Cystic lung disease lymphangioleiomyomatosis (LAM) in a male patient.

Lymphangioleiomyomatosis (LAM) is characterised by the proliferation of abnormal smooth muscle cells (LAM cells) in the lungs, lymph nodes and other organs. We report the case of a man in his 50s who had right-sided pleural effusion. On performing a diagnostic tap, the fluid was milky white in colour. An intercostal chest tube was inserted and after complete drainage of fluid, a high-resolution CT (HRCT) was done. HRCT revealed multiple cysts throughout both of the lungs. On subsequent bronchoscopy-guided transbronchial lung biopsy and histochemical staining, diagnosis of LAM was made. We started the patient on oral sirolimus. On subsequent follow-up, subjective and objective improvements were observed.

Explaining citizens' resistance to use digital contact tracing apps: A mixed-methods study.

Governments worldwide are using digital contact tracing (DCT) apps as a critical element in their COVID-19 pandemic lockdown exit strategy. Despite substantial investment in research and development, the public's acceptance of DCT apps has been phenomenally low, signaling resistance among potential users. Little is known about why people would resist using the DCT app, a useful innovation that can potentially save millions of human lives. This study explores the determinants and consequences of citizens' resistance to use DCT apps using a sequential two-stage mixed-methods approach. The preliminary qualitative study analyzed interviews of 24 Indian smartphone users who chose not to use or discontinued the DCT app after an initial trial. In the quantitative stage, an integrated model based on innovation resistance theory and distrust theory was tested using the survey data collected from 194 non-adopters of the DCT app from India. The findings revealed that the factors, distrust, value barrier, information privacy concerns, and usage barrier predicted the resistance to the DCT app, and resistance, in turn, predicted intention to use. Additionally, distrust was found to be a key mediator between innovation barriers and resistance. The insights from this study could help the developers and policymakers formulate strategies for implementing DCT interventions during future disease outbreaks.

Genetic insight into Birt-Hogg-Dubé syndrome in Indian patients reveals novel mutations at FLCN.

BACKGROUND: Birt-Hogg-Dubé syndrome (BHDS) is a rare monogenic condition mostly associated with germline mutations at FLCN. It is characterized by either one or more manifestations of primary spontaneous pneumothorax (PSP), skin fibrofolliculomas and renal carcinoma (chromophobe). Here, we comprehensively studied the mutational background of 31 clinically diagnosed BHDS patients and their 74 asymptomatic related members from 15 Indian families. RESULTS: Targeted amplicon next-generation sequencing (NGS) and Sanger sequencing of FLCN in patients and asymptomatic members revealed a total of 76 variants. Among these variants, six different types of pathogenic FLCN mutations were detected in 26 patients and some asymptomatic family members. Two of the variants were novel mutations: an 11-nucleotide deletion (c.1150_1160delGTCCAGTCAGC) and a splice acceptor mutation (c.1301-1G > A). Two variants were Clinvar reported pathogenic mutations: a stop-gain (c.634C > T) and a 4-nucleotide duplication (c.1329_1332dupAGCC). Two known variants were: hotspot deletion (c.1285delC) and a splice donor mutation (c.1300 + 1G > A). FLCN mutations could not be detected in patients and asymptomatic members from 5 families. All these mutations greatly affected the protein stability and FLCN-FNIP2 interaction as observed by molecular docking method. Family-based association study inferred pathogenic FLCN mutations are significantly associated with BHDS. CONCLUSION: Six pathogenic FLCN mutations were detected in patients from 10 families out of 15 families in the cohort. Therefore, genetic screening is necessary to validate the clinical diagnosis. The pathogenic mutations at FLCN affects the protein-protein interaction, which plays key roles in various metabolic pathways. Since, pathogenic mutations could not be detected in exonic regions of FLCN in 5 families, whole genome sequencing is necessary to detect all mutations at FLCN and/or any undescribed gene/s that may also be implicated in BHDS.

Comparison of diagnostic accuracy of digital chest X-ray images between PACS and WhatsApp Messenger in resource poor setting.

PURPOSE: Many underserved remote locations without specialists would benefit from the ability to quickly and easily share images of radiographs with trained radiologists using WhatsApp messenger. However, there is limited evidence on the role of WhatsApp messenger for sharing chest x-ray (CXR) images to aid diagnosis and management. The objective of the study was to determine the diagnostic accuracy and inter-observer agreement of WhatsApp messenger images of digital CXR compared to viewing on Picture Archiving and Communication System (PACS) monitor. METHODS: Two pulmonologists reported 400 WhatsApp messenger images of digital CXR each. After a wash period of two weeks, they reviewed the original CXR images on PACS and again reported their findings. Diagnostic agreement was measured using kappa value, diagnostic accuracy was evaluated by sensitivity and specificity. RESULTS: The diagnostic agreement between WhatsApp and PACS images for both the readers was high in case of normal CXR (0.84), Pneumonia (0.85) and Active Koch's (0.79) and Old Koch's (0.71). The inter-observer agreement between two readers on WhatsApp images was good in cases of normal chest x-ray (0.74), Active Koch's (0.61) and Pneumonia (0.74) and low in COPD (0.31) and Pleural Effusion (0.28) and Carcinoma Lung (0.40). In terms of radiological lesion, inter-observer agreement between two readers on WhatsApp images was good in terms of the zonal involvement, moderate in case of infiltrates, consolidation, nodules, and fibrosis, fair in cavity, effusion (0.28) and poor in hilar lymphadenopathy (0.14). The sensitivity in the diagnosis of nodules, effusion and hilar lymphadenopathy was <50% in both the readers. CONCLUSION: CXR transmission via WhatsApp is able to identify clinical findings similar to viewing the same image on a PACS monitor in cases of Pneumonia and normal subjects. Active and old Koch's has good comparability whereas; diagnostic agreement is poor in COPD, cavity, pleural effusion and hilar lymphadenopathy, requiring more caution during interpretation.

Mobile digital x-ray and portable cartridge based nucleic acid amplification test machines for point-of-care diagnosis of TB in rural India.

BACKGROUND: There is no experience of point-of-care (POC) microbiological confirmation for TB in India in field settings. METHODS: Under the TB-Free Haryana project, a mobile van-mounted digital x-ray and portable GeneXpert system screened all presumptive TB patients with strong clinic-radiological suspicion for TB. RESULTS: Of 1673 x-rays, 215 (13%) had findings suggestive of TB, 109 had strong clinical suspicion and were eligible for POC GeneXpert, in whom a test was performed in 82 (75%) cases; 59 (72%) tested positive and were initiated on treatment within 24 h. CONCLUSIONS: A mobile van equipped with digital x-ray and POC GeneXpert is feasible and has a good success rate with potential for replication.

Implementing upfront mobile digital chest x-ray for tuberculosis diagnosis in India-feasibility and benefits.

BACKGROUND: The Tuberculosis (TB) Control Program in India changed the TB diagnostic algorithm and recommended sputum testing and chest x-ray (CXR) for presumptive TB up front. There is no experience of testing this algorithm in routine field settings. METHODS: In a public-private partnership (PPP), a private hospital provided mobile digital CXR services (mounted on a van) to complement the existing diagnostic services of sputum microscopy and GeneXpert testing. All presumptive TB patients (cough >2 weeks) underwent CXR and sputum microscopy, and GeneXpert testing if eligible (smear-negative CXR suggestive of TB). RESULTS: All 2973 presumptive TB patients underwent CXR and sputum microscopy; 471 (15.8%) had abnormal CXR findings suggestive of TB, 129 (4.3%) were smear positive and 17 were extrapulmonary TB. Of the remaining 325 with smear-negative and CXR suggestive of TB, 147 (45.2%) underwent GeneXpert testing, yielding 32 positives (21.8%). Of the remaining 178 with no GeneXpert test done, 106 (60.0%) had CXR definitely suggesting TB (clinically diagnosed TB). Thus a total of 284 cases of TB (161 microbiologically confirmed, 106 clinically diagnosed, 17 extrapulmonary TB) were identified, giving a potential diagnostic yield of 19.6%. CONCLUSIONS: Systematic screening with mobile digital X-ray service via a PPP model integrated into the national program is feasible and scalable with a high yield.

Pulmonary cryptococcosis: Report of the first confirmed autochthonous case in Nepal.

We report a case of cryptococcal pneumonia in a 53 years old female Nepali patient with uncontrolled diabetes mellitus type 2 who empirically received multiple broad-spectrum antibiotics and standard anti-tubercular treatment as well as glucocorticosteroids for suspected sarcoidosis before the diagnosis was finally established. To our knowledge, this is the first confirmed autochthonous case of cryptococcosis reported from Nepal.

Fifth year of a public-private partnership to improve the case detection of tuberculosis in India: A role model for future action?

BACKGROUND: There is limited access to radiology facilities in most parts of India leading to significant under diagnosis and underreporting of smear negative clinically diagnosed tuberculosis (CDTB). Public Private Partnership (PPP) has a lot to contribute towards addressing this gap through providing access to chest x-ray (CXR) in far-off locations. METHOD: Mobile vans equipped with digital CXR equipment and support staff were provided by a Corporate Hospital working closely with government, with scheduled visits to government peripheral health institutes. Patients received upfront CXR and sputum microscopy along with GeneXpert in accordance with the revised TB diagnostic algorithm prescribed by the national program, Following a successful pilot in 2014 in district Rewari, "TB free Haryana" was launched in November 2015 with a phased roll out in 16 districts by 2018. RESULTS: The pilot initiative in 2014 confirmed practical and clinical feasibility and revealed a high rate (30% of people screened) of CDTB i.e. symptomatic cases with radiologic abnormalities compatible with Pulmonary TB. In the first year (2016), 5 districts were covered and a total of 3340 CXRs were carried out. There was an increase in the case notification rates of new CDTB (smear negative) in 2016 compared to 2015 (p = 0.036); yielding an additional 180 cases and an 11.67% increase in case detection. Scale up to a total of 13 and 16 districts took place successfully in 2017 and 2018 respectively; with 6268 CXRs and 8021 CXRs done in the respective years. CONCLUSION: PPP can involve Corporate Hospitals to improve the existing diagnostic infrastructure and provides access to CXR in a not-for-profit sustainable collaboration, with scale-up to the state level; and potential to replicate this initiative in other states.

A Case of Sirolimus-Induced Interstitial Lung Disease After Liver Transplantation.

Mammalian target of rapamycin inhibitors (mTORis) are immunosuppression agents that are commonly used in solid organ transplantation. Available mTORis include sirolimus and everolimus. Interstitial lung disease (ILD) is an uncommon side effect of mTORis in liver transplantation. A high index of suspicion is needed, and timely intervention can reverse the disease. We present a case of sirolimus-induced ILD that improved after discontinuation of sirolimus.

Barber's neck manipulation causing bilateral diaphragmatic paralysis and type-2 respiratory failure.

This is a case report of an unusual cause of bilateral diaphragmatic palsy. A 54-year-old gentleman, presented to us with exertional dyspnea and chest heaviness for the past 6 months which had increased in the last 6 days. Dyspnea increased on lying down. He was diagnosed as pneumonia on the basis of X-ray and chest CT scan, received treatment for the same and responded to the therapy. However, breathlessness and hypercapnia persisted. He had unexplained hypercapnia for which extensive investigations were carried out. Neurological and cardiac assessments were essentially normal. On revisit clinical examination, he was found to have paradoxical diaphragmatic movement with respiration. Ultrasound of chest detected no diaphragmatic movement. Detailed history elicited that patient was fond of neck massage and neck cracking wherein his barber would bend his neck with jerk to either side after a haircut.After considering all possible etiologies; we concluded that it was a case of diaphragm palsy induced by barber neck manipulation, leading to Type-2 respiratory failure. The fact that the vital clues to the diagnosis were elicited by detailed history and thorough examination reinforces that history and clinical examination for doctors shall remain a very important tool for clinical diagnosis.

Bullet in tracheobronchial tree without lung contusion removed by fibreoptic bronchoscopy in two parts.

A person presented with multiple gunshot injury. Chest x-ray & CT whole body trauma protocol was done which showed multiples pellets of bullet in abdomen and one bullet in elbow according to entry wound. There was an entry wound without any bullet in left maxillofacial region however there was no exit wound. A bullet was noticed in tracheobronchial tree. There was no pneumothorax any signs of chest trauma or any pneumomediastinum. It is assumed that the bullet first hit the left cheek (maxilla) and lost its momentum. As the patient lost consciousness and had a fall leading to inhalation (aspiration) of bullet in the airway. As per ballistic experts it was basically a jacketed metallic bullet. As bullet moved in airway, the outer metallic core reached the trachea near carina and the soft metallic core slipped more distally to right main bronchus and bronchus intermedius. While inspection the outer metallic capsule was seen in trachea just above carina which was hollow and was gently removed with the help of foreign body forceps. The core was removed with dormia basket without any mucosal tear. The favorable outcome can be attributed as patient had no lung contusion or chest trauma and bullet was inhaled which was not very old. The evolution of bronchoscopy started with rigid one but the fibreoptic bronchoscopy (FOB) has revolutionized the pulmonary interventions. The FOB can be used with minimal traumas under local anesthesia resulting in markedly reduced morbidity and mortality.

Comparison of clinical and cost-effectiveness of two strategies using mobile digital x-ray to detect pulmonary tuberculosis in rural India.

BACKGROUND: Medanta - The Medicity, a multi-super specialty corporate hospital in Gurugram, Haryana launched a "TB-Free Haryana" Campaign; mobile van equipped with a digital CXR machine to screen patients with presumptive Tuberculosis (TB). OBJECTIVES: In this study, we aimed to assess the (1) yield and cost analysis of two strategies using mobile digital x-ray to detect Pulmonary TB in rural Haryana. METHODS: An observational study was conducted on all individuals screened by either of the two case finding strategies using a mobile x-ray unit (MXU) mounted on a mobile van in District Mewat, Haryana during Jan-March 2016. RESULTS: Strategy 1: Out of 121 smear negative cases, x-rays were suggestive of TB in 39(32%), of which 24 were started on TB treatment. Cost of identifying a smear negative TB was US$ 32. Strategy 2: Out of 596 presumptive TB, chest x-rays were suggestive of TB in 108 (18%), of which 67 were started on TB treatment (56 were smear negative TB). Cost of detecting any case of TB was US$ 08 (1 USD = 64 INR). CONCLUSION: The study reports a new initiative within a PPM model to improve the diagnosis of PTB by filling the gap in the current diagnostic infrastructure. We believe there is potential for replication of strategy 2 model in other states, although further evidence is required.

The clinical utility of cycle of threshold value of GeneXpert MTB/RIF (CBNAAT) and its diagnostic accuracy in pulmonary and extra-pulmonary samples at a tertiary care center in India.

BACKGROUND: There are knowledge gaps in the in-depth analysis of the most promising and robust diagnostic tool, GeneXpert MTB/RIF (CBNAAT). The cycle of threshold (CT) value of the CBNAAT test and its clinical implications has not been explored much. AIMS AND OBJECTIVES: The study aimed at (a) estimating the diagnostic accuracy and incremental yield of Xpert MTB/RIF in various specimens (b) establishing the association between CT value category (high, medium, low, very low) and culture time-to-positivity (TTP). METHODS: A total of 1000 samples, both pulmonary and extra-pulmonary were collected from presumptive TB cases in a large tertiary care hospital. Sensitivity and specificity of CBNAAT was calculated with culture as the gold standard. The association of CT value with culture TTP was also studied. RESULTS: The overall sensitivity of CBNAAT was 88.5%, with bronchial washing specimen being the most sensitive (92.3%) and pleural fluid being the least (66.7%). In smear negative individuals, the sensitivity of CBNAAT was 80.9%. The additional yield of CBNAAT over smear microscopy was 10.9%. It was observed that as we move from high to very low CT category, culture positivity decreases significantly (p<0.001), whereas time taken for culture growth increases (p<0.001). CONCLUSION: CBNAAT is a robust test for accurate diagnosis of tuberculosis both pulmonary and extra-pulmonary, smear negative as well, especially in resource-limited settings. The correlation between CT value and culture TTP has potential in predicting bacillary load, though further studies are required.

Efficacy of high zinc biofortified wheat in improvement of micronutrient status, and prevention of morbidity among preschool children and women - a double masked, randomized, controlled trial.

BACKGROUND: Biofortification of staple food crops with zinc (Zn) can be one of the cost-effective and sustainable strategies to combat zinc deficiency and prevent morbidity among the target population. Agronomic approaches such as application of Zn fertilizers to soil and/or foliar spray seem to be a practical tool for Zn biofortification of wheat. However, there is a need to evaluate its efficacy from randomized controlled trials. This study aimed to evaluate the efficacy of zinc biofortified wheat flour on zinc status and its impact on morbidity among children aged 4-6 years and non-pregnant non lactating woman of child bearing age (WCBA) in Delhi, India. METHODS: In a community based, double-masked randomized controlled trial, 6005 participants (WCBA and child pairs) were enrolled and randomly allocated to receive either high zinc biofortified wheat flour (HZn, 30 ppm zinc daily) or low zinc biofortified wheat flour (LZn, 20 ppm zinc daily) for 6 months (WCBA @ 360 g/day and children @ 120 g/day). Baseline and endline blood samples were obtained for assessing hematological markers; zinc status and data on compliance and morbidity were collected. RESULTS: Compliance rates were high; ~ 88% of the WCBAs in both the groups consumed 50% or more of recommended amount of biofortfied wheat flour during the follow up. Similarly 86.9% children in HZn and 87.5% in LZn consumed 50% or more of recommended wheat flour intake. There was no significant difference in mean zinc levels between the groups at end study. This observation might be due to a marginal difference in zinc content (10 ppm) between the HZn and LZn wheat flour, and a short intervention period. However a positive impact of bio-fortification on self-reported morbidity was observed. Compared to children in LZn group, children in HZn group had 17% (95% CI: 6 to 31%, p = 0.05) and 40% (95% CI: 16 to 57%; p = 0.0019) reduction in days with pneumonia and vomiting respectively. WCBA in the HZn group also showed a statistically significant 9% fewer days with fever compared to LZn group. CONCLUSIONS: Biofortified wheat flour had a good compliance among children and WCBAs. Significant improvement on some of the self-reported morbidity indicators suggests that evaluating longer-term effects of biofortification with higher grain zinc content would be more appropriate. TRIAL REGISTRATION: http://ctri.nic.in/Clinicaltrials/ , CTRI/2014/04/004527, Registered April 7, 2014.

Syringo-pleural shunt: a rare cause of recurrent pleural effusion.

case of a patient with reported spinal tuberculosis (TB) that developed syringomyelia, a rare sequelae of the disease is presented in this report. He underwent syringo-pleural shunting for syringomyelia. After 15 years, the patient presented with recurrent pleural effusion. Diagnostic thoracentesis revealed fluid of transudative nature. Pleural fluid was positive for beta-2 transferrin. Pleural biopsy was negative for any malignancy or TB. Computed tomography scan focusing on the shunt showed that it was undisplaced. Video-assisted thoracoscopic exploration showed the distal tip of the shunt adherent to the lung parenchyma. The effusion resolved after the dislodgement of distal tip from lung parenchyma.

Diabetic ketoacidosis following mumps.

A-13-year-old girl presented with diabetic ketoacidosis with convincing clinical signs of parotitis (fever, drooling of saliva, inability to swallow with development of bilateral parotid swelling) and pancreatitis (fever, abdominal pain and vomiting), along with high serum amylase and positive mumps IgM titer. This suggests that mumps virus may have been the causative factor, probably as a result of concomitant involvement of the pancreas.

Comparison of cardiac autonomic activity between malnourished and healthy children.

Severe malnutrition can significantly compromise autonomic nervous system. However, less is known about the cardiac autonomic activity in mild and moderate grades of malnutrition in children. Therefore, the objective of this study was to assess the effect of mild/moderate malnutrition on heart rate variability (HRV), a non invasive tool to estimate the cardiac autonomic activity. A cross sectional, community based study was conducted in which 35 malnourished children (mean age: 6.06 +/- 2.04 yrs), on the basis of anthropometric parameters, were enrolled in the study group by random samplings, who were the children of urban slum dwellers and 35 age and sex matched healthy children, were taken as controls. Grading of malnutrition was done according to Indian Academy of Pediatrics (IAP) classification. Anthropometry, basal heart rate (BHR), blood pressure were determined. Time domain and frequency domain indices of HRV were assessed using RMS Polyrite D (version 2.4). Weight, height, mid arm circumference (MAC) and body mass index (BMI) were found to be statistically lower in the study group. There was a strong negative correlation between MAC and LF component (P < 0.01). BHR was found to be increased in the malnourished group (P = 0.027). Low frequency (LFnu) & LF-HF ratio were found to be increased (P = 0.000 & P = 0.001 respectively) while high frequency (HFnu) component was decreased (P = 0.000) in malnourished group. Our results suggested that impaired cardiac autonomic nerve function characterized by sympathetic over activity may occur in malnourished children. This study also enables us to compare, in future works, HRV in pediatric subjects with different grades of malnutrition.

Correlation of anthropometric parameters with QTc interval in malnourished children.

Various studies have reported the effect of severe malnutrition on electrolyte levels and electrocardiographic parameters. However, only a few have reported these findings in mild and moderate grades of malnutrition in children. Therefore, the objective of this study was to assess the effect of malnutrition (mainly mild and moderate grades) on corrected QT interval (QTc) and QT dispersion (QTcd) and electrolyte changes. A total of 20 malnourished children in the age group of 2-11 yrs were enrolled in the study group and 20 age and sex matched healthy children were taken as controls. Anthropometry, serum levels of albumin & electrolytes were determined. QTc and QTcd (difference between maximum & minimum corrected QT interval) were measured with the help of RMS Polyrite D. Our results have shown that body weight, height, body mass index (BMI), serum levels of albumin, potassium & calcium were lower (P<0.01) in malnourished children. QTc (P<0.01) & QTcd (P<0.01) were significantly greater in malnourished children than controls. We concluded that increase in QTc and QTcd intervals is associated with electrolyte disturbances in malnourished children. Electrolyte disturbance correction and appropriate nutrition therapy followed by further cardiac evaluation must be taken into account in the management of these patients.