Y. Golowa

Bone density changes with enzyme therapy for Gaucher disease

Gaucher disease is the most common lysosomal storage disease. Enzyme replacement therapy engenders improvement in hematological and visceral parameters; however, improvement in bone density (BMD) with treatment has not been confirmed. This study presents follow-up of BMD in the first ten patients in Israel treated with low-dose recombinant enzyme for up to 108 months. BMD at femoral neck and lumbar spine was determined by dual-energy X-ray absorptiometry (DEXA) at the start of the trial, after 3–6 months, after 18–24 months, and at the most recent follow-up. BMD in all patients was very low at onset and never normalized. There was a decrease in BMD in all patients at 3–6 months. Older patients (four women, two men; >30 years of age) showed some improvement in BMD during treatment. Younger patients (four females; 18–23 years of age) did not show a statistically significant improvement. These findings might reflect the failure of patients with Gaucher disease to achieve expected peak bone density at appropriate chronological milestones despite treatment. Nonetheless, the z-scores of the older patients were better than those of the younger patients, implying some catch-up period. Yet, some patients with Gaucher disease evince rapid onset of osteoporosis in early adulthood. Enzyme treatment per se, as well as attendant improved well-being and increased physical activity, may induce amelioration in BMD at this later stage. One may consider adding anti-osteoporosis therapy in young adults to induce earlier “catch up” to peak bone mass, and then enzyme replacement in later adulthood to prevent decrements in bone mass related to Gaucher cell infiltration.

Value of Noncontrast CT Immediately after Transarterial Chemoembolization of Hepatocellular Carcinoma with Drug-eluting Beads

PURPOSE To retrospectively evaluate the presence and distribution patterns of contrast agent retention in the liver on noncontrast computed tomography (CT) immediately following chemoembolization with drug-eluting beads (DEBs). MATERIALS AND METHODS From 2008 to 2010, 95 patients with 224 liver lesions had chemoembolization performed with DEBs and a noncontrast CT examination of the liver performed immediately after embolization. Of these, 85 patients with 193 lesions were included. The postembolization CT scan was reviewed by a diagnostic radiologist, and the presence of contrast agent retention within the lesion was assessed. Varying patterns of contrast agent retention were defined. RESULTS Of the 193 lesions included, 146 (76%) retained contrast medium. Aside from some contrast medium in vessels, very little if any contrast medium was seen in the surrounding liver. Various patterns of contrast agent retention were noted within lesions. In a single case, repeat imaging was obtained 6 hours later, which demonstrated washout of contrast agent in a lesion that had retained contrast agent on the postprocedure CT scan. Of significance, 13 additional foci of contrast agent retention were identified on postchemoembolization CT scans that, on retrospective review of preprocedure imaging, represented enhancing lesions not previously identified. CONCLUSIONS Noncontrast CT after chemoembolization with DEBs demonstrates contrast agent retention in 76% of cases, without significant contrast medium seen in the adjacent liver parenchyma. The presence or absence of contrast agent retention may prove to be useful in evaluating accurate targeting of a lesion.

Image-guided percutaneous ablation of lung malignancies: A minimally invasive alternative for nonsurgical patients or unresectable tumors.

Lung cancer remains the malignancy with the highest mortality and second highest incidence in both men and women within the United States. Image-guided ablative therapies are safe and effective for localized control of unresectable liver, renal, bone, and lung tumors. Local ablative therapies have been shown to slow disease progression and prolong disease-free survival in patients who are not surgical candidates, either due to local extent of disease or medical comorbidities. Commonly encountered complications of percutaneous ablation of lung tumors include pneumothorax, pleural inflammation, pleural effusions, and pneumonia, which are usually easily managed. This review will discuss the merits of image-guided ablation in the treatment of lung tumors and the underlying mechanism, procedural techniques, clinical utility, toxicity, imaging of tumor response, and future developments, with a focus on radiofrequency ablation.

Transfemoral Transcaval Core-Needle Liver Biopsy: An Alternative to Transjugular Liver Biopsy.

PURPOSE To describe the technique and outcome of transfemoral transcaval (TFTC) core-needle liver biopsies. MATERIALS AND METHODS Retrospective chart review was performed on 121 patients who underwent transvenous liver biopsies at a single institution between February 2014 and July 2015, yielding 66 total TFTC liver biopsies for review (65.2% male; mean age, 53.2 y ± 15.0). From August 2014 through July 2015, TFTC biopsies accounted for 64 of 77 (83%) transvenous biopsies. Hepatic tissue was obtained directly through the intrahepatic inferior vena cava from a femoral venous approach. Procedural complications were classified according to Society of Interventional Radiology guidelines. RESULTS Of the 66 biopsies, technical success was achieved in 64 cases (97.0%). Histopathologic diagnoses were made in 63 cases (95.5%). Fragmented or limited specimens in which a pathologic diagnosis was still made occurred in four cases (6.1%). Complications occurred in two cases (3.0%). Venous pressure measurements were requested in 60 cases, and all were successfully obtained. CONCLUSIONS TFTC core-needle liver biopsies are feasible and safe as demonstrated in this series of patients.

Catheter directed interventions for inferior vena cava thrombosis

Inferior vena cava (IVC) thrombosis, although similar in many aspects to deep venous thrombosis (DVT), has distinct clinical implications, treatments and roles for endovascular management. Etiologies of IVC thrombosis vary from congenital malformations of the IVC to acquired, where indwelling IVC filters have been implicated as a leading cause. With an increasing incidence of IVC thrombosis throughout the United States, clinicians need to be educated on the clinical signs and diagnostic tools available to aid in the diagnosis as well as available treatment options. Untreated IVC thrombus can result in serious morbidity and mortality, both in the acute phase with symptoms related to venous outflow occlusion and embolism, and in the long-term, sequelae of post-thrombotic syndrome (PTS) related to chronic venous occlusion. This manuscript will discuss the clinical presentation of IVC thrombosis, diagnostic and treatment options, as well as the role of endovascular management.

Portal hypertensive vaginal bleeding

A 44-year-old woman with hepatitis C cirrhosis presented with a week of heavy vaginal bleeding. Her obstetric history was significant for three cesarean sections. Her gynecologist made an initial diagnosis of menometrorrhagia exacerbated by thrombocytopenia and coagulopathy. Computed tomography (CT) angiography revealed splenic vein thrombosis and engorged pelvic veins which arose as collaterals from the splenic vein (Fig. 1). Hysteroscopy could not identify a culprit lesion due to the rapidity of bleeding. A transjugular intrahepatic portosystemic shunt (TIPS) was created and thrombectomy of the splenic vein was performed and the residual partially occlusive thrombus was then stented. Hepatopedal flow was then noted from splenic vein to portal vein and through the TIPS. Hysteroscopy showed persistently engorged varices. Venous embolization of the varices was performed with a combination of embolization coils and a vascular plug (Fig. 2). Recovery was uneventful, and she was followed for 2 years in our clinic without further vaginal bleeding.