1/30. Pulmonary storage with emphysema as a sign of Niemann-Pick type C2 disease (second complementation group). Report of a case.A case is described of Niemann-Pick type C2 disease presenting an infantile pneumopathic phenotype known to occur in this recently established, second, minor complementation group of Niemann-Pick type C (NPC) disease. However, the pulmonary involvement was unique, being dominated, in addition to the usual storage macrophage infiltration of the alveolar and septal compartments, by irregular emphysema attributed to storage cell migration into the bronchiolar lumen. The latter modified considerably the X-ray findings and hindered the initial clinical diagnosis. Otherwise, the storage phenotype, including the range of stored lipids, storage distribution, and cell and organ pathology, was found to be identical to that in the whole Niemann-Pick type C disease group dominated by NPC1. The biochemical findings (cholesterol esterification level) corresponded to the classical biochemical phenotype. Emphysema should thus be considered as a variant of the pulmonary NPC2 storage process, governed most probably by an epigenetic mechanism responsible for storage macrophage migration into the bronchiolar compartment.- - - - - - - - - - ranking = 1keywords = emphysema (Clic here for more details about this article) |
2/30. Intra-operative and post-operative hypercapnia leading to delayed respiratory failure associated with transanal endoscopic microsurgery under general anaesthesia.We present an unusual case of hypercapnia and surgical emphysema during transanal endoscopic microsurgery, which led to delayed post-operative ventilatory failure. The hypercapnia and surgical emphysema were secondary to rectal insufflation with carbon dioxide used to facilitate visualization and resection of a rectal tumour. Despite a return to wakefulness after surgery, the patient's level of consciousness deteriorated in the recovery area as a result of hypercapnia. The PaCO2 rose to 16.8 kPa because of absorption of carbon dioxide from the surgical emphysema. On close examination, surgical emphysema was identified in unusual areas, including the anterior abdominal wall, both loins, both groins and the left thigh. Reventilation was required until these unusual carbon dioxide stores had dissipated. We discuss the need for prolonged post-operative vigilance in patients with surgical emphysema secondary to carbon dioxide insufflation, and the risk of delayed ventilatory failure.- - - - - - - - - - ranking = 1keywords = emphysema (Clic here for more details about this article) |
3/30. Corticosteroid therapy for hemolytic anemia and respiratory failure due to mycoplasma pneumoniae pneumonia.This is a report of hemolytic anemia and respiratory failure due to Mycopkisma pneumoniae pneumonia. His chest CT scans showed bilateral diffuse thickened bronchovascular bundles and emphysematous changes. The pulmonary function test supported the diagnosis of chronic obstructive pulmonary disease (COPD). He was diagnosed as coldagglunitin-associated hemolytic anemia and M. pneumonzae pneumonia in inapparent COPD. Corticosteroid administration was remarkabily effective for hemolytic anemia and beneficial for acute exacerbation of COPD.- - - - - - - - - - ranking = 0.2keywords = emphysema (Clic here for more details about this article) |
4/30. mediastinal emphysema and bilateral pneumothoraces with chronic GVHD in patients after allogeneic stem cell transplantation.It is difficult to treat lung complications caused by chronic graft-versus-host disease (GVHD) after allogeneic stem cell transplantation (allo-SCT). We retrospectively analyzed the characteristics of five patients with mediastinal emphysema (ME) and bilateral pneumothoraces (BP) caused by chronic lung GVHD after allo-SCT. Four of these patients had undergone unrelated SCT, and three had had HLA-identical unrelated donors. All patients received total body irradiation (TBI) during conditioning. immunosuppressive agents were administered as GHVD prophylaxis, but two patients developed acute GVHD and all the five developed chronic GVHD. The onset of lung complications was 99-1915 days (median, 202 days) after SCT. The onset of ME and BP was 6-48 days (median, 23 days) after the onset of lung complications. immunosuppressive agents were initially beneficial on the lung complications, but the patients later showed no response to therapy, and all died from respiratory failure 7-195 days (median, 28 days) after the development of ME and BP. The results suggest that these complications progress rapidly, are resistant to treatment, and have a poor prognosis. It is therefore important to start prophylaxis and treatment as early as possible.- - - - - - - - - - ranking = 1keywords = emphysema (Clic here for more details about this article) |
5/30. Surgical emphysema: a rare presentation of foreign body inhalation.An 11-year-old girl with an almond lodging in the tracheobronchial tree is described. She presented with an uncommon symptom of subcutaneous emphysema The x-ray revealed left-sided pneumothorax and pneumomediastinum. Intercostal drain was inserted, but she developed respiratory failure and was ventilated. After initial stabilization for 60 hours, she deteriorated again and her x-ray revealed right-sided collapse. After removal of the foreign body, she was discharged but presented again with stridor necessitating tracheostomy. tracheal stenosis was found and required end-to-end anastomosis. The authors feel that, while foreign bodies are uncommon in this age group with emphysema as a rarer manifestation, this cause should be kept in mind, even in the absence of forthcoming history. A high index of suspicion for tracheobronchial foreign body is required in atypical presentations of acute pediatric respiratory distress.- - - - - - - - - - ranking = 1.2keywords = emphysema (Clic here for more details about this article) |
6/30. A case of congenital cutis laxa (generalized elastolysis).cutis laxa (generalized elastolysis) is characterized by sagging folds of loose skin on the face and trunk. Although grouped with hyperelastic skin conditions such as ehlers-danlos syndrome, cutis laxa shows an almost total lack of skin elasticity. Because it is a generalized state, it may present as an abnormality in the connective tissue of more than one organ system. The authors report a patient who had end-stage renal failure related to heavy chain disease, a recognized association of cutis laxa. Although the patient's vascular abnormalities were significant, she died of respiratory failure at 41 years of age from cutis laxa-induced emphysema.- - - - - - - - - - ranking = 0.2keywords = emphysema (Clic here for more details about this article) |
7/30. Pneumatic chest wall compression. A cause of respiratory failure from massive subcutaneous emphysema.subcutaneous emphysema rarely causes significant adverse clinical consequences. Two patients had development of massive subcutaneous emphysema during positive-pressure ventilation that resulted in chest wall compression and respiratory failure. drainage of the subcutaneous air produced dramatic improvement. subcutaneous emphysema is potentially fatal in ventilated patients. Specific decompression of subcutaneous tissues is indicated in such extreme cases.- - - - - - - - - - ranking = 1.4keywords = emphysema (Clic here for more details about this article) |
8/30. Kyphoscoliosis and respiratory failure. A patient treated with assisted ventilation for 27 years.A 62-year-old woman had had kyphoscoliosis since age 12 years. Respiratory failure developed at age 35 years following a respiratory infection. A tracheostomy was done and she required assisted ventilation using a respiratory (Bird). Many attempts at weaning her from the ventilator were unsuccessful. She has remained dependent on assisted ventilation for more than 27 years but has had a relatively comfortable and meaningful life. In 1983, a left pleuroscopy was done related to recurrent pneumothorax and numerous small bullae were seen on the lung surface. Recent investigation using computed tomographic scanning has shown patchy areas of emphysema in both lungs. Emphysema is not a feature of kyphoscoliosis and it is suggested that intermittent positive pressure applied to the lung over long duration may cause the lung destruction of emphysema.- - - - - - - - - - ranking = 0.4keywords = emphysema (Clic here for more details about this article) |
9/30. Infantile lobar emphysema in association with congenital heart disease.Three cases of congenital heart disease (CHD) are presented; all infants developed the unusual complication of lobar emphysema (LE). In all cases delay in reaching the correct diagnosis resulted in two of the infants undergoing a second operation to resect the emphysematous lobe. The chest radiograph in all cases was dominated by the heart disease and the radiological signs of LE were initially missed. The radionuclide ventilation/perfusion lung scan was used in two of these infants to provide conclusive proof and to emphasise the clinical significance of the condition. Lobar emphysema in association with CHD should be recognised early and lobectomy performed at the same time as surgical correction of the heart defect.- - - - - - - - - - ranking = 1.4keywords = emphysema (Clic here for more details about this article) |
10/30. Severe bronchospasm followed by respiratory arrest during thallium-dipyridamole imaging.We describe the occurrence of sudden severe bronchospasm and respiratory arrest following dipyridamole infusion in a patient with chronic obstructive pulmonary disease predominantly of the emphysematous type. The severe reaction was unexpected because the patient had tolerated well withdrawal of aminophylline derivatives for 48 hours and was receiving chronic prednisone 20 mg qd. Although the diagnostic and prognostic gains from dipyridamole imaging far outweigh the small risk associated with the test, patients with chronic pulmonary obstructive disease must be closely monitored during thallium-dipyridamole imaging.- - - - - - - - - - ranking = 0.2keywords = emphysema (Clic here for more details about this article) |
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