Recurrence Pattern of Pulmonary Langerhans Cell Histiocytosis After Lung Transplantation: A Case Report.

Pulmonary Langerhans cell histiocytosis is an uncommon indication for lung transplantation. The recurrence of the primary pathology after transplantation is rare. Extra-pulmonary involvement of Langerhans cell histiocytosis before transplantation and resuming of smoking after transplantation are risk factors for recurrence. Herein, we present a 48-year-old woman with a recurrence pattern 24 months after bilateral sequential lung transplantation. On thoracic computed tomography, progressive parenchyma infiltrates were present without clinical symptoms. Infiltrations regressed and respiratory status of the patient was improved with steroid therapy. No events had occurred at follow-up of 31 months.

A case of acute fibrinous and organizing pneumonia during early postoperative period after lung transplantation.

Acute fibrinous and organizing pneumonia (AFOP) is a distinct histologic pattern usually classified under the term chronic lung allograft dysfunction. We present a 48-year-old female patient who experienced AFOP during the 2nd week of double lung transplantation for pulmonary Langerhans cell histiocytosis and secondary pulmonary hypertension. During the 8th day after transplantation, fever and neutrophilia developed together with bilateral consolidation. Infection markers were elevated. Despite coverage of a full antimicrobial spectrum, the situation progressed. The patient was diagnosed with AFOP with transbronchial biopsy. The infiltration resolved and the patient improved dramatically with the initiation of pulse corticosteroid treatment. AFOP should be suspected when there is a pulmonary consolidation after lung transplantation, even in the very early post-transplantation period. Several causes, such as alveolar damage and drug reactions, should be considered in the differential diagnosis.

Absence of the right iliac vein and an unusual connection between both common femoral veins.

The aim of the study is to report a case of a rare congenital anomaly of the venous system and to emphasize its clinical importance. We describe a case of aplasia of the right common and external iliac veins in a healthy seven-year-old boy who was referred for Doppler ultrasound examination for further evaluation of an abnormal varicosity in the suprapubic region. Colour Doppler ultrasound revealed a dilated, arch-shaped vein. Contrast-enhanced magnetic resonance angiography showed the absence of the right common iliac vein and external iliac vein. It also clearly demonstrated the aberrant venous structure, originating from the right common femoral vein and draining to the left common femoral vein. In conclusion, in our case, the patient's life was threatened because the aberrant venous connection crossing within subcutaneous fatty tissue was not protected from external trauma and possible abdominal surgical interventions. Additional attention should be given to avoid such injuries, which can cause significant haemorrhage.

Prevalence of hepatic pseudolesions around the falciform ligament in a paediatric population.

This study investigated the prevalence and features of hepatic pseudolesions around the falciform ligament in 320 children undergoing contrast-enhanced abdominal multidetector computed tomography. Aberrant venous drainage was investigated using thin-section computed tomography images. Pseudolesions around the falciform ligament were detected in 63 patients (20%). The longest diameter was in the craniocaudal direction in 41 (65%) patients (lesions). An inferior vein of Sappey supplying the pseudolesions was seen in 11 patients (17%). Thirteen patients of the 63 patients (21%) with pseudolesions who had a history of extrahepatic malignancy were also examined by ultrasound; in two of the 13 patients the lesions were isoechoic and, in the remainding 11 patients, they were hyperechoic. It was concluded that hepatic pseudolesions around the falciform ligament occur frequently in the paediatric population on CT images. Detection of the longest diameter in the craniocaudal direction and the presence of an inferior vein of Sappey and fatty infiltration should be sufficient to exclude true lesions and make further evaluations unnecessary.

Predictive value of Doppler ultrasound in childhood pneumonia.

This study aimed to determine the predictive value of intercostal and pulmonary artery Doppler flow patterns in the outcome of childhood pneumonia. Pneumonia was classified according to type of pleural effusion and the ultrasound features of consolidations. Doppler flow patterns of intercostal and pulmonary arteries were analysed and correlated with pneumonia type and hospital stay. Of 83 pneumonia cases, 55 were uncomplicated and 28 were complicated. Pleural effusion was present in 54 cases, with 29 non-septated and 25 septated cases. Patients with uncomplicated pneumonia did not have abnormal Doppler flow patterns, compared with 64% (18 of 28) of patients with complicated pneumonia. Doppler ultrasound patterns in childhood pneumonia were correlated with pneumonia type and may be predictive of pneumonia outcome.

Current management of esophageal perforation: 20 years experience.

Esophageal perforations are surgical emergencies associated with high morbidity and mortality rates. No single strategy has been sufficient to deal with the majority of situations. We aim to postulate a therapeutic algorithm for this complication based on 20 years of experience and also on data from published literature. We performed a retrospective clinical review of 44 patients treated for esophageal perforation at our hospital between January 1989 and May 2008. We reviewed the characteristics of these patients, including age, gender, accompanying diseases, etiology of perforation, diagnosis, location, time interval between perforation and diagnosis, treatment of the perforation, morbidity, hospital mortality, and duration of hospitalization. Perforation occurred in the cervical esophagus in 14 patients (31.8%), thoracic esophagus in 18 patients (40.9%), and abdominal esophagus in 12 patients (27.3%). Management of the esophageal perforation included primary closure in 23 patients (52.3%), resection in 7 patients (15.9%), and nonsurgical therapy in 14 patients (31.8%). In the surgically treated group, the mortality rate was 3 of 30 patients (10%), and 2 of 14 patients (14.3%) in the conservatively managed group. Four of the 14 nonsurgical patients were inserted with covered self-expandable stents. The specific treatment of an esophageal perforation should be selected according to each individual patient. To date, the most effective treatment would appear to be operative management. With improvements in endoscopic procedures, the morbidity and mortality rates of esophageal perforations are significantly decreased. We suggest that minimally invasive techniques for the repair of esophageal perforations will be very important in the future treatment of this condition.

Extralobar pulmonary sequestration mimicking neuroblastoma.

Extralobar pulmonary sequestration is a congenital pulmonary malformation, which rarely may be present in an intra-abdominal location. We describe a 1-week-old newborn with an intra-abdominal sequestration, which presented to us as an antenatally diagnosed suprarenal mass. Intra-abdominal extralobar sequestration should also be kept in differential diagnosis in cases of masses in the suprarenal location.

Technetium-99m 2-methoxy-isobutyl-isonitrile uptake scintigraphy in detection of the bone marrow infiltration in multiple myeloma: correlation with MRI and other prognostic factors.

Whole-body scintigraphy with Technetium-99m 2-methoxy-isobutyl-isonitrile ((99m)Tc-MIBI) has been proposed as a useful method for demonstrating the areas of active bone marrow infiltration in multiple myeloma (MM). In this study, we compared the (99m)Tc-MIBI scan with magnetic resonance imaging (MRI), skeletal X-ray survey, and biochemical markers of disease activity in MM to determine its potential in predicting the extension of the disease. Twenty-four myeloma patients had undergone to the (99m)Tc-MIBI scan. Only two patients showed negative results in the (99m)Tc-MIBI scan; one had clinically active disease, and the other was on remission. MRI was performed to 18 clinically active patients, and 16 of them showed positive myelomatous bone marrow involvement. No significant difference was found between the (99m)Tc-MIBI scan and MRI in predicting the extension of bone marrow infiltration in MM (p = 0.11). (99m)Tc-MIBI scores were correlated with bone marrow neoplastic plasma cell ratio (p = 0.005), serum paraprotein level (p < 0.001), serum lactate dehydrogenase (p = 0.031), and beta-2 microglobulin (p = 0.045). The (99m)Tc-MIBI scan showed disease activity better than the skeletal X-ray survey (x2 = 5.299, p = 0.021). A significant decrease was found in posttreatment (99m)Tc-MIBI scores of the patients with positive overall response (p = 0.016). The (99m)Tc-MIBI scan is a noninvasive test that can show the extension of the disease in MM. It seems that the (99m)Tc-MIBI scan and MRI show extension and intensity of the myelomatous bone marrow infiltration equally well. The (99m)Tc-MIBI scan can be an alternative to MRI when it is not available or if there is any limitations for its usage.

Bladder tumors: virtual MR cystoscopy.

Virtual cystoscopy is a promising new technique based on computer-simulated rendering of the inner surface of the urinary bladder using volumetric magnetic resonance (MR) imaging data, thus enabling maneuvers that normally are not possible with conventional cystoscopy. Due to several distinct advantages over conventional cystoscopy such as minimal invasiveness, evaluation of the urethral orifice from a cranial point of view and an opportunity to observe diverticula formations and the inner urethral space, gadolinium-enhanced MR cystoscopy has a great potential for competing with conventional cystoscopy under some clinical circumstances. The recent improvement in MR scanners has significantly facilitated virtual cystoscopic evaluation of the urinary bladder lumen by MR imaging. Volumetric data associated with powerful postprocessing procedures allow imaging of the inner urinary bladder surface with excellent detail. In this article, imaging techniques and clinical applications of gadolinium-enhanced virtual MR cystoscopy are presented.

Successful medical treatment of abdominal aortic aneurysms in a patient with Behcet's disease: imaging findings.

Arterial manifestations of Behcet's disease consist of aneurysm formation, stenosis and occlusion. Aneurysms in Behcet's disease most commonly involve the pulmonary arteries and have been shown to resolve with medical treatment. However, this regression pattern with medical therapy has not been reported for aortic aneurysms to date. We present a 43-year-old man with bilateral abdominal aortic aneurysms resulting from Behcet's disease resolving with medical therapy.

Genetic polymorphisms and endothelial dysfunction in patients with essential hypertension: a cross-sectional case-control study.

BACKGROUND: Both in animal models and humans an association between endothelial constitutive nitric oxide synthase (ecNOS) gene polymorphism and the development of hypertension has been found. However, the relation between ecNOS polymorphism and endothelial function in patients with hypertension has not been systematically studied. Genes of the renin-angiotensin system include the angiotensin-converting enzyme (ACE) gene, and the angiotensin II type 1 receptor (ATIR) gene, and have been associated with essential hypertension. However, no consistent data are available about the relation between polymorphisms of these genes and the presence of endothelial dysfunction in such patients. OBJECTIVES: To assess the presence of genetic polymorphisms and of endothelial dysfunction in patients with essential hypertension. To determine the effects of gene polymorphisms on endothelial dysfunction in these subjects. METHODS: In 129 patients with essential hypertension and the same number of age-matched controls polymorphisms of the ecNOS gene, ACE gene, and AT1R gene were analysed by polymerase chain reactions. Endothelial function was assessed by maximal endothelial dependent vasodilation in response to reactive hyperaemia using high resolution ultrasound examinations of the brachial arteries. To assess correlation between genetic markers, endothelial function, and the presence of hypertension both univariate and multivariate analyses were used including Pearson's and Spearman's correlation coefficients, and multiple logistic regressions. RESULTS: The size of endothelium-dependent vasodilation between patients and controls differed by 16% (p<0.02). However, the presence of genetic polymorphisms of the ecNOS, ACE, and AT1R genes did not significantly differ between patients and controls. Neither were there any statistically significant differences in endothelial function between various genotypes of the three genes. This was so for both the patients and the controls, although in all of these comparisons the controls overall displayed a slightly better endothelial function than the patients did. Multiple regression analysis with endothelial dysfunction as dependent and the presence of gene polymorphisms as independent variables did not reveal any significant correlation either. CONCLUSION: A significant relation between endothelial dysfunction and essential hypertension was demonstrated. However, no relations between genetic markers and the presence of essential hypertension or between endothelial dysfunction and genetic markers were established. The failure of our study to demonstrate the latter may be due to confounders. Also, other genes may be more important in the pathogenesis of endothelial dysfunction and essential hypertension. The current study underscores that endothelial dysfunction and hypertension are not simple genetic disorders, and that they are, essentially, multicausal.

Does the endothelial function change in renal transplant patients with longer duration of exposure and with higher cumulative doses of cyclosporine?

OBJECTIVE: Administration of cyclosporine (CsA) is one potential cause of endothelial dysfunction in renal transplant patients. We sought to investigate endothelial functional changes with respect to the cumulative dose and duration of exposure to CsA. METHODS: Sixty-six renal recipients and 25 healthy controls were included in the study. The recipients were classified according to their time of CsA exposure: group 1 (0 to 36 months); group 2 (36 to 72 months); and group 3 (over 72 months). Endothelial function of the brachial artery was evaluated using high-resolution vascular ultrasound. Endothelium-dependent and -independent vasodilatation (EDD and EID, respectively) were assessed by assessing the responses to reactive hyperemia and using sublingual isosorbide dinitrate (ISDN), respectively. RESULTS: There were no statistically significant differences between the groups with regard to their demographic, clinical, and most biochemical characteristics. Baseline measurements of the diameter of the brachial artery were similar in all groups. The values of mean brachial artery EDD and EID responses in groups 1, 2, and 3 were less than those in the control group (P < .05, P < .05, and P < .05, respectively). Mean brachial artery EDD and EID in group 1 were significantly impaired compared to groups 2 and 3 (for EDD: P < .05 and P < .05, respectively; for EID: P < .05 and P < .05, respectively). In contrast there was no difference between groups 2 and 3 with respect to these parameters. There were mild to moderate positive correlations between the cumulative doses of CsA and EDD and EID (r = .26 and r = .52, P < .05, respectively). CONCLUSION: Endothelial dysfunction was more prominent in the first 36-month period than later despite the longer exposure to and higher cumulative doses of CsA. This finding may reflect an extended effect of the uremic state on endothelial function or more intense doses of CsA in early posttransplant period.

Acute torsion of a wandering spleen: imaging findings.

Wandering spleen is a rare entity characterized by incomplete fixation of the spleen by lienorenal and gastrosplenic ligaments. It can migrate to the lower abdomen or pelvis and can be congenital or acquired. We report a case of torsion of a wandering spleen for which there was correlative imaging by ultrasonography, Doppler ultrasonography, computed tomography, magnetic resonance imaging, and angiography. To our knowledge, this is the first reported case in which all these modalities were used in the diagnostic evaluation.

US versus conventional radiography in the diagnosis of sternal fractures.

PURPOSE: To investigate the value of ultrasonography (US) in the diagnosis of sternal fractures. MATERIAL AND METHODS: Twenty-three patients (mean age 35.4 years) with a clinical suspicion of sternal fracture after blunt chest trauma were retrospectively reviewed. At admission, a.p. and lateral chest radiographies and sternal US were obtained. Sternal fractures were classified as nondisplaced or displaced. US and conventional radiographic findings were compared. RESULTS: In 3/23 (13.0%) of the patients, no fracture was found by radiography or by US. Both radiography and US demonstrated sternal fractures in 16/23 (69.6%) of the patients. Sternal fractures were detected only by US while the conventional radiography was negative in 2/23 (8.7%) cases. Also in 2/23 (8.7%) of the patients with US positive for fracture, radiographies were suspicious. In 2 patients, the degree of fracture displacement on US was lesser than that found by radiography. CONCLUSION: US was better than lateral radiography to diagnose sternal fractures; however, conventional radiography remains the standard means of demonstrating grade of displacement.