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Query: UNIPROT:Q86TM3 (cage)
 29,987 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Homozygous achondroplasia is a rare yet distinct clinical entity. Most infants succumb to an early death as a result of respiratory compromise due to upper airway obstruction, thoracic cage deformity, and/or cervicomedullary compression. The successful cervicomedullary decompression of a 16-week-old infant with homozygous achondroplasia is described. This report suggests that homozygous achondroplasia is not universally fatal and that these infants are potentially viable if managed by aggressive respiratory and surgical measures.
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PMID:Foramen magnum decompression in an infant with homozygous achondroplasia. Case report. 274 57

We studied prospectively 26 young patients with achondroplasia to test two hypotheses: that respiratory problems may be the result of occult spinal cord compression, and that achondroplastic patients with cord compression might have occult respiratory abnormalities. Respiratory abnormalities were present in 85%, the majority caused by a primary problem of the pulmonary system, such as small thoracic cage or obstructed airway. Three patients had hypoxemia, recurrent cyanotic spells, and episodes of respiratory distress explainable only by cervicomedullary cord compression; in each patient, respiratory problems were alleviated by decompressive surgery. Another six patients with cervicomedullary compression had, in addition, at least one primary pulmonary cause of respiratory problems. After decompressive surgery the respiratory problems improved in three and were unchanged in three. Reconstructed sagittal CT images proved the most sensitive technique for detecting craniocervical stenosis as a cause of cervicomedullary cord compression, although some degree of stenosis was present in nearly all of the patients.
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PMID:Cervicomedullary compression in young patients with achondroplasia: value of comprehensive neurologic and respiratory evaluation. 355 99

Achondroplasia, the most common type of dwarfism, involves several physical characteristics that can cause pre- and intraoperative respiratory problems. The characteristics include rib-cage deformities, midface hypoplasia, choanal stenosis, muscular hypotonia, foramen magnum compression, and cervical abnormalities. Specific pre- and postoperative strategies for dealing with achondroplastic dwarfs are discussed.
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PMID:Achondroplasia: pre- and postsurgical considerations for midface advancement. 813 Feb 46

Acetylcholine often affects cardiac action potential repolarization only during augmented adrenergic tone, i.e., the phenomenon of accentuated antagonism. Since chronic exercise involves repeated changes in autonomic outflow, we determined whether it also influenced adrenergic/cholinergic interactions in isolated canine cardiac tissue. Using standard micro-electrode techniques in thin ventricular subendocardial slices isolated from exercised (EX: 8-10 wk daily exercise) and sedentary (SED): 8-10 wk cage rest) dogs, we examined transmembrane potential responses to isoproterenol (ISO: 10(-8), 10(-7), 10(-6) M) and to ISO in the presence of ACH (10(-5) M). Control transmembrane characteristics at BCL = 500 ms were similar for EX (N = 8 dogs) and SED (N = 9 dogs). ISO (10(-6) M) decreased action potential duration at 50% repolarization (APD50): EX = -29 +/- 15 ms; SED = 11 ms and at 90% repolarization (APD90): EX = -37 +/- 17 ms; and SED = -24 +/- 14 ms (P > 0.05, EX vs SED). ACH alone did not alter APD. With ACH (10(-5) M), delta APD50 with ISO (10(-6) M) was -5 +/- ms and 0 +/- 5 ms for EX and SED, respectively; delta APD90 was -8 +/- 4 ms and -8 +/- 7 ms for EX and SED, respectively (P > 0.05, EX vs SED). Thus, ACH antagonized ISO-mediated acceleration of repolarization equally in both groups. Chronic daily exercise does not influence adrenergic/cholinergic interactions at the cellular level.
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PMID:Accentuated antagonism in canine subendocardium is not altered by chronic exercise. 926 57

The authors describe a radiographic method to quantify a surgical procedure of thoracic expansion in a 2-year-old patient with achondroplasia, small chest cage, and severe restrictive lung disease. The patient had undergone three surgical procedures of thoracic expansion since birth. The authors utilized computer-generated lung volume histograms after spiral computed tomographic scanning and three-dimensional imaging of the lungs to calculate his lung volumes before and after the third surgical thoracic expansion. The lung volumes, calculated by the histograms, were 363 mL and 406 mL before and after surgery, respectively. This 40-mL difference in the patient's lung volumes (4 mL/kg) accounted for a significant clinical improvement. Lung volume histograms obtained by this radiographic method are very helpful in substantiating a successful surgical chest expansion or provide an explanation for an unsuccessful repair.
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PMID:Lung volume histograms after computed tomography of the chest with three-dimensional imaging as a method to substantiate successful surgical expansion of the rib cage in achondroplasia. 960 82

We designed a closed-system cage with vent ports that would allow continuous airflow in the occupied cage to ensure adequate ventilation and gas exchange. In this system, the metabolic heat loads of mice generate upward thermal air currents. Heat exits via the exhaust port, and room air enters via the intake port, providing adequate ventilation. Simulations based on computational fluid dynamics (CFD) helped us to optimize the cage's design. CFD simulations and smoke visualizations with a feeder-trough assembly illustrated the one-pass air circulation pattern and the lack of air recirculation, turbulence, and dead air space in our system. We used hot-film anemometry and smoke-test methodologies to show that adequate ventilation was provided. In a room with still air (0 air changes per hour [ACH]), a cage fitted with double wire-cloth filters (40 mesh size) and occupied by five mice has at least 12 ACH, whereas the same cage occupied by one mouse has 6 ACH. After five mice had occupied the cage for a week, its average temperature was 0.58C, relative humidity was 34%, and ammonia concentration was 3 ppm higher than that of the room. Our novel vented microisolation cage provides adequate intracage ACH, isolates mice from environmental contaminants, and contains allergenic particles within the cage in an environment appropriate for the species.
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PMID:A novel vented microisolation container for caging animals: microenvironmental comfort in a closed-system filter cage. 1117 11

Achondroplasia is a rare disorder occurring 1 in 1 5,000 to 1 in 40,000 live births. It is, however, the commonest cause of short-limbed dwarfism. It is a genetic disorder and inherited as an autosomal dominant trait but most cases (80%) are due to mutations of fibroblast growth factor receptor 3 (FGFR3). These individuals have normal mental and sexual development, and life span may be normal. Certain gynaecological problems like infertility, menorrhagia, dysmenorrhoea, leiomyomata and early menopause are more common in these patients. Information regarding obstetric behaviour in achondroplastic females is scarce in literature. However, problems such as pre-eclampsia, polyhydramnios, respiratory compromise, contracted pelvis necessitating lower section caesarean section, prematurity and foetal wastage, etc, have been reported. General anaesthesia is preferred to regional anaesthesia because of the spinal abnormalities. There is increased neonatal mortality due to hydrocephalus and thoracic cage abnormality. Such a patient is considered high risk in terms of anaesthesia and obstetric outcome and there is enough room for prenatal counselling and diagnosis. Here a case of achondroplasia with pregnancy is reported. The patient, an achondroplastic dwarf presented with 30 weeks pregnancy. She was prenatally screened with ultrasonography to rule out affection in baby. She had a caesarean section for contracted pelvis.
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PMID:Pregnancy in an achondroplastic dwarf: a case report. 1649 57

The environment in which a laboratory animal is housed can significantly influence its behaviour and welfare, acting as a potential confounding factor for those studies in which it is utilised. This study investigated the impact of two Individually Ventilated Cage (IVC) housing systems on anxiety-related behaviour and welfare indicators in two common strains of laboratory mice. Subjects were juvenile female C57BL/6J and BALB/c mice (N=128) housed in groups of four in two different IVC systems for 7weeks. System One had air delivery at the cage 'cover' level at 75 ACH (Air Changes/Hour) and System Two had air delivery at the 'animal' level at 50 ACH. Mice were assessed twice a week (e.g. bodyweight) or at the end of the study (e.g. anxiety tests). Our results showed significant differences in anxiety-related behaviour between strains and housing systems. Mice in System Two, regardless of strain, defecated more in the Elevated Plus Maze (EPM), spent less time in the open arms of the EPM, and less time in the central zone of the Open Field (OF). Strain differences in anxiety-like behaviour were seen in the increased defecation by BALB/c mice in the OF and EPM and less time spent in the open arms of the EPM compared to C57BL/6J mice. These results suggest that different IVC housing systems can influence mouse behaviour in different ways, with mice of both strains studied exhibiting more anxiety-related behaviour when housed in System Two (air entry at the 'animal' level at 50 ACH), which could impact upon experimental data.
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PMID:The effect of two different Individually Ventilated Cage systems on anxiety-related behaviour and welfare in two strains of laboratory mouse. 2418 92