Hb Calais [β76(E20)Ala→Pro]: A Family Study of a Variant With Decreased Oxygen Affinity

Secondary to the detection of a chronic anemia with a slightly increased Hb F level in a 7‐year-old boy carrying a hemoglobin (Hb) variant, we investigated the members of his family and found that they were related to the original case of Hb Calais. In the present study, we report the clinical and biological impacts of this Hb variant in various members of three generations of this family.     

Proposed graphical system of evaluating disc–condyle displacements of the temporomandibular joint in MRI

The aim of this preliminary study was to standardize the reading of magnetic resonance imaging (MRI) of the temporomandibular joint (TMJ). The MRI was conducted on a control group of eight subjects. In this study, the analysis of joint relationships was limited to the oblique sagittal plane, during the movement of controlled opening. The sections were analyzed by a computer-assisted quantitative method of graphical evaluation. The total surface area (TS) of the disc section was divided into an anterior surface area (AS) and a posterior area (PA), in accordance with a line joining the center of the condyle (C) to the center of the articular tubercle of the temporal bone (T). The disc–condyle relationships in the chosen plane of section were evaluated by the AS/TS ratio. If TS remains relatively constant, the AS/TS values on average decrease from 0.5 to 0.3 between the closed mouth position and the 25 mm open position. The results show good intra-observer reproducibility (p=0.26), evaluated by Wilcoxon’s test. In conclusion, this protocol enables the displacement of the disc–condyle assembly during mouth opening to be described. Observed on a larger sample, the AS/TS would be able to show a range of variability in functional disc positions.     

Effects of the Ebbinghaus figure on grasping are not only due to misjudged size

It is not evident how the small effects of the flankers of the Ebbinghaus figure on peak grip aperture (PGA) should be interpreted. One interpretation is that the flankers influence the estimated size, which in turn influences the grasp. If this interpretation is correct, then only the size-dependent aspects of the grasping movement should depend on the spatial positions of the flankers. An alternative interpretation is that the effect on grip aperture is caused by a change in judgement of the required precision, in which case various aspects of the grasping movement could be influenced by the size and position of the flankers. We presented subjects with a display consisting of a central disk surrounded by four large or small flankers. The array of circular flankers could be rotated by 45°. There were two tasks: to reproduce the perceived size of the central disk, and to grasp the central disk. As in other studies, the reproduced size and the PGA were both influenced by the size of the flankers. The effect on reproduced size settings was independent of the flankers spatial position. Nevertheless, the flankers position did influence the final grip aperture and the grip orientation at PGA and at movement offset. Because the flankers changed more than only the PGA, we conclude that the effect of the flankers on prehension cannot only be because of misjudgement of the size of the central disk.     

Are home ventilators able to guarantee a minimal tidal volume?

Objective  

The aim of the study was to evaluate the ability of home ventilators to maintain a minimal tidal volume during different conditions associated with alveolar hypoventilation.     

Changes in the Number of CD8+ T Lymphocytes in the Peripheral Blood of Patients with Various Autoimmune Diseases after Autologous Hematopoietic Stem Cell Transplantations and their Relations to the Survival Times

The changes in the number of CD8⁺ T lymphocytes were studied before (0 day) and then 30 days after the autologous hematopoietic stem cell transplantations (AHSCT) in 14 therapy refractory patients with autoimmune diseases. The years of survival and the clinical states were also evaluated. The number of CD8⁺ T cells was determined by an hematologic automat and by flow cytometry. Longer than 5-year survival times were found in 6 cases, whereas there was no progression (improvement) in 2 cases, and 4 patients were lost. The increase in the number of CD8⁺ cytotoxic T cells was gradual in the first 2 months and reached the significantly highest values among all subtypes of lymphocytes. It was of a special interest that in all the 4 patients who died, the numbers of CD8⁺ T cells were less than 150/μl on the 30th day after AHSCT, whereas all the 10 patients with a higher cell number survived. These results suggest that the early monitoring of the number (not only the ratio) of regenerating CD8⁺ T cells in the peripheral blood can be a useful and quantitative laboratory measurement after AHSCT, and it has a significant relation also to the survival times of transplanted patients.     

Early intermittent noninvasive ventilation for acute chest syndrome in adults with sickle cell disease: a pilot study

Purpose

Alveolar hypoxia and hypoxic vasoconstriction lead to trapping of sickle cells within the pulmonary vasculature. Improving alveolar ventilation and oxygenation may improve the outcome of acute chest syndrome (ACS).

Methods

Prospective randomized single-center open study from November 1998 to February 2002 to test whether noninvasive ventilation (NIV) was more effective than oxygen alone in improving oxygenation on day 3 in adults with ACS and to evaluate the effects on pain, transfusion requirements, and length of stay.

Results

Seventy-one consecutive ACS episodes in 67 patients were randomly allocated to oxygen (n = 36) or NIV (n = 35) for 3 days in a medical step-down unit. Baseline respiratory rate and pain score were higher in the NIV group. NIV promptly lowered the respiratory rate, raised $ {\text{Pa}}_{{\text{O}_{2}}} $ , and decreased alveolar–arterial oxygen gradient $ (({\text{A}} - {\text{a}})_{{{\text{O}}_{ 2} }} ) $ , which remained unchanged with oxygen alone. $ {\text{Pa}}_{{{\text{CO}}_{ 2} }} $ significantly worsened only in the oxygen group. On day 3, the groups did not differ regarding the proportion of episodes with normal $ {\text{Pa}}_{{{\text{O}}_{ 2} }} $ (35% with NIV and 25% with oxygen; P = 0.5) or $ (({\text{A}} - {\text{a}})_{{{\text{O}}_{ 2} }} ) $ . Patient satisfaction and compliance were lower with NIV. No differences were noted in pain relief, transfusions, or length of stay. In the subgroup of patients with severe hypoxemia $ ( {\text{Pa}}_{{{\text{O}}_{ 2} }} \le 6 5\,{\text{mmHg)}} $ , physiological variables also improved faster with NIV, the differences being slightly more pronounced.

Conclusions

Respiratory rate and gas exchange improved faster with NIV. However, NIV failed to significantly reduce the number of patients remaining hypoxemic at day 3, and was associated with greater patient discomfort.