Systemic onset juvenile rheumatoid arthritis presenting with absence of B lymphocytes

Juvenile rheumatoid arthritis (JRA) is a complex disease involving the interactions of several cell populations with different mediators. Herein, we report a five-year-old girl with systemic-onset JRA. At admission, peripheral blood flowcytometric analysis showed the percentages of CD19⁺ and CD20⁺ B cells were <1%. These values returned to normal on the tenth day of steroid treatment. This is the first report of JRA presented with absence of B lymphocytes in the literature and suggested that lymphocytes subset analysis could change with treatment in patients with JRA. Different clinical signs and symptoms reflecting aspects of JRA are critical for the etiology of the disease and to identify new strategies for treatment.     

Takayasu arteritis: association with focal segmental glomerulosclerosis

We herein report on a 29-year-old woman who presented with edema and proteinuria. In light of her blood pressure differences and, finally, with the angiographic findings, Takayasu arteritis (TA) was the diagnosis. Renal biopsy showed focal segmental glomerulosclerosis (FSGS) and other possible etiologic alternatives were excluded. This was a very rare association and we could not find any other cases reported on TA accompanied with FSGS before. A poor treatment response was observed at the end of a 1-year therapy.     

Oxidative status in rheumatoid arthritis

The insufficiency of antioxidant defense systems and the acceleration of the oxidative reactions can be results of the pro-oxidant/antioxidant imbalance in rheumatoid arthritis (RA). The aim of our study was to investigate the changes in oxidant status by measuring two different parameters; one was the level of malondialdehyde (MDA) as an index of lipid peroxidation and the other was total oxidative status; we could then compare our results with the antioxidant status, superoxide dismutase (SOD) enyzme activities. All were assessed in 22 patients with active RA and 18 age- and gender-matched control subjects. While serum MDA levels were significantly increased in patients with RA compared to the control group (p<0.03), the total oxidative status levels were decreased in patients with RA compared to the control group (p<0.008), and serum SOD activities did not show any statistical difference between the two groups. In conclusion, the increased MDA levels in our study may be important as a marker but are not sufficient to conclude that there was an increase in oxidative stress in RA patients because supporting results were not obtained from SOD and oxidative status measurements. These results give further support to the concept of oxygen free radicals playing a role in the pathogenesis of chronic inflammatory disorders, but we also consider that there is a more complex relationship than has been assumed. We think that further studies are needed to clarify these conflicting results.     

Immunoglobulin E: a new diagnostic clue for Behçet’s disease?

Behçet’s disease (BD) is a multisystemic inflammatory disorder of unknown etiology. Hyperimmunoglobulinemia has been demonstrated in BD; however, its clinical importance has not yet been discovered. In a few reports, an association between BD and plasma immunoglobulin E (IgE) has been noted. In this study, we had three objectives: (1) to investigate plasma levels of IgE and their association with levels of acute phase reactants, (2) to determine whether treatment has any effect upon IgE levels, and (3) to assess whether higher serum levels of IgE provide a clue for the diagnosis of BD. Fifty-two patients with BD, without any accompanying disease or condition that might lead to a rise in IgE, were compared with 51 age-matched healthy controls. Plasma levels of IgE, IgA, IgG, and IgM were analyzed by means of nephelometric analysis. Results showed that among the 52 patients, 23 (44%) had IgE levels that were higher than the upper limits of normal. IgE was not correlated with age, erythrocyte sedimentation rate, or C-reactive protein (p=0.59, p=0.57, and p=0.757, respectively). These relationships remained nonsignificant when multiple regression analysis was performed, with R ² of 0.187 and p=0.057. Moreover, treatment of patients with elevated IgE did not produce any predictable IgE response. We conclude that Behçet’s patients commonly exhibit elevated plasma IgE levels. However, elevated IgE levels are not correlated with levels of acute phase reactants or disease activity. We suggest that IgE levels might be an independent diagnostic clue in Behçet’s patients.     

Childhood vasculitides in Turkey: a nationwide survey

Aim

The aims of this study were to evaluate the characteristics of childhood vasculitides and to establish the first registry in Turkey, an eastern Mediterranean country with a white population.

Patients and methods

A questionnaire was distributed to the main referral centers asking for the registration of the Henoch-Schönlein purpura (HSP) patients in the last calendar year only and 5 years for other vasculitides. Demographic, clinical, and laboratory data were assessed.

Results

Vasculitic diseases were registered from 15 pediatric centers. These centers had a fair representation throughout the country. In the last calendar year, incidences were as follows: HSP 81.6%, Kawasaki disease (KD) 9.0%, childhood polyarteritis nodosa (C-PAN) 5.6%, Takayasu arteritis (TA) 1.5%, Wegener’s granulomatosis 0.4%, and Behçet disease 1.9%. There was no clear gender dominance. The mean age was 11.05±4.89 years. Acute phase reactants were elevated in almost all, highest figures being in C-PAN. Renal involvement was present in 28.6% of HSP and 53% of the C-PAN patients. Abdominal aorta was involved in all TA patients. Among the C-PAN patients, 25% had microscopic PAN with necrotizing glomerulonephritis; antineutrophil cytoplasmic antibody was positive in those who were studied. Among the patients, 12.5% and 15% had classic PAN and cutaneous PAN, respectively. The remaining majority were classified as systemic C-PAN diagnosed with biopsies and/or angiograms demonstrating small to midsize artery involvement. The overall prognosis was better than reported in adult series.

Conclusion

This is the largest multicenter study defining the demographic data for childhood vasculitides. The distribution of childhood vasculitides was different in our population where KD is much less frequent, whereas HSP constitutes an overwhelming majority. C-PAN was more frequent as well.     

The value of magnetic resonance cholangiography in the preoperative assessment of living liver donors

ObjectiveThe purpose of this study was to investigate the ability of magnetic resonance cholangiography (MRC) in the depiction of biliary anatomy of living liver donors by using intraoperative cholangiography (IOC) as a gold standard.Materials and MethodsBetween 2004 and 2006, 86 potential adult-to-adult living donor liver transplantation donors had preoperative MRC at our institution. Of these, 24 potential donors were excluded due to various clinical factors. A total of 62 of these individuals were selected for liver donation and included in the study. MRC was performed on a 1.5-T scanner with breath-hold, rapid acquisition with relaxation enhancement (RARE) sequence with half-Fourier acquisition (HASTE; Siemens) and free-breathing, three-dimensional turbo spin-echo sequence with respiratory triggering. Thin- and thick-slab imaging techniques were employed with half-Fourier RARE MRC. IOC was performed in all 63 cases. The images of IOC and MRC were classified according to a modified Huang classification, independently. The results of the MRC were then compared with the IOC results.ResultsIOC was used as the reference standard; a total of 43 (69.3%) liver donors were considered to have normal biliary anatomy, whereas 19 (30.7%) were considered to have variants of biliary anatomy. Compared with IOC, MRC correctly revealed biliary anatomy in 59 of 62 (95.1%) donors. The sensitivity, specificity, positive predictive value and negative predictive value of MRC in distinguishing normal and any type of variant biliary anatomy were 84.2%, 100%, 100%, and 93.4%, respectively.ConclusionMRC is an effective imaging technique for the preoperative evaluation of the biliary anatomy in living liver donors. However, MRC and IOC should be considered complementary to one another in order to avoid complications.     

An unusual cause of cholecystitis： Heterotopic pancreatic tissue in the gallbladder

Gallbladder localization of heterotopic pancreas （HP） is uncommon and very rarely gives rise to symptoms. Herein we report a case of HP found in the gallbladder neck presented with signs and symptoms of cholecystitis. The patient was a 40-year old male, suffering from epigastric pain, abdominal fullness and fever. On physical examination, the right upper abdomen was tender with a positive Murphy＇s sign. Ultrasonographic examination showed a hydropic gallbladder without stones and he underwent a cholecystectomy. Pathological examination revealed an intramural nodule （9 ram） in the neck region which is consisted of acini, ducts and islet cells of an aberrant pancreatic tissue. Although HP is encountered rarely in the gallbladder and is found incidentally during pathological studies, this case emphasizes that HP might cause symptoms and present clinically as cholecystitis. For this reason, in patients presenting with symptomatic gallbladder diseases, including cholecystitis without any other pathology, HP should be taken into consideration before it is diagnosed as ＂idiopathic＇：     

Is familial Mediterranean fever (FMF) common in patients with negative appendectomy?

Objectives

Familial Mediterranean fever (FMF) is an autosomal-recessive disease characterized by recurrent attacks of fever with serositis. Differential diagnosis of a FMF abdominal attack with acute abdomen is difficult. Acute appendicitis is the most common cause of acute abdominal pain that requires surgical treatment. The aim of this study was to investigate frequency of FMF in patients with negative appendectomy.

Methods

We assessed 278 patients (female/male 127/151) who were operated with preoperative diagnosis of acute appendicitis. In 250 of the patients, definitive diagnosis of acute appendicitis was established by histo-pathological examination. Patients with negative appendectomy were assessed for FMF by rheumatologist.

Results

Negative appendectomy was detected in 28 patients (M/F 5/23, mean age 25.3 ± 8.4 years). Negative appendectomy ratio was 10.1 %. Among 28 patients two had FMF (7.7 %).

Conclusions

FMF were established in 7.7 % of patients with negative appendectomy. Our study suggests patients having negative appendectomy should be evaluated for FMF. Further large sample studies are needed to define the real prevalence of FMF among negative appendectomy patients.     

Fasting, non-fasting glucose and HDL dysfunction in risk of pre-diabetes, diabetes, and coronary disease in non-diabetic adults

We determined in non-diabetic persons the risk of fasting and non-fasting glucose levels for pre-diabetes, diabetes, and coronary heart disease (CHD), including the roles of serum C-reactive protein (CRP) and HDL cholesterol, and delineated risk profiles of the pre-diabetic states. Over 7¼ years, 2,619 middle-aged Turkish adults free of diabetes and CHD were studied prospectively. Using different serum glucose categories including impaired fasting glucose (IFG, 6.1–6.97 mmol/L) and impaired glucose tolerance (IGT), outcomes were analyzed by Cox regression. IFG was identified at baseline in 112 and IGT in 33 participants. Metabolic syndrome components distinguished individuals with IFG from those with normoglycemia. Participants with IGT tended to differ from adults in normal postprandial glucose categories in regard to high levels of triglycerides, apoA-I, and CRP. Diabetes risk, adjusted for sex, age, waist circumference, CRP, and HDL cholesterol, commenced at a fasting 5.6–6.1 mmol/L threshold, was fourfold at levels 6.1–6.97 mmol/L. Optimal glucose values regarding CHD risk were 5.0–6.1 mmol/L. Fasting and postprandial glucose values were not related to CHD risk in men; IGT alone predicted risk in women (HR 3.74 [1.16;12.0]), independent of age, systolic blood pressure, non-HDL cholesterol, waist circumference, smoking status, and CRP. HDL cholesterol was unrelated to the development of IFG, IGT, and diabetes, while CRP elevation independently predicted the development of diabetes. IGT independently predicts CHD risk, especially in women. HDL dysfunction associated with low-grade inflammation is a co-determinant of pre-diabetic states and their progression to diabetes.