Reno-vascular hypertension in childhood: a nationwide survey

Renovascular disease accounts for 8–10% of all cases of paediatric hypertension, whereas, in adults, its incidence is approximately 1%. The Turkish Paediatric Hypertension Group aimed to create the first registry database for childhood renovascular hypertension in Turkey. Twenty of the 28 paediatric nephrology centres in Turkey responded to the survey and reported 45 patients (27 girls, 18 boys) with renovascular hypertension between 1990 and 2005. The age at presentation ranged from 20 days to 17 years. The mean blood pressure at the diagnosis was 169/110 mmHg. Chief complaints of symptomatic patients were headache (38%), seizure (18%), epistaxis (4%), growth retardation (4%), cognitive dysfunction (4%), polyuria (2%), palpitation (2%), and hemiplegia (2%). Renovascular hypertension was found incidentally in 11 children. The diagnosis of renovascular hypertension was established with conventional angiography in 39 patients, MR angiography in three, CT angiography in two, and captopril diethylene triamine penta-acetic acid (DTPA) scintigraphy in one patient. Twenty-one children had bilateral renal artery stenosis and 24 had unilateral renal artery stenosis. Of these, 14 (31%) had fibromuscular dysplasia; 12 (27%) Takayasu’s arteritis; six (13%) neurofibromatosis; two (5%) Williams syndrome; one (2%) Kawasaki disease; one (2%) mid-aortic syndrome; one (2%) extrinsic compression to the renal artery, and eight (18%) unspecified bilateral renal artery stenosis. Hypertension was controlled with antihypertensive drugs in 17 patients. Percutaneous transluminal angioplasty (PTRA) or surgery had to be performed in 28 patients: PTRA in 16 patients, PTRA + surgery in one patient and surgery in 11 patients (four nephrectomies). The importance of vasculitic disease, especially Takayasu’s arteritis, should not be underestimated in children with renovascular hypertension.     

Association of macroscopic gut inflammation with disease activity, functional status and quality of life in ankylosing spondylitis

The aim of this cross-sectional study was to evaluate the frequency of intestinal inflammation and its association with disease activity, functional status and quality of life in patients with ankylosing spondylitis (AS). A total of 25 patients with AS had undergone ileocolonoscopy and concomitant histological study. Clinical and demographical parameters, BASDAI, BASFI, and SF-36 scores were compared between patients with and without macroscopic gut inflammation (MGI). Colonoscopic study revealed MGI in 9 patients and macroscopically normal gut mucosa in 16 patients. On histological examination, of 25 patients 20 had gut inflammation, mostly in ileum. BASDAI score was higher (P < 0.05), SF-36 pain and physical scores, and chest expansion measurement were lower (P = 0.00, P = 0.01, P = 0.01), duration of morning stiffness was longer (P = 0.01) in patients with MGI. Serum C-reactive protein, erytrocyte sedimentation rate levels were similar between groups (P > 0.05). There is high prevalence of histological gut inflammation in AS patients. More active disease should suggest gut inflammation in AS patients.     

Relation between intracranial volume and the surface area of the foramen magnum

Several investigators have estimated the intracranial volume (ICV) in the past which indirectly reflects the brain volume. Most of these studies have been made on the dry skulls using linear dimensions, packing methods or occasionally radiological methods. It is also reported that the etiology of cerebellar tonsillar herniation is closely related to the size of the foramen magnum (FM). In the present study the ICVs have been estimated in 28 dry skulls using filling water method and the surface area of FMs were measured planimetry method. The estimated mean ICV was 1,311 +/- 133 cm. Surface area of FM was 760 +/- 144 mm. Antero-posterior and lateral direct roentgenograms of the skulls were also taken and the width, height and length (WHL) of the skull were measured by means of the cephalometry on radiograms. The relationship between the ICV, WHL and surface area of FM were analyzed statistically. The ICV, WHL and surface area of FM was correlated well (r = 0.271, P < 0.005; r = 0.265, P < 0.005, respectively) and a regression formula was proposed. Our results showed that skulls with larger ICV and WHL have larger FM surface area. We also suggested a regression formula that could be used to predict the surface area of FM regarding to the ICV and WHL values. In the next step, we took roentgenograms of skulls and obtain ICV measuring the width, length and height of skulls by means of cephalometry and investigate the relation between the findings of cephalometry and surface area of FM. The cephalometry could apply on living subjects and, thereby, our findings could provide some data to evaluate the etiology of Arnold Chiari malformation and achondroplasia for living subjects.     

Synthesis and Biological Evaluation of Novel Bromophenol Derivatives as Carbonic Anhydrase Inhibitors

Here, we provide an alternative synthesis of the natural bromophenol 3,4‐dibromo‐5‐(2,3‐dibromo‐4,5‐dihydroxybenzyl)‐6‐(ethoxymethyl)benzene‐1,2‐diol ( 3 ) and the first synthesis of (4,5‐dihydroxy‐2‐methylphenyl)(3,4‐dihydroxyphenyl)methanone ( 18 ) and its brominated derivatives 19 – 21 . The compounds were characterized and tested against the two most studied members of the pH regulatory enzyme family, carbonic anhydrase (CA). The inhibitory potencies of the novel compounds and two natural bromophenols 2 , 3 were analyzed at the human isoforms hCA I and hCA II as targets and the K_I values were calculated. The K_I values of the novel compounds were measured in the range of 13.7–32.7 µM for the hCA I isozyme and 0.65–1.26 µM for the hCA II isozyme. The structurally related compound 14 was also tested in order to understand the structure–activity relationship, and the clinically used sulfonamide acetazolamide (AZA) was tested for comparison reasons. All of the compounds exhibited competitive inhibition with 4‐nitrophenylacetate as substrate. The compounds showed strong inhibitory activity against hCA I, being more effective as compared to the clinically used AZA (K_I: 36.2 µM), but rather less activity against hCA II.     

Relationship between Structural and Functional Assessment of the Visual System in Mildly Disabled Relapsing-Remitting Multiple Sclerosis Patients

Studies that explored the anterior visual pathway in the patients with multiple sclerosis (MS) have demonstrated contradictory results about the correlation between structural and functional status of optic nerve and retina. We aimed to investigate the functional and structural findings in our cohort of mildly disabled relapsing-remitting MS patients. A total of 134 eyes (80 eyes of the patients with MS and 54 eyes of the control group) were investigated. Eyes of MS patients were divided into two groups—as eyes with history of optic neuritis (ON group) and without history of optic neuritis (NON group). Ophthalmological investigation including visual evoked potentials, standard automated perimetry, and optical coherence tomography were performed for all participants. Retinal and macular thicknesses were significantly decreased in ON and NON groups compared with controls. Also, visual evoked potential latencies and visual field loss were worse in the both MS groups compared with control group. We did not find any correlation between visual evoked potentials and retinal or macular thickness values but visual field parameters were correlated between retinal and macular layer loss in the NON group. According to our results and some previous studies, although both functional and structural changes were detected in patients with MS, functional status markers do not always show parallelism (or synchrony) with structural changes, especially in eyes with history of optic neuritis.     

Volumetric evaluation of the relations among the cerebrum,cerebellum and brain stem in young subjects: a combination of stereology and magnetic resonance imaging

The Cavalieri estimator using a point grid is used to estimate the volume of three-dimensional structures based on two-dimensional slices of the object. The size of the components of intracranial neural structures should have proportional relations among them. The volume fraction approach of stereological methods provides information about volumetric relations of the components of structures. The purpose of our study is to estimate the volume and volume fraction data related to the cerebrum, cerebellum and brain stem. In this study, volume of the total brain, cerebrum, cerebellum and brain stem were estimated in 24 young Turkish volunteers (12 males and 12 females) who are free of any neurological symptoms and signs. The volume and volume fraction of the total brain, cerebrum, cerebellum and brain stem were determined on magnetic resonance (MR) images using the point-counting approach of stereological methods. The mean (+/-SD) total brain, cerebrum and cerebellum volumes were 1,202.05 +/- 103.51, 1,143.65 +/- 106.25 cm(3) in males and females, 1,060.0 +/- 94.6, 1,008.9 +/- 104.3 cm(3 )in males and females, 117.75 +/- 10.7, 111.83 +/- 8.0 cm(3) in males and females, respectively. The mean brain stem volumes were 24.3 +/- 2.89, 22.9 +/- 4.49 cm(3) in males and females, respectively. Our results revealed that female subjects have less cerebral, cerebellar and brain stem volumes compared to males, although there was no statistically significant difference between genders (P > 0.05). The volume ratio of the cerebrum to total brain volume (TBV), cerebellum to TBV and brain stem to TBV were 88.16 and 88.13% in males and females, 9.8 and 9.8% in males and females, 2.03 and 2.03% in males and females, respectively. The volume ratio of the cerebellum to cerebrum, brain stem to cerebrum and brain stem to cerebellum were 11.12 and 11.16% in males and females, 2.30 and 2.31% in males and females, 20.7 and 20.6% in males and females, respectively. The difference between the genders was not statistically significant (P > 0.05). Our results revealed that the volumetric composition of the cerebrum, cerebellum and brain stem does not show sexual dimorphism.     

Combined transbronchial needle aspiration and PET/CT for mediastinal staging of lung cancer

In this study, we aimed to evaluate the performance of transbronchial needle aspiration (TBNA) combined with positron emission tomography/computed tomography (PET/CT) for the staging of lung cancer. Twenty-five patients having lymphadenopathies greater than 1 cm on thorax CT and maximum standardized uptake value (SUVmax) ≥ 2.5 on PET/CT were included in this prospective study performed between March 2006 and March 2008. Forty-three lymphnode stations were sampled by using TBNA. Surgical histology, as confirmed by mediastinoscopy, was accepted as the "gold standard". The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of combined TBNA and PET/CT for correct lymph node staging were 67%, 100%, 100%, 76% and 84%; respectively. The initial clinical staging was downstaged after TBNA in 13/19 (69%) patients with adequate TBNA samples, whereas staging was correct in 17/19 (89%) patients assessed by combined TBNA and PET/CT. Staging was completed by TBNA, without mediastinoscopy, in 6/25 (24%) patients. Among the clinical factors that were assessed, only the PET SUVmax was associated with positive TBNA results [odds ratio (OR) 1.27, 95% CI 1.004-1.61, p= 0.046]. A PET SUVmax ≥ 5 was eleven times more likely in patients with positive TBNA results [OR 10.68, 95% CI 1.91-59.62, p< 0.01]. In conclusion, the combination of TBNA with PET/CT increased the sensitivity of TBNA. Combined TBNA and PET/CT may also allow adequate mediastinal staging of lung cancer in most patients with enlarged lymph nodes, and reduce the need for mediastinoscopy. The SUVmax cut off point for a positive TBNA result was ≥ 5.     

Temperament and character personality profile in relation to suicide attempts in patients with schizophrenia

Introduction

Schizophrenia is associated with a significant risk of suicide, and suicide still remains one of the main causes of death in schizophrenic patients. Beside classic risk factors for suicidality, temperament and character traits have been researched and considered as risk factors for suicidal behavior in recent years.

Method

Subjects were 94 patients with schizophrenia who were under treatment. All patients were in a stable phase of the illness. Patients with lifetime suicide attempt (n = 46) and without suicidal attempt (n = 48) were compared with each other in terms of temperament and character traits by using the Temperament and Character Inventory.

Results

Harm avoidance and persistence scores were higher in suicidal schizophrenic patients compared with nonsuicidal schizophrenic patients. The scores of self-directedness and cooperativeness were lower in suicidal schizophrenic patients compared with nonsuicidal schizophrenic patients. These 4 variables remained significant predictors of lifetime suicidal attempts in a logistic regression model.

Conclusion

To the best of our knowledge, the present study is the first that specifically compares schizophrenic patients with and without suicidal behavior by the Cloninger temperament and character model. Our data indicate that schizophrenic patients will show a greater risk for suicide according to certain personality configurations. However, to establish causal relationships between personality and suicidality in schizophrenia, longitudinal studies are warranted within a multifactorial interactive framework of biologic and clinical variables.     

Frequency of functional gastrointestinal disorders in children with familial Mediterranean fever

Clinical Rheumatology - Familial Mediterranean fever (FMF) is characterized by self-limiting fever episodes usually accompanied by serositis, arthralgia, and arthritis. Functional gastrointestinal...     

Changes in soluble tumor necrosis factor receptor type 1 levels and early renal function decline in patients with diabetes

The relationship between serial changes in soluble tumor necrosis factor receptor type 1 (TNFR1) levels and an early decline in estimated glomerular filtration rate (eGFR) decline remains to be defined. We found that in patients with an early decline in renal function (n = 30), soluble TNFR1 values increased (2,595 ± 683 vs 3,596 ± 1,203 pg/mL, P < 0.001) as eGFR decreased (89 ± 1 vs 51 ± 2 mL/min/1.73m², P < 0.001) over an 8‐year period. In contrast, there were no significant changes in soluble TNFR1 levels in patients with stable renal function (n = 17). In a multilevel mixed effects regression model, changes in soluble TNFR1 levels were found to be independently associated with eGFR decline (Z = ?4.31, P < 0.001). An early decline in eGFR is associated with an increase in soluble TNFR levels; however, the factors driving this increase and the possible pathological role that soluble TNFR1 plays in progressive diabetic kidney disease remain to be determined.