Association between age at disease onset of anti-neutrophil cytoplasmic antibody-associated vasculitis and clinical presentation and short-term outcomes

Objectives: ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- A nd older-onset patients are still incompletely understood. Methods: We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: <65 years or ≥65 years. We adjusted associations for the type of AAV and the type of ANCA (anti-MPO, anti-PR3 or negative). Results: A total of 1338 patients with AAV were included: 66% had disease onset at <65 years of age [female 50%; mean age 48.4 years (s.d. 12.6)] and 34% had disease onset at ≥65 years [female 54%; mean age 73.6 years (s.d. 6)]. ANCA (MPO) positivity was more frequent in the older group (48% vs 27%; P = 0.001). Younger patients had higher rates of musculoskeletal, cutaneous and ENT manifestations compared with older patients. Systemic, neurologic,cardiovascular involvement and worsening renal function were more frequent in the older-onset group. Damage accrual, measured with the Vasculitis Damage Index (VDI), was significantly higher in older patients, 12% of whom had a 6 month VDI ≥5, compared with 7% of younger patients (P = 0.01). Older age was an independent risk factor for early death within 6 months from diagnosis [hazard ratio 2.06 (95% CI 1.07, 3.97); P = 0.03]. Conclusion: Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients

Kawasaki disease presenting as acute intestinal obstruction

Kawasaki disease (KD) is an acute febrile illness of childhood associated with vasculitis of medium-sized arteries especially the coronary arteries. Typical clinical features involving the skin, mucous surfaces, etc., occur sequentially over a few days. We report a rare presentation of KD as a surgical abdomen in a 2-year-old boy. Awareness of this presentation is important as it can otherwise lead to a delay in starting potentially life-saving intervention like intravenous immunoglobulins for cardiac complications kept cryptic by the manifest acute abdomen

Oxygenative and Dehydrogenative [3 + 3] Benzannulation Reactions of α,β-Unsaturated Aldehydes and γ‑Phosphonyl Crotonates Mediated by Air: Regioselective Synthesis of 4‑Hydroxybiaryl-2-carboxylates

Regioselective synthesis of 4-hydroxybiphenyl-2-carboxylates via the base-mediated oxygenative [3 + 3] benzannulation reaction of α,β-unsaturated aldehydes and γ-phosphonyl crotonates is reported. A hydroxyl group is installed in the final product on the originally phosphorus-bound carbon via a novel oxygenative and dehydrogenative transformation. The reaction proceeds rapidly in an open flask, uses atmospheric oxygen as an oxidant, and affords good yields of substituted biaryl phenols

Oxygenative and Dehydrogenative [3 + 3] Benzannulation Reactions of α,β-Unsaturated Aldehydes and γ‑Phosphonyl Crotonates Mediated by Air: Regioselective Synthesis of 4‑Hydroxybiaryl-2-carboxylates

Regioselective synthesis of 4-hydroxybiphenyl-2-carboxylates via the base-mediated oxygenative [3 + 3] benzannulation reaction of α,β-unsaturated aldehydes and γ-phosphonyl crotonates is reported. A hydroxyl group is installed in the final product on the originally phosphorus-bound carbon via a novel oxygenative and dehydrogenative transformation. The reaction proceeds rapidly in an open flask, uses atmospheric oxygen as an oxidant, and affords good yields of substituted biaryl phenols

Evaluation of HRCT chest features in COVID 19 patients with pre-existing comorbidity and clinical correlation

Background: High resolution CT scan of chest has a major role in diagnosis and prognosis of COVID 19 patients. This study attempts to assess the pattern and severityof theCT chest manifestations in COVID patients with pre-existing comorbidity as compared to those without comorbidity. Methods:This is a retrospective study. HRCT findings of 572 COVID19 pneumonia patients in the period for 5 months were analysed. And the medical record of the same patients was searched for the presence of any pre-existing comorbidity.The CT severity score and the CT pattern of the two groups of patients (those with comorbidity and those without comorbidity) were compared to find out the severity of HRCT chest manifestation in patients with comorbidity.Results: Among them 513 patients were male. The age group in the studied population range between 2-87 years with mean age 38.75 years. Out of 572 patients 22.5% (n- 129) patients had pre-existing comorbidity.Out of 129 patients in the comorbidity group majority (66.6%) showedpositive findings in high resolution CT of chest, but out of 443 patients in the non-comorbidgroup majority (46.8%) had negativechest findings. Severe CT severity score(>15/25) was found in 33.3% of patients with comorbidity while in 2.5% of cases without comorbidity. There was a strong correlation between the clinical and CT severity score.Conclusion: Larger percentage of Covid patients with comorbidity showed positive chest findings in high resolution CT of chest. And the severe (>15/25) CT severity scorewas seen in increased proportion of Covid patients with comorbidity as compared to those without comorbidity

Unusual Cause and Association of Gastrointestinal Bleed in a Young Boy

A 4-year old child, previously operated case of tetralogy of Fallot present with recurrent episodes of massive lower gastrointestinal bleed of one year duration. Endoscopic evaluation revealed multiple bluish vascular lesions in the duodenum and proximal jejunum and a single rectal polyp. Histology of the lesion was suggestive of venous malformation consistent with blue rubber bleb nevus syndrome (BRBNS). Child underwent endoscopic snaring and surgical resection with end to end anastomosis. At six months’ follow-up child was asymptomatic without any bleed episodes

Update on the Pathology of Pediatric Liver Tumors: A Pictorial Review

Liver tumors in children are uncommon and show remarkable morphologic heterogeneity. Pediatric tumors may arise from either the epithelial or mesenchymal component of the liver and rarely may also show both lines of differentiation. Both benign and malignant liver tumors have been reported in children. The most common pediatric liver tumors by age are benign hepatic infantile hemangiomas in neonates and infants, malignant hepatoblastoma in infants and toddlers, and malignant hepatocellular carcinoma in teenagers. Here, we provide an up-to-date review of pediatric liver tumors. We discuss the clinical presentation, imaging findings, pathology, and relevant molecular features that can help in the correct identification of these tumors, which is important in managing these children