mechanism did not occur in another individual who had received the same batch of material.
Bovine blood coagulation factor IX (Christmas factor) contains four asparagine-linked sugar chains in one molecule. The sugar chains were quantitatively liberated as radioactive oligosaccharides from the polypeptide moiety by hydrazinolysis followed by N-acetylation and NaB3H4 reduction. The structures of these sugar chains were determined by sequential exoglycosidase digestion in combination with methylation analysis. Bovine factor IX contained two unique penta- and tetrasialyl triantennary sugar chains with the structures shown below in addition to tetra-, tri-, and disialyl biantennary sugar chains of Sia alpha 2 leads to 3 Gal beta 1 leads 3(Sia alpha 2 leads to 6)GlcNAc beta 1 leads to 2Man alpha 1 leads to 6[Sia alpha 2 leads to 3Gal beta 1 leads to 3(Sia alpha 2 leads to 6)GlcNac beta 1 leads to 2Man alpha 1 leads to 3]Man beta 1 leads to 4GlcNAc beta 1 leads to 4GlcNAc, Sia alpha 2 leads to 6Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 6[Sia alpha 2 leads to 3Gal beta 1 leads to 3(Sia alpha 2 leads to 6)GlcNAc beta 1 leads to 2Man alpha 1 leads to 3]Man beta 1 leads to 4GlcNAc beta 1 leads to 4GlcNAc, and Sia alpha 2 leads to 6Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 6(Sia alpha 2 leads to 6Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 3)Man beta 1 leads to 4GlcNAc beta 1 leads to 4GlcNAc and their partially desialized forms.
The 3M Company (Minnesota Mining and Manufacturing Company) has produced about three billion plastic Christmas bow pins over 20 years. When it was reported that inhalation of these pins had resulted in two deaths, the company sought otolaryngologic consultation to suggest design modifications which would decrease the risk of aspiration asphyxia. The circular base of the bow pin was modified into the form of a cross so that it would not completely obstruct the airway. Barium was added to render the bow pin radiopaque. When the unmodified bow pin was inserted into the larynx of ten cats, six died. The modified pin caused no deaths in nine cats. We regard this as an example of responsible cooperation between industry and medicine.
A high-resolution three-dimensional Fourier transform technique and prototype bilateral dual phased-array surface coil technique was used to make inner ear structures visible on MR. Multiplanar reformatted images, parallel to the plane of the vestibular aqueduct, allowed viewing of the entire endolymphatic sac/vestibular aqueduct on one section, producing a "Christmas tree" shape. The reformation was obtained using a double oblique angle, 45 degrees from true sagittal and 70 degrees from the orbital-meatal axis.
A 4-month-old boy presented with an apparently spontaneous uniocular hyphema as the initial manifestation of Christmas disease (hemophilia B). Although it is uncommon for patients with hemophilia to experience major bleeds in infancy, and the ocular involvement is usually limited to periocular hemorrhages, we recommend that a coagulation profile be obtained in all patients with intraocular hemorrhage to rule out this disease.
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The partial thromboplastin time test provides a convenient and sensitive screening procedure for deficiencies of thromboplastic factors, especially factors VIII and IX. The test is carried out after preincubating the plasma for 10 minutes with kaolin, and Inosithin is used as a platelet substitute. The ;normal range' of the test has been estimated in terms of the differences encountered between random normal plasmas tested in pairs, because individual patients are usually tested against single control subjects. A patient's partial thromboplastin time should be regarded as abnormal if it is more than six seconds longer than the control time. In the diagnosis of haemophilia, patients' plasmas with concentrations of factor VIII as low as about 20% might be regarded as being within the range of normal, if the selected control subject's factor VIII happened to lie near the lower end of the normal range. When mild haemophilia is suspected, discrimination may be improved by diluting both the patient's and the control plasmas 1 in 20 in haemophilic plasma. With the test modified in this way the clotting time is prolonged, though the range of differences among normal subjects is unaltered, and plasmas with factor VIII concentrations below about 30%, i.e., in undiluted plasma, would be unlikely to be regarded as normal. The partial thromboplastin time may be similarly modified as a screening test for factor IX deficiency.Some clinical examples are reported.
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