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Budd-Chiari syndrome (BCS), also known as hepatic venous outflow tract obstruction includes a group of conditions characterized by obstruction to the outflow of blood from the liver secondary to involvement of one or more hepatic veins (HVs), inferior vena cava (IVC) or the right atrium. There are a number of conditions that lead to BCS-ranging from hypercoagulable states to malignancies. In up to 25% patients, no underlying disorder is identified. Diagnosis of BCS is based on a combination of clinical and imaging features. A major part of the literature in BCS has been devoted to interventions; however, a detailed description of various imaging manifestations of BCS is lacking. In this review, we highlight the importance of various imaging modalities in the diagnosis of BCS.

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.

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SummaryBilateral adrenal lesions include a spectrum of disorders: neoplastic disorders (metastases, lymphoma, bilateral phaeochromocytoma, adrenocortical carcinoma and myelolipoma); longstanding congenital adrenal hyperplasia and macronodular adrenal hyperplasia; infections such as tuberculosis, histoplasmosis and blastomycosis; adrenal haemorrhage; adrenals in hypoperfusion complex and paediatric masses. Bilateral distribution in addition to other imaging characteristics can help narrow differential diagnoses in case of adrenal masses. We present a pictorial review highlighting the adrenal lesions that can present bilaterally.