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Rachmilewitz E.A.,Wolfson Medical Center | Giardina P.J.,New York Presbyterian Hospital

The purpose of this article is to set forth our approach to diagnosing and managing the thalassemias, including β-thalassemia intermedia and β-thalassemia major. The article begins by briefly describing recent advances in our understanding of the pathophysiology of thalassemia. In the discussion on diagnosing the condition, we cover the development of improved diagnostic tools, including the use of very small fetal DNA samples to detectsingle point mutations with great reliability for prenatal diagnosis of homozygous thalassemia. In our description of treatment strategies, we focus on how we deal with clinical manifestations and long-term complications using the most effective current treatment methods for β-thalassemia. The discussion of disease management focuses on our use of transfusion therapy and the newly developed oral iron chelators, deferiprone and deferasirox.We also deal with splenectomy and how we manage endocrinopathies and cardiac complications. In addition, we describe our use of hematopoietic stem cell transplantation, which has produced cure rates as high as 97%, and the use of cord blood transplantation. Finally, we briefly touch on therapies that might be effective in the near future, including new fetal hemoglobin inducers and gene therapy. © 2011 by The American Society of Hematology. Source

Kernberg O.,New York Presbyterian Hospital
International Journal of Psychoanalysis

The main proposal of this paper is that normal mourning is not completed after six months to a year or two as suggested in earlier literature, but may bring about a permanent alteration of psychological structures that affect various aspects of the mourning persons' lives. These structural consequences of mourning consist in the setting up of a persistent internalized object relationship with the lost object that affects ego and superego functions. The persistent internalized object relationship develops in parallel to the identification with the lost object, and the superego modification includes the internalization of the value systems and life project of the lost object. A new dimension of spiritual orientation, the search for transcendental value systems, is one consequence of this superego modification. © 2010 Institute of Psychoanalysis. Source

Halmi K.A.,New York Presbyterian Hospital
BMC Psychiatry

Background: Treatment resistance is an omnipresent frustration in eating disorders. Attempts to identify the features of this resistance and subsequently develop novel treatments have had modest effects. This selective review examines treatment resistant features expressed in core eating disorder psychopathology, comorbidities and biological features. Novel treatments addressing resistance are discussed.Description: The core eating disorder psychopathology of anorexia nervosa becomes a coping mechanism likely via vulnerable neurobiological features and conditioned learning to deal with life events. Thus it is reinforcing and ego syntonic resulting in resistance to treatment. The severity of core features such as preoccupations with body image, weight, eating and exercising predicts greater resistance to treatment. Bulimia nervosa patients are less resistant to treatment with treatment failure related to greater body image concerns, impulsivity, depression, severe diet restriction and poor social adjustment. For those with binge eating disorder overweight in childhood and high emotional eating predicts treatment resistance. There is suggestive data that a diagnosis of an anxiety disorder and severe perfectionism may confer treatment resistance in anorexia nervosa and substance use disorders or personality disorders with impulse control problems may produce resistance to treatment in bulimia nervosa. Traits such as perfectionism, cognitive inflexibility and negative affect with likely genetic influences may also affect treatment resistance. Pharmacotherapy and novel therapies have been developed to address treatment resistance. Atypical antipsychotic drugs have shown some effect in treatment resistant anorexia nervosa and topiramate and high doses of SSRIs are helpful for treatment of resistant binge eating disorder patients. There are insufficient randomized controlled trials to evaluate the novel psychotherapies which are primarily based on the core psychopathological features of the eating disorders.Conclusion: Treatment resistance in eating disorders is usually predicted by the severity of the core eating disorder psychopathology which develops from an interaction between environmental risk factors with genetic traits and a vulnerable neurobiology. Future investigations of the biological features and neurocircuitry of the core eating disorders psychopathology and behaviors may provide information for more successful treatment interventions. © 2013 Halmi; licensee BioMed Central Ltd. Source

Rotman S.R.,New York Presbyterian Hospital | Bishop T.F.,New York Medical College

Background and Aims: Anecdotal reports and studies of select populations suggest that the use of proton pump inhibitors (PPIs) has increased since their introduction. We sought to determine recent trends in PPI use in the U.S. outpatient setting and characteristics of patients and physicians that may predict their use. Methods: We used data from the National Ambulatory Medical Care Survey (NAMCS) and the National Hospital Ambulatory Medical Care Survey (NHAMCS) to estimate the prevalence of visits in which patients used PPIs from 2002 to 2009. We tested for associations between PPI use and patient, physician, and practice characteristics using data from 2009. We also estimated the prevalence of visits in which PPIs were used by patients without gastrointestinal complaints, diagnoses, or other indications for their use and tested for associations between patient and physician characteristics and PPI use in patients with no documented indication. Results: PPIs were used in 4.0% of visits in 2002 and 9.2% in 2009 (p<0.001 for trend across years). The use of omeprazole (0.9% in 2002 to 3.9% in 2009, p<0.001), esomeprazole (0.9% in 2002 to 2.3% in 2009, p<0.001), and pantoprazole (0.6% in 2002 to 1.6% in 2009, p<0.001) increased significantly over the study period. Among visits by patients using PPIs, 62.9% documented no gastrointestinal complaints, gastrointestinal diagnoses, or other indicated reason for their use. Conclusions: We found that PPI use increased significantly from 2002 to 2009 as did documented indications for their use. Newly-prescribed PPI use did not change from 2006 to 2009. More research is needed to determine whether PPIs are overused in the U.S. outpatient setting. © 2013 Rotman, Bishop. Source

Stessin A.M.,Tri Institutional MD PhD Program | Sherr D.L.,New York Presbyterian Hospital
Cancer Epidemiology Biomarkers and Prevention

Background: Several reports showed incomplete adoption of adjuvant radiotherapy (RT) for resectable gastric cancer since the publication of Intergroup 0116 trial results. The aims of this study were to identify demographic factors associated with omission of adjuvant RT and assess the impact of this omission on survival. Methods: SEER database was queried for cases of resected gastric cancer. Multivariate analyses with logistic and Cox regressions were used to examine (a) likelihood of receiving adjuvant RT for different patient and county demographics and (b) effect of demographics on survival outcomes. Results: A total of 7,348 patients met the study criteria. Adjuvant RT was used in 33.1% of cases diagnosed in 1998-2001 and in 45.3% of cases in 2002-2007 (P < 0.001). Controlling for independent covariates, African Americans were 8.9% less likely to receive adjuvant RT than Caucasians or Asians (P < 0.001). Correspondingly, overall survival rates were significantly lower for African Americans than other races (HR = 1.38, P < 0.001). Furthermore, both the likelihood of receiving RT and the survival rates were significantly affected by county demographics: percent of population without high school education, percent of households below the poverty line, and median household income. Survival rates were highest among Asians, but this finding did not reflect more frequent use of RT. Conclusions: Race and socioeconomic factors are significant predictors of treatment and survival outcomes for patients with resectable gastric cancer. Impact: The findings of this and similar studies may aide the medical community in designing more effective strategies to ameliorate the standards of care nationwide. ©2011 AACR. Source

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