Neurofibromatosis Type 1 Diagnosed in a Child Based on Multiple Juvenile Xanthogranulomas and Juvenile Myelomonocytic Leukemia

An association between juvenile xanthogranuloma (JXG), neurofibromatosis type 1 (NF1), and juvenile myelomonocytic leukemia (JMML) has been described in the literature but has only been documented in approximately 20 cases. We diagnosed a patient with NF1 at 25 months of age, before any cutaneous stigmata of this disease had appeared, because we decided to screen for the NF1 gene mutation because of his presentation with multiple JXGs and moderate macrocephaly (2.5 standard deviations) at 9 months of age and JMML diagnosed at 20 months of age. The child is well today after treatment with chemotherapy and allogenic bone marrow transplantation. With increased awareness, patients with JXG and NF1 who develop symptoms possibly related to JMML, such as paleness, skin bleeding, cough, unexplained fever, and hepatosplenomegaly, should be further evaluated. We also emphasize that multiple JXG lesions can be an early marker of NF1.     

Teaching psychiatric ethics

In the last decade, we have witnessed a burgeoning of interest in ethical issues amongst psychiatrists. Teaching of the subject, however, remains at a rudimentary stage. Various approaches to such instruction are available, particularly modelling (students observe their experienced counterpart), the case method (examining specific clinical situations which involve a need for ethical decision-making), and the seminar approach (trainees are exposed to a core body of knowledge, mainly theoretical in nature). Faced with these different teaching models, the University of Oxford Department of Psychiatry has opted for a blend of all three approaches, which incorporates two goals: an increase in the trainees' sensitivity to the many intricate moral dilemmas facing the psychiatric profession; and their familiarity with salient concepts in moral philosophy which constitute a basis for ethical reasoning and which have a bearing on clinical practice. The teaching programme comprises the following: a pair of trainees prepares a presentation on an aspect of psychiatric ethics under the supervision of a senior psychiatrist. A moral philosopher assumes the role of discussant of the ethical problems raised by the trainees; this is followed by a general discussion. Topics have included involuntary hospitalization, dual loyalty, suicide, psychiatric diagnosis, and ethical issues in various spheres of psychiatric practice such as sex therapy, psychotherapy and child psychiatry. The approach has worked effectively and proved rewarding to all participants involved.     

Comparative toxicity of total lymphoid irradiation and immunosuppressive drug treated patients with intractable rheumatoid arthritis

Outcomes were compared between consecutive patients who had received either total lymphoid irradiation (TLI) or immunosuppressant treatment for intractable rheumatoid arthritis (RA). There were 33 TLI and 32 immunosuppressive recipients; all patients had failed standard therapy. Average followup from the start of therapy was 2.7 years for TLI and 5.9 years for immunosuppressive recipients. Final disability levels were the same in both groups; mortality was equal in both groups as well. There were more hospitalizations for infections in the TLI group and the infecting organisms tended to be staphylococcus or gram negative organisms. Apart from infections, there were more adverse effects reported in the immunosuppressive therapy group.     

High risk factors for rehospitalization within six months

Objective. 1. To identify risk factors associated with psychiatric rehospitalization within six months, using global clinical assessments and demographic information and; 2. To determine if risk factors for a hospital in a rural region are similar to those reported for urban hospitals. Method. The setting was a psychiatric unit within a general hospital. All adult admissions for one year were assigned scores on the North Carolina Functional Assessment Scale (NCFAS) and the Global Assessment of Functioning (GAF) scale. Patients were interviewed six months after discharge to determine if they had been rehospitalized and to assign new NCFAS and GAF scores. Results. Significant risk of rehospitalization was predicted by: 1. NCFAS score >90; 2. history of prior hospitalization; 3. nursing home residence; 4. referral from a small community hospital and; 5. non-compliance with outpatient appointments. Conclusions. Global assessments and demographic information collected during an index admission can generate factors to identify patients at risk for rehospitalization within six months. History of prior admissions and non-compliance with outpatient treatment, reported as risk factors in urban settings, were found also to be risk factors in a rural region.He was formerly Assistant Professor, Center for Health Sciences Statistics, East Carolina University School of Medicine.