Platelet dysfunction in primary thrombocythemia using the platelet function analyzer, PFA-100

We measured platelet function by standard aggregometric tests and by the PFA-100 (Dade Behring, Newark, DE) in samples from 55 patients with primary thrombocythemia (PT) and 26 healthy volunteers. Platelet function was evaluated in platelet-rich plasma by aggregation tests. PFA-100 studies (closure time) were performed in citrated whole blood using collagen-adenosine diphosphate (ADP) and collagen-epinephrine cartridges. Plasma levels of von Willebrand factor (vWF) also were measured. The mean +/- SD closure time for patients vs volunteers for the collagen-epinephrine cartridge was prolonged (210.8 +/- 62.2 vs 118.1 +/- 19.6 seconds; P < .001); results were abnormal for 38 patients (69%). Results with the collagen-ADP cartridge also were abnormal in patients (134.3 +/- 58.4 seconds) vs volunteers (87.3 +/- 15.6 seconds; P < .001); closure time was prolonged in 23 patients (42%). A decreased response to epinephrine (38.4% +/- 34.2% vs 82.5% +/- 10.3%; P < .001), the main defect detected by platelet aggregation tests, affected 32 patients (58%). Platelet response to collagen also was abnormal (52.0% +/- 34.6% vs 86.0% +/- 10.1%; P < .01) but affected only 21 patients (38%). vWF levels for patients were normal. The results seem to confirm that platelet function in patients with PT is abnormal and show that platelet function can be assessed by an easy, reproducible, and sensitive method, the PFA-100. Closure time usually was prolonged; this feature could be applied in the diagnosis of PT.     

Persistent hyperinsulinaemic hypoglycaemia of infancy: 15 years' experience at the Royal Children's Hospital (RCH), Melbourne

OBJECTIVE: To review the clinical outcome and report our experience of patients with persistent hyperinsulinemic hypoglycaemia of infancy (PHHI). METHODS: Retrospective case series of 14 patients diagnosed with PHHI at the Royal Children's Hospital in Melbourne in the period between 1988 and 2004. Diagnoses of insulinoma, Beckwith syndrome and transient hyperinsulinism were excluded. RESULTS: Six patients (43%) presented on day 1, with a median presenting age of 2.75 months; ten (71.4%) presented with seizures. All were initially treated with diazoxide, of whom six (43%) recovered and are off medication, while four patients (28.5%) required surgery (twice in one patient). One developed permanent diabetes mellitus. Developmental delay occurred in nine (64%), and was severe in two (14.5%) patients, possibly associated with delayed treatment. CONCLUSIONS: PHHI is a rare but severe condition. The aim of management of PHHI should be on the one hand to prevent mental retardation and developmental delay due to severe and persistent hypoglycaemia, and on the other hand to avoid the induction of diabetes mellitus following surgery. Satisfactory outcome depends on early effective medical treatment and judicious use of surgery by an experienced operator.     

Marital status, marital transitions, well-being, and spinal cord injury: an examination of the effects of sex and time

Kalpakjian CZ, Houlihan B, Meade MA, Karana-Zebari D, Heinemann AW, Dijkers MP, Wierbicky J, Charlifue S. Marital status, marital transitions, well-being, and spinal cord injury: an examination of the effects of sex and time.

Objective

To examine the applicability of marital resource (marriage has substantial benefits for well-being over not being married) or marital crisis (marital dissolution leads to poorer well-being) models to the spinal cord injury (SCI) population by studying the effects of sex, marital status, and marital transitions on well-being.

Design

Prospective cohort study from the SCI Model Systems National Database.

Setting

Community.

Participants

Men (n=4864) and women (n=1277) who sustained traumatic SCI and completed a minimum of 1 follow-up interview beginning at 1 year through 15 years postinjury.

Interventions

None.

Main Outcomes Measures

Life satisfaction, depressive symptoms, and self-perceived health status by using linear mixed models for longitudinal data.

Results

In general, well-being improved over time since injury. Hypothesis testing supported the marital crisis model, as marital loss through being or becoming separated or divorced and being or becoming widowed, had the most consistent and negative impact across well-being outcomes, whereas being or becoming married had an advantage for only lower depression symptoms over time. However, marital dissolution or loss did not have a uniformly adverse impact on well-being outcomes, and this effect often was moderated by sex, such that widows had higher depressive symptoms and poorer self-perceived health than widowers, but separated or divorced women had higher life satisfaction and self-perceived health than men. Irrespective of sex, being separated or divorced versus being single was associated with higher depressive symptoms over time.

Conclusions

Results support the marital crisis model and that women and men can experience marital dissolution differently. All marital loss does not result in compromised well-being and all marriage does not enhance well-being, highlighting complex dynamics worthy of further investigation in this population.     

Surgical management of cervical chyloma following parathyroidectomy

Although rare, chylomas can present as a neck mass, especially in the post-operative setting. Here, we present a case of a persistent cervical chyloma following parathyroidectomy and propose a management algorithm for this clinical entity.     

Takotsubo cardiomyopathy associated with peritonitis in peritoneal dialysis patient

Takotsubo cardiomyopathy (TTC) is characterized by clinical and electrocardiographic features that mimic acute myocardial infarction, normal or mildly elevated cardiac enzymes, distinctive left ventricular wall motion abnormalities, and absence of significant obstructive coronary artery disease. Often there is a history of emotional stress and usually encountered in postmenopausal women. Excessive catecholamine stimulation plays an important role in the pathogenesis of TTC. Usually, this condition is reversible within several weeks to months. Only two cases of TTC were described in patients on hemodialysis. To our knowledge, we report the first case of TTC in peritoneal dialysis and the first case associated with peritonitis.     

Borna Disease Virus and the Brain

Viruses with the ability to establish persistent infection in the central nervous system (CNS) can induce progressive neurologic disorders associated with diverse pathological manifestations. Clinical, epidemiological, and virological evidence supports the hypothesis that viruses contribute to human mental diseases whose etiology remains elusive. Therefore, the investigation of the mechanisms whereby viruses persist in the CNS and disturb normal brain function represents an area of research relevant to clinical and basic neurosciences. Borna disease virus (BDV) causes CNS disease in several vertebrate species characterized by behavioral abnormalities. Based on its unique features, BDV represents the prototype of a new virus family. BDV provides an important model for the investigation of the mechanisms and consequences of viral persistence in the CNS. The BDV paradigm is amenable to study virus–cell interactions in the CNS that can lead to neurodevelopmental abnormalities, immune-mediated damage, as well as alterations in cell differentiated functions that affect brain homeostasis. Moreover, seroepidemiological data and recent molecular studies indicate that BDV is associated with certain neuropsychiatric diseases. The potential role of BDV and of other yet to be uncovered BDV-related viruses in human mental health provides additional impetus for the investigation of this novel neurotropic infectious agent.