The role of therapeutic plasma exchange in pemphigus vulgaris

Background The treatment of pemphigus, an autoimmune bullous disease, is based on the combination of corticosteroids and adjuvant therapies, such as immunosuppressive drugs, anti‐inflammatory drugs and immunomodulatory procedures, such as intravenous immunoglobulin and therapeutic plasma exchange (TPE). Objective This study aims to assess our experience with TPE as a steroid‐sparing modality in moderate and severe intractable pemphigus patients. Methods A retrospective evaluation for all intractable pemphigus patients treated by TPE in a university‐affiliated tertiary referral medical centre between the years 1998 and 2008. Treatment protocol included three TPE treatments weekly for 1–3 months, combined with monthly pulse therapy of dexamethasone and/or cyclophosphamide. Maintenance therapy was based on once/bi weekly TPE treatments or monthly intravenous immunoglobulin. Results Seven patients were included in the study, four with severe pemphigus vulgaris and three with moderate disease. Six of the seven patients responded to TPE: Four patients (57%) achieved complete remission and two patients (28%) achieved partial remission on minimal therapy. Mild adverse effects related to TPE were observed in two patients and included dizziness and mild headache. Conclusion TPE is a well‐tolerated effective steroid‐sparing agent in recalcitrant pemphigus patients.     

A randomized prospective study on the effect of short and long Buserelin treatment in women with repeated unsuccessful in vitro fertilization (IVF) cycles due to inadequate ovarian response

Fifty four women with repeated unsuccessful in vitro fertilization (IVF) cycles due to inadequate ovarian response to stimulation with human menopausal gonadotropins (hMG) participated in this study. They were randomized to receive either gonadotropin releasing hormone agonist (GNRHa), Buserelin, prior to and during induction of ovulation by hMG (Group I—long protocol), or GnRHa starting on the first day of the cycle together with induction of ovulation by hMG (Group II—short protocol). Mean follicular phase serum luteinizing hormone (LH) and progesterone (P) levels were significantly lower in Group I than in Group II (P<0.01). Cancellation rate was significantly lower in Group I than in Group II (P<0.01). The long GNRHa protocol resulted in statistically significant lower cancellation rates, more oocytes per pickup (OPU), more embryos trans-ferred per patient, and a higher pregnancy rate. Significantly more hMG ampoules and more treatments days were required in the long GNRHa protocol. Our data demonstrate that the use of GNRHa prior to and during ovarian stimulation with hMG offers a very good alternative for patients with repetitive unsuccessful IVF cycles due to inadequate response.     

Can late survival of patients with moderate ischemic mitral regurgitation be impacted by intervention on the valve?

BACKGROUND: Ischemic mitral regurgitation is known to be associated with poor long-term outcome after coronary artery bypass grafting; however, our ability to alter that outcome with intervention on the valve is unclear. The decision to address the valve is most challenging for patients with only moderate mitral regurgitation, particularly with the popularization of off-pump surgery. We therefore reviewed early and late outcomes of patients undergoing revascularization with or without mitral valve surgery. METHODS: Patients with moderate mitral regurgitation undergoing revascularization with and without mitral surgery between January 1991 and September 1996 were identified retrospectively. Operative notes were reviewed and patients with structural valve disease excluded. Perioperative events and late outcomes as determined by telephone contact and search of the social security death index (survival data 97% complete) were compared. RESULTS: One hundred seventy-six patients with moderate mitral regurgitation underwent revascularization alone (n = 142) or with mitral repair or replacement (n = 34). Those undergoing revascularization alone had a higher serum creatinine, somewhat less mitral regurgitation, and lower New York Heart Association functional class preoperatively. Operative mortality was greater with valve surgery (21% vs 9%, p = 0.047). Actuarial survival of both groups at 5 years was similar (52% vs 58%, p = NS); however, when stratified by preoperative functional class, those with more advanced heart failure preoperatively had superior late survival if their mitral valve was intervened upon. CONCLUSIONS: The late survival of patients with ischemic mitral regurgitation undergoing coronary revascularization remains poor; however, intervention on the mitral valve appears to benefit those with symptomatic heart failure.     

The role of the family physician in eating disorders

The steady increase in Eating Disorders (ED) during the last three decades has raised increasing concern over the early diagnosis and treatment of this disease. Due to the multi-factorial etiology of ED, treatment is complex, prolonged, and demands a team approach. Family physicians are often approached first for consultation following the patient's initial physical complaints. For this reason, the general practitioner can play a critical role in the early diagnosis and treatment of these disorders. By asking the appropriate questions and providing relevant information, the family physician (FP) may be able to refer the patient to treatment necessary for recovery, as well as assist in this process. Because the family physician typically has previous acquaintance with the patient and her family, he or she may be of considerable import to creating a therapeutic liaison and support framework with the professional team. This article will discuss the potential involvement and significance of the family physician in the successful diagnosis, prevention, and treatment of ED. The importance of the FP in the decision for hospitalization, referral, and follow-up is emphasized, as well as the pivotal role of the FP as a multi-disciplinary team coordinator.     

Nocturnal acute laryngospasm in children: a possible epileptic phenomenon

Respiratory difficulties are not uncommon during epileptic activity in all age groups. Laryngospasm, as an isolated manifestation of epileptic disorder, is a rare phenomenon described previously in only two patients. We report our experience with five children in whom nocturnal laryngospasm was the only clinical manifestation of their epileptic disorder. All children underwent extensive workup and the diagnosis was made by sleep-deprived electroencephalography (two cases) and sleep study (three cases). All patients were treated with carbamezapine with prompt resolution of their laryngospasm.     

Unexplained fever in neonates may be associated with hepatitis B vaccine.

AIM: To investigate whether hepatitis B vaccination has increased the number of cases of unexplained neonatal fever. METHOD: The files of all infants born from 1 January 1991 to 31 December 1992, in whom a diagnosis of "injected antibiotic" or "disease of temperature regulation" was recorded, were reviewed. Those who had unexplained fever of 38 degrees C or higher during the first three days of life were divided into two groups: infants who did not receive the hepatitis B vaccine (1991) and infants who did (1992). RESULTS: In 1992 the incidence of unexplained fever in hepatitis B vaccinated neonates was significantly higher than in the 1991 group of pre-vaccination neonates (35 out of 5819 (0.6%) vs 14 out of 5010 neonates (0.28%) respectively, p=0.013). CONCLUSIONS: The increase in the number of cases of unexplained neonatal fever seems to be associated with the introduction of routine hepatitis B vaccination on the first day of life. The possibility that an excess number of neonates will undergo unnecessary procedures and treatment to diagnose unexplained fever justifies planning a controlled study to determine whether these preliminary findings point to a significant problem.     

Motivation for giving birth after breast cancer

BACKGROUND: Breast cancer at a young age threatens the natural developmental tasks that characterize this phase in life including parenthood. The dilemma of whether to give birth arises due to the potential medical, psychological and social implications of pregnancy, birth and child rearing after breast cancer. The purpose of this study was to investigate the positive and negative motivations toward childbirth of breast cancer survivors and their husbands. METHOD: Thirty breast cancer survivors and 13 husbands were compared to 29 healthy women and 15 husbands. The study included qualitative questions and quantitative measures including: a demographic and medical questionnaire, the Parenthood Motivation Questionnaire--Revised, the ENRICH Marital Satisfaction Scale, the Brief Symptom Inventory, the Impact of Events Scale, and the Mental Adjustment to Cancer Questionnaire. RESULTS: The experience of having breast cancer did not hinder overall positive motivations toward childbirth, nor did it increase overall negative motivations toward childbirth, among women and their husbands. However, there were several differences between the groups, which may reflect the illness experience. For example, breast cancer survivors and their husbands reported more negative motivations toward childbirth due to health concerns than did healthy women and their husbands.     

Differential responses of visceral and subcutaneous fat depots to nutrients

Increased visceral adiposity is a pivotal component of the metabolic syndrome. Differential gene expression patterns of fat-derived peptides (FDPs) in visceral fat and subcutaneous fat have been characterized in the fasting state. Here we examined whether delivery of nutrients differentially affects the expression of FDPs in visceral fat versus subcutaneous fat (in the fed state). We increased the rate of glucose flux into adipose tissue of normal rats (n = 16) by hyperglycemia or hyperinsulinemia using the clamp technique. Glucose uptake was associated with increased expression of FDPs, including resistin ( approximately 5-fold), adiponectin ( approximately 2-fold), leptin ( approximately 15-fold), plasminogen activating inhibitor-1 ( approximately 10-fold), and angiotensinogen ( approximately 4-fold) in visceral fat, but markedly less in subcutaneous fat. Cytokine expression derived mainly from vascular/stromal/macrophage components of adipose tissue was less dramatically increased. Infusion of glucosamine amplified the results obtained by increasing glucose uptake into adipose tissue, suggesting that flux through the hexosamine biosynthetic pathway may serve as a mechanism for "nutrient sensing." Nutrient-dependent expression of FDPs in visceral fat was also associated with increased plasma levels of several FDPs. Because a biologic sensing pathway can dynamically couple daily food intake to abnormal plasma levels of important FDPs, we challenge the practice of obtaining plasma levels after fasting to assess risk factors for metabolic syndrome.