- Episodic Breathlessness in Patients with Advanced Cancer: Characteristics and Management. [Review]
- DDrugs 2018 Feb 22
- The aim of this review is to present the way in which episodic breathlessness (EB) has been recognized over the years, with regard to definition, characteristics, and management of these acute episod...
The aim of this review is to present the way in which episodic breathlessness (EB) has been recognized over the years, with regard to definition, characteristics, and management of these acute episodes that have serious consequences for patients. EB is characterized by a sudden increase in intensity of dyspnea over a short duration of time, leading to high levels of anxiety. A significant aggravation of dyspnea may occur in patients with a background of dyspnea or intermittently even without basal breathlessness. Often, known precipitating factors may trigger EB. Flares of breathlessness are accompanied by degrees of psychological distress, although it is unclear whether psychological factors may precede or be induced by EB. In any case, there is a reinforcing circuit. The duration of EB ranges from 10-30 min. Given the specific temporal pattern, requiring rapid intervention, substances with a short onset of action are suitable to overlap this phenomenon. Short-onset opioids could provide a clinical effect overlapping the onset and duration of an episode, resembling what has been largely reported for breakthrough pain. Although data are still insufficient to suggest specific recommendations, strategies such as avoiding exertion, pacing or using devices, or keeping calm have been described. Few controlled studies have investigated the effects of different formulations of opioids. Some data were gathered from studies assessing the pre-emptive use of rapid onset opioids, such as transmucosal preparations of fentanyl, followed by a provocative test, while other studies attempted to reproduce real-life conditions, given as needed. All these trials were insufficiently powered to address the efficacy of fentanyl products over oral morphine or placebo, reflecting the difficulties in patient recruiting and finalizing the studies. Strategies to prevent the occurrence of EB should be taken into consideration, including optimization of the condition of persistent dyspnea or treating psychologic or environmental causes.
- Use of endoscopic-guided electrocautery ablation for treatment of tracheal liposarcoma in a dog. [Journal Article]
- JAJ Am Vet Med Assoc 2018 Mar 01; 252(5):581-585
- CASE DESCRIPTION A 7-year-old 44-kg (97-lb) neutered male Great Pyrenees was referred for evaluation because of episodic dyspnea with cyanosis of 1 to 2 weeks' duration. Three days prior to evaluatio...
CASE DESCRIPTION A 7-year-old 44-kg (97-lb) neutered male Great Pyrenees was referred for evaluation because of episodic dyspnea with cyanosis of 1 to 2 weeks' duration. Three days prior to evaluation, the clinical signs had worsened, including 1 episode of collapse. CLINICAL FINDINGS Thoracic radiography and CT revealed a well-delineated soft tissue mass, located approximately 1.5 cm cranial to the carina and occupying almost 90% of the tracheal lumen. A CBC and serum biochemical analysis were performed, and all results were within reference limits. TREATMENT AND OUTCOME Tracheoscopy confirmed the presence of a broad-based bilobate mass that was protruding from the right dorsal aspect of the trachea and occupied almost the entire tracheal lumen. The mass was successfully resected by endoscopic-guided electrocautery ablation. Findings of histologic evaluation were consistent with a diagnosis of liposarcoma. Immediately following the ablation procedure, the previously noted clinical signs of respiratory tract disease resolved. On follow-up examination 12 months later, no regrowth of the mass was evident on thoracic helical CT and tracheoscopy. CLINICAL RELEVANCE Endoscopic-guided electrocautery ablation of tracheal liposarcoma was a safe and effective minimally invasive treatment for the dog of this report. The procedure was brief and appeared to be well tolerated, resulting in immediate improvement of clinical signs.
- [Severe lactic acidosis requiring continuos haemodiafiltration in a young patient with unrecognized metabolic abnormality. Case report]. [Case Reports]
- GIG Ital Nefrol 2018; 35(1)
- CONCLUSIONS: The presence of not-otherwise-explained lactic acidosis in young patients has to place the suspect of an underlying and unknown metabolic derangement; in these cases, the involvement of the nephrologist appears to be pivotal for the differential diagnosis of the abnormalities of the acid-base balance, and for setting the best treatment.
- Sudden cardiac death preceded by ST segment elevations during ECG patch monitoring. [Journal Article]
- JEJ Electrocardiol 2017 Oct 18
- A 58-year-old male underwent ambulatory ECG monitoring with continuous patch monitoring (Zio XT Patch) for the complaint of episodic dyspnea. In the period of monitoring the patient suffered sudden c...
A 58-year-old male underwent ambulatory ECG monitoring with continuous patch monitoring (Zio XT Patch) for the complaint of episodic dyspnea. In the period of monitoring the patient suffered sudden cardiac death (SCD) with continuous ECG recording showing pronounced ST segment elevations followed by bradycardia and death. This report highlights the growing potential of continuous ST segment monitoring, and features the infrequent entity of ischemic electromechanical dissociation with bradyarrhythmia as a cause of SCD.
- A case of relapsing polychondritis mimicking lung cancer. [Journal Article]
- TTTuberk Toraks 2017; 65(3):245-248
- Relapsing polychondritis (RP) is a rare autoimmune disease, characterized by episodic inflammatory attacks on cartilaginous tissue. Elastic cartilage of the ears and nose, hyaline cartilage of the pe...
Relapsing polychondritis (RP) is a rare autoimmune disease, characterized by episodic inflammatory attacks on cartilaginous tissue. Elastic cartilage of the ears and nose, hyaline cartilage of the peripheral joints, vertebral fibrocartilage, tracheobronchial cartilage, and tissues rich in proteoglycan such as those in the eyes, heart, blood vessels, and inner ear are more likely to be affected. A 35-year-old male presented with complaints of hoarseness, tinnitus and dyspnea for 19 years, with a history of several diagnostic and therapeutic interventions for laryngeal and respiratory tract. He was diagnosed to have inflammation of the tracheobronchial cartilage, cardiac valvulopathy and conductive hearing loss after intensive diagnostic studies. He responded well to low-dose steroids in combination with methotrexate. RP is a diagnostically challenging condition and may cause significant morbidities during diagnosis. RP should be considered in differential diagnosis of airway obstruction as a rare condition.
- Effect of Prophylactic Fentanyl Buccal Tablet on Episodic Exertional Dyspnea: A Pilot Double-Blind Randomized Controlled Trial. [Journal Article]
- JPJ Pain Symptom Manage 2017; 54(6):798-805
- CONCLUSIONS: This study supports that prophylactic FBT was associated with a reduction of exertional dyspnea and was well tolerated. Our findings support the need for larger trials to confirm the therapeutic potential of rapid-onset opioids.
- Diagnosis and Management of Asthma in Adults: A Review. [Review]
- JAMAJAMA 2017 Jul 18; 318(3):279-290
- CONCLUSIONS: Asthma is characterized by variable airway obstruction, airway hyperresponsiveness, and airway inflammation. Management of persistent asthma requires avoidance of aggravating environmental factors, use of short-acting β2-agonists for rapid relief of symptoms, and daily use of inhaled corticosteroids. Other controller medications, such as long-acting bronchodilators and biologics, may be required in moderate and severe asthma. Patients with severe asthma generally benefit from consultation with an asthma specialist for consideration of additional treatment, including injectable biologic agents.
- Haemoglobinuria And Portal Venous Thrombosis In A Young Male. [Case Reports]
- JAJ Ayub Med Coll Abbottabad 2017 Apr-Jun; 29(2):353-354
- Paroxysmal nocturnal haemoglobinuria is a non-malignant stem cell disorder due to acquired somatic mutations in cell surface anchored proteins CD55 and CD59. Both have a compliment inhibitory role an...
Paroxysmal nocturnal haemoglobinuria is a non-malignant stem cell disorder due to acquired somatic mutations in cell surface anchored proteins CD55 and CD59. Both have a compliment inhibitory role and their deficiency leads to intravascular haemolysis. This paper reports a challenging case of a 25 years old male who presented with generalized weakness, exertional dyspnoea and episodic early morning haematuria. Recently, he started developing progressive abdominal distention and dull generalized abdominal pain. Investigations revealed haemoglobin 3.5 g/dl with 10% reticulocytes, total bilirubin 54.5 mg/dl, LDH 3155 U/L, negative Coomb's test and erythroid hyperplasia on bone marrow biopsy. Urine complete exam was significant for haemoglobinuria without red blood cells. Doppler scan of abdomen showed portal vein thrombosis. Loss of expression of CD14, CD16, CD55 and CD59 on leukocytes and erythrocytes was seen on PNH analysis, confirming paroxysmal nocturnal haemoglobinuria. He was managed with blood transfusions and was advised folic acid and bone marrow transplant.
- Peripheral Neuropathy, Episodic Rhabdomyolysis, and Hypoparathyroidism in a Patient with Mitochondrial Trifunctional Protein Deficiency. [Journal Article]
- JRJIMD Rep 2017 Jul 07
- A combination of unexplained peripheral neuropathy, hypoparathyroidism, and the inability to cope with metabolic stress could point to a rare inborn error of metabolism, such as mitochondrial trifunc...
A combination of unexplained peripheral neuropathy, hypoparathyroidism, and the inability to cope with metabolic stress could point to a rare inborn error of metabolism, such as mitochondrial trifunctional protein (MTP) deficiency.Here, we describe a 20-year-old woman who was known since childhood with axonal motor sensory polyneuropathy of unknown origin. She presented with progressive dyspnoea, and increased muscle weakness, preceded by 6 days of fever, vomiting, and diarrhoea. Laboratory testing showed rhabdomyolysis, and hypocalcaemia with low parathyroid levels. The patient was intubated because of respiratory insufficiency and a viral and bacterial pneumonia was diagnosed. She was discharged after 16 days of admission. Metabolic screening, performed at the time of rhabdomyolysis, showed increased concentrations of long-chain 3-hydroxyacyl carnitine species, together with elevated urinary excretion of 3-hydroxy dicarboxylic acids. Decreased activity of long-chain 3-hydroxyacyl-CoA dehydrogenase and long-chain 3-ketoacyl-CoA thiolase in peripheral lymphocytes and fibroblasts confirmed a MTP deficiency. Sequence analysis of the HADHB gene showed two heterozygous variants: c.209+1G>C (splicing defect) and c.980T>C (p.Leu327Leu). When the acylcarnitine profile was repeated after the episode of rhabdomyolysis had resolved it showed no abnormalities.Our case illustrates a cluster of peripheral neuropathy, episodic rhabdomyolysis, and hypoparathyroidism in a patient with MTP deficiency caused by mutations in the HADHB gene. It stresses the importance of performing metabolic screening when patients are most symptomatic, as normal results can be found at times when no metabolic stress is present. Screening is relatively easy and timely diagnosis has important implications for treatment.
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- Middle lobe syndrome: an intriguing presentation of tracheobronchial amyloidosis. [Journal Article]
- BCBMJ Case Rep 2017 May 22; 2017
- Pulmonary involvement in amyloidosis is a distinct rarity. This clinical entity usually presents as tracheobronchial amyloidosis (TBA). A 32-year-old, never-smoker man presented with episodic dyspnoe...
Pulmonary involvement in amyloidosis is a distinct rarity. This clinical entity usually presents as tracheobronchial amyloidosis (TBA). A 32-year-old, never-smoker man presented with episodic dyspnoea and wheezing along with cough and mucoid sputum. The chest radiograph was suggestive of a middle lobe syndrome (MLS). High-resolution CT (HRCT) of the chest confirmed the presence of MLS. In addition, HRCT showed circumferential thickening of the trachea and the main bronchi, with thickening of the posterior membranous wall of trachea. Fibrebronchoscopy, done to evaluate MLS, visualised multiple small polypoidal lesions in the lower part of trachea and carina. Endobronchial biopsies showed homogeneous, acellular amorphous deposit in the subepithelial region, which was congophilic in nature. A diagnosis of TBA presenting as MLS was made. To the best of our knowledge, this is the first detailed report of MLS as a presentation of TBA in the English literature.