Wilson's Temperature Syndrome

OBJECTIVE: To identify through case study the presentation and possible pathophysiological cause of complex regional pain syndrome and its preferential response to stellate ganglion blockade. SETTING: Complex regional pain syndrome can occur in an extremity after minor injury, fracture, surgery, peripheral nerve insult or spontaneously and is characterised by spontaneous pain, changes in skin temperature and colour, oedema, and motor disturbances. Pathophysiology is likely to involve peripheral and central components and neurological and inflammatory elements...

BACKGROUND: Campylobacter jejuni is a major bacterial cause of food-borne enteritis, and its lipooligosaccharide (LOS) plays an initiating role in the development of the autoimmune neuropathy, Guillain-Barré syndrome, by induction of anti-neural cross-reactive antibodies through ganglioside molecular mimicry. RESULTS: Herein we describe the existence and heterogeneity of multiple LOS forms in C. jejuni strains of human and chicken origin grown at 37 °C and 42 °C, respectively, as determined on sodium dodecyl sulphate-polyacrylamide electrophoresis gels with carbohydrate-specific silver staining and blotting with anti-ganglioside ligands, and confirmed by nuclear magnetic resonance (NMR) spectroscopy...

The 2008 annual report of the Regional Infant and Child Mortality Review Committee (RICMRC) is presented. This committee has as its mission the review of infant and child deaths so that information can be transformed into action to protect young lives. The 2008 review area includes South Dakota's Minnehaha, Turner, Lincoln, Moody, Lake, McCook, Union, Hansen, Miner and Brookings counties. Within our region in 2008, there were six infant deaths labeled as Sudden Unexpected Infant Deaths (SUID), of which two met the criteria for the Sudden Infant Death Syndrome (SIDS)...

The response to infection from porcine reproductive and respiratory syndrome virus (PRRSV) for 2 genetically diverse commercial pig lines was investigated. Seventy-two pigs from each line, aged 6 wk, were challenged with PRRSV VR-2385, and 66 litter-mates served as control. The clinical response to infection was monitored throughout the study and pigs were necropsied at 10 or 21 d postinfection. Previous analyses showed significant line differences in susceptibility to PRRSV infection. This study also revealed significant line differences in growth during infection...

The mission of the Regional Infant and Child Mortality Review Committee (RICMRC) is to review infant and child deaths so that information can be transformed into action to protect young lives. The 2007 review area includes South Dakota's Minnehaha, Turner, Lincoln, Moody, Lake, McCook, Union, Hansen, Miner and Brookings counties. Although there were no deaths in 2007 that met the criteria of the Sudden Infant Death Syndrome (SIDS) in our region, there were three infant deaths associated with unsafe sleeping environments (including adult co-sleeping) that either caused or potentially may have caused these infants' deaths...

OBJECTIVE: Familial cold autoinflammatory syndrome (FCAS) is caused by mutations in the CIAS1 gene, leading to excessive secretion of interleukin-1beta (IL-1beta), which is associated with cold-induced fevers, joint pain, and systemic inflammation. This pilot study was conducted to assess the safety and efficacy of rilonacept (IL-1 Trap), a long-acting IL-1 receptor fusion protein, in patients with FCAS. METHODS: Five patients with FCAS were studied in an open-label trial...

RATIONALE: Prenatal cigarette smoke (CS) exposure, increased environmental temperature, and hypoxic episodes have been postulated as major risk factors for sudden infant death syndrome. OBJECTIVES: To test the hypothesis that maternal CS exposure disrupts eupneic breathing and depresses breathing responses of neonatal rats to thermal and hypoxic challenges. METHODS: Experiments were performed on 1-week-old rat pups exposed prenatally to CS (n = 39) or room air (sham; n = 30)...

The 2006 annual report of the Regional Infant and Child Mortality Review Committee (RICMRC) is attached. This committee's mission is to review infant and child deaths so that information can be transformed into action to protect young lives. The 2006 review region includes South Dakota's Minnehaha, Turner, Lincoln, Moody, Lake, McCook, Union, Hansen, Miner and Brookings counties. Although there was only one death meeting the criteria for Sudden Infant Death Syndrome (SIDS) in our region, there were five infant deaths associated with unsafe sleeping environments that either caused or potentially caused these infants' deaths...

The use of sustained release tri-iodothyronine (SR-T3) in clinical practice, has gained popularity in the complementary and alternative medical community in the treatment of chronic fatigue with a protocol (WT3) pioneered by Dr. Denis Wilson. The WT3 protocol involves the use of SR-T3 taken orally by the patient every 12 hours according to a cyclic dose schedule determined by patient response. The patient is then weaned once a body temperature of 98.6 degrees F has been maintained for 3 consecutive weeks. The symptoms associated with this protocol have been given the name Wilson's Temperature Syndrome (WTS)...

Hospital emergency department (ED) syndromic surveillance has been proposed for early detection of a large-scale biologic terrorist attack. However, questions remain regarding its usefulness. The authors examined the use of active syndromic surveillance at hospital EDs in Virginia for early detection of disease events and analyzed the effectiveness of the cumulative sum (CUSUM) algorithm in identifying disease events from syndromic data. Daily chief-complaint data were collected for 10 months at seven hospital EDs in southeastern Virginia...

PURPOSE: To test the hypothesis that subtle abnormalities of the autonomic nervous system underlie the chronic symptoms reported by many Gulf War veterans, such as chronic diarrhea, dizziness, fatigue, and sexual dysfunction. METHODS: Twenty-two ill Gulf War veterans and 19 age-, sex-, and education-matched control veterans underwent measurement of circadian rhythm of heart rate variability by 24-hour electrocardiography, ambulatory blood pressure recording, Valsalva ratio testing, sympathetic skin response evaluation, sweat imprint testing, and polysomnography...

BACKGROUND: Information on the clinical features of the severe acute respiratory syndrome (SARS) will be of value to physicians caring for patients suspected of having this disorder. METHODS: We abstracted data on the clinical presentation and course of disease in 10 epidemiologically linked Chinese patients (5 men and 5 women 38 to 72 years old) in whom SARS was diagnosed between February 22, 2003, and March 22, 2003, at our hospitals in Hong Kong, China. RESULTS: Exposure between the source patient and subsequent patients ranged from minimal to that between patient and health care provider...

Patients with Down syndrome (DS) and Alzheimer's disease (AD) share a number of characteristic neuropathologic lesions. Several lines of evidence suggest that mitochondria and the oxidative stress response are involved in the pathogenesis of both conditions. In the process of investigating the stress response in DS, we discovered a defective basal expression of a major mitochondrial heat shock protein, chaperonin 60 (Cpn60) in non-transformed dermal fibroblast cell lines from DS individuals. Such a defect was not present in control cells that had been cultured under identical physiological growth conditions...

AIMS: A failure of the arousal mechanism is a key feature in the apnoea theory for sudden infant death syndrome (SIDS). In infants studied at an age when the incidence of SIDS is highest, we evaluated whether in utero smoke exposed infants have altered arousal response to standardised auditory stimuli, and/or sleep pattern, as recorded on overnight complex sleep polysomnography. METHODS: A standardised sequence of audiology stimuli was applied binaurally to 20 in utero smoke and non-smoke exposed infants aged 8-12 weeks during a rapid eye movement (REM) and NREM epoch, in a controlled (temperature, position, pacifier use, noise) sleep environment...

Smith-Lemli-Opitz syndrome (SLOS; MIM 270400) is a genetic disorder characterized by hypocholesterolemia and elevated 7-dehydrocholesterol (7DHC) levels resulting from mutations affecting 7-dehydrocholesterol reductase. We describe a colorimetric assay for 7DHC with potential application to large-scale screening for SLOS. Reaction of 7DHC and its esters with the Liebermann-Burchard reagent resulted in a brief initial absorbance at 510 nm (pink color) followed by an absorbance at 620 nm (blue color) after 2 min, while cholesterol samples were essentially colorless...

OBJECTIVE: To investigate the molecular mechanisms of nucleoside analogue reverse transcriptase inhibitor (NRTI)-associated mitochondrial dysfunction. METHODS: Peripheral blood samples were collected from 10 healthy individuals, 10 HIV-infected, NRTI-treated patients with lipoatrophy, and four HIV-infected patients naive to all antiretrovirals. DNA was isolated from the leukocytes and the mitochondrial genome analyzed for DNA depletion, deletions and point mutations...

OBJECTIVE: To investigate the cause and outcome of high fever in Jamaican children with homozygous sickle cell disease. DESIGN: Retrospective review of febrile episodes in a three year period (1 September 1993 to 31 August 1996). SETTING: Sickle cell clinic, an outpatient clinic in Kingston run by the Medical Research Council Laboratories (Jamaica). PATIENTS: Patients with homozygous sickle cell disease under 17 years of age presenting with an axillary temperature >/= 39...

OBJECTIVE: To relate clinical features to autonomic laboratory indices used in the diagnosis of Complex Regional Pain Syndrome type I (CRPS I) (reflex sympathetic dystrophy) to generate improved diagnostic criteria. DESCRIPTION: CRPS I is a chronic pain syndrome, characterized by diffuse limb pain with allodynia and prominent vasomotor and sudomotor dysfunction. METHODS: We conducted a prospective study on 102 patients referred for possible CRPS I...

We report a modified copper stain that can be completed in 2 hours, rather than overnight, which is required for other stains. This is useful in the diagnosis of chronic cholestatic syndromes, Wilson's disease, Indian childhood cirrhosis, and other conditions associated with copper retention.

Because of the critical role of neutrophils in host defenses, it was hypothesized that stimulation of neutrophil production and function with Filgrastim would improve the outcome of hospitalized patients with community-acquired pneumonia. To test this hypothesis, a randomized, placebo-controlled, multicenter trial of Filgrastim (300 micrograms/day up to 10 days) as an adjunct to antibiotics was conducted for these patients. Outcome measures included time to resolution of morbidity (TRM, a composite measure of temperature, respiratory rate, blood oxygenation, and chest radiograph), 28-day mortality, length of stay, and adverse events...