Henoch Schonlein purpura in childhood: clinical analysis of 254 cases over a 3-year period

We aimed to evaluate the patients who were diagnosed as Henoch Schonlein purpura (HSP) for disease characteristics and prognosis of those with joint, gastrointestinal (GI), and renal involvement. Two hundred and fifty-four children who were followed up with the diagnosis of HSP in the Pediatric Nephrology Clinics of Meram Medical Faculty of Selcuk University and Medical Faculty of Gazi University between January 2003 and June 2006 were retrospectively evaluated. The clinical follow-up and treatment regimens of patients in whom renal biopsy was performed were evaluated in detail. The study group consisted of 254 children, 147 boys (57.8%) and 107 girls (42.2%), and the ratio of boys to girls was 1.37. The percentages of skin, joint, GI, and renal manifestations were 100%, 66%, 56%, and 30%, respectively. Eight patients had intussusception. Five of them recovered with steroid treatment only while three patients were operated on. Sixty-four patients (44%) with GI involvement had severe disease and were successfully treated with steroids. Renal biopsy was performed in 26 patients. Among those 26 patients, two of them recovered spontaneously within 3 and 4 weeks. Ten patients improved with only steroid treatment while 12 patients recovered with steroid and cyclophosphamide treatment. Two patients were resistant to steroid and cyclophosphamide treatment and were treated with cyclosporine A. We believe that steroid therapy given to the HSP patients with GI manifestations might be helpful to prevent probable complications such as GI bleeding and intussusception. In addition, combined therapy with steroid and cyclophosphamide can usually be an appropriate treatment for patients with nephrotic proteinuria.     

DNA methylation-independent loss of RARA gene expression in acute myeloid leukemia

The retinoic acid receptor (RAR) alpha gene (RARA) encodes 2 major isoforms and mediates positive effects of all-trans retinoic acid (ATRA) on myelomonocytic differentiation. Expression of the ATRA-inducible (RARalpha2) isoform increases with myelomonocytic differentiation and appears to be down-regulated in many acute myeloid leukemia (AML) cell lines. Here, we demonstrate that relative to normal myeloid stem/progenitor cells, RARalpha2 expression is dramatically reduced in primary AML blasts. Expression of the RARalpha1 isoform is also significantly reduced in primary AML cells, but not in AML cell lines. Although the promoters directing expression of RARalpha1 and RARalpha2 are respectively unmethylated and methylated in AML cell lines, these regulatory regions are unmethylated in all the AML patient cell samples analyzed. Moreover, in primary AML cells, histones associated with the RARalpha2 promoter possessed diminished levels of H3 acetylation and lysine 4 methylation. These results underscore the complexities of the mechanisms responsible for deregulation of gene expression in AML and support the notion that diminished RARA expression contributes to leukemogenesis.     

Patients with paroxysmal atrial fibrillation but not paroxysmal supraventricular tachycardia display evidence of platelet activation during arrhythmia

It is well known that chronic atrial fibrillation (CAF) and paroxysmal atrial fibrillation (PAF) are associated with a hypercoagulable state. However, pathological hemostatic changes during the paroxysmal supraventricular tachycardia (PSVT) have not yet been elucidated. To determine platelet activity in patients with PSVT, PAF and CAF, we examined the levels of β-thromboglobulin (BTG) and platelet factor 4 (PF4) during tachyarrhythmia attacks. We measured the levels of BTG and PF4, as an index of platelet activation in 15 patients with PAF (9 men, mean age 45 ± 11), and 14 patients with PSVT (8 men, mean age 40 ± 10). Levels were compared to 22 age and sex-matched healthy controls in sinus rhythm and with 25 patients with CAF (16 men, mean age 51 ± 12). Blood samples were taken during arrhythmia and 24 hours after conversion to sinus rhythm. Patients taking medications or have clinical conditions that may affect the BTG and PF4 levels were excluded.

In patients with PAF, BTG and PF4 levels were significantly higher than in controls (?p<0.009, and p = 0.002, respectively), and in patients with PSVT (?p<0.04, and p = 0.009, respectively), however, BTG and PF4 levels were significantly lower than CAF patients (?p = 0.002, and p = 0.02, respectively). Moreover, BTG and PF4 levels were significantly decreased 24 hours after conversion to sinus rhythm (?p<0.0001, and p = 0.004, respectively). Although BTG and PF4 levels in patients with PSVT were significantly lower than in patients with PAF (?p = 0.04, and p = 0.009, respectively) and CAF (?p = 0.0001, and p = 0.0001, respectively), BTG and PF4 levels were similar to controls and did not change significantly after recovery to sinus rhythm (?p = NS for all).

These results indicate that there was no platelet activation in patients with PSVT during tachyarrhythmia but significantly increased platelet activity in PAF and CAF patients. There was a significant decrement of the platelet activity to a level of control subjects twenty-four hours after cardioversion of PAF.     

Hearing status in brucellosis.

BACKGROUND: Although brucellosis can lead to multisystem complications, involvement of the ear in brucellosis is rarely reported in the literature. The purpose of this study was to assess the hearing status of patients with brucellosis. STUDY DESIGN: Thirty-two patients with brucellosis were included in the study. Pure tone and speech audiometry and tympanometry were performed in the patients. RESULTS: The mean pure tone averages of the patients were within normal limits and were similar in both ears (P > 0.05). The pure tone averages of the patients with or without anti-Brucella treatment were not significantly different (P > 0.05) and were within the normal limits. When the hearing levels of these patients were compared at the frequencies of 250, 500, 1000, 2000, 4000, and 8000 Hz, there was no significant difference as well (P > 0.05). CONCLUSIONS: Peripheral brucellosis does not appear to be associated with hearing loss.     

Fibromyalgia: its prevalence in haemodialysis patients and its relationships with clinical and laboratory parameters.

OBJECTIVE: Our aim was to determine the prevalence of fibromyalgia syndrome (FS) in chronic haemodialysis (HD) patients and to identify possible links between FS and various clinical and laboratory parameters. METHODS: We studied 122 chronic HD patients and 89 healthy age- and sex-matched controls, classified according to the American College of Rheumatology (ACR) classification criteria for FS. Age, sex, causes of renal failure, length of time on dialysis and marital status were recorded, and questions were asked about symptoms related to FS. All subjects completed the Fibromyalgia Impact Questionnaire (FIQ). Laboratory data obtained in the preceding 6 months were re-evaluated. RESULTS: Nine (7.4%) of the 122 HD patients and four of the 89 control subjects (4.5%) fulfilled the ACR criteria for definite FS (P = 0.56). The mean ages of the subjects who had definite FS and those who did not were similar. Most of the subjects diagnosed with definite FS were female (11 out of 13). The HD patients had higher FIQ scores than the controls, regardless of FS diagnosis. Among the HD patients, those with definite FS had a significantly higher mean FIQ score than all the other HD patients combined. In the all HD patients group, fatigue, irritable bowel syndrome and personal histories of depression were correlated with FS, whereas duration of HD, aetiology of renal failure, laboratory parameters and hepatitis B or C virus infection were not. CONCLUSION: The prevalence of FS appeared to be similar in chronic HD patients and the general population; also, FS-related symptoms appear to be similar in HD patients and the general population who have FS. No laboratory parameter was correlated with frequency of FS.     

Acute lymphoblastic leukemia following optic glioma treated by radiotherapy and surgery

A 14-year-old girl was diagnosed as having acute lymphoblastic leukemia following 5000 cGy cranial radiotherapy for treatment of optic glioma. In the absence of underlying predisposing factors, development of acute leukemia was attributed to the oncogenic effect of radiation.