Sneddon's syndrome: clinical and laboratory analysis of 10 cases

Sneddon's syndrome is characterized by livedo reticularis and cerebrovascular lesions. We report the cases of women (mean age, 36.2 +/- 8.1 years) diagnosed with Sneddon's syndrome based on the presence of livedo reticularis and characteristic cerebrovascular findings. Seven of these patients had cerebral infarcts on cranial computed tomography scan. Antiphospholipid antibodies were positive in 6 of these cases. Three cases had abnormal levels of antithrombin III. Analyses of chromosome 6 revealed no abnormalities. In 3 of the cases, investigation of the pedigrees revealed autosomal dominant traits. Two cases had epilepsy, and 3 had migraine. One case with migraine also had myasthenia gravis. In addition, we detected inferior altudinal hemianopia in 2 cases, cognitive functional disorder in 3 and depression in 2. Based on these findings, the entire vascular, haematologic, neurologic, and dermatologic systems should be evaluated in patients diagnosed with Sneddon's syndrome.     

Incidence and outcome of chronic graft-versus-host disease using National Institutes of Health consensus criteria.

Chronic graft-versus-host disease (cGVHD), a common complication after stem cell transplant (SCT), has an impact on morbidity and survival. Previous classification of cGVHD has not been reproducible or prognostic for nonrelapse mortality (NRM). Recently the National Institutes of Health (NIH) consensus criteria were proposed, but the ability of this classification to predict outcome of various subtypes of cGVHD is unknown. Patients (N = 110) undergoing an SCT for a hematologic malignancy and surviving until day 100 posttransplant from 2001 to 2003 were studied. The overall survival (OS) using a landmark analysis at day 100 was 44% versus 66% (no GVHD vs. GVHD, P = .026). The OS of patients with various types of GVHD as proposed by the NIH criteria were significantly different (P < .0001). In a univariate analyses, this was more apparent when patients with any acute features of GVHD were compared to classic cGVHD (3-year OS 46% vs. 68%, P = .033). The 3-year NRM for the entire cohort was 21%, and was not affected by presence or absence of GVHD or subtypes of GVHD. In a multivariable analysis, extensive cGVHD (hazard ratio [HR] 0.35, P = .015) and having any acute feature of GVHD after day 100 (HR 3.36, P = .0144) were significant independent predictors of survival. The OS with different NIH subtypes of GVHD after day 100 from SCT varies, and is superior for patients with classic cGVHD.     

Human rhinovirus C infections in pediatric hematology and oncology patients

Children with cancer and HSCT recipients are at high risk for common viral infections. We sought to define the viral etiology of ARI and identify risk factors. Nasal wash samples were collected from pediatric hematology–oncology patients and HSCT recipients with ARI during the 2003–2005 winter seasons. Real‐time RT‐PCR was performed to detect Flu A, influenza B, RSV, PIV 1‐3, human MPV, and HRV. HRV specimens were sequenced and genotyped. Seventy‐eight samples from 62 children were included. Viruses were detected in 31 of 78 samples (40%). HRV were detected most frequently, in 16 (52%) including five HRVC; followed by seven (22%) RSV, five (16%) Flu A, four (13%) MPV, and two (6%) PIV2. There was a trend toward higher risk of viral infection for children in day care. Only 8% of the study children had received influenza vaccine. HRV, including the recently discovered HRVC, are an important cause of infection in pediatric oncology and HSCT patients. Molecular testing is superior to conventional methods and should be standard of care, as HRV are not detected by conventional methods.     

Differences in end-of-life preferences between congestive heart failure and dementia in a medical house calls program

OBJECTIVES: To compare end-of-life preferences in elderly individuals with dementia and congestive heart failure (CHF). DESIGN: Retrospective case-control study. SETTING: Geriatrician-led interdisciplinary house-call program using an electronic medical record. PARTICIPANTS: Homebound individuals who died while under the care of the house-call program from October 1996 to April 2001. MEASUREMENTS: Medical records review for demographics, functional status, advance medical planning, hospice use, and place of death. RESULTS: Of 172 patients who died in the program, 29 had CHF, 79 had dementia, 34 had both, and 30 had neither. Patients with CHF were younger (82.6 vs 87.0, P=.011) and less functionally dependent (activities of daily living score 9.1 vs 11.5, P=.001). Time from enrollment to death was not significantly different (mean+/-standard deviation=444+/-375 days for CHF vs 325+/-330 days for dementia, P=.113). A do-not-resuscitate (DNR) directive was given in 62% of patients with CHF and 91% with dementia (P<.001). Advance medical planning discussions were not significantly different (2.10 in CHF vs 1.65 in dementia, P=.100). More patients with CHF participated in their advance medical planning than those with dementia (86% vs 17%, P<.001). Hospice was used in 24% of CHF and 61% of dementia cases (P<.001). Finally, 45% of patients with CHF and 18% of patients with dementia died in the acute hospital (P=.006). Multivariate analysis showed that the fact that more patients with CHF were involved in their medical planning was not significant in predicting end-of-life preferences. Alternatively, Caucasian ethnicity was an independent predictor of having a documented DNR and death outside of the acute hospital. CONCLUSION: In the months before death, patients with CHF were more likely to have care plans directed at disease modification and treatment, whereas dementia patients were more likely to have care plans that focused on symptom relief and anticipation of dying. Several factors may contribute to this difference.     

Effect of Intraoperative Valsalva Maneuver Application on Bleeding Point Detection and Postoperative Drainage After Thyroidectomy Surgeries

The purpose of this paper was to analyze the effect of Valsalva maneuver application before finalizing thyroidectomy operations on the identification of bleeding points and postoperational drainage. One hundred patients (age range, 24–76 years) with multinodular goiter, recurrent multinodular goiter, toxic diffuse multinodular goiter, or papillary thyroid cancer were included in the study and were divided into 2 groups of 50 randomly. Both groups underwent thyroidectomy operation, only 1 group received intraoperative Valsalva maneuver application (twice, 30 seconds of 30-cm PEEP). The size of the thyroid gland, the duration of operation, hospital stay, and drain usage were reported. Postoperational occurrences of drainage, hematoma, reoperation, and additional complications were compared between the groups. Valsalva maneuver application helped to identify minor bleeding points in 32% of the cases. There was no significant difference between the study groups regarding the thyroid gland size, operation duration, hospital stay, and the duration of drain usage (P > 0.05 for all). The amount of drainage as well as the frequencies of hematoma, reoperation, and further complications was not significantly different between the study groups (P > 0.05 for all). Intraoperative application of Valsalva maneuver is only useful to detect minor bleeding points in some patients during thyroidectomy operations, but it had no effect on the duration of postoperative drain usage, the amount of drainage, and risk of hematoma. Therefore, intraoperative application of Valsalva maneuver has no beneficial effect on postoperative hemorrhagic complication after thyroidectomy operations.Key words: Drainage, Hemostasis, Thyroidectomy, Valsalva maneuverThyroidectomy is one of the most commonly performed operations worldwide.¹ Though a series of improvements were introduced to the operation process, still several postoperative complications remain to be faced such as seroma, wound infection, skin flap complications, and hematoma.^2,3Despite the richness of the blood vessels in the thyroid area,⁴ the rate of postoperational bleedings thus hematoma events are low (1–5% of the cases).⁵ However, once not controlled, hematoma can result in airway compression and create a life-threatening situation for the patient.⁶ Hence, preventive measures have been emphasized and employed to avoid postoperational bleedings after thyroidectomy. These approaches range from exercises to avoid Valsalva maneuver kind of forces such as coughing or straining at opening bowel⁷ to the use of drains after the operation. However, recent literature indicates that the drain usage does not have a significant effect on the recovery period or on the duration of the hospital stay.^3,5,8,9 Furthermore, it may cause wound infection and contribute to the discomfort of the patients.^5,8,10,11 Thus a proper hemostasis and an early detection of potential bleeding sites are crucial steps for a successful operation and a steady recovery period.⁴ Several techniques have been used to detect and treat bleeding points immediately after the operation to prevent postoperational hematoma. These techniques involve the use of hydrogen peroxide, water in the wound, Valsalva maneuver, and Trendelenburg tilt.^4,7 Recently, the success of Valsalva maneuver and Trendelenburg tilt application on bleeding point detection was demonstrated by Moumoulidis et al.⁴ However, no further information is provided until now regarding the postoperational evaluation of the patients.In this paper, we aim to analyze the effect of the Valsalva maneuver application on the identification of bleeding points before finalizing the surgery and its influence on the postoperative drainage, complications, and recovery process.