Augmented mannose-binding lectin levels in primary membranous nephropathy: A pilot study.

There is evidence to suggest that M-type phospholipase A2 (PLA2R) antibodies activate the mannose-binding lectin (MBL) cascade, resulting in glomerular damage and proteinuria in patients with primary membranous nephropathy (PMN). Furthermore, there are few reports indicating that aberrant MBL activation is associated with endothelial dysfunction and accelerated atherosclerosis. While PMN is a common cause of adult nephrotic syndrome, and patients are at increased risk of cardiovascular disease (CVD), there is a lack of research that explores the factors that contribute to this condition. This study aims to determine the MBL levels in PMN and their relation to the clinical activity and endothelial dysfunction in PMN. The MBL levels of 22 biopsy-confirmed PMN patients were assessed at baseline and after 6 months of immunosuppressive therapy. In order to evaluate endothelial dysfunction in PMN patients, flow-mediated vasodilation (FMD) was measured at baseline and after treatment. A total of 22 healthy controls were included in this study to measure MBL levels and FMD. A significant difference was observed between MBL levels in PMN patients and healthy controls (p < .01). MBL levels decreased significantly after immunosuppressive therapy (p = .04). The baseline MBL levels and FMD levels exhibited a strong correlation (Spearman correlation coefficient [ρ] = 0.51: p = .01). In conclusion, the study signals the activation of the MBL cascade and its association with endothelial dysfunction in PMN patients.

Autoimmune disorders of the gastrointestinal tract: Review of radiological appearances.

Autoimmune gastrointestinal (GI) disorders comprise a heterogeneous group of diseases with non-specific clinical manifestations. These are divided into primary and secondary. A high index of clinical suspicion complemented with endoscopic and radiological imaging may allow early diagnosis. Due to the relatively low incidence of autoimmune disorder, the imaging literature is sparse. In this review, we outline the pathogenesis, classification, and imaging appearances of autoimmune GI disorders.

Radiation dose analysis of computed tomography coronary angiography in Children with Kawasaki disease.

BACKGROUND: There is evolving role of computed tomography coronary angiography (CTCA) in non-invasive evaluation of coronary artery abnormalities in children with Kawasaki disease (KD). Despite this, there is lack of data on radiation dose in this group of children undergoing CTCA. AIM: To audit the radiation dose of CTCA in children with KD. METHODS: Study (December 2013-February 2018) was performed on dual source CT scanner using adaptive prospective electrocardiography-triggering. The dose length product (DLP in milligray-centimeters-mGy.cm) was recorded. Effective radiation dose (millisieverts-mSv) was calculated by applying appropriate age adjusted conversion factors as per recommendations of International Commission on Radiological Protection. Radiation dose was compared across the groups (0-1, 1-5, 5-10, and > 10 years). RESULTS: Eighty-five children (71 boys, 14 girls) with KD underwent CTCA. The median age was 5 years (range, 2 mo-11 years). Median DLP and effective dose was 21 mGy.cm, interquartile ranges (IQR) = 15 (13, 28) and 0.83 mSv, IQR = 0.33 (0.68, 1.01) respectively. Mean DLP increased significantly across the age groups. Mean effective dose in infants (0.63 mSv) was significantly lower than the other age groups (1-5 years 0.85 mSv, 5-10 years 1.04 mSv, and > 10 years 1.38 mSv) (P < 0.05). There was no significant difference in the effective dose between the other groups of children. All the CTCA studies were of diagnostic quality. No child required a repeat examination. CONCLUSION: CTCA is feasible with submillisievert radiation dose in most children with KD. Thus, CTCA has the potential to be an important adjunctive imaging modality in children with KD.

Multimodality imaging of myocardial tuberculosis presenting as right ventricular cardiomyopathy: A case report.

An 18-year male with no significant past medical history presented with features of right-sided heart failure. Haematological investigations revealed raised erythrocyte sedimentation rate (23 mm/h). Transthoracic echocardiography (TTE) demonstrated biventricular systolic dysfunction with thickened free wall of right ventricle (RV). Whole-body fluorodeoxyglucose (FDG)-positron emission tomography (PET) computed tomography (FDG-PET-CT) showed intensely increased FDG uptake in the thickened RV wall and an FDG avid lesion in the left medial temporal lobe. Cardiac magnetic resonance imaging (CMR) characterized thickening and suggested an infiltrative disease (isointense on TI, hyperintense on myocardial oedema sensitive sequence with transmural enhancement on late gadolinium sequence). Magnetic resonance imaging (MRI) brain showed a thick rim enhancing ring lesion in the body of hippocampus. Endomyocardial biopsy revealed moderately dense lymphomononuclear inflammation with the formation of granulomas. Since tuberculosis is endemic in our country and considering all laboratory, imaging and biopsy findings a working diagnosis of tuberculosis was considered, and standard anti-tubercular treatment was started. There was an improvement in clinical status and follow-up imaging (TTE, CMR, brain MRI and FDG-PET-CT) showed improvement in biventricular function with regression in myocardial thickening and resolution of brain lesion, confirming the diagnosis of tuberculosis.

Aortopulmonary window with anomalous ascending aortic origin of left pulmonary artery: Hitherto unreported pattern.

Anomalous ascending aortic origin of left pulmonary artery in association with aortopulmonary window has not been reported so far in literature and is the main highlight of the present case.

Congenitally corrected transposition with absent pulmonary valve: Hitherto unreported association.

Absent pulmonary valve has usually been described in association with tetralogy of Fallot. Present case highlights its association with congenitally corrected transposition which has not been reported so far in literature.

Aneurysmal right ventricular outflow in an adult patient with unrepaired Tetralogy of Fallot and an absent pulmonary valve.

BACKGROUND: Absent pulmonary valve (PV) is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot (TOF). Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. DISCUSSION: Here, we describe the case of a 37 year old gentleman who presented with a diagnosis of TOF with absent PV, hugely dilated right and left pulmonary arteries and an aneurysmal right ventricular outflow. CONCLUSION: TOF with absent PV surviving into adulthood is a rarity. An aneurysmal right ventricular outflow tract in an unrepaired case of TOF has not been described before. Massively dilated left and right pulmonary arteries as in this case can cause significant and even life-threatening airway compromise, thus necessitating early surgical repair.

Contrast enhanced ultrasound versus multiphasic contrast enhanced computed tomography in evaluation of gallbladder lesions.

AIM: To compare the diagnostic performance of contrast enhanced ultrasound (CEUS) and multiphasic contrast enhanced computed tomography (CECT) in differentiating benign and malignant gallbladder (GB) lesions. METHODS: This prospective ethical review board approved study comprised consecutive patients with GB lesions who underwent CEUS and multiphasic CECT at a tertiary care referral center. The enhancement patterns at CEUS and CT were compared. The quantitative CEUS parameters including arrival time (AT), AT in liver, time to peak enhancement, and washout time (WT) were assessed. The diagnostic performance of CEUS and CT features was calculated using receiver operating characteristic analysis. A subgroup analysis was performed for patients with GB wall thickening. Multivariate analysis was performed to identify features significantly associated with malignancy. RESULTS: Over the study period, 30 patients (mean age, 52.8 ± 12.2 years, 17 females) with GB lesions were evaluated. Benign and malignant diseases were present in 13 and 17 patients, respectively. There was excellent agreement between CEUS and CT findings. Among the quantitative CEUS features, only WT was significantly associated with malignancy in the overall group (p < 0.001) and wall thickening subgroup (p = 0.007). WT within 53.5 s and 51.5 s had sensitivity of 88.2% and 81.8% and specificity of 84.5% and 100% in diagnosing malignant lesions in the overall group (AUC 0.900) and the wall thickening subgroup (area under curve, AUC 0.927), respectively. At multivariate analysis, features that were significantly associated with malignant lesions in the overall group were disruption of GB wall (CEUS), intralesional non-enhancing areas (CEUS), liver involvement (CEUS or CT), and arterial phase hyperenhancement (CT) in the overall group and disruption of GB wall (CEUS), WT (CEUS), and liver involvement (CEUS or CT) in the wall thickening subgroup. CONCLUSION: CEUS is a useful adjunct to CT in evaluation of GB lesions. Its utilization in patients with GB wall thickening may improve detection of malignancy.

Pulmonary Artery Pseudoaneurysm in COVID-19-Associated Pulmonary Mucormycosis: Case Series and Systematic Review of the Literature.

Literature on COVID-19-associated pulmonary mucormycosis (CAPM) is sparse. Pulmonary artery pseudoaneurysm (PAP) is an uncommon complication of pulmonary mucormycosis (PM), and rarely reported in CAPM. Herein, we report five cases of CAPM with PAP managed at our center and perform a systematic review of the literature. We diagnosed PM in those with clinico-radiological suspicion and confirmed it by microbiology or histopathology. We encountered five cases of CAPM with PAP (size ranged from 1 × 0.8 cm to ~ 4.9 × 4.8 cm). All subjects had diabetes and were aged 55-62 years (75% men). In two cases, COVID-19 and mucormycosis were diagnosed simultaneously, while in three others, COVID-19 preceded PM. One subject who underwent surgery survived, while all others died (80% mortality). From our systematic review, we identified one additional case of CAPM with PAP in a transplant recipient. CAPM with PAP is rare with high mortality. Early diagnosis and multimodality management are imperative to improve outcomes.

Gallbladder reporting and data system (GB-RADS) for risk stratification of gallbladder wall thickening on ultrasonography: an international expert consensus.

The Gallbladder Reporting and Data System (GB-RADS) ultrasound (US) risk stratification is proposed to improve consistency in US interpretations, reporting, and assessment of risk of malignancy in gallbladder wall thickening in non-acute setting. It was developed based on a systematic review of the literature and the consensus of an international multidisciplinary committee comprising expert radiologists, gastroenterologists, gastrointestinal surgeons, surgical oncologists, medical oncologists, and pathologists using modified Delphi method. For risk stratification, the GB-RADS system recommends six categories (GB-RADS 0-5) of gallbladder wall thickening with gradually increasing risk of malignancy. GB-RADS is based on gallbladder wall features on US including symmetry and extent (focal vs. circumferential) of involvement, layered appearance, intramural features (including intramural cysts and echogenic foci), and interface with the liver. GB-RADS represents the first collaborative effort at risk stratifying the gallbladder wall thickening. This concept is in line with the other US-based risk stratification systems which have been shown to increase the accuracy of detection of malignant lesions and improve management.

Validation of the provisional seven-item criteria for the diagnosis of polyarteritis nodosa.

The 1990 American College of Rheumatology (ACR) criteria for the classification of polyarteritis nodosa (PAN) have many pitfalls and performed poorly when used for diagnostic purposes. Recently, a provisional seven-item diagnostic criteria for PAN was proposed. To validate the provisional seven-item diagnostic criteria for PAN in a cohort of PAN patients from a tertiary care centre in India. Clinical details of patients diagnosed as PAN as per the European Medicines Agency algorithm between 2005 and 2020 were collected retrospectively. Age and sex-matched anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) patients were included in the non-PAN group. Patients with a deficiency of adenosine deaminase 2 (DADA2) were included as a separate group. The sensitivity, specificity, positive and negative predictive values (PPV and NPV) for ACR criteria, the Ministry of Health, Labour and Welfare (MHLW) in Japan diagnostic criteria and the seven-item diagnostic criteria were calculated. Thirty-seven PAN, 14 DADA2 and 37 AAV patients were included in the analysis. The sensitivity, specificity, PPV and NPV of the seven-item criteria were 83.7%, 96.8%, 97.3% and 81.1% respectively. For the ACR criteria, sensitivity was 82.9% and specificity was 79.5%. The sensitivity, specificity for MHLW criteria were 77.3% and 90% respectively. The sensitivity and specificity of seven-item criteria for DADA2 patients were 58.8% and 88.2% respectively. There was very poor agreement between the ACR criteria and the seven-item and MHLW criteria and fair agreement between seven-item and MHLW criteria (κ = 0.279). The provisional seven-item criteria for PAN performed well with high specificity and PPV.

Deficiency of Adenosine Deaminase 2 in Adults and Children: Experience From India.

OBJECTIVE: Deficiency of adenosine deaminase 2 (DADA2) is a potentially fatal monogenic syndrome characterized by variable manifestations of systemic vasculitis, bone marrow failure, and immunodeficiency. Most cases are diagnosed by pediatric care providers, given the typical early age of disease onset. This study was undertaken to describe the clinical phenotypes and treatment response both in adults and in children with DADA2 in India. METHODS: A retrospective analysis of pediatric and adult patients with DADA2 diagnosed at various rheumatology centers across India was conducted. Clinical characteristics, diagnostic findings, and treatment responses were analyzed in all subjects. RESULTS: In total, 33 cases of DADA2 were confirmed in this cohort between April 2017 and March 2020. Unlike previous studies, nearly one-half of the confirmed cases presented during adulthood. All symptomatic patients exhibited features of vasculitis, whereas constitutional symptoms and anemia were more common in pediatric patients. Cutaneous and neurologic involvement were common, and 18 subjects had experienced at least one stroke. In addition, the clinical spectrum of DADA2 was expanded by recognition of novel features in these patients, including pancreatic infarction, focal myocarditis, and diffuse alveolar hemorrhage. Treatment with tumor necrosis factor inhibitors (TNFi) was initiated in 25 patients. All of the identified disease manifestations showed marked improvement after initiation of TNFi, and disease remission was achieved in 19 patients. Two cases were complicated by tuberculosis infection, and 2 deaths were reported. CONCLUSION: This report presents the first case series of patients with DADA2 from India, diagnosed by adult and pediatric care providers. The findings raise awareness of this syndrome, particularly with regard to its presentation in adults.

Percutaneous glue embolization as a primary treatment for visceral pseudoaneurysms.

Aim: To evaluate the feasibility and outcome of percutaneous glue embolisation as primary treatment for pseudoaneurysms in selective patients.Material and methods: A review of the pseudoaneurysm cases managed with percutaneous glue at our institute was analyzed in this study. A total of 21 patients over a period of last one year who were treated for pseudoaneurysm at varied sites primarily with percutaneous n-butyl cynoacrylate were retrospectively evaluated. Site of pseudoaneurysm was Pulmonary (seven cases), renal (four cases), pelvic (4four cases) and miscellaneous abdomen (six cases). Combined ultrasound and fluoroscopy approach was used in 17 cases, CT guidance in three cases and combined ultrasound and CT guidance in one case. Patients were evaluated for therapeutic response (radiological and clinical) and procedure related complications.Results: Complete occlusion of the pseudoaneurysm was seen in all patients. Two patients showed mild pneumothorax and one patient showed reflux into the intercostal artery. All the patients showed symptomatic improvement and discharged in stable condition.Conclusions: Percutaneous glue embolisation of the pseudoaneurysms as a primary treatment is feasible and can be safely carried out in select group of patients with good therapeutic response and minimal complications.

Comparison between computerized tomography-guided bronchial width measurement versus conventional method for selection of adequate double lumen tube size.

Background: Selection of adequate size double lumen tube (DLT) is complicated by marked inter-individual variability in morphology and dimensions of tracheobronchial tree. Computerized tomography (CT)-guided left bronchus width measurement has been used to predict adequate size DLT in European and Singapore population; however, no such data exist for Indian population who are racially different. We compared the effect of DLT size selection based on CT-guided bronchial width measurement to the conventional method of DLT selection on the adequacy of both lungs isolation and on the safety margin of right-sided DLT. Methods: Fifty-five adults scheduled to undergo thoracotomy were enrolled in this prospective observational study. An appropriate size left- or right-sided DLT with outer diameter 0.5-1 mm smaller than the CT-measured bronchial width was selected for the isolation of lungs. Adequacy of separation was checked using fiberoptic bronchoscope. The safety margin of selected right-sided DLT size was calculated from CT-measured right upper lobe bronchus width and diameter of right upper lobe ventilation slot of the DLT. Results: Adequate separation of lungs was achieved in 92.7% of studied population, 90.9% in males, and 95.4% in females. Among these, 54.9% patients required different sized DLT as compared to conventional method. Overall safety of margin of right-sided DLTs was comparable between two methods of DLT selection (median [IQR] 4.8 (3.5-6.8) vs. 6.59 (3.5-7.8), P = 0.317). DLT size with adequate isolation of lung correlated with height, tracheal width (TW) on chest X-ray, and age of the patients. A formula to calculate DLT size based on these variable was derived. Conclusion: CT-measured bronchial width predicts the appropriate DLT size better than conventional method. In the absence of CT scan facility, patient height, age, and chest X-ray TW may be used to predict DLT size with reasonable accuracy.

Cross-sectional Imaging of Gallbladder Carcinoma: An Update.

Gallbladder Carcinoma (GBCA) is the most common biliary tract malignancy. As the disease is often diagnosed clinically in an advanced stage, the survival rates are dismal. Imaging studies allow for an early diagnosis of malignancy, though the findings may be indistinguishable from non-malignant disease processes affecting the gallbladder. Attempts have been made to make a specific diagnosis of GBCA at an early stage on imaging studies. Ultrasonography (US) is the most commonly employed technique for gallbladder evaluation. Gallbladder wall thickening is the most common finding of early GBCA and in this context, US is non-specific. Recently, contrast enhanced ultrasound has been shown to be effective in differentiating benign from malignant disease. Multidetector computed tomography represents the most robust imaging technique in evaluation of GBCA. It provides relatively sensitive evaluation of mural thickening, though it is not entirely specific and issues in differentiating GBCA from xanthogranulomatous cholecystitis do arise. Due to its superior soft tissue resolution, Magnetic Resonance Imaging (MRI) provides excellent delineation of gallbladder and biliary tree involvement. When coupled with functional MRI techniques, such as diffusion-weighted and perfusion imaging, it provides a useful problem solving tool for interrogating the malignant potential of nonspecific gallbladder lesions and detection of metastases. Positron emission tomography has a role in detection of distant metastases and following patients following treatment for malignancy. We review the current role of various imaging modalities in evaluating patients with GBCA.