Prospective diagnostic test accuracy comparison of computed tomography during arterial portography and Primovist magnetic resonance imaging in the pre-operative assessment of colorectal cancer liver metastases.

OBJECTIVES: To assess and compare the accuracy and inter-observer agreement for the detection of liver lesions using Primovist magnetic resonance imaging (pMRI) and computed tomography during arterial portography (CTAP). METHODS: Patients evaluated at St George Hospital Liver Unit for colorectal liver metastases (CRCLM) underwent CTAP as part of standard staging. pMRI was added to the pre-operative assessment. Two radiologists reported CTAP and two reported pMRI. The sensitivity and specificity of CTAP and pMRI were calculated using histopathology as the gold standard. RESULTS: Complete data were available for 62 patients corresponding to 219 lesions confirmed on histopathology. Agreement on the detection of lesions between the two radiologists that reported pMRI was higher than for CTAP (Kappa = 0.80 versus 0.74). Specificity of lesion detection for pMRI was 0.88 and 0.83 for CTAP (P = 0.112). Sensitivity for pMRI was 0.83 and 0.81 for CTAP. For patients who had chemotherapy before evaluation, pMRI had a significantly higher specificity than CTAP (0.79 versus 0.63, P = 0.011). CONCLUSIONS: pMRI is less invasive, has a good inter-observer agreement, has comparable sensitivity and specificity to CTAP in the pre-chemotherapy population and demonstrates better specificity in patients assessed post-chemotherapy. pMRI is a valid alternative to CTAP in the assessment of CRCLM.

Crystalglobulinemia in Multiple Myeloma: A Rare Case Report of Survival and Renal Recovery.

RATIONALE: Crystalglobulinemia is a rare complication of monoclonal gammopathy wherein crystallized immunoglobulins deposit in various organs causing occlusive vasculopathy, endothelial damage, and thrombosis. It should be differentiated from light chain cast nephropathy without crystalline nephropathy through timely diagnosis with a kidney biopsy. PRESENTING CONCERNS OF THE PATIENT: We report a case of a 74-year-old female with polyarthralgia, chest pain, petechial rash, and acute kidney injury. DIAGNOSES: Kidney biopsy revealed eosinophilic casts in the tubular lumen and similar occlusive crystalline deposits within the glomerular vasculature and interlobular arteries. Bone marrow biopsy and serum electrophoresis confirmed immunoglobulin G (IgG) κ multiple myeloma. INTERVENTIONS: Dialysis was initiated for severe oligoanuric acute kidney injury. The patient was treated with 5 sessions of plasmapheresis and 11 cycles of clone reduction chemotherapy with CyBorD (cyclophosphamide, bortezomib, and dexamethasone). OUTCOMES: This patient achieved excellent kidney recovery and is no longer dialysis dependent. TEACHING POINTS: Crystalglobulinemia should be suspected in patients with rapidly progressive acute kidney injury and monoclonal gammopathy. Timely investigation with kidney biopsy to differentiate this condition from light chain cast nephropathy and initiation of appropriate treatment can lead to remission of disease and excellent recovery of kidney function.

JUSTIFICATION: La cristalglobulémie, une complication rare de la gammapathie monoclonale, se caractérise par la présence de dépôts d'immunoglobulines cristallisées dans différents organes, ce qui entraîne une vasculopathie occlusive, des lésions endothéliales et une thrombose. Un diagnostic précoce par biopsie rénale doit être posé pour différencier la cristalglobulinémie de la néphropathie en chaînes légères sans néphropathie cristalline. PRÉSENTATION DU CAS: Nous présentons le cas d'une patiente de 74 ans souffrant de polyarthralgie, de douleurs thoraciques, d'une éruption pétéchiale et d'insuffisance rénale aiguë. DIAGNOSTIC: La biopsie rénale a révélé la présence de cylindres éosinophiles dans la lumière tubulaire et de dépôts cristallins occlusifs similaires dans le système vasculaire des glomérules et dans les artères interlobulaires. La biopsie de la moelle osseuse et l'électrophorèse sérique ont confirmé la présence d'un myélome multiple à immunoglobulines G (IgG) κ. INTERVENTIONS: L'insuffisance rénale aiguë oligoanurique grave a nécessité l'amorce d'une dialyse. Le traitement a consisté en cinq séances de plasmaphérèse et onze 11 cycles de chimiothérapie de réduction des clones avec CyBorD (cyclophosphamide, bortézomib et dexaméthasone). RÉSULTATS: Le rétablissement rénal est excellent et la patiente n'est plus sous dialyse. LEÇONS TIRÉES: Une cristalglobulinémie doit être suspectée chez les patients qui présentent une gammapathie monoclonale et une insuffisance rénale aigüe rapidement progressive. Le diagnostic rapide par biopsie rénale pour différencier cette affection d'une néphropathie à chaînes légères et l'initiation précoce du traitement approprié peut mener à une rémission et à une excellente récupération de la fonction rénale.

Multifocal cutaneous mucormycosis complicating polymicrobial wound infections in a tsunami survivor from Sri Lanka.

A man injured in the tsunami of Dec 26, 2004, returned to Sydney for management of his soft-tissue injuries. Despite broad-spectrum antibiotics, surgical wound debridement, and vigilant wound care, his condition worsened. Muscle and fat necrosis developed in a previously debrided thigh wound, and necrotising lesions arose from previous abrasions. Histological analysis showed mucormycosis in three non-contiguous sites, and Apophysomyces elegans was isolated from excised wound tissue. Wound infections, both bacterial and fungal, will undoubtedly add to the morbidity and mortality already recorded in tsunami-affected areas. Other cases [correction] of cutaneous mucormycosis might develop in survivors, but this disease can be difficult to diagnose and even harder to treat, particularly in those remaining in affected regions.

Deletion of the antiphospholipid syndrome autoantigen ß2 -glycoprotein I potentiates the lupus autoimmune phenotype in a Toll-like receptor 7-mediated murine model.

OBJECTIVE: The BXSB.Yaa mouse strain is a model of systemic lupus erythematosus that is dependent on duplication of the Toll-like receptor 7 gene. The objective of this study was to systematically describe the amplified autoimmune phenotype observed when the soluble plasma protein ß2 -glycoprotein I (ß2 GPI) gene was deleted in male BXSB.Yaa mice. METHODS: We generated BXSB.Yaa and NZW mouse strains in which the ß2 GPI gene had been knocked out by backcrossing the wild-type strains with C57BL/6 ß2 GPI(-/-) mice for 10 generations. Sex- and age-matched mice of the various strains were housed under identical conditions and were killed at fixed time intervals. Serum and tissue specimens were collected at various time points. Lupus-associated autoantibodies, inflammatory cytokines, and the type I interferon (IFN) gene signature were measured. Flow cytometric analyses of lymphocyte populations were performed. The severity of glomerulonephritis was graded by 2 independent renal histopathologists. RESULTS: Male BXSB.Yaa ß2 GPI(-/-) mice developed significant lymphadenopathy and splenomegaly compared with age-matched controls. Male BXSB.Yaa ß2 GPI(-/-) mice also had significantly higher levels of autoantibodies, increased levels of inflammatory cytokines including tumor necrosis factor α, interleukin-6, and BAFF, and more severe glomerulonephritis. The type I IFN gene signature in male BXSB.Yaa ß2 GPI(-/-) mice was significantly higher than that in control mice. Male BXSB.Yaa ß2 GPI(-/-) mice also had marked dysregulation of various B cell and T cell populations in the spleens and lymph nodes and a disturbance in apoptotic cell clearance. CONCLUSION: Deletion of ß2 GPI accelerates and potentiates the autoimmune phenotype in male BXSB.Yaa mice.