Effect of Sequential Intravenous Pulse Cyclophosphamide-Azathioprine in Systemic Sclerosis-Interstitial Lung Disease: An Open-Label Study.

BACKGROUND: Systemic sclerosis (SSc) is a rare connective tissue disorder of unknown aetiology. Pulmonary involvement contributes substantially to its morbidity and mortality. Treatment of pulmonary disease due to SSc remains unsatisfactory. We examined the effect of sequential six-month intravenous pulse therapy with cyclophosphamide (CYC) followed by azathioprine and low-dose corticosteroids on SSc associated interstitial lung disease (SSc-ILD). METHODS: In a single-centre, prospective, observational, open-labelled study; nine patients (eight females, one male) with SSc-ILD were treated with intravenous pulse CYC (600mg/m(2) body surface area) at monthly interval for six cycles with oral prednisolone 10mg daily. Subsequently, azathioprine (2-3mg/Kg) was administered while continuing with the same dose of prednisolone. Primary end-points were forced vital capacity (FVC) and high resolution computed tomography (HRCT) scan of thorax score. Secondary end-points were quality of life measured by health assessment questionnaire-disability index (HAQ-DI) and six-minute walk distance (6WMD) test. RESULTS: After one year of observation, the FVC showed significant improvement (p=0.003). The 6WMD also improved significantly (p=0.0028). However, change in HRCT scan scoring and HAQ-DI score was not significant. CONCLUSIONS: Intravenous, pulse CYC followed by azathioprine along with low-dose corticosteroids produces significant improvement in FVC and 6WMD at 12-month follow-up without significant change in radiological manifestations and health status.

Genetic analysis of familial spontaneous pneumothorax in an Indian family.

Familial spontaneous pneumothorax is one of the phenotypes of Birt-Hogg-Dubé syndrome (BHDS), an autosomal dominant condition associated with folliculin (FLCN). We investigated clinical and genetic data of an Indian family having two patients suffering from spontaneous pneumothorax in the absence of skin lesions or renal tumors. HRCT scan of patient's lung revealed paracardiac cysts, and DNA sequencing of all 14 exons of FLCN from patients showed the presence of heterozygous "C allele" deletion in the poly-cytosine (poly-C) tract of exon 11 leading to truncated folliculin. This mutation was also observed in four asymptomatic members of the family. Our results confirmed the presence of deletion mutation in poly-C tract of FLCN in members of BHDS family. This is the first report of genetic insight in a BHDS family from India but in-depth studies with a larger sample set are necessary to understand mechanism of familial pneumothorax.

Isolated Endobronchial Capillary Haemangioma: A Rare Cause of Haemoptysis in Adult.

Endobronchial capillary haemangioma is a very rare benign tumour in adults. The clinical presentation and management of adult capillary haemangiomas involving the tracheo-bronchial tree is not yet established. We present a case of an isolated capillary haemangioma of the left main bronchus detected during the evaluation of an adult male presented with haemoptysis. The lesion was managed successfully bronchoscopically.

A Study of Depression in Adult Patients with Bronchial Asthma Presenting to a Tertiary Care Hospital in Eastern India.

BACKGROUND: Bronchial asthma is a serious global health problem. Depression, the most common mood disorder, is often found to be higher among people with chronic health conditions like bronchial asthma. METHODS: Patients with newly diagnosed to have bronchial asthma (n = 100) who fulfilled the study criteria were evaluated for depression with Beck Depression Inventory (BDI) score. Severity and level of bronchial asthma control were determined as per Global Initiative for Asthma (GINA) guidelines. Subjective asthma severity was assessed by Perceived Control of Asthma Questionnaire. Follow-up evaluation was done after three months of asthma management with the same study tools. RESULTS: In our study population, 65% asthma patients showed depression on first visit (95% Confidence interval [CI] 55.65-74.35). Correlation coefficient between subjective asthma severity and severity of depression was -0.945 (p < 0.001) while correlation coefficient between objective asthma severity and depression severity was 0.066 (p = 0.515). In follow-up visit after asthma management 63% patients still had depression (95% CI 53.54-72.46). Correlation coefficient between objective asthma control and depression severity was 0.1 (p = 0.320). Correlation coefficient between subjective asthma severity and severity of depression was -0.979 (p < 0.001). CONCLUSIONS: Our observational study suggests that depression is highly prevalent in asthma patients. There is a high inverse correlation between depression and patient's perception of asthma control. However, no significant correlation could be observed between objective measures of asthma severity and depression.

Ancient schwannoma of vagus nerve mimicking hamartoma.

Schwannomas arising from vagus nerve sheath are rare mediastinal neurogenic tumours. Schwannomas usually arise from left hemithorax. Unlike a hamartoma, radiologically, calcification is rarely seen in schwannomas. We present the rare case of an ancient schwannoma arising from vagus nerve sheath from the right hemithorax presenting with gross calcification.

Leveraging metal node-linker self-assembly to access functional anisotropy of zirconium-based MOF-on-MOF epitaxial heterostructure thin films.

Chemically robust, functional porous materials are imperative for designing novel membranes for chemical separation and heterogeneous catalysts. Among the array of potential materials, zirconium (Zr)-based metal-organic frameworks (MOFs) have garnered considerable attention, and have been investigated for applications related to gas separation and storage, and catalysis. However, a significant challenge with Zr-MOFs lies in their processibility, particularly in achieving homogenous thin films and controlling functional anisotropy. The recent developments in MOF thin film fabrication methodologies do not yield a solution to achieve mild reaction condition growth of Zr-MOF thin films with epitaxial MOF-on-MOF geometry (i.e. functional anisotropy). In the current work, we have devised a straightforward methodology under room temperature conditions, which enables epitaxial, oriented MOF-on-MOF thin film growth. This achievement is accomplished through a stepwise self-assembly approach involving Zr nodes and linkers on a functionalized substrate. This de novo developed strategy of functionality design is demonstrated for UiO-66 (University of Oslo) type Zr-MOFs. We have demonstrated the precise placement of chemical functionalities within the thin film structure, allowing for controlled chemical diffusion and regulation of diffusion selectivity.

Pulmonary renal syndrome in a case of Wegener's granulomatosis.

We report a case of a 42-year-old patient who presented with Wegener's granulomatosis complicated by pulmonary renal syndrome, i.e., diffuse alveolar haemorrhage and rapidly progressive crescentic glomerulonephritis. The patient was treated with plasmapheresis and immunosuppressive drugs--intravenous cyclophosphamide and methyl prednisolone. The clinical, haematological and biochemical parameters improved substantially and remission is achieved.

A roadmap to enhance gas permselectivity in metal-organic framework-based mixed-matrix membranes.

Performing gas separation at high efficiency with minimum energy input and reduced carbon footprint is a major challenge. While several separation methods exist at various technology readiness levels, porous membrane-based separation is considered as a disruptive technology. To attain sustainability and required efficiency, different approaches of membrane design have been explored. However, the selectivity-permeation trade-off and membrane aging have restricted further advancement. In this regard, a new generation composite made of organic polymers and metal-organic framework (MOF) fillers shows substantial promise. Organic polymer matrix allows easy processibility, but it has poor permselectivity for gas molecules. Metal-organic frameworks are excellent sieving materials; however, they suffer from poor processibility issues. A combination of these two components makes an ideal sieving membrane, which can potentially outnumber the existing energy intensive distillation strategies. In this perspective, we have discussed key indices that regulate gas permselectivity by a careful selection of the existing literature. While the target gas flux and selectivity values have been a part of many previous reviews and articles, we have presented a concise discussion on the interface design of the MOF-polymer membrane, morphology, and orientation control of MOF fillers in the matrix. Following this, a future roadmap to overcome challenges related to MOF-polymer interfacial defects is outlined.

Squamous cell carcinoma lung with progressive systemic sclerosis.

Association between progressive systemic sclerosis (PSS) and lung malignancy is rare yet well recognised. In order of frequency bronchioloalveolar carcinoma (BAC) is most common followed by squamous cell carcinoma, oat cell carcinoma, anaplastic carcinoma of lung. We present a 55 year old non-smoker male patient with PSS, who presented to us with progressively increasing shortness of breath and dry cough. Initially clinico-radiologically he was provisionally diagnosed as a case of non-resolving pneumonia. Subsequent investigations revealed it is a case of squamous cell carcinoma of lung with PSS.

Hydatid lung disease: an analysis of five years cumulative data from Kolkata.

BACKGROUND AND OBJECTIVE: There is scarcity of clinical data regarding hydatid lung disease from Indian subcontinent. In this retrospective study we analyzed the cumulative data over five year's period from two tertiary care hospitals of Kolkata to determine the presentation, treatment and outcome of hydatid disease of lung. METHOD: We analyzed 106 patients on the basis of clinical findings, radiological findings [chest X-ray--PA and lateral view, CT scan of thorax and upper abdomen, ultrasonography (USG) of thorax and upper abdomen] and serological test IgG ELISA (enzyme linked immunosorbent assay) against echinococcus granulosus antigen along with the mode of diagnosis, indications and types of surgery, and outcome of treatment with chemotherapy (albendazole), surgery and combined surgery with chemotherapy. RESULT: There was female preponderance with median age of presentation 33 years with interquartile range of 7. At the time of diagnosis 14.15% patients were asymptomatic. Cough was the commonest symptom (73.58%) followed by chest pain (54.72%). Single sharply demarcated round or oval homogenous opacity was the commonest radiological sign (81.13%). Complicated cysts such as lung abscess, pleural involvement, pneumonitis and fibrosis were noted in 10.38%, 13.21%, 7.55% and 11.32% cases respectively. We found that serological test was only 77.01% sensitive. Eighty-two (86.32%) patients underwent surgery, and 56.10% of them received additional chemotherapy with albendazole. The types of operation performed were pericystectomy (in 91.46% cases), lobectomy (in 6.10% cases) and pneumonectomy (in 2.44% cases). Perioperative mortality was nil but morbidity was found in 10.98% cases with empyema (8.54%), the commonest complication. Thirteen inoperable patients were treated with albendazole, among them 8 showed reduction of cyst-size, 3 showed no change and 2 patients showed obliteration of cyst. CONCLUSION: Surgery is a safe and effective way of treatment for thoracic hydatid cyst along with perioperative albendazole therapy. There is a scope for chemotherapy with albendazole in inoperable cases.

Effects of polyethylene terephthalate microplastic on germination, biochemistry and phytotoxicity of Cicer arietinum L. and cytotoxicity study on Allium cepa L.

Accumulation of plastic materials in terrestrial systems threatens to contaminate food chains. The aim of the current study is to determine the impact of microplastics synthesized from PET plastics (control, 50, 250, 500, 750, 1000 mg/L) with respect to morphological, biochemical impact on Cicer arietinum using standardized 72 h assay and cytotoxicity study on Allium cepa root tips. The synthesized microplastics were characterized by Scanning Electron Microscope (SEM) and Fourier Transform Infrared spectroscopy (FTIR) studies. Germination studies clearly revealed that there is a sharp decrease in germination with increasing the concentration of microplastics. Both pigment and carbohydrate levels increased up to 500 mg/L concentration, although protein levels increased with increase of microplastic dose. Catalase activity also increased with increasing microplastic concentration. Finally, cytotoxicity studies revealed significant chromosomal aberration at higher dose of microplastics. Therefore, it may be concluded that the microplastics have significant biological and structural adverse effects on plant metabolism.

Psychological profile of multi drug resistance TB patients: A qualitative study from a Tertiary care Centre of Kolkata.

INTRODUCTION: There has been a new challenge to the already existing threat of tuberculosis (TB) and that is drug resistance TB (DR-TB). The causal relationships between mental disorders and TB are complicated and relatively unexplored. For this reason a qualitative study was done on DR-TB patients attending R G Kar Medical College. MATERIALS AND METHODS: The study population consisted of the patients who are registered for the DR-TB regimen are followed up four times with General Health Questionnaire (GHQ). Those scoring poorly were sent for expert evaluation by psychologist, who counselled them, and followed them up after in-depth interviews. These records of in-depth interview were analysed as qualitative research inputs. RESULTS: In our study out of 165 patients, (4.8%) needed interventions. The domains emerging from the study are worried about future and as well as family, disbelief about the diagnosis, embarrassment regarding the diagnosis, fear of death, blaming fate for the disease, stigma, suicidal ideation. CONCLUSION: This study finds out the important domains of psychogical problems among the patients and also advocates a psychologist to remain at DR-TB centres.

Mesothelioma with superior vena cava obstruction in young female following short latency of asbestos exposure.

An 18 years female was admitted with right-sided chest pain, dry cough, and low-grade fever and weight loss for last 1 month. On examination, patient had features of superior vena cava (SVC) syndrome with right-sided pleural effusion. Chest X-ray showed mediastinal widening with nonhomogenous opacity mainly in the periphery of right upper and mid zone with right-sided pleural effusion. Ultrasonography thorax confirmed mild pleural effusion. Pleural fluid analysis showed lymphocytic, exudative, low adenosine deaminase with negative for Pap smear. Contrast-enhanced computed tomography (CT) thorax revealed large extensive nodular soft tissue lesion along right mediastinum as well as costal pleura, with enlarged pretracheal lymphadenopathy and SVC obstruction. CT guided Tru-cut biopsy report came as malignant epithelial tumor with polygonal shape, abundant eosinophilic cytoplasm and nuclei with prominent nucleoli suggestive of mesothelioma of epithelioid type. The tumor cell expressed calretinin, WT-1, and immunonegative for thyroid transcription factor-1.

Rare Endobronchial metastasis from uterine leiomyosarcoma.

Uterine sarcomas are rare and represent approximately 3.2% of all invasive uterine cancers. The annual incidence rate is less than two per 100,000 women. The median age at which uterine sarcoma diagnosed is 56 years. The most common histologic pattern is leiomyosarcoma (LMS) which originates from the myometrium or myometrial vessels. Uterine LMSs are aggressive tumors with high rates of recurrence. The most common mode of spread is hematogenous, with lymphatic spread being rare. Recurrences of up to 70% are reported in stage I and II disease with the site of recurrence being distal, most commonly the lungs or the upper abdomen. But the intra bronchial spread is extremely rare. Here we are reporting a case of uterine LMS with endobronchial metastasis causing whole lung collapse.

Common Presentation with Uncommon Diagnosis: Multifocal Epithelioid Hemangioendothelioma.

A young female patient presenting with recurrent hemoptysis, neck swelling, and mediastinal mass mimicking lymphadenopathy was admitted to the Institute of Post Graduate Medical Education and Research and SSKM hospital, Kolkata, India. Clinical features, radiological studies, fibre optic bronchoscopy, and fine needle aspiration cytology from the neck swelling created a diagnostic dilemma until surgical resection and immunohistochemistry reports confirmed the diagnosis of multifocal epithelioid hemangioendothelioma, a rare vascular tumor with intermediate malignancy potential. Because it is a slow-progressing disease and due to the non-availability of standard chemotherapy, the patient, and her legal guardian, opted for palliative care only. She was asymptomatic for four years but again presented with hemoptysis, reappearance of the neck swelling on the same side, and a mediastinal mass compressing the superior vena cava and right pulmonary artery. This report describes the diagnostic problems and therapeutic challenges in the management of this rare tumor over a four-year follow-up period. The clinical course emphasizes the highly unpredictable nature of this tumor.

Reference equation for spirometry interpretation for Eastern India.

INTRODUCTION: Spirometry measurements are interpreted by comparing with reference values for healthy individuals that have been derived from multiple regression equations from earlier studies. There are only two such studies from Eastern India, both by Chatterjee et al., one each for males and females. These are however single center and approximately two decades old studies. AIMS: (1) to formulate a new regression equation for predicting FEV1 and FVC for eastern India and (2) to compare the results to the previous two studies by Chatterjee et al. MATERIALS AND METHODS: Healthy nonsmokers were recruited through health camps under the initiative of four large hospitals of Kolkata. Predicted equations were derived for FEV1, FVC and FEV1/FVC in males and females separately using multiple linear regression, which were then compared with the older equations using Bland-Altman method. RESULTS: The Bland-Altman analyses show that the mean bias for females for FVC was 0.39 L (95% limits of agreement 1.32 to -0.54 L) and for FEV1 was 0.334 L (95% limits of agreement of 1.08 to -0.41 L). For males the mean bias for FEV1 was -0.141 L, (95% limits of agreement 0.88 to -1.16 L) while that for FVC was -0.112 L (95% limits of agreement 0.80 to -1.08 L). CONCLUSION: New updated regression equations are needed for predicting reference values for spirometry interpretation. The regression equations proposed in this study may be considered appropriate for use in current practice for eastern India until further studies are available.

Assessment of spontaneous pneumothorax in adults in a tertiary care hospital.

CONTEXT: Pneumothorax continues to be a major cause of morbidity and mortality among respiratory patients, but there is a paucity of data regarding etiology, clinical profile, management, and outcome of spontaneous pneumothorax (SP), from this part of the world. AIMS: To assess the patients of spontaneous pneumothorax in adults with special reference to the etiology, clinical presentation, management, and outcome of SP. SETTINGS AND DESIGN: Prospective, observational study conducted in a tertiary care institution over a period of one year. MATERIALS AND METHODS: All adult patients of SP attending the department of pulmonary medicine in a tertiary hospital were studied and detailed clinical, radiological, and management data were recorded and analyzed. RESULTS: Sixty consecutive patients, who satisfied the inclusion criteria were included in the study. Among them 10 had primary spontaneous pneumothorax (PSP) and 50 had secondary spontaneous pneumothorax (SSP). The overall male to female ratio was 4:1. The mean age of the PSP patients was 26.3 ± 2.19 years, whereas, that of the SSP patients was 53.42 ± 2.07 years (P < 0.0001). Seventy percent of the patients were smokers. The most common clinical manifestation of PSP was chest pain (80%) in contrast to dyspnea in SSP (96%). The most common cause of SSP (42%) was found to be chronic obstructive pulmonary disease (COPD) followed by pulmonary tuberculosis (30%). The cases were managed with intercostal tube drainage (85%), simple aspiration (8.33%), and observation (6.67%). Full expansion of the lung was noted in 91.67% of the cases. CONCLUSION: Spontaneous pneumothorax was more common in men. SSP was far more common in this study, and the predominant underlying cause of SSP was COPD, which surpassed tuberculosis as the leading cause of SSP. This is in contrast to the results from previous studies done in our country. Intercostal tube drainage was the mainstay of treatment and the response was good.

Habitual physical activity score as a predictor of the 6-min walk test distance in healthy adults.

BACKGROUND: The 6-min walk test (6MWT) is a simple, inexpensive test of functional exercise capacity. The 6MWT distance (6MWD) in healthy adults varies geographically, emphasizing the need for population-specific reference equations. The purpose of the present study was to investigate the influences of the habitual physical activity (HPA) score and other anthropometric and demographic parameters on the variability of the 6MWD among healthy adults and to propose a reference equation. METHODS: This was a prospective, cross-sectional, observational study. The 6MWT was conducted in a 30-m hospital corridor on 201 healthy volunteers, 125 men and 76 women, aged 20-60 years. The HPA in the previous 6 months was assessed using Baecke's questionnaire. Univariate analysis followed by multiple regression analysis was performed to analyze the significance levels of different probable predictors. RESULTS: The 6MWD was significantly greater in more active than in less active subjects (663.8±55.4m vs. 599.9±67.8m, p<0.001). The regression analysis showed that the subject's age in years (p=0.017), gender (p=0.006), height in cm (p=0.004), weight in kg (p<0.001), total activity score (TS) (p<0.001), and absolute difference in heart rate before and after exercise (p<0.001) could explain 48.9% of the variability in the 6MWD in healthy adults. CONCLUSIONS: The HPA score is probably the most appropriate variable to include in the reference equation predicting the 6MWD in healthy adults from the Indian subcontinent.

Primary Lymphoepithelioma-like Carcinoma of the Lung in a 13 year old Girl.

Lymphoepithelioma-like carcinoma, a large cell carcinoma with pronounced lymphocyte infiltration, is a rare entity mostly seen in the nasopharynx. But primary pulmonary LELC in children & adolescents is extremely rare. Here we present a 13 year old girl with chief complaint of fever, cough, shortness of breath and heaviness in the left side of chest. She underwent left upper lobectomy. Histopathological examination of the biopsy specimen confirmed it to be a case of large cell carcinoma of lung, lymphoepithelioma type, invading pleura (stage pT3N0M0) with high serum titre of anti Epstein-Barr virus IgG antibody.