HLA class 1 associations in Henoch Schonlein purpura: increased and decreased frequencies

Henoch Schonlein purpura (HSP) is the most common vasculitis of childhood. Susceptibility to HSP and associated clinical heterogeneity in HSP may be conferred by a number of genetic loci, including the major histocompatibility complex. We aimed to investigate the implications of the human leukocyte antigen (HLA) class 1 alleles in susceptibility to HSP and determine the possible associations with renal, gastrointestinal (GI), and joint manifestations of the disease. 110 children with HSP (66 boys, 44 girls) and 250 unrelated healthy controls were enrolled in the study. The mean age was 8.65 ± 3.59 years. HSP was diagnosed on the basis of clinical and laboratory data according to the American College of Rheumatology classification. The diagnosis was supported with skin and/or kidney in most of the patients. Clinical and laboratory findings revealed: skin involvement in 110 (100%), joint manifestations in 82 (74.5%), GI symptoms in 58 (52.7%), and hematuria and/or proteinuria in 36 (32.7%) patients. HLA class 1 alleles were identified by DNA amplification, hybridized with specific primer sequences. Comparison of frequencies between patients and controls were made by using the Fisher’s exact test. Odds ratio (OR) was used as the measure of association. HLA A2, A11, and B35 antigens showed an increased risk for predisposition to HSP (OR = 1.714, 95%CI = 1.088–2.700, p = 0.020; OR = 2.185, 95%CI = 1.289–3.703, p = 0.003; and OR = 2.292, 95%CI = 1.451–3.619, p = 0.000, respectively), while HLA A1, B49, and B50 antigens revealed decreased risk for predisposition to HSP (OR = 4.739, 95%CI = 1.828–12.345, p = 0.001; OR = 3.268, 95%CI = 0.955–11.236, p = 0.047; and OR = 7.462, 95%CI = 0.975–55.555, p = 0.024, respectively). Considering the renal involvement and severity of proteinuria, there was no association with HLA class 1 alleles. Our results suggest that the increased frequency of HLA A2, A11, and B35 alleles in unselected pediatric HSP patient population and miscarrying of HLA A1, B49, and B50 could be considered as a risk factor for susceptibility to HSP.     

Melatonin protects myocardium from ischemia-reperfusion injury in hypertensive rats: role of myeloperoxidase activity

Increased levels of reactive oxygen species, alterations in nitric oxide synthesis, and increased migration of neutrophils to the ischemic tissue play an important role in the pathophysiology of myocardial ischemia-reperfusion (IR) injury. In this study, we have evaluated the effects of melatonin on myeloperoxidase (MPO) activity, tissue glutathione (GSH), lipid peroxidation levels, and blood pressure in L-NAME-induced hypertensive rats with or without IR. NOS inhibitor L-NAME was administrated before inducing cardiac ischemia for 15 days intraperitoneally. For the cardiac ischemia, the left coronary artery was ligated for 30 min, and reperfusion was performed for 120 min after the ischemia. L-NAME treatment in non-ischemic animals increased blood pressure and lipid peroxidation, and decreased glutathione level in myocardial tissue significantly as compared with non-L-NAME-treated animals. Melatonin reversed L-NAME-induced blood pressure elevation and oxidative changes. Cardiac IR increased MDA levels and MPO activity and decreased GSH levels as compared with non-ischemic animals. L-NAME treatment did not change in IR-induced MDA and GSH levels as compared with ischemic control animals. However, MPO activity was significantly higher than control ischemic animals. MDA levels and MPO activity resulting from ischemic injury in melatonin-treated animals were significantly less than L-NAME-treated animals. Taken together-the ischemic and non-ischemic control and melatonin-treated animals-this study shows that neutrophil migration plays an important role on the development of ischemic injury in hypertensive rats.     

Using two-holed needles for both arterial and venous accesses to the arteriovenous fistula to improve flow during hemodialysis

This prospective study compared methods using both arterial and venous needles with back eyes with those using only arterial needle with back eye for arteriovenous fistula cannulation. Sixty-one patients receiving hemodialysis (HD) via an arteriovenous fistula were evaluated. All patients underwent arteriovenous fistula puncture using only arterial needle with back eye in first 3 months and both arterial and venous needles with back eyes in following 3 months. Arterial and venous pressures, blood flow velocities, total blood volume cleared, and Kt/V values were compared. Mean blood flow velocity, arterial pressure, Kt/V, and cleared total blood volume values were higher and venous pressure was lower in patients who underwent cannulation using both needles with back eyes than in those with only the arterial needle with back eye. For arteriovenous fistula cannulation, using both arterial and venous needles with back eyes provides adequate HD more successfully.     

Resveratrol, a Red Wine Constituent Polyphenol, Protects Gastric Tissue Against the Oxidative Stress in Cholestatic Rats

This experimental study was designed to determine the effects of resveratrol on the level of malondialdehyde (MDA), reduced glutathione (GSH), and nitric oxide (NO) in gastric tissue after bile duct ligation (BDL). Swiss albino rats were divided into three groups: Group 1, sham (n = 7); Group 2, BDL (BDL only group; n = 7); and Group 3, BDL plus resveratrol (n = 7). Animals in the resveratrol group were treated with 10 mg/kg resveratrol (i.p.) once a day throughout 28 days. In the resveratrol group, levels of MDA and NO in gastric tissue were significantly lower than in the BDL-only group (P < 0.001). The level of GSH in the resveratrol group was significantly higher than in the BDL-only group (P < 0.001). The present study demonstrates that intraperitoneal administration of resveratrol maintains antioxidant defenses and reduces oxidative gastric damage. This effect of resveratrol may be useful to preserve gastric tissue under oxidative stress due to cholestasis.     

Asymmetric dimethylarginine level in hyperglycemic gestation

We aimed to evaluate plasma asymmetric dimethylarginine (ADMA) concentrations and its relation with insulin sensitivity/resistance indices in pregnant women with different degrees of carbohydrate intolerance. This study included a two step approach; 50 g glucose challenge test (GCT) followed by 100 g oral glucose tolerance test (OGTT) was used for diagnosis of carbohydrate intolerance within 24-28th weeks of gestation. Pregnant women with positive GCT but negative OGTT (AGCT group, n=30) and gestational diabetics (GDM group, n=58) were compared to healthy pregnant controls (n=50). Plasma ADMA concentration and its relationship with glucose and insulin levels and insulin sensitivity/resistance indices (HOMA-IR, QUICKI, ISIOGTT) were evaluated. Both AGCT and GDM groups were found to have similarly higher plasma ADMA levels than control subjects (3.60±1.21; 4.00±1.70; 2.65±0.82 μmol/l, respectively, P=0.001). ADMA was significantly but slightly correlated with insulin sensitivity/resistance indices and moderately correlated with 2-h insulin level. The 2-h insulin value of the OGTT was the independent influencing constant for ADMA (R=0.57, P=0.0001). In conclusion, plasma asymmetric dimethylarginine level was higher in cases with abnormal glucose challenge test but normal OGTT as well as in gestational diabetics, compared to pregnant women with normal glucose tolerance. The elevated ADMA level in pregnant women with carbohydrate intolerance may possibly be due to elevated insulin level.     

Serum IGF-1 and IGFBP-3 levels in healthy children between 0 and 6 years of age

Objective: Along with growth hormone (GH) levels, measurements of serum insulin-like growth factor-1 (IGF-1) and IGF-binding protein-3 (IGFBP-3) are used in the diagnosis of GH deficiency and in monitoring the efficacy and safety of long-term GH treatment. The purpose of the present study was to establish reference values for serum IGF-1 and IGFBP-3 in healthy Turkish children less than 6 years of age.Methods: This study was designed as a multicenter project. Five hundred sixty-seven healthy children younger than 6 years of age from different geographical regions of Turkey, with weight and height values between the 10th and 90th percentiles according to the national standards were included in the study. In addition to anthropometric parameters, serum IGF-1 and IGFBP-3 levels were measured in all subjects.Results: Although not statistically significant, the serum IGF-1 levels in infants at age 6 months were lower than those in infants at age 3 months. The IGF-1 levels showed a slow increase with age. Serum IGF-1 levels were lower in girls as compared to boys only at age 6 months. No correlation was found between either serum IGFBP-3 levels and body mass index (BMI) or serum IGFBP-3 and weight and height standard deviation scores (SDS). A weak correlation was observed between serum IGF-1 and IGFBP-3 concentrations.Conclusions: The age- and gender-specific reference values for serum IGF-1 and IGFBP-3 reported in this study will aid in the diagnosis of GH deficiency and in the monitoring of children receiving GH treatment.Conflict of interest:None declared.     

Takayasu arteritis in children

OBJECTIVE: To retrospectively evaluate the clinical features, angiographic findings, and outcomes of children with Takayasu arteritis (TA) in Turkey. METHODS: Clinical, laboratory, and angiographic findings and outcomes of 19 children with TA were evaluated with a retrospective chart review. The criteria for inclusion were those proposed by the American College of Rheumatology. RESULTS: Mean followup period was 35.89 +/- 40.75 months (range 1-168, median 30). There were 14 girls and 5 boys. The mean age at diagnosis was 12.84 +/- 2.69 years (range 8-17, median 13). The most common complaints on admission were headache (84%), abdominal pain (37%), claudication of extremities (32%), fever (26%), and weight loss (10%). One patient presented with visual loss. Examination on admission revealed hypertension (89%), absent pulses (58%), and bruits (42%). Angiography revealed type I in 13 patients (aortic arch, descending thoracic, and abdominal aorta), type II in 4 (descending thoracic aorta and abdominal aorta), and type IV in 2 (diffuse aortic and pulmonary artery). The most commonly involved vessels were the renal, subclavian, and carotid arteries. All patients received corticosteroid therapy, and further immunosuppressive therapy was added in 15 patients. Fourteen of the 17 hypertensive patients had renal artery stenosis and 9 underwent surgery or interventional therapy. Thoraco-abdominal bypass graft was performed in 2 patients who had abdominal aortic stenosis. CONCLUSION: Hypertension is the most common clinical feature at presentation. Corticosteroid and immunosuppressive therapy was effective in the control of disease activity. Angioplasty or bypass grafting was successfully performed when needed.     

The effect of growth hormone treatment on bone mineral density in prepubertal girls with Turner syndrome: a multicentre prospective clinical trial

Background Patients with Turner syndrome (TS) are treated with GH to increase adult height. Although it is well established that GH promotes longitudinal bone growth, the effects of GH treatment on bone density are less clear. Objective To determine how GH treatment affects trabecular bone mineral density (BMD) in girls with TS at prepubertal ages in a prospective multicentre study. Patients and method Twenty‐two patients with TS in the prepubertal period with a mean age of 9·8 ± 2·5 (range 3·6–12·8) years were included in the study. All girls with TS underwent measurement of areal BMD using dual‐energy X‐ray absorptiometry (DXA) to obtain pretreatment anteroposterior (AP) lumbar spine values at L1–L4. Patients received GH (Genotropin) subcutaneously for 1 year at a dose of 0·05 mg/kg/day. Height and weight were measured at 3‐monthly intervals. The AP lumbar spine areal BMD was remeasured using the same technique after 1 year of treatment. Lumbar spine BMD Z‐scores and volumetric BMD (vBMD) Z‐scores were calculated using national standards. Results The height SDS of our cases showed a significant increase with GH therapy. The pretreatment lumbar spine (L1–L4) BMD Z‐score was –1·2 ± 1·2 SD and the vBMD Z‐score was –0·8 ± 1·6 SD. There were no significant changes in these values after 1 year of GH treatment. Prepubertal TS girls more than 11 years of age had lower vBMD Z‐scores (–1·7 ± 1·7 SD) than the girls aged less than 11 (–0·1 ± 1·0 SD) (P < 0·05) at the onset of therapy. No significant changes were observed in these values after 1 year of GH therapy. Conclusions Osteopaenia becomes apparent in prepubertal TS patients as they reach pubertal age. BMD evaluation may be necessary in these prepubertal TS girls at diagnosis. Short‐term GH therapy in these TS patients does not have a significant effect on bone density when measured at a site with a predominance of trabecular bone.     

Psychiatric comorbidity in medication overuse headache patients with pre-existing headache type of episodic tension-type headache.

BACKGROUND: Medication overuse headache (MOH) mostly evolves from migraine and episodic tension-type headache (ETTH). Chronic tension-type headache (CTTH) is another headache type that evolves over time from ETTH. It is well known that psychiatric comorbidity is high in MOH patients. AIM: To investigate the frequency of psychiatric comorbidity, and the intensity of depression and anxiety in MOH patients evolving from ETTH and to compare results with CTTH patients and MOH patients evolving from migraine. METHODS: Twenty-eight CTTH (Group C) and 89 MOH patients were included into the study. MOH patients were divided into two groups according to their pre-existing headache types: MOH patients with pre-existing ETTH (Group E, n = 31), and with pre-existing migraine (Group M, n = 58). All patients were interviewed with a psychiatrist and SCID-CV and SCID-II were applied. Beck Anxiety Inventory and Beck Depression Inventory scales were also performed. RESULTS: Eleven patients (39.3%) in Group C, 21 patients (67.7%) in Group E, and 31 patients (53.7%) in Group M were diagnosed to have comorbid psychiatric disorders. The psychiatric comorbidity was found significantly higher in Group E than Group C. In Group E, mood disorders were found significantly higher, but the difference between the two groups with regard to anxiety disorders was insignificant. Mean depression scores were significantly higher in Group E than Group C. The mostly diagnosed type was obsessive-compulsive personality disorder in all the three groups, and was statistically significant in Group M than Group C. CONCLUSION: Psychiatric comorbidity in MOH patients with pre-existing ETTH is common as in those with pre-existing migraine headache and MOH with regard to developing psychiatric disorders should be interpreted as a risk factor in chronic daily headache patients.     

A Behçet's disease patient with intracardiac thrombus, pulmonary artery aneurysms complicating recurrent pulmonary thromboembolism

Intracardiac thrombus and pulmonary embolism is a very rare manifestation of Beh?et's disease. A twenty-years-old man was admitted to hospital due to dyspnea, haemoptysis, fever and partially loss of vision. On dynamic thorax computed tomography (CT), there was aneurysmatic dilatation and thrombus in bilateral pulmonary artery segments and also findings of pulmonary thromboembolism. A diagnosis of Beh?et's disease was made based on his clinical course and radiological findings. During treatment, the patient was admitted two times to the hospital because of recurrent pulmonary thromboembolism. At the 10th months of follow up, partially dissolution of the thrombi and pulmonary defects were observed and right ventricular thrombus was revealed by dynamic thorax CT. On a follow up period of 16 months the patient is still under treatment and doing well. We present this case because Beh?et's disease is a rarely considered cause of recurrent pulmonary embolism and intracardiac thrombus which is seen under treatment.