Iclal Ocak

Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)

ARPKD/CHF is an inherited disease characterized by non-obstructive fusiform dilatation of the renal collecting ducts leading to enlarged spongiform kidneys and ductal plate malformation of the liver resulting in congenital hepatic fibrosis. ARPKD/CHF has a broad spectrum of clinical presentations involving the kidney and liver. Imaging plays an important role in the diagnosis and follow-up of ARPKD/CHF. Combined use of conventional and high-resolution US with MR cholangiography in ARPKD/CHF patients allows detailed definition of the extent of kidney and hepatobiliary manifestations without requiring ionizing radiation and contrast agents.

The HLA Class II Allele DRB1*1501 Is Over-Represented in Patients with Idiopathic Pulmonary Fibrosis

Background Idiopathic pulmonary fibrosis (IPF) is a progressive and medically refractory lung disease with a grim prognosis. Although the etiology of IPF remains perplexing, abnormal adaptive immune responses are evident in many afflicted patients. We hypothesized that perturbations of human leukocyte antigen (HLA) allele frequencies, which are often seen among patients with immunologic diseases, may also be present in IPF patients. Methods/Principal Findings HLA alleles were determined in subpopulations of IPF and normal subjects using molecular typing methods. HLA-DRB1*15 was over-represented in a discovery cohort of 79 Caucasian IPF subjects who had lung transplantations at the University of Pittsburgh (36.7%) compared to normal reference populations. These findings were prospectively replicated in a validation cohort of 196 additional IPF subjects from four other U.S. medical centers that included both ambulatory patients and lung transplantation recipients. High-resolution typing was used to further define specific HLA-DRB1*15 alleles. DRB1*1501 prevalence in IPF subjects was similar among the 143 ambulatory patients and 132 transplant recipients (31.5% and 34.8%, respectively, p = 0.55). The aggregate prevalence of DRB1*1501 in IPF patients was significantly greater than among 285 healthy controls (33.1% vs. 20.0%, respectively, OR 2.0; 95%CI 1.3–2.9, p = 0.0004). IPF patients with DRB1*1501 (n = 91) tended to have decreased diffusing capacities for carbon monoxide (DLCO) compared to the 184 disease subjects who lacked this allele (37.8±1.7% vs. 42.8±1.4%, p = 0.036). Conclusions/Significance DRB1*1501 is more prevalent among IPF patients than normal subjects, and may be associated with greater impairment of gas exchange. These data are novel evidence that immunogenetic processes can play a role in the susceptibility to and/or manifestations of IPF. Findings here of a disease association at the HLA-DR locus have broad pathogenic implications, illustrate a specific chromosomal area for incremental, targeted genomic study, and may identify a distinct clinical phenotype among patients with this enigmatic, morbid lung disease.

The aortic root: Comparison of measurements from ECG-gated CT angiography with transthoracic echocardiography

Purpose The purpose of this study was to compare the measurements of the aortic root obtained from electrocardiographically (ECG)-gated computed tomography (CT) angiography (CTA) to the measurements obtained from transthoracic echocardiography (TTE). Materials and Methods This was a retrospective study in a patient population scanned at our institution between December 2005 and January 2007 with retrospectively ECG-gated CTA. ECG-gated CTA was performed with a 64-section helical CT scanner (Light speed, VCT, GE, Milwaukee, WI). Sixty-eight patients; 51 men and 17 women were included in this study. Aortic root diameters were measured by using double oblique reconstruction from axial source images. The TTE measurements of the aortic root were obtained from the reports that were performed within 2 months of CTA. Results The average aortic root diameter measured by TTE was 33±4.1 mm; on CTA it was 36.9±3.8 mm. The median difference between the 2 measurements was 3.9 mm which was significant (P<0.0001). In patients whose aortic root measurements with CTA were normal, the TTE measurements were also normal. However, in the group of patients with dilated aortic roots by CTA, TTE measurements were significantly lower and many were normal. In the group of patients with dilated aortic root by TTE, the CTA measurements of the aortic root were similarly increased. Conclusions Retrospective comparison of TTE and CTA measurements of the aortic root reveal that TTE measurements are substantially lower or even normal in patients found to have dilated aortic root by CTA.

Granulomatosis with Polyangiitis Presenting with Coronary Artery and Pericardial Involvement

Granulomatosis with polyangiitis is a systemic disease resulting in necrotizing vasculitis of small- and medium-sized vessels. Cardiac involvement is rare and when present usually manifests with pericarditis and coronary artery vasculitis. We report here a case of granulomatosis with polyangiitis involving the native coronary arteries, bypass graft, and pericardium with interesting imaging findings on contrast-enhanced CT and MRI. A 57-year-old man with a history of chronic headaches presented to the emergency room with syncope. Contrast-enhanced CT demonstrated extensive soft tissue attenuation around the native coronary arteries and bypass graft. Contrast-enhanced MRI demonstrated enhancing nodular soft tissue surrounding the coronary arteries, bypass graft, and pericardium. Pericardial biopsy revealed a necrotizing granulomatous pericarditis with vasculitis concerning for granulomatosis with polyangiitis. The patient demonstrated MPO-positive and PR-3 negative serologies. After being discharged on rituximab and prednisone, follow-up CT 3 years later showed significant improvement of the soft tissue thickening surrounding the coronary arteries, bypass graft, and pericardium.

Prevalence of aortic root dilation in patients with CT angiography of the aorta.

PURPOSE To investigate the prevalence of aortic root dilation in patients who underwent CT angiography of the thoracic aorta. MATERIALS AND METHODS In 95 patients, 64-slice multislice computed tomography was performed for evaluation of the thoracic aorta. Measurements of the annulus, sinuses of valsalva (SOV), sinotubular junction (STJ), and maximum ascending aorta (AAo) were made by double oblique multiplanar reformation (MPR). For the AAo, STJ, and SOV, dilation was defined as greater than 40 mm; for annulus, the dilation criterion was greater than 27 mm. RESULTS Overall, 52 patients were diagnosed with a dilated AAo. Of those patients with dilated AAo, 28 patients had a dilated annulus, 27 patients had dilated SOV, and 11 patients had STJ dilation. Forty-three patients presented with normal AAo; 12 patients had annulus dilation; 12 patients had SOV dilation; and 4 patients had STJ dilation. In patients with dilated AAo, 38% also had a dilated annulus, 52% showed SOV dilation, and 21% presented with STJ dilation, compared to 28% annulus dilation, 28% SOV dilation, and 9% STJ dilation in patients with an AAo of normal caliber. CONCLUSION Our data indicate a higher prevalence of aortic root dilation among patients with dilated AAo.

Imaging findings of lipomatosis: a comprehensive review.

Lipomatosis is a benign and non-encapsulated mature form of fat tissue proliferation, which can be isolated or multiple, symmetrical or infiltrative, and may be associated with several syndromes. Lipomatosis has been reported to involve almost every part and organ in the body. In this review, we present imaging findings at common locations of lipomatosis and summarize lipomatosis-related syndromes.

Delayed recurrence of ulcerative colitis manifested by tracheobronchitis, bronchiolitis, and bronchiolectasis

Ulcerative colitis can cause inflammation of small and large airways, characterized by mucosal inflammation, tracheobronchial stenosis, bronchiestasis, and bronchiolitis. We present a case of tracheobronchitis and bronchiolitis associated with ulcerative colitis in a 58-year-old nonsmoking man, 17 years after the total colectomy and complete resolution of intestinal findings. Computed tomography demonstrated wall thickening of trachea and left main stem bronchus, and multiple bronchi around the both hilum with mild to moderate stenosis. Fiberoptic bronchial biopsy showed inflammation of the airways, similar to histologic findings of ulcerative colitis within colon.

Talcoma: A Diagnostic Challenge in Differential Diagnosis of Pleural Masses

Talcoma is a pleural mass which may develop as a rare complication following talc pleurodesis. Talc pleurodesis is performed to obliterate the pleural space to prevent recurrent pleural effusions or persistent pneumothoraces. The present report describes a case of a patient who developed enlarging pleural mass (talcoma) following talc pleurodesis.