Lungen- und Atemwegsmedizin

Background: Patients with thalassemia major require regular blood transfusions which causes iron overload which untreated leads to respiratory, cardiac, hepatic and endocrine dysfunction.

Objectives: This study was undertaken to assess cardiopulmonary functions in thalassemia major patients and its correlation to serum ferritin.

Methods: A hospital based observational study was conducted over a period of 11 months, between November 2015 and September 2016. 47 thalassemia major patients were included along with 20 age and sex matched controls were taken as controls.

Results: Mean age in our study population was 9.44 ± 2.030 years. The mean Sr. ferritin level was 3217+1351.853 ng/dl. 18 (38%) patients had low FEV1 and FVC; suggesting restrictive pattern along with small airway obstruction suggested by reduced FEF25% to 75%. We observed that as the serum ferritin level increased, mean value of FEV1, FVC, FEV1/FVC, FEF (25% to 75%) decreased and it was statistically significant in cases with ferritin >3000 ng/ml (p value<0.05). In echocardiography interventricular septal thickness and posterior wall diameter were significantly increased in thalassemia patients as compared to controls. The mean value of EF was lower among cases than in controls and this decrease in EF was statistically significant (p<0.05) but there was no systolic dysfunction.16 patients out of 23 (69%) had dilated cardiomypathy in group where sr ferritin was >3000 ng/dl and 5 patients out of 17 (31%) had dilated cardiomypathy in ferritin levels of 2000 to 3000 ng/dl. So the cardiovascular complications were found to be higher in the high-ferritin group (serum ferritin>3000 ng/dl) than in low-ferritin group (serum ferritin 1000-2000). In our study among 47 Thalassemic patients, 15 (40%) were found to have both cardiac pathology as well as pulmonary dysfunction. This finding was statistically significant.

Conclusion: At the end of our study on thalassemic patients, we concluded that all the PFT parameters were decreased with increasing Sr. ferritin level. Most of the echocardiographic parameters were found to be significantly deranged with reference to increase in Sr. ferritin levels. Nearly half of the patients were found to have both pulmonary and cardiac involvement.

Sarcomatoid carcinoma is a rare histologic type of non-small cell lung cancers (NSCLC) which represents only 0.3 to 3% of primitive lung cancers.

In 2004, the World Health Organization (WHO) defined in its classification a new entity, sarcomatoid carcinoma, as “any proliferation that can offer permanently epithelial-mesenchymal morphological transition.”

The term sarcomatoid carcinoma is generic; it includes various entities such as spindle cell carcinomas which are without well-known clinical and immunohistochemical features.

We report the case of a 53-year-old smoker patient, with a history of bullous emphysema discovered 8 years ago and who was hospitalized for exploration of a large pleuro parenchymal mass. The functional signs were dyspnea and alteration of the condition. Percutaneous Computed Tomography (CT) guided biopsy of the mass concluded to a pulmonary spindle cell carcinoma. Staging revealed adrenal nodule with mesenteric and peritoneal invasions. The patient died two months after diagnosis despite an attempt of chemotherapy based on carboplatin/docetaxel.

Objectives: This study describes the un-sedated endoscopic airway findings in infants with obstructive sleep apnea confirmed by polysomnography (PSG). Methods: A retrospective chart review was performed on infants who had undergone both a documented airway endoscopy and PSG from 2006 to 2015 at our center. Demographics, comorbidities, findings of airway endoscopy and findings on PSG were extracted from the electronic medical record. Regression analyses were performed to determine the relationship between AHI, endoscopic airway findings and gestational age. Results: We identified 39 patients with PSG in room air and confirmed obstructive sleep apnea (Apnea Hypopnea Index (AHI) >1/hour) who had undergone unsedated airway endoscopy. The median gestational age at time of PSG was 40 weeks and for endoscopy was 40 weeks. Median AHI on PSG was 17.2/hour. Laryngomalacia [87.2%], pharyngomalacia [33.3%] and tracheomalacia [10.3%] were the three most prevalent findings on endoscopy. Surgically correctable fixed airway obstruction was uncommon. Prevalence of pharyngomalacia decreased with increased gestational maturity (p=0.05). As the postmenstrual age at PSG completion increased, there was a trend towards a decline of the AHI (p=0.087). Twenty-two (56.4%) patients had a follow up PSG performed. Using paired t test, there was a significant decrease in AHI (Δ13.41: -40.9, 15.8, 15.8) from 23.4/hour (1.3-62) to 10.0/hour (0-32), despite no interim surgical intervention. Conclusions: Dynamic airway collapse, including laryngomalacia and pharyngomalacia, were the most common findings in obstructive sleep apnea during infancy. The decreased prevalence of pharyngomalcia and trend towards improvement of AHI with time suggests airway immaturity contributes to obstructive sleep apnea observed during infancy and improves with age.

Background: Thoracic wall reconstruction is commonly performed by using muscle flaps or prosthetic materials. We try through this article to show the possibility of myoplasty in extended thoracic wall resections.
Case report: A 33-year-old woman had been treated by chemo-radiotherapy, for undifferentiated carcinoma of the nasopharyngeal type. The CT-scan completed by magnetic resonance showed a 10 cm sternal mass centered by the manubriosternal articulation with an infiltration of left and right second sternocostal joints and intercostal muscles. The diagnosis of a single sternal metastasis of nasopharyngeal carcinoma was established.

After the resection of anterior arc of right ribs (from the first to the third), resection of internal right clavicle edge, transverse sternotomy above xiphoid process, resection of anterior arc of left ribs (from the first to the third), resection of internal left clavicle edge, sternal tumor and sternal body were removed en-bloc without a 2 cm residual extension which was marked by metallic clips. A myocutaneous plasty using pectoralis major and pectoralis minor muscles covered the chest wall defect after the release of the pectoralis major from its humeral attachment.

The postoperative course was uneventful. Residual tumor was treated by radiotherapy with no disease recurrence sign for 3 years later.
Conclusion: The choice of muscle to use depends on the location and the extent of the defect to be repaired. The knowledge of the anatomy of the muscles is essential to obtain a good quality flap.

Background: The relationship between p73 gene G4C14-to-A4T14 polymorphism and non-small cell lung cancer risk is unclear. Now we performed a meta-analysis to clarify the association of p73 polymorphism with nonsmall cell lung cancer.
Methods: To assess the association between p73 polymorphism and non-small cell lung cancer deeply, we searched Pubmed, Embase, CNKI, Wanfang and CBM databases. All analyses were done using RevMan 5.3 software which provided by the Cochrane Collaboration and Stata version 12.0. The statistical heterogeneity among studies was assessed with the chi-square-based Q test. We used the random effects model as well as the fixed effects model to calculate the pooled ORs.
Results: Our meta-analysis included 6 studies with a total of 1658 patients with non-small lung cancer and 2328 cancer-free control subjects. In all comparisons, we find none of genetic models shows significant relation with the risk of non-small lung cancer (recessive model: OR: 1.16, 95%CI: 0.94-1.43; dominant model: OR: 0.63, 95%CI: 0.37-1.06; co-dominant model: OR: 1.63, 95%CI: 0.94-2.83; allelic model: OR: 1.20, 95%CI: 0.98-1.48). However, when we proceeded to subgroup analysis according country, significantly increased risk was observed in a recessive models (OR: 1.35, 95%CI: 1.15-1.59), in a co-dominant model: (OR: 2.49, 95%CI: 1.76-3.53), in an allelic model (OR, 1.41, 95%CI, 1.24-1.61). Significantly decreased risk was observed in a dominant model (OR: 0.42, 95%CI: 0.30-0.59).
Conclusions: Our results indicate that p73 gene G4C14-to-A4T14 polymorphism is associated with the risk of non-small cell lung cancer in China. However, a large gene-to-environment research is required to confirm this conclusion.

Background: Alaghwar region is the lowest area in the world inhabited by permanent population at the level of the Dead Sea area, which is -420 m (-1,378 ft) below sea level. The reduced barometric pressure, density of air and the degree of acclimatization are associated with the low altitude. These factors are essential for the evaluation of a lung function tests at different altitudes.
Objectives: The main aim of this study was to assess the effect of this unique feature of very low altitude (LA) on the performance of lung function.
Methods: A comparative cross sectional design was chosen as the epidemiological design, and the standard cluster sampling technique was used to select the study population. Study was conducted on 1493 subjects (319 exposed to LA, and 1174 living at HA). Data were collected using predesigned questionnaire on personal and sociodemographic characteristics and smoking habit; as well as measurements of forced spirometry [time-volume curve and flow volume curve), and anthropometric measurements, using standard techniques and equipment. The data was analyzed using SPSS software (IBM, version 22), and multiple regression statistical subroutine, was used. Level of significance for the present study was 0.05.
Results: After allowing for the effect of age, height, gender and smoking habit, people residing in low altitude area had significantly higher lung function indices compared to those residing at high altitude areas. Smoking was found to have significant negative effect on the different indices of lung function mainly of obstructive type.
Conclusions: The increased barometric pressure, decreased density of air and the degree of acclimatization have shown an increment in most pulmonary function indices at low altitude. Basically high altitude may play a role in altering ventilatory function. However, additional factors like smoking should be considered.

Pulmonary mucormycosis is a relatively uncommon infection that is mostly observed among diabetic patients. We present here three cases of pulmonary mucormycosis as the cause of non-resolving pneumonia in diabetic patients. The goals of our review are to better characterize the population at risk, presenting symptoms, radiological appearance, diagnostic methods and to demonstrate the efficacy of medical therapy.

Introduction: National Hospital for Respiratory Diseases (NHRD) at Welisara is the Premier tertiary care hospital in Sri Lanka for respiratory diseases. No studies have been done in its OPD set up to collect data which could have been invaluable for future planning and implementation. This study was done to gather such vital information in view of improving services rendered by NHRD OPD. Few parameters like socio-demographic details, distance to the hospital, cost for the visit, having a referral letter given by a doctor, number of persons accompanied the patient and reasons to visit NHRD were studied.
Methodology: This was a descriptive cross sectional study of patients who attended OPD of NHRD. Interviewer administered questionnaire was given to randomly selected 420 patients who were above 18 years of age and who were on their first visit to the hospital.
Results: Majority of patients were less educated and were coming from a low socio-economic background. Both sexes presented equally to the OPD. 68% of patients were coming from within 20 km of distance from the hospital and for 62%, the cost for the visit was found to be 200 rupees or less. Only for 7% of people, NHRD was the nearest hospital. 95% of patients has presented with respiratory related conditions while only 2% had a referral letter with them. Most of the patients (around 80%) were accompanied by 2 or 3 persons. More than 70% visited NHRD due to its reputation as the leading hospital in the country for respiratory diseases.
Conclusion: Middle aged less educated people with a low socio-economic status were the people who mostly visited the NHRD OPD. Most had respiratory system related presenting complaints. This is of significance and should be considered in giving health education to these patients. Majority of the patients resided close by to the hospital and their cost for the visit roughly showed similarities to the distances they travel. Though most patients visit NHRD after using nearby health facilities, lack of a proper referral system and accompained too many people can worsen the overcrowding at OPD. Strengthening the usage of nearby healthcare facilities can reduce this as NHRD was found to be the nearest hospital for very few of this population. Most common reason for visiting NHRD OPD was the reputation NHRD has gained as the leading hospital of the country for Respiratory disease, which should be conserved.

Introduction/Background: Chronic obstructive pulmonary disease (COPD) is associated with osteoporosis and vertebral fractures. It is still unclear whether the presence of fractures and changes in bone mineral density (BMD) are associated with disease severity and prognosis. The aim of this study was to evaluate low BMD, and morphometric vertebral fractures (MVF) in patients with COPD compared with two control groups and correlate these parameters with indices of COPD severity (FEV1 and GOLD) and prognosis (BODE).
Methodology: This was a cross-sectional study in COPD patients (disease group, DG) that undergone BMD and vertebral fracture assessment (VFA). Two control groups were used, one group of smokers individuals without COPD (smokers group, SG), and another group of healthy never smokers individuals (never smokers group, NSG).
Results: DG comprised 121 patients (65 women, mean age 67.9 ± 8.6 years). Altered BMD was observed in 88.4% of the patients in the DG which was more prevalent when compared to control groups (p<0.001). The BMD values were lower in the DG than in controls (p<0.05). BMD was associated with the worst degree of obstruction (FEV1), GOLD, and BODE (p<0.05). The prevalence of MVF was high (57.8%) and greater than that in the SG (23.8%), and NSG (14.8%; p<0.001). The prevalence of fractures was not associated with FEV1, GOLD, or BODE.
Conclusions: This study showed a high prevalence of low BMD in COPD patients and an association with a worse degree of FEV1, GOLD, and BODE. MVF in patients with COPD were also higher but were not associated with disease gravity and prognosis.

A 45-year-old Saudi female with history of SLE on hydroxychloroquine 400 mg daily and prednisolone 5 mg daily Presented to ER by epigastric pain, diarrhoea 3 motions per day and generalized body aches associated with infrequent cough for past 2 days not preceded by flu symptoms without contact to sick people for last month, without history of recent travel and without contact to animals.