Extrapulmonary sequestration mimicking mediastinal cyst: report of two identical cases

Extrapulmonary sequestration (EPS) is a rare congenital anomaly usually diagnosed during the first six months of life. It is mostly found between the left lower lobe of the lung and the diaphragm. Mediastinal presentation is extremely rare. We describe two identical cases of extrapulmonary sequestration mimicking mediastinal cystic mass in two boys. These emphasize the need to keep this diagnosis in mind when dealing with children presenting with anterior mediastinal mass.     

Anxiety and depression in patients with rheumatoid arthritis

Rheumatoid arthritis (RA) mostly follows a painful, progressively disabling course, and individuals with RA experience more psychological distress than healthy individuals. The objective of the present study is to examine the prevalences of accompanying anxiety and depression in RA cases. The study included 82 RA cases and 41 age- and sex-matched healthy volunteers as the control group. Psychiatric examinations of all cases of the patient and control groups were performed according to DSM-IV criteria. Hamilton Anxiety Scale or Hamilton Depression Scale was applied to those who were found to have anxiety or depression. Total prevalence of anxiety, depression, and mixed anxiety-depressive disorder was found to be 70.8% (n=58) in the patient group and 7.3% (n=3) in the control group, and the difference was significant (p<0.001). Of the RA patients, 41.5% (n=34) was found to have depression, 13.4% (n=11) anxiety, and 15.9% (n=13) mixed anxiety-depressive disorder. The disease duration in patients with anxiety was shorter than the RA patient with depression (p<0.05). The disease duration was positively correlated with the degree of depression and negatively correlated with the degree of anxiety (r=0.341, p<0.05; r=−0.642, p<0.05, respectively). The results of our study suggest that prevalences of anxiety and mainly depression, increase in RA cases. When the clinical picture in RA cases becomes complicated with anxiety or depression, some problems at patients’ adaptation and response to treatment may be possible. RA cases should be monitored for accompanying anxiety or depression during follow-up.     

Subclavian artery pseudoaneurysm in Behcet’s disease

Behcet’s disease (BD) is an autoimmune, multisystemic, and chronic inflammatory disease. Although it affects all systems, involvement of the vascular system is of vital importance. Pseudoaneurysm ruptures in the arteries are the major causes of sudden deaths in BD. Although pseudoaneurysms in aorta and pulmonary arteries are rare, it is even more rare in the subclavian arteries. In our case, a pulsatile mass in the right clavicular area was determined to be associated with BD and this case is presented because subclavian artery pseudoaneurysm is seen rarely in BD.     

T helper 1 type cytokines polymorphisms: association with susceptibility to Behçet’s disease

The pathogenesis of Behçet’s disease (BD) is not fully understood and immunological abnormalities and genetic factors have been investigated. Because serum concentrations of mainly T helper 1 (Th1) type cells have been reported to be increased in BD, we aimed to investigate whether certain cytokine polymorphisms might represent a risk factor for developing BD. We genotyped 80 patients with BD and 105 healthy controls for interleukin (IL)-1 α (T/C ?889), IL-1 β (C/T ?511, T/C +3962), IL-1R (C/T pst11970), IL-1RA (T/C mspa111100), IL-2 (T/G ?330), IL-12 (C/A ?1188), interferon (IFN)-γ (A/T UTR 5644), and TNF-α (G/A ?238) polymorphisms. Analyses of cytokine polymorphisms were performed with PCR-SSP. The genotype and allele frequencies of the patients and controls were compared and the association between the polymorphisms of the cytokines with the clinical findings was investigated. Genotype distribution showed significant differences between the patients and the controls for the IL-1 α ?889, IL-1 β ?511, IL-1 β +3962, IL-1R, IL-12, IFN- γ, and TNF-α cytokines. We didn’t observe significant difference in genotypic frequencies of IL-1RA and IL-2 in our study. Comparison of the IL-1 α ?889, IL 1 β ?511, and IL 1 β +3962 genotype frequencies showed significant increase in CC genotype between the patients and the controls. The individuals with IL-1R TT polymorphism had a higher risk for BD compared to patients with CT/CC polymorphism. Comparison of IL-12, IFN- γ, and TNF-α, genotype frequencies showed significant increase in CA, AA, and AA genotypes between the patients and controls, respectively. The frequencies of genotypes according to the clinical features of the patients with BD did not show a significant difference (p?>?0.05). Our study suggests that development of BD might be determined by various cytokine gene polymorphisms. However, further studies on larger numbers of cases are needed before definite conclusions can be drawn.     

Unusual presentation of triple A syndrome mimicking Sjögren’s syndrome

Triple A syndrome is a rare autosomal recessive disorder characterized by alacrima, achalasia, and adrenal insufficiency. Sjögren’s syndrome (SS) is a chronic inflammatory disorder manifested primarily by diminished lacrimal and salivary gland secretions, resulting in symptoms of dry eyes and dry mouth, the so-called “sicca complex”. However, a variety of other manifestations also can occur, which can be termed “nonexocrine manifestations”. One of the frequent nonexocrine manifestations is dysphagia. In this paper, we present an unusual form of Triple A disease mimicking Sjögren’s syndrome, which leads to a challenging diagnosis.     

DNA repair gene XRCC1 and XPD polymorphisms and their association with coronary artery disease risks and micronucleus frequency

Coronary artery disease (CAD) is a multifactorial process that appears to be caused by the interaction of environmental risk factors with multiple predisposing genes. In this study, we investigated the effects of the XPD Lys751Gln and XRCC1 Arg399Gln polymorphisms on the presence and the severity of CAD. We also investigated the presence of DNA damage in the peripheral lymphocytes of patients with CAD by using the micronucleus (MN) test and the effect of XPD Lys751Gln and XRCC1 Arg399Gln polymorphisms on this damage. The study population consisted of 147 patients with angiographically documented CAD and 48 healthy controls. No association between XPD Lys751Gln or XRCC1 Arg399Gln polymorphisms and the presence or the severity of CAD was observed. On the other hand, a significantly higher frequency of MN was observed in CAD patients compared with controls (5.7 ± 1.9 vs 5.0 ± 2.1, respectively, P = 0.018). We found an elevated frequency of MN in CAD patients with the XPD 751Gln allele (Gln/Gln genotype) or the XRCC1 399Gln (Arg/Gln or Gln/Gln genotypes) allele compared with the XPD 751Lys (Lys/Lys genotype) allele or XRCC1 399 Arg (Arg /Arg genotype) allele, respectively. These preliminary results suggest that XPD Lys751Gln and XRCC1 Arg399Gln polymorphisms may not be a significant risk factor for developing CAD. In addition, our results indicate that the MN frequency is associated with presence, but not severity, of CAD and is related to the XRCC1 Arg399Gln and XPD Lys751Gln polymorphisms, suggesting an elevated frequency of MN in CAD patients with the XPD 751Gln or XRCC1 399Gln alleles.     

Complete surgical resolution of bilateral total opthalmoplegia without visual field defect in an acromegalic patient presented with pituitary apoplexy

Pituitary apoplexy (PA), which is one of the most serious life-threatening complications of pituitary adenoma, is characterized by abrupt onset of headache, nausea, vomiting, visual disturbances and oculomotor paresis. Combination of oculomotor cranial nerve paralysis with normal visual fields is very rare in PA. We report a 60-year-old acromegalic man presented with panhypopituitarism and bilateral total opthalmoplegia without a visual field defect. At initial evaluation his clinical findings were compatible with adrenal crisis and eye examination revealed total opthalmoplegia, bilateral ptosis and normal vision. MRI showed a large heterogeneous mass in the pituitary fossa. Although clinical findings due to adrenal crisis improved after glucocorticoid therapy there was no improvement in opthalmoplegia and ptosis. The patient underwent transsphenoidal excision of the pituitary mass. Histological examination revealed an adenoma with large areas of hemorrhagic infarction and most of the cells were positive for GH in immunohistochemical analysis. Although opthalmoplegia was severe at presentation, total recovery was achieved 3 months after transsphenoidal surgery. Therefore the presented case clearly demonstrates that opthalmoplegia without a visual field defect due to PA has a good prognosis and early diagnosis and treatment including surgical decompression are crucially important.     

Levosimendan Improves Renal Function in Patients with Acute Decompensated Heart Failure: Comparison with Dobutamine

Background Levosimendan is a relatively new cardiac inotropic agent with calcium sensitizing activity. This study was conducted to investigate the effects of levosimendan (L) and dobutamine (D) on renal function in patients hospitalized with decompensated heart failure (HF). Method The present study included 88 consecutive patients hospitalized with acutely decompensated HF (New York Heart Association (NYHA) Class 3–4) requiring inotropic therapy. Patients were randomized 2:1 to either L or D for intravenous inotropic support. Diuretic therapy was kept constant during infusions. Renal function values, including serum creatinine (CR), blood urea nitrogen, 24-h urinary output levels and calculated glomerular filtration rate (GFR) were measured just prior to and 24 h after the infusions in all patients, and 48 and 72 h after the infusions in every second patient in both groups. The pre and post-infusion values of renal function and left ventricular ejection fraction (LVEF) were evaluated. Results LVEF increased significantly in both groups. Those in L showed a significant improvement in calculated GFR after 24 h, whereas those in D showed no significant change (median in change in L:+15.3%, median change in D: −1.33%). Furthermore, in the L group a significant improvement was observed in calculated GFR after 72 h compared to baseline levels, whereas in D no significant change (median change in L:+45.45%, median change in D: +0.09%) was seen. Both agents improved 24-h urinary output. Conclusion Levosimendan seems to provide beneficial effects in terms of improvement in renal function compared to dobutamine in patients with heart failure who require inotropic therapy.     

Large vesicula seminalis cyst: a very rare cause of constipation and male infertility

We report a 35-year-old male patient with chronic constipation and infertility for 4 years. Spermiogram revealed severe oligospermia. An external mass compressing the rectum was found during rectal examination and flexible rectosigmoidoscopy. Abdominal computed tomography showed a presacral cystic mass which displaced the bladder anteriorly. The cyst was completely removed with open surgery. Histopathologic analysis revealed a cystic lesion covered with squamous epithelium including polymorphonuclear leukocytes, macrophages and sperm cells. After the operation, the patient's symptoms were relieved. We considered that the constipation was caused by external compression by the vesicula seminalis cyst. In cases of constipation with infertility, vesicula seminalis cyst should be kept in mind.     

Treatment of mitochondrial neurogastrointestinal encephalomyopathy with dialysis

OBJECTIVE: To study the effect of continuous ambulatory peritoneal dialysis on nucleoside levels and clinical course in a patient with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE). Patient We studied a patient with genetically verified MNGIE, who prior to treatment had lost weight progressively, developed amenorrhea, vomited multiple times daily, and had abdominal pain. Intervention The patient was treated with peritoneal dialysis for 3 years, and the effect on symptoms and plasma concentrations of thymidine and deoxyuridine were monitored. RESULTS: Dialysis stopped vomiting and reduced abdominal pain, and the patient gained 5 kg in weight and started to menstruate again. Symptoms returned if dialysis was paused. Dialysis did not affect plasma nucleoside levels. CONCLUSIONS: This study shows an unambiguous clinical benefit of peritoneal dialysis on gastrointestinal symptoms in MNGIE. Dialysis did not affect nucleoside levels, indicating elevated thymidine and deoxyuridine levels are not solely responsible for the pathogenesis of MNGIE.