Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case report.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterised by platelet aggregation causing microvascular occlusion. Early diagnosis and utilization of plasmapheresis can provide an improvement in prognosis. CASE REPORT: A 17 year old male with classical findings of TTP was later diagnosed as systemic lupus erythematosus (SLE). Plasmapheresis resulted in the significant amelioration of the course. CONCLUSION: The coexistence of TTP and SLE may facilitate a better understanding of in the pathophysiology of TTP. These association may provide the role of autoimmunity in TTP. SLE should be considered in the differential diagnosis of patients with TTP because of therapeutic implications.     

Somatostatin infusion withdrawal in the diagnosis of childhood growth hormone deficiency

OBJECTIVE: An evaluation of growth hormone (GH) testing for GH deficiency (GHD) in childhood is confounded by the lack of a world-wide consensus on the definition of GHD. Although a single GH test remains the most powerful biochemical tool in the evaluation of a child with growth failure, the test remains far from ideal. Withdrawal of somatostatin (SS) infusion is followed by a rebound rise of GH thought to be mediated by endogenous GH-releasing hormone (GHRH) function. This study was designed to compare the GH response to 90 min SS infusion in children with normal GH secretion versus children with GH deficiency. METHODS: Ten children with GHD and 10 healthy controls (NC) have been evaluated for GH response to somatostatin infusion withdrawal (SSIW) and compared with response of two provocative tests, glucagon plus propranolol test and L-Dopa test. All children received constant infusion of somatostatin for 90 min (3 microg/kg per h, Stilamin, Serono, Aubonne, Switzerland). In order to determine GH, blood samples were obtained 90 min before the SS infusion and 0, 15, 30, 45, 60, 75, and 90 min after the cessation of infusion. RESULTS: Growth hormone peak levels with SSIW were significantly lower in GH deficient children than in healthy children (2.5 +/- 1.2 ng/dL, vs 21.9 +/- 5.3 ng/dL, respectively, P < 0.01). No adverse effects were observed during or after somatostatin infusion. CONCLUSION: In the present study, SSIW elicited a significant GH rise in healthy children but not in children with GH deficiency. Although further controlled studies using more data are necessary to expand these findings, the results suggested that children with GH deficiency can be reliably discriminated from healthy children by SSIW.     

Low scores on the Benton Facial Recognition Test associated with vertebrobasilar insufficiency

Background

Decreased posterior cerebral circulation has been observed in patients with vertebrobasilar insufficiency (VBI). Reduced cerebral perfusion may have an impact on mental performance as measured by the Benton Facial Recognition Test (BFRT). We evaluated the usefulness of BFRT in identifying cognitive decline in patients with VBI by correlating test performance with total blood flow in the vertebrobasilar system and other variables such as educational level and gender.

Materials and methods

Thirty-three participants without dementia (mini-mental state examination; MMSE >27) and cranial magnetic resonance imaging abnormality, but with atherosclerotic risk factors were involved in the study. Nineteen subjects had a total vertebrobasilar flow volume less than 200 ml/min (Group I), and 14 subjects had a flow volume more than 200 ml/min (Group II).

Results

The groups were similar in regard to gender, age, and educational level. BFRT results were 19.53 ± 3.12 and 22.36 ± 2.73 for Groups I and II, respectively (p = 0.01). The educational level was the main factor affecting the BFRT score in Group I (p = 0.04).

Discussion

BFRT is clearly impaired in VBI as measured by Doppler ultrasound examination. We concluded that the test appears to adequately distinguish cognitive levels between VBI and other patients. Additionally, our results suggest that education is associated with BFRT results, and for normative purposes, gender consideration is unnecessary. Further studies are needed to investigate the association between VBI and memory dysfunction in early dementia.     

Effectiveness of Palosuran in Bleomycin-Induced Experimental Scleroderma

Systemic sclerosis (SSc) is a disease characterized by skin and internal organ involvement. There is progressive accumulation of extracellular matrix components in the skin and involved organs. Tissue fibrosis is the prominent reason for mortality, and still, there is no satisfactory treatment. The aim of this study was to evaluate the effects of urotensin-II (U-II) antagonist palosuran in an animal model of scleroderma. We also planned to measure U-II, endothelin-1 (ET-1), and transforming growth factor-β1 (TGF-β1) levels, as well as the association of these levels with dermal thickness. Twenty-four male mice were included in this study and they were divided into three groups—group 1: control group, group 2: fibrosis group, and group 3: fibrosis + palosuran treatment group. Fibrosis + palosuran treatment in group 3 reduced ET-1, U-II, and TGF-β1 levels. In total, the diminished values were statistically significant in the ET-1 and TGF-β1 levels (p?<?0.05). Dermal thickness was higher in the fibrosis group, when compared with the other groups. There was no significant relationship between dermal thickness and ET-1, U-II, or TGF-β1 levels (p?>?0.05). It is believed that U-II is an important mediator in SSc, and its antagonism with palosuran could be a new treatment choice in SSc.     

Urotensin Inhibition with Palosuran Could Be a Promising Alternative in Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a progressive and a life-threatening disease with its high morbidity and mortality ratios. On searching for new shining targets in pathogenesis, we noticed, in our previous studies, urotensin-II (UII) in systemic sclerosis with potent angiogenic and pro-fibrotic features. Owing to the mimicking properties of UII with endothelin-1 (ET1), we attempted to investigate the effect of palosuran in a PAH rat model. Thirty rats were randomly divided into three groups, with each group comprising 10 rats: group 1 (control group) received the vehicle subcutaneously, instead of monocrotaline (MCT) and vehicle; group 2 (MCT group) received subcutaneous MCT and vehicle; and group 3 (MCT + palosuran group) received subcutaneous MCT and palosuran. Serum UII, ET1, transforming growth factor-β1 (TGF-β1) levels, pulmonary arteriolar pathology of different diameter vessels, and cardiac indices were evaluated. The ET1, TGF-β1, and UII levels were significantly diminished in the treatment group, similar to the controls (p?<?0.001). Right ventricular hypertrophy index and mean pulmonary arterial pressure scores were also significantly reduced in the treatment group (p?=?0.001). Finally, in the 50–125-μm diameter arterioles, in contrast to Groups 3 and 1, there was a statistically significant thickness (p?<?0.01) in the arteriolar walls of rats in Group 2. The treatment effect on arteries of more than 125-μm diameters was found to be valuable but not significant. Owing to its healing effect on hemodynamic, histological, and biochemical parameters of MCT-induced PAH, palosuran as an antagonist of UII might be an optional treatment alternative for PAH.     

Assessments of the associations of thrombus localization with accompanying disorders,risk factors,D-dimer levels,and the red cell distribution width in pulmonary embolism

OBJECTIVE:

Pulmonary embolisms occur as a wide spectrum ranging from clinically asymptomatic thrombi to massive thrombi that lead to cardiogenic shock. The purpose of this study was to determine the associations of thrombus localization with risk factors, accompanying disorders, D-dimer levels and the red blood cell distribution width in patients with pulmonary embolism.

MATERIAL AND METHODS:

In 148 patients diagnosed with pulmonary embolism, the presence and anatomical localization of the thrombus were assessed via computed tomographic pulmonary angiography. The accompanying disorders, risk factors, serum D-dimer levels, and red blood cell distribution width of the patients were retrospectively evaluated. ClinicalTrials.gov: NCT02388841.

RESULTS:

The mean age of the patients was 54±16.0 years, and 48 patients were ≥65 years of age. The most frequent accompanying disorders were chronic obstructive pulmonary disease (22%) and malignancy (10.1%), and the most frequent risk factors were recent operation (14.1%) and immobilization (18.2%). Thrombi were most frequently observed in the right pulmonary artery (37.8%). In 31% of the patients, the thrombus was localized to the main pulmonary arteries. Immobile patients exhibited a higher proportion of thrombi in the main pulmonary arteries than mobile patients. The mean D-dimer level and the mean red blood cell distribution width in the patients with thrombi in the main pulmonary arteries were higher than those in the patients with thrombi in more distal pulmonary arterial branches.

CONCLUSION:

Significant associations of proximally localized thrombi with immobilization, the D-dimer levels, and the red blood cell distribution width were observed.     

Lead Exposure Is Related to Impairment of Aortic Elasticity Parameters

Arterial hypertension is one of the physical complications of chronic lead exposure. Hypertension has effects on aortic elastic properties. The aim of this study was to evaluate the aortic elastic properties in workers occupationally exposed to lead. Forty‐one workers who were exposed to lead and 39 healthy controls were included in the study. All patients underwent transthoracic echocardiography for detecting aortic elastic parameters. There were no differences in baseline characteristics between the lead‐exposure group and controls. Aortic strain (9.4%±4.5% vs 12.4%±4.2%, P=.004) and aortic distensibility (0.45±0.21 cm²/dyn vs 0.55±0.20 cm²/dyn, P=.046) were decreased in patients with lead exposure compared with controls. There was a negative significant weak correlation between aortic strain and (r=−0.294, P=.008) lead levels. There was no significant correlation between aortic distensibility and any other echocardiographic parameters. This study suggests that chronic exposure to lead is related to impairment of aortic elasticity parameters.

Lead contamination (such as that emitted from house paint, gasoline, batteries, and other sources) may cause a wide variety of body organ complications.¹ Despite the still manifested divergences of opinion, it seems that chronic exposure to lead represents a risk for arterial hypertension development. Functional changes within the arterial wall both in smooth muscles and the endothelium might result in arterial hypertension caused by chronic exposure to lead compounds.² Blood pressure (BP) was found to be increased in workers with blood lead concentrations of 7 μg/L on average.³ According to the World Health Organization (2000), the level of 400 μg/L is accepted as safe to avoid possible adverse health effects, but American Conference Governmental and Industrial Hygienists suggest an even lower value of <300 μg/L.Hypertension has effects on the aorta (decreased aortic distensibility and increased aortic stiffness). Aortic strain is a simple and useful parameter of transthoracic echocardiography. Some studies have suggested that aortic elastic parameters can be used as an independent predictor of all‐cause and cardiovascular mortality in hypertensive patients.⁴, ⁵ In previous studies, the relationship between lead exposure and arterial hypertension has been demonstrated, and findings suggest that arterial hypertension and organ complications of arterial hypertension are more frequent in workers with lead exposure. Until now, there have been no data on the effects of lead exposure on aortic stiffness. The aim of this study was to evaluate the effects of lead on aortic elasticity parameters.     

The Effect of Strut Allograft and Its Position on Vancouver Type B1 Periprosthetic Femoral Fractures: A Biomechanical Study

The aim of this study is to assess the biomechanical advantage of adding strut allograft and the effect of its position on the construct in Vancouver type B1 fractures. Fifteen forth-generation synthetic femurs were used and created a fracture model at the tip of prosthesis, and subsequently fixated with a lateral plate only, lateral plate and medial strut, lateral plate and anterior strut. Rotational and axial tests were performed. In all loading tests, the plate with medial strut group was stiffer than the other constructs and had higher failure load values and had less displacement in the fracture site. A combination of a plate with a medial strut allograft provides more mechanical stability on periprosthetic femoral fractures near the tip of a total hip arthroplasty.