Differential Regulation of Retinoic Acid Metabolism in Fanconi Anemia.

Fanconi anemia (FA) is a rare genetic disease characterized by heterogeneous congenital abnormalities and increased risk for bone marrow failure and cancer. FA is caused by mutation of any one of 23 genes, the protein products of which function primarily in the maintenance of genome stability. An important role for the FA proteins in the repair of DNA interstrand crosslinks (ICLs) has been established .

Lung Ultrasonography for the Diagnosis of SARS-CoV-2 Pneumonia in the Emergency Department.

Study Objective: Accurate diagnostic testing to identify severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is critical. Although highly specific, SARS-CoV-2 reverse transcriptase-polymerase chain reaction (RT-PCR) has been shown in clinical practice to be affected by a noninsignificant proportion of false-negative results. This study seeks to explore whether the integration of lung ultrasonography with clinical evaluation is associated with increased sensitivity for the diagnosis of coronavirus disease 2019 pneumonia, and therefore may facilitate the identification of false-negative SARS-CoV-2 RT-PCR results.

Long-term evolution of the electrical stimulation for cochlear implant adult patients. The role of a progressive adaptation method.

In clinical experience patients who do not frequently check the cochlear implant (CI) maps have poor auditory performance and poor acceptance of map variations over time. The aim of the present study was to evaluate the effect of CI map optimization on both speech perception and subjective hearing in a group of CI patients with a long-standing map. 14 patients (6 females and 8 males; age range 16-77, mean age 45.

Does nephrectomy during radical adrenalectomy for stage II adrenocortical cancer affect patient outcome?

Purpose: To evaluate if including nephrectomy in the standard surgical approach to stage II adrenocortical cancer (i.e., adrenalectomy) might modify oncologic outcome of patients.

18F-FDG PET/CT in the post-operative monitoring of patients with adrenocortical carcinoma.

Context: The role of (18)F-labeled 2-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET)/computed tomography (CT) in the post-operative monitoring of patients with adrenocortical carcinoma (ACC) is still unclear.

Objective: To assess the accuracy of FDG PET/CT to diagnose ACC recurrence in a real world setting.

Design And Methods: Retrospective evaluation of data of 57 patients with presumed ACC recurrence at CT scan who underwent FDG PET/CT within a median time of 20 days.

Major prognostic role of Ki67 in localized adrenocortical carcinoma after complete resection.

Background: Recurrence of adrenocortical carcinoma (ACC) even after complete (R0) resection occurs frequently.

Objective: The aim of this study was to identify markers with prognostic value for patients in this clinical setting.

Design, Setting, And Participants: From the German ACC registry, 319 patients with the European Network for the Study of Adrenal Tumors stage I-III were identified.

Peritonectomy and hyperthermic intraperitoneal chemotherapy: cost analysis and sustainability.

Background: Malignancies of the peritoneum remain a challenge in any hospital that accepts to manage them, due not only to difficulties associated with the complexity of the procedures involved but also the costs, which - in Italy and other countries that use a diagnosis-related group (DRG) system - are not adequately reimbursed.

Material And Methods: We analyzed data relative to 24 patients operated on between September 2010 and May 2013 with special regard to operating room expenditure, ICU stay, duration of hospitalization, and DRG reimbursement. The total costs per patient included clinical, operating room, procedure, pathology, imaging, ward care, allied healthcare, pharmaceutical, and ICU costs.

Surgical remission of Cushing's syndrome reduces cardiovascular risk.

Objective: Recent studies have questioned the reversibility of complications of Cushing's syndrome (CS) after successful surgical treatment. The aim of this study was to assess the outcome of patients with CS who achieved disease remission compared with those patients with persistent hypercortisolism and matched controls.

Design: A retrospective study of 75 patients with CS followed at an academic center.

Management of adrenal cancer: a 2013 update.

Adrenocortical carcinoma (ACC) is a devastating tumor for either patients or their families because of short life expectancy and severe impact on quality of life. Due to the rarity of ACC, with a reported annual incidence of 0.5-2 cases per million population, progress in the development of treatment options beyond surgery has been limited.

Prognostic role of overt hypercortisolism in completely operated patients with adrenocortical cancer.

Background: Although prognostic parameters are important to guide adjuvant treatment, very few have been identified in patients with completely resected adrenocortical carcinoma (ACC).

Objective: To assess the prognostic role of clinical symptoms of hypercortisolism in a large series of patients with completely resected ACC.

Design, Setting, And Participants: A total of 524 patients followed at referral centers for ACC in Europe and the United States entered the study.

Mitotane levels predict the outcome of patients with adrenocortical carcinoma treated adjuvantly following radical resection.

Context: Mitotane plasma concentrations ≥ 14 mg/l have been shown to predict tumor response and better survival in patients with advanced adrenocortical carcinoma (ACC). A correlation between mitotane concentrations and patient outcome has not been demonstrated in an adjuvant setting.

Objective: To compare recurrence-free survival (RFS) in patients who reached and maintained mitotane concentrations ≥ 1 4 mg/l vs patients who did not.

Influence of the CYP2B6 polymorphism on the pharmacokinetics of mitotane.

Objective: The aim of this study was to assess the potential impact of the pharmacogenetic variability of CYP2B6 and ABCB1 genes on the pharmacokinetics of mitotane.

Methods: A retrospective analysis was carried out on 27 patients with adrenocortical carcinoma on postoperative adjunctive mitotane. CYP2B6 and ABCB1 polymorphisms were genotyped and tested for an association with plasma trough concentration after 3, 6, 9, and 12 months of therapy.

A prospective evaluation of short-term and long-term results from colonic stenting for palliation or as a bridge to elective operation versus immediate surgery for large-bowel obstruction.

Background: The efficacy and safety of self-expandable metallic stent (SEMS) placement as a bridge to elective surgery or definitive palliation versus emergency operation to treat colorectal obstruction is debated. This study aimed to evaluate the outcomes of patients with colorectal obstruction treated using different strategies.

Methods: Subjects admitted to the authors' department with colorectal obstruction (n = 134) were studied prospectively.

Management of adjuvant mitotane therapy following resection of adrenal cancer.

Whenever adrenal cancer (ACC) is completely removed we should face the dilemma to treat by means of adjuvant therapy or not. In our opinion, adjuvant mitotane is the preferable approach in most cases because the majority of patients following radical removal of an ACC have an elevated risk of recurrence. A better understanding of factors that influence prognosis and response to treatment will help in stratifying patients according to their probability of benefiting from adjuvant mitotane, with the aim of sparing unnecessary toxicity to patients who are likely unresponsive.

Metronomic chemotherapy may be active in heavily pre-treated patients with metastatic adreno-cortical carcinoma.

Objective: The potential benefit of further chemotherapy approaches in patients with adrenocortical carcinoma (ACC) showing progressive disease after 2 chemotherapy lines is actually unknown. This study provides explorative information on the activity of metronomic chemotherapy in heavily pre-treated ACC patients.

Design And Methods: We tested the activity of cytotoxic treatments administered on a metronomic schedule in metastatic ACC patients showing disease progression after treatment with gemcitabine and capecitabine scheme.

Mitotane treatment for adrenocortical carcinoma: an overview.

Adrenocortical carcinoma (ACC) is a rare aggressive endocrine neoplasm characterized by a 5-year survival of less than 50%. Due to the widespread use of imaging techniques in clinics, ACC is increasingly recognized as an incidentally discovered tumor. Mostly characterized by poor prognosis, ACC is often diagnosed at an advanced stage of disease.

Cortisol secretion, bone health, and bone loss: a cross-sectional and prospective study in normal non-osteoporotic women in the early postmenopausal period.

Objective: The aim of the study was to evaluate the relationship between cortisol secretion, bone health, and bone loss in a cohort of normal women in the early postmenopausal period.

Methods: We measured lumbar and hip bone mineral density (BMD) by dual-energy X-ray absorptiometry (DXA) and heel ultrasound parameters in 82 healthy, nonosteoporotic (lumbar T-score ≥-2.0) women (median age 52.

Phase II study of weekly paclitaxel and sorafenib as second/third-line therapy in patients with adrenocortical carcinoma.

Background: There is a strong rationale in the use of antiangiogenic therapy in the management of adrenocortical carcinoma (ACC). Metronomic administration of chemotherapy and antiangiogenic drugs can be synergistic in targeting endothelial cells.

Objective: We assessed the activity of sorafenib plus metronomic paclitaxel as second/third-line therapy in advanced ACC patients.

Metronomic therapy concepts in the management of adrenocortical carcinoma.

Metronomic chemotherapy is the administration of cytotoxic drugs at low doses, on a frequent or continuous schedule, with no extended interruption. This treatment approach can target tumor cells indirectly since it can affect the endothelium of the growing tumor vasculature and stimulates the anticancer immune response. Both the antiangiogenetic and the immunomodulatory roles of metronomic chemotherapy favor a tumor dormancy, a condition that may improve the patient outcome.

Percutaneous transhepatic biliary drainage and occlusion balloon in the management of duodenal stump fistula.

Background: Duodenal stump fistula (DSF) after gastrectomy is a complication with a high mortality rate. We report a series of patients with postoperative DSF treated with percutaneous transhepatic biliary drainage and occlusion balloon (PTBD-OB). The aim of this study is to explore the feasibility and efficacy of PTBD-OB in the treatment of DSF.

Pros and cons of dexamethasone suppression test for screening of subclinical Cushing's syndrome in patients with adrenal incidentalomas.

The results of dexamethasone suppression tests (DST) in the screening of subclinical hypercortisolism are not readily comparable. Aim of the present study was to review the effectiveness of overnight 1-mg DST and 8-mg DST to look for functional autonomy of clinically inapparent adrenal adenomas. Sixty-eight consecutive patients with clinically inapparent adrenal adenomas were enrolled.

Coexistence in competition models with density-dependent mortality.

We consider a two-competitor/one-prey model in which both competitors exhibit a general functional response and one of the competitors exhibits a density-dependent mortality rate. It is shown that the two competitors can coexist upon the single prey. As an example, we consider a two-competitor/one-prey model with a Holling II functional response.

[Duodenal perforation post-ERCP: diagnostically and therapeutic management].

Objective: Author's experience with periduodenal perforation after ERCP and there systematic approach is presented.

Methods: A retrospective study of 6 instances of duodenal perforation related to endoscopic retrograde cholangiopancreatography. The study follows these parameters: type of perforations, clinical presentation, diagnostic methods, time to diagnosis, methods of management, surgical procedures, length of stay, mortality and morbidity.

A comparison of active compression-decompression cardiopulmonary resuscitation with standard cardiopulmonary resuscitation for cardiac arrests occurring in the hospital.

Background: Recent studies have demonstrated improved cardiopulmonary circulation during cardiac arrest with the use of a hand-held suction device (Ambu CardioPump) to perform active compression-decompression cardiopulmonary resuscitation (CPR). The purpose of this study was to compare active compression-decompression with standard CPR during cardiac arrests in hospitalized patients.

Methods: All patients over the age of 18 years who had a witnessed cardiac arrest while hospitalized at our center were enrolled in this trial; they were randomly assigned according to their medical-record numbers to receive either active compression-decompression or standard CPR.