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Methodist DeBakey Cardiovascular Journal 2024Apical hypertrophic cardiomyopathy (HCM) is a rare variant of HCM. A 43-year-old female with a past medical history significant for hypertension and kidney...
Apical hypertrophic cardiomyopathy (HCM) is a rare variant of HCM. A 43-year-old female with a past medical history significant for hypertension and kidney transplantation presented with recurrent syncopal episodes and dyspnea on exertion. Electrocardiogram showed characteristic diffuse giant T-waves inversion, and cardiac magnetic resonance showed HCM with circumferential apical thickening. This case highlights the rapid development of apical HCM and its challenging diagnostic characteristics.
Topics: Humans; Female; Cardiomyopathy, Hypertrophic; Adult; Electrocardiography; Disease Progression; Magnetic Resonance Imaging; Predictive Value of Tests; Magnetic Resonance Imaging, Cine; Apical Hypertrophic Cardiomyopathy
PubMed: 38911827
DOI: 10.14797/mdcvj.1386 -
SAGE Open Medical Case Reports 2024Pancreatitis can produce several complications such as pseudocyst, which can happen in acute and chronic pancreatitides. Pseudocysts are typically found in the abdomen...
Pancreatitis can produce several complications such as pseudocyst, which can happen in acute and chronic pancreatitides. Pseudocysts are typically found in the abdomen but can rarely extend into the mediastinum. Atypical symptoms such as dyspnea, dysphagia, coughing, vomiting, abdominal or chest pain, and hemoptysis are usually the notable complaints. CT scan, MRI, and endoscopic ultrasound are valuable diagnostic modalities. Drainage and surgical removal of the pseudocyst are the treatment options. Herein, we outline the case of a young female with episodic chest and epigastric discomfort, dysphagia, and weight loss. Previously, she was incorrectly diagnosed with gastroesophageal reflux disease and peptic ulcer. A mediastinal pseudocyst secondary to chronic pancreatitis was found to be the cause. The patient underwent surgical removal of the pseudocyst and a pancreaticojejunostomy. Significant improvement was noticed at follow-up. This article highlights the possibility of such unusual conditions and the importance of a proper assessment while treating patients with epigastric pain.
PubMed: 38911178
DOI: 10.1177/2050313X241262139 -
Integrative Cancer Therapies 2024To observe the clinical efficacy of Chinese herbal medicine combined with Liuzijue exercise on the physiological symptoms and quality of life (QoL) in postoperative...
Chinese Herbal Medicine Combined With Liuzijue Exercise in Physiological Rehabilitation After Video-assisted Lung Lobectomy for Cancer: A Prospective Propensity Score Matching Study.
OBJECTIVE
To observe the clinical efficacy of Chinese herbal medicine combined with Liuzijue exercise on the physiological symptoms and quality of life (QoL) in postoperative patients with early-stage lung cancer.
METHODS
One hundred and eighty-three lung cancer patients who underwent video-assisted thoracoscopic surgery (VATS) were categorize into either a traditional Chinese medicine treatment group (CM) or a control group (non-traditional Chinese medicine treatment, NC), among whom 73 underwent Chinese herbal medicine and Liuzijue therapy, while 110 underwent no comprehensive treatment with traditional Chinese medicine. The propensity score matching (PSM) method with a 1:2 ratio was used to balance the baseline characteristics and evaluate the efficacy of CM in improving postoperative symptoms and QoL.
RESULTS
Cough, dyspnea, chest pain, and fatigue were the most common clinical symptoms after VATS. Except for chest pain, they were all correlated with the scope of operation ( < .05). After PSM, 165 patients were identified in the matched cohort, and the covariates of gender, age, operative site, and scope of operation were balanced between the 2 groups ( > .05). In the domain of global health status, the improvement in QoL in CM was greater than that in NC (6.06 ± 15.83 vs -1.06 ± 14.68, = .005). In terms of symptoms, improvements in cough (1.69 ± 3.15 vs 0.38 ± 2.63, = .006), dyspnea during climbing stairs (-10.30 ± 16.82 vs -1.82 ± 17.97, = .004), and pain (-0.76 ± 1.32 vs -0.08 ± 1.31, = .002) in CM were better than in NC.
CONCLUSION
Comprehensive treatment with traditional Chinese medicine (TCM) can provide therapeutic benefits in physiological rehabilitation after VATS for cancer.
Topics: Humans; Male; Female; Drugs, Chinese Herbal; Lung Neoplasms; Propensity Score; Middle Aged; Thoracic Surgery, Video-Assisted; Quality of Life; Prospective Studies; Aged; Exercise Therapy; Medicine, Chinese Traditional; Treatment Outcome; Combined Modality Therapy
PubMed: 38907709
DOI: 10.1177/15347354241261977 -
Journal of Cardiothoracic Surgery Jun 2024Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (PMAIF) is a rare complication of infective endocarditis or aortic valve surgery. Surgical treatment is... (Review)
Review
BACKGROUND
Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (PMAIF) is a rare complication of infective endocarditis or aortic valve surgery. Surgical treatment is suggested, but the long-term follow-up of conservative management remains unclear.
CASE PRESENTATION
A 33-year follow-up of a patient who developed PMAIF six years after aortic valve replacement is reported. The patient presented to our center with dyspnea, and the echocardiography revealed an ejection fraction of 20% and a PMAIF measuring 7 × 10 mm. Despite being advised to undergo surgery, the patient declined due to fear of surgical outcomes. Consequently, conservative treatment with close observation but without surgery was initiated. During the 33-year follow-up period, the patient did not experience any adverse health effects.
CONCLUSION
Surgical intervention should be considered whenever the PMAIF is diagnosed. However, in any case that the surgery was not applicable, conservative management might lead to long-term survival, based on this and similar case reports in the literature.
Topics: Humans; Aneurysm, False; Mitral Valve; Aortic Valve; Male; Follow-Up Studies; Heart Aneurysm; Echocardiography; Heart Valve Prosthesis Implantation; Middle Aged
PubMed: 38907323
DOI: 10.1186/s13019-024-02885-7 -
Journal of Cardiovascular Imaging Jun 2024
PubMed: 38907321
DOI: 10.1186/s44348-024-00007-4 -
Journal of Cardiothoracic Surgery Jun 2024Pulmonary arteriovenous malformation (PAVM), also known as pulmonary arteriovenous fistula, is a rare vascular developmental anomaly. Most cases of PAVM are associated... (Review)
Review
BACKGROUND
Pulmonary arteriovenous malformation (PAVM), also known as pulmonary arteriovenous fistula, is a rare vascular developmental anomaly. Most cases of PAVM are associated with hereditary hemorrhagic telangiectasia (HHT). Hemothorax associated with PAVM is even rarer, and management concerning this complication still challenges.
CASE PRESENTATION
A 55-year-old man with sudden onset of dyspnea and chest pain was admitted to our hospital. He had a medical history of epistaxis, intraperitoneal germ cell tumor and PAVM. Chest unenhanced CT revealed the left-sided pleural effusion together with partial passive atelectasis and gradual increase at the interval of six days. Diagnostic thoracocentesis further revealed hemorrhagic effusion. CT angiography (CTA) showed tortuously dilated lumen of the left lower pulmonary artery and PAVM with the formation of aneurysm. Due to his family's refusal of surgery, the patient underwent transcatheter embolization therapy. However, the left pleural effusion did not significantly reduce and there was a slow drop in hemoglobin value even after interventional treatment, indicating the possibility of ongoing active bleeding. Eventually, the patient received lobectomy of the left lower lobe with a satisfactory outcome.
CONCLUSIONS
Massive hemothorax resulting from PAVM rupture into the pleural space can lead to fatal outcomes. CTA can accurately diagnose this pathologic condition. Transcatheter embolization is frequently used in the treatment of PAVM, but it may be challenging to achieve the desirable effect in patients with hemothorax. Combined with our case and literature review, direct radical surgery can lead to a successful outcome when PAVM complicated with hemothorax and a large diameter of the draining vein.
Topics: Humans; Hemothorax; Male; Middle Aged; Pulmonary Artery; Pulmonary Veins; Arteriovenous Fistula; Arteriovenous Malformations; Computed Tomography Angiography; Embolization, Therapeutic; Rupture, Spontaneous; Tomography, X-Ray Computed
PubMed: 38907280
DOI: 10.1186/s13019-024-02867-9 -
BMC Neurology Jun 2024Congenital myasthenic syndromes (CMS) are among the most challenging differential diagnoses in the neuromuscular domain, consisting of diverse genotypes and phenotypes.... (Review)
Review
BACKGROUND
Congenital myasthenic syndromes (CMS) are among the most challenging differential diagnoses in the neuromuscular domain, consisting of diverse genotypes and phenotypes. A mutation in the Docking Protein 7 (Dok-7) is a common cause of CMS. DOK7 CMS requires different treatment than other CMS types. Regarding DOK7's special considerations and challenges ahead of neurologists, we describe seven DOK7 patients and evaluate their response to treatment.
METHODS
The authors visited these patients in the neuromuscular clinics of Tehran and Kerman Universities of Medical Sciences Hospitals. They diagnosed these patients based on clinical findings and neurophysiological studies, which Whole Exome Sequencing confirmed. For each patient, we tried unique medications and recorded the clinical response.
RESULTS
The symptoms started from birth to as late as the age of 33, with the mean age of onset being 12.5. Common symptoms were: Limb-girdle weakness in 6, fluctuating symptoms in 5, ptosis in 4, bifacial weakness in 3, reduced extraocular movement in 3, bulbar symptoms in 2 and dyspnea in 2 3-Hz RNS was decremental in 5 out of 6 patients. Salbutamol was the most effective. c.1124_1127dupTGCC is the most common variant; three patients had this variant.
CONCLUSION
We strongly recommend that neurologists consider CMS in patients with these symptoms and a similar familial history. We recommend prescribing salbutamol as the first-choice treatment option for DOK7 patients.
Topics: Humans; Myasthenic Syndromes, Congenital; Male; Female; Muscle Proteins; Adult; Young Adult; Adolescent; Child; Mutation
PubMed: 38907197
DOI: 10.1186/s12883-024-03713-0 -
The Journal of Allergy and Clinical... Jun 2024Breathlessness is a disabling symptom, with complexity that is often under recognised and under treated in asthma.
BACKGROUND
Breathlessness is a disabling symptom, with complexity that is often under recognised and under treated in asthma.
OBJECTIVE
To highlight the burden of breathlessness in people with severe compared with mild-to-moderate asthma and identify psychophysiological correlates of breathlessness.
METHODS
This was a cross-sectional study of people with mild-to-severe asthma, who attended two in-person visits to complete a multidimensional assessment. The proportion of people with mild-to-moderate versus severe asthma who reported physically limiting breathlessness (modified Medical Research Council [mMRC] dyspnoea score ≥2) was compared. Psychophysiological factors associated with breathlessness in people with asthma were identified via a directed acyclic graph and explored with multivariate logistic regression to predict breathlessness.
RESULTS
144 participants were included, of which, 74 (51%) had mild-to-moderate asthma and 70 (49%) severe asthma. Participants were predominantly female (n=103, 72%) with a median (quartile 1, quartile 3) age of 63.4 (50.5,69.5) years and body mass index (BMI) of 31.3 (26.2, 36.0) kg/m. The proportion of people reporting mMRC ≥2 was significantly higher in those with severe- (n=37, 53%) compared with mild-to-moderate (n=21, 31%) asthma (p=0.013). Dyspnoea-12 Total (8.00 [4.75, 17.00] versus 5.00 [2.00, 11.00], p=0.037) score was also significantly higher in the severe asthma group. Significant predictors of physically limiting breathlessness were: BMI, asthma control, exercise capacity, and hyperventilation symptoms. Airflow limitation and type-2 inflammation were poor breathlessness predictors.
CONCLUSION
Over half of people with severe asthma experience physically limiting breathlessness despite treatment. Targeting psychophysiological factors, or traits, associated with breathlessness may help relieve this distressing symptom, which is of high priority to people with asthma.
PubMed: 38906398
DOI: 10.1016/j.jaip.2024.06.019 -
European Clinical Respiratory Journal 2024Pulmonary lymphangitis carcinomatosa is a rare and severe manifestation of metastatic disease that causes pulmonary symptoms and radiologic patterns similar to...
INTRODUCTION
Pulmonary lymphangitis carcinomatosa is a rare and severe manifestation of metastatic disease that causes pulmonary symptoms and radiologic patterns similar to interstitial lung diseases.
CASE PRESENTATION
We report a case of a 78-year-old woman who presented to our department with insidiously developed symptoms of fatigue, dry cough, and severe dyspnea for 3 months. Chest radiography showed bilateral interstitial changes. On suspicion of interstitial lung disease, bronchoscopy and transbronchial cryobiopsy were carried out. Surprisingly, histopathological investigation revealed pulmonary lymphangitis carcinomatosa originating from primary breast adenocarcinoma.
CONCLUSION
To achieve an accurate diagnosis and prevent delay of initiation of proper treatment a thorough diagnostic approach is necessary. In case of doubt, biopsy should be performed to secure clarification. In this case report we discuss the diagnostic value of transbroncial cryobiopsy for this purpose.
PubMed: 38903731
DOI: 10.1080/20018525.2024.2365510 -
Frontiers in Oncology 2024SMARCA4-deficient undifferentiated thoracic tumor (SMARCA4-UT) is a rare malignant tumor characterized by inactivation of the gene and the presence of undifferentiated...
SMARCA4-deficient undifferentiated thoracic tumor (SMARCA4-UT) is a rare malignant tumor characterized by inactivation of the gene and the presence of undifferentiated or rhabdoid morphology in the tissue. This tumor is highly invasive, typically diagnosed at advanced stages III or IV, and commonly involves thoracic structures, such as the mediastinum and chest wall. Reported cases are limited and treatment guidelines have not yet been established. Here, we present a rare case of surgically treated non-metastatic SMARCA4-UT. The patient presented with blood-tinged sputum, dyspnea, and a history of heavy smoking, and underwent surgery after preoperative evaluation ruled out contraindications. The tumor was successfully removed along with the relevant lymph nodes; analysis determined it to be stage IIB T3N0M0. No recurrence was detected at two months post-surgery. However, four months after surgery, the tumor recurred and invaded the adjacent ribs. The diagnosis, differential diagnosis, and treatment of SMARCA4-deficient undifferentiated lung tumors is considered. The combination of chemotherapy and immunotherapy has shown efficacy, and other treatments such as anti-angiogenic drugs, histone deacetylase inhibitors (HDACi), enhancer of zeste 2 polycomb repressive complex 2 subunit (EZH2) inhibitors, and oxidative phosphorylation (OXPHOS) inhibitors may also be beneficial in treating SMARCA4-UT.
PubMed: 38903719
DOI: 10.3389/fonc.2024.1399868