Effect on fetal mouse development of exposure to MR imaging and gadopentetate dimeglumine

Pregnant mice were exposed to one of five regimens at 9.5 days of gestation: no treatment (group 1), intraperitoneal injection of normal saline (group 2), intraperitoneal injection of gadopentetate dimeglumine (group 3), intraperitoneal injection of gadopentetate dimeglumine and magnetic resonance (MR) exposure (group 4), and MR exposure alone (group 5). At 18 days of gestation, the mice were sacrifice and fetuses were removed and examined for the following end points: litter size, number alive or dead, fetal weight, extremity morphology, eye and ear development, and appearance of the head. A total of 739 fetuses were analyzed: group 1 (n = 161), group 2 (n = 149), group 3 (n = 142), group 4 (n = 136), and group 5 (n = 151). The only statistically significant difference was a lower mean fetal weight in the saline-injection group compared with the control group. The results show that MR exposure with and without gadopentetate dimeglumine had no adverse effect on the end points analyzed.     

Extreme hypogammaglobulinemia disclosing large granular lymphocytes with hepatosplenorenal infiltration

We report a case of large granular lymphocytosis, or chronic "natural killer" lymphocytosis, a newly described entity. We were able to demonstrate the proliferative character of the disease by the finding of karyotype abnormalities. This case was remarkable for the pre-existence, for at least three years, of severe hypogammaglobulinaemia, for the very slow course of the proliferative process and for the progressive and tumoral infiltration of the spleen and liver, then kidney.     

Walking modality affects respiratory muscle action and contribution to respiratory effort

We hypothesized that walking at increased speed or increasing gradient might have different effects on chest wall kinematics and respiratory muscle power components, and contribute differently to respiratory effort sensation. We measured the volumes of chest wall compartments by optoelectronic plethysmography, esophageal, gastric and transdiaphragmatic (P _di) pressures, and the sensation of the respiratory effort by a Borg scale in five normal subjects walking both at ascending gradient with constant speed (AG) and at ascending speed with constant gradient (AS). Chest wall kinematics, evaluated by displacement of chest wall compartments, did not show any significant difference between AS and AG. Muscle power, calculated as the product of mean flow and mean pressure, increased similarly, but its partitioning into pressure and velocity of shortening differed in the two modes. A greater increase in the pressure developed by the abdominal muscles (P _abm) (4.06-fold), and in the velocity of shortening of both rib cage inspiratory muscles (v _rcm,i) (2.01-fold) and the diaphragm (v _di) (1.90-fold) was associated with a lower increase in the pressure developed by the rib cage inspiratory muscles (P _rcm,i) (1.24-fold) and P _di (0.99-fold) with AG. Instead, with AS, a lower increase in P _abm (2.12-fold), v _rcm,i (1.66-fold) and v _di (1.54-fold) was associated with a greater increase in P _rcm,i (1.56-fold) and P _di (1.97-fold). A combination of P _abm and v _di during AG (Wald ²=23.19, P<0.0000), with the addition of P _rcm,i during AS (Wald ²=29.46, P<0.0000), was the best predictor of Borg score. In conclusion, the general strategy adopted by respiratory centers during different walking modes does not differ in terms of ventilation, chest wall kinematics, and respiratory muscle power production, whereas it does in terms of partitioning of power into pressure and velocity of shortening, and respiratory muscle contribution to respiratory effort sensation. Combinations of different patterns of flow and pressure generation made the respiratory effort sensation similar during AS and AG modes.     

Impaired antibody responses in the hyperimmunoglobulin E syndrome

Patients with the hyper-IgE (HIE) syndrome have recurrent bacterial infections with Staphylococcus aureus and other polysaccharide encapsulated organisms. To determine whether an impairment of the antibody response to polysaccharide antigens contributes to infections in this syndrome, we measured serum antibody to the teichoic acid of S. aureus and to the capsular polysaccharide of Haemophilus influenzae type b. Compared to control subjects who had no history of S. aureus infections (N = 14), sera from patients with HIE (N = 9) lacked the expected elevation of serum antibody to teichoic acid (p greater than 0.05) and had significantly lower levels of this antibody than sera from 14 patients with atopic dermatitis, complicated by recurrent cutaneous S. aureus infections (p less than 0.01). After immunization with the capsular polysaccharide of Haemophilus influenzae type of vaccine, the antibody response of patients with HIE was significantly impaired compared to that of age-matched control subjects (p = 0.01). Although patients with HIE syndrome had normal total IgG levels, most patients with HIE but not patients with atopic dermatitis had IgG2 subclass deficiency. Defective antibody responses in patients with HIE were not restricted to polysaccharide antigens because the serum levels of antitetanus toxoid antibody in these patients were significantly lower than that of control subjects (p less than 0.001). Impaired antigen-specific antibody responses in patients with HIE syndrome may contribute to their increased susceptibility to infection.     

Proxy‐reported health‐related quality of life of patients with juvenile idiopathic arthritis: The pediatric rheumatology international trials organization multinational quality of life cohort study

Objective

To investigate the proxy‐reported health‐related quality of life (HRQOL) and its determinants in patients with juvenile idiopathic arthritis (JIA).

Methods

In this multinational, multicenter, cross‐sectional study, HRQOL of patients with JIA was assessed through the Child Health Questionnaire (CHQ) and was compared with that of healthy children of similar age from the same geographic area. Potential determinants of HRQOL included demographic data, physician's and parent's global assessments, measures of joint inflammation, Childhood Health Assessment Questionnaire (CHAQ), and erythrocyte sedimentation rate.

Results

A total of 6,639 participants (3,324 with JIA and 3,315 healthy) were enrolled from 32 countries. The mean ± SD physical and psychosocial summary scores of the CHQ were significantly lower in patients with JIA than in healthy children (physical: 44.5 ± 10.6 versus 54.6 ± 4.0, P < 0.0001; psychosocial: 47.6 ± 8.7 versus 51.9 ± 7.5, P < 0.0001), with the physical well‐being domain being most impaired. Patients with persistent oligoarthritis had better HRQOL compared with other subtypes, whereas HRQOL was similar across patients with systemic arthritis, polyarthritis, and extended oligoarthritis. A CHAQ score >1 and a pain intensity rating >3.4 cm on a 10‐cm visual analog scale were the strongest determinants of poorer HRQOL in the physical and psychosocial domains, respectively.

Conclusion

We found that patients with JIA have a significant impairment of their HRQOL compared with healthy peers, particularly in the physical domain. Physical well‐being was mostly affected by the level of functional impairment, whereas the intensity of pain had the greatest influence on psychosocial health.     

Failure of resistive breathing training to improve pulmonary function tests in patients with chronic obstructive pulmonary disease

The effects on pulmonary function tests and exercise tolerance of resistive breathing training (RBT) were assessed in 16 subjects with stable chronic obstructive pulmonary disease (COPD) who had received no benefit from previous programs of breathing retraining (BR) and medical therapy (MT). 16 male patients with mild degree stable COPD underwent detailed evaluation of pulmonary function tests, blood gas analysis and exercise tolerance test before and after a monthly program of RBT. The patients had received no physiological effects from previous monthly programs of BR and MT. No change in pulmonary function tests, blood gas analysis and exercise tolerance test was observed after RBT. Only maximal static expiratory pressure increased significantly after RBT. We conclude that RBT does not improve pulmonary function tests in subjects who received no physiological benefit from BR.