Case report: A patient with primary biliary cirrhosis and autoimmune haemolytic anaemia

Diseases of an autoimmune nature are well recognized in association with primary biliary cirrhosis. Although autoimmune thyroiditis and many rheumatological conditions are well described in primary biliary cirrhosis, autoimmune haematological diseases have been less well reported. We report on a 66 year old North American Indian man with coincident primary biliary cirrhosis and warm antibody haemolytic anaemia. This case report supports the suggestion of an association between autoimmune haemolytic anaemia and primary biliary cirrhosis.     

Working memory and preparation elicit different patterns of slow wave event-related brain potentials

Some event-related brain potential (ERP) studies of working memory have used delayed match-to-sample designs in which a stimulus (S1) is held in memory for comparison with a subsequent stimulus (S2). During the S1-S2 interval, ERP slow negativities varied with both the type and amount of material held in working memory. One interpretation is that these slow waves index working memory operations. An alternative explanation is that they only reflect general preparatory processing for the response to S2. To decide between these explanations, we used two visual processing tasks that required similar preparation for S2. In one task, visual memory rehearsal operations were required. During the S1-S2 interval, there were clear differences between the amplitudes, topographies, and the effect of information load on the slow waves in the two tasks, thus ruling out preparation only as an explanation.     

NORMALIZED STRESS–STRAIN RELATIONSHIP IN HUMAN HAIR PERTURBATION BY HYPOTHYROIDISM

SUMMARY.— A short review is given of the observations which have been made on the mechanical properties of human hair under axial tension.
Stress–strain curves of dry single fibres of human hair from patients with hypothyroidism and acromegaly revealed a distinctive alteration in the high yield region. The biophysical basis for this anomalous behaviour is not known.
Attention is directed to the potential value of further studies of the physical characteristics of hair with special reference to mass screening programmes for evidence of disease. This aim is furthered by the technique introduced in this paper for distinguishing the abnormal from the normal.     

CONTINUOUS NEGATIVE CHEST-WALL PRESSURE THERAPY FOR ASSISTING VENTILATION IN OLDER CHILDREN WITH PROGRESSIVE RESPIRATORY INSUFFICIENCY1

Abstract. Continuous negative chest-wall pressure (CNP) was used to assist ventilation in 14 children, 6 months to 14 years of age, who had progressive respiratory insufficiency caused by diffuse bilateral alveolar disease. Before the start of CNP therapy, each child had a respiratory rate>50/min, arterial oxygen tension (PaO₂)<70 mmHg (FIO₂≥50%), and arterial carbon dioxide tension (PaCO₂)<45 mmHg. The mean intrapulmonary right-to-left shunt was 28.7±3.8%. Within 6 hours after therapy was started, PaO₂ increased from 55.4±15.9 to 81.6±17.7 mmHg (p<0.005). This improvement was sustained and within 24 hours permitted a decrease in fractional concentration of inspired oxygen (FIO₂) from 51.8±6.2 to 41.0±8.4% (p<0.001) and in respiratory rate from 78.1±23.0 to 56.4±21.3 (p<0.01). There was a concomitant decrease in intrapulmonary right-to-left shunt. Four of the 14 patients developed pneumothorax that was successfully decompressed. Ten patients survived. These observations establish CNP therapy as an effective means of improving arterial oxygenation in spontaneously breathing older children. Of added significance, this mode of therapy eliminates the need for endotracheal intubation and prolonged use of muscle relaxants and sedatives. It also minimizes exposure to high FIO₂, thereby minimizing the hazards of pulmonary oxygen toxicity.     

The fitness of human translocation carriers

A simple method to estimate the mean fitness of human translocation carriers is described. The method is based on information about the inheritance pattern of the translocations. This kind of data can be obtained from the surveys of the chromosome constitutions of new-born babies. The following fitness estimates are obtained: D/D translocation carriers, 0-94; D/G translocation carriers, 0-83; and reciprocal translocation carriers, 0-67. The estimates are rather uncertain due to the scarcity of information, but they are consistent with the expected results. More accurate estimates will be obtained in the future when more data on translocation carriers are available and the cytological classification of the different translocations becomes more exact.     

Hemoglobin Zurich. I. A New Hemoglobin Anomaly Associated with Acute Hemolytic Episodes with Inclusion Bodies after Sulfonamide Therapy

A new abnormal hemoglobin was observed in 15 members over four generations of a large Swiss family and has been termed "Hemoglobin Zürich."The discovery of this hemoglobin was prompted by a severe hemolytic crisisin two members of the family after sulfonamide therapy. During this episode,virtually all erythrocytes and reticulocytes contained a single large inclusionbody which was visible with Giemsa and brilliant cresyl blue stains. Outsidethe hemolytic episode, the erythrocytes revealed no morphologic abnormalities.The results of enzyme studies were all within normal limits. The associationof a hemoglobinopathy with a drug-induced inclusion body anemia withoutany demonstrable enzyme defect is a new entity. The anomalous hemoglobinis inherited as a dominant character and affects both sexes. Thus far, onlythe heterozygous form has been observed.

Submitted on April 9, 1962 Accepted on June 20, 1962     

TIME-DEPENDENCY OF NEUROHYPOPHYSEAL PEPTIDE ATTENUATION OF PUROMYCIN AMNESIA IN MICE

[Ile³, Arg⁸]. vasopressin (arginine-vasotocin), as well as the C-terminal tripeptides of the neurohypophyseal hormones arginine and lysine vasopressin, Pro-Arg-Gly-NH₂ and Pro-Lys-Gly-NH₂, were protective against puromycin-induced amnesia in mice when administered 24h before training. The N-protected tripeptide derivative, Z-Pro-Lys-Gly-NH₂, was effective when given 5 days before training. The effectiveness of all peptides to attenuate puromycin-induced amnesia decreased as the interval between training and peptide treatment increased, indicating that the peptides influence memory processes, rather than general arousal. Z-Pro-Lys-Gly-NH₂ was active at 24h after training, when the other peptides were no longer effective. Although it seems clear that neurohypophyseal hormones per se can attenuate puromycin-induced amnesia, these results are in line with the possibility that some portion of hormone action may be mediated via formation of longer-lived hormone fragments in the CNS.