A case of Maffucci's syndrome in an 18-month old boy is presented, in which the vascular malformation consists of lymphangioma. This is a rare variant of Maffucci's syndrome. So far only six other cases have been reported in which lymphangiomas featured. We believe that the case described here is only the second case to be reported for which a lymphangioma is the only vascular malformation in the syndrome. The literature of the other six cases was reviewed and various features identified enabling this particular disease to be classified as a distinct variant of Maffucci's syndrome.

Maffucci lymphangioma syndrome: an unusual variant of Ollier's disease, a case report and a review of the literature.

Journal of Pediatric Orthopedics. Part B

to detect the role of procalcitonin, erythrocyte sedimentation rate to c-reactive protein (ESR/CRP) ratio, neutrophils-to-lymphocyte ratio (NLR), and platelets-to-lymphocyte ratio (PLR) in the diagnosis of infection in systemic lupus erythematosus (SLE) patients with fever, their diagnostic value to differentiate between infection and disease activity, and their correlation with disease activity.

Forty SLE patients and forty healthy control cases were included in the study. Disease activity was assessed by the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2&#xa0;K), and quality of life was assessed by Lupus QoL. A bacterial infection was detected by clinical symptoms and positive culture results. Laboratory tests were done for all patients and controls: complete blood count (CBC), ESR, CRP, and procalcitonin (PCT). NLR, PLR, and ESR/CRP ratios were calculated.

There was a statistically significant difference between infected SLE patients and non-infected SLE patients regarding PCT (p&#x2009;&lt;&#x2009;0.001), ESR (p&#x2009;=&#x2009;0.002), CRP (p&#x2009;=&#x2009;0.005), ESR/CRP ratio (0.002), and NLR (p&#x2009;=&#x2009;0.023). PCT, ESR, CRP, and NLR were positively correlated with the presence of infection in SLE patients, while the ESR/CRP ratio was negatively correlated. There was no significant correlation with the SLEDAI-2&#xa0;K score. Logistic regression analysis revealed that PCT was the best significant predictor of infection (OR 224.37, 95% CI 8.94-5631.35). PCT was a good predictor of infection, with a cut-off value of 0.90 ng/ml, which gave the best combination of sensitivity (84.62%) and specificity (85.71%).

PCT, ESR/CRP ratio, and NLR provide good diagnostic markers for the diagnosis of infection and can distinguish between infection and disease flare in SLE patients with fever.

Infection versus disease activity in systemic lupus erythematosus patients with fever.

BMC rheumatology.

IgA nephropathy (IgAN) is considered the most common primary glomerulonephritis worldwide with a predilection for Asian-Pacific populations and relative rarity in those of African descent. Perhaps 20%-50% of patients progress to kidney failure. The pathogenesis is incompletely understood. Mesangial deposition of immune complexes containing galactose-deficient IgA1 complexed with anti-glycan IgG or IgA antibodies results in mesangial cell activation and proliferation, inflammatory cell recruitment, complement activation, and podocyte damage. Diagnosis requires a biopsy interpreted by the Oxford criteria. Additional pathologic features include podocytopathy, thrombotic microangiopathy, and C4d staining. Biomarkers predicting adverse outcomes include proteinuria, reduced GFR, hypertension, and pathology. Acceptable surrogate endpoints for therapeutic trials include ongoing proteinuria and rate of eGFR decline. The significance of persisting hematuria remains uncertain. The mainstay of therapy is supportive, consisting of lifestyle modifications, renin-angiotensin inhibition (if hypertensive or proteinuric), sodium-glucose-transporter 2 inhibition (if GFR reduced or proteinuric), and endothelin-receptor antagonism (if proteinuric). Immunosuppression should be considered for those at high risk after maximal supportive care. Corticosteroids are controversial with the most positive results observed in Chinese. They carry a high risk of serious side effects. Similarly, mycophenolate may be most effective in Chinese. Other immunosuppressants are of uncertain benefit. Tonsillectomy appears efficacious in Japanese. Active areas of investigation include B-cell inhibition with agents targeting the survival factors BAFF and APRIL and complement inhibition with agents targeting the alternate pathway (Factors B and D), the lectin pathway (MASP-2), and the common pathway (C3 and C5). Hopefully soon, the who and the how of immunosuppression will be clarified, and kidney failure can be forestalled.

Contemporary review of IgA nephropathy.

Frontiers in Immunology

Aspiration under anesthesia: what happens after we sound the glucagon-like peptide-1 receptor agonist alarm?

Canadian Journal of Anaesthesia

Direct oral anticoagulants (DOACs), comprising apixaban, rivaroxaban, edoxaban, and dabigatran, are commonly used medications to treat patients with atrial fibrillation and venous thromboembolism. Decisions about how to manage DOACs in patients undergoing a surgical or nonsurgical procedure are important to decrease the risks of bleeding and thromboembolism.

For elective surgical or nonsurgical procedures, a standardized approach to perioperative DOAC management involves classifying the risk of procedure-related bleeding as minimal (eg, minor dental or skin procedures), low to moderate (eg, cholecystectomy, inguinal hernia repair), or high risk (eg, major cancer or joint replacement procedures). For patients undergoing minimal bleeding risk procedures, DOACs may be continued, or if there is concern about excessive bleeding, DOACs may be discontinued on the day of the procedure. Patients undergoing a low to moderate bleeding risk procedure should typically discontinue DOACs 1 day before the operation and restart DOACs 1 day after. Patients undergoing a high bleeding risk procedure should stop DOACs 2 days prior to the operation and restart DOACs 2 days after. With this perioperative DOAC management strategy, rates of thromboembolism (0.2%-0.4%) and major bleeding (1%-2%) are low and delays or cancellations of surgical and nonsurgical procedures are infrequent. Patients taking DOACs who need emergent (&lt;6 hours after presentation) or urgent surgical procedures (6-24 hours after presentation) experience bleeding rates up to 23% and thromboembolism as high as 11%. Laboratory testing to measure preoperative DOAC levels may be useful to determine whether patients should receive a DOAC reversal agent (eg, prothrombin complex concentrates, idarucizumab, or andexanet-&#x3b1;) prior to an emergent or urgent procedure.

When patients who are taking a DOAC require an elective surgical or nonsurgical procedure, standardized management protocols can be applied that do not require testing DOAC levels or heparin bridging. When patients taking a DOAC require an emergent, urgent, or semiurgent surgical procedure, anticoagulant reversal agents may be appropriate when DOAC levels are elevated or not available.

Perioperative Management of Patients Taking Direct Oral Anticoagulants: A Review.

JAMA

Chronic kidney disease (CKD) is common in patients with heart failure (HF) and is associated with high morbidity and mortality. There has been remarkable progress in the treatment of HF over recent years with the establishment of guideline-directed medical therapies including: (1) Beta-blockers, (2) renal angiotensin aldosterone system (RAAS) inhibition (i.e., angiotensin-converting enzyme inhibitor [ACEi], aldosterone receptor blocker [ARB] or angiotensin receptor-neprilysin inhibitor [ARNI]); (3) mineralocorticoid receptor antagonists (MRA), and (4) sodium-glucose cotransporter-2 inhibitors (SGLT2i). However, there are challenges to the implementation of these medications in patients with concomitant CKD due to increased vulnerability to common side-effects (including worsening renal function, hyperkalaemia, hypotension), and most of the pivotal trials which provide evidence of the efficacy of these medications excluded patients with severe CKD. Patients with CKD and HF often have regular healthcare encounters with multiple professionals and can receive conflicting guidance regarding their medication. Thus, despite being at higher risk of adverse cardiovascular events, patients who have both HF and CKD are more likely to be under-optimised on evidence-based therapies. This review is an updated summary of the evidence available for the management of HF (including reduced, mildly reduced and preserved left ventricular ejection fraction) in patients with various stages of CKD. The review covers the evidence for recommended medications, devices such as implantable cardioverter-defibrillator (ICD), cardiac resynchronization therapy (CRT), intravenous (IV) iron, and discusses how frailty affects the management of these patients. It also considers emerging evidence for the prevention of HF in the cohort of patients with CKD. It synthesises the available evidence regarding when to temporarily stop, continue or rechallenge medications in this cohort. Chronic HF in context of CKD remains a challenging scenario for clinicians to manage, which is usually complicated by frailty, multimorbidity and polypharmacy. Treatment should be tailored to a patients individual needs and management in specialised cardio-renal clinics with a multi-disciplinary team approach has been recommended. This review offers a concise summary on this expansive topic.

Maffucci lymphangioma syndrome: an unusual variant of Ollier's disease, a case report and a review of the literature.

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Maffucci lymphangioma syndrome: an unusual variant of Ollier's disease, a case report and a review of the literature.

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