Cyclosporin Treatment Improves Skin Findings in Omenn Syndrome

Omenn syndrome is a combined immunodeficiency characterized by a generalized erythematous skin rash, enlarged lymph nodes, hepatosplenomegaly, severe susceptibility to infections, eosinophilia, and hyperimmunoglobulinemia E. A 3‐month‐old girl was admitted to our hospital with a history of recurrent sepsis. Physical examination revealed severe erythroderma, hepatosplenomegaly, lymphadenopathy, and failure to thrive. Laboratory findings revealed leukocytosis, lymphocytosis with high CD3 T‐cells, a high CD4:CD8 ratio, absence of CD19 B‐cells, high eosinophil count, and low immunoglobulin levels. A heterozygote RAG1 gene mutation was found. She had itchy, scaling, ichthyosiform erythroderma and protracted diarrhea. Cyclosporin treatment up to 10 mg/kg effectively resolved erythroderma and lowered total eosinophil counts, and she gained weight during treatment. Since extensive erythroderma with generalized itching causes patient discomfort in Omenn syndrome, cyclosporin treatment can be considered while waiting for treatment with hematopoietic stem cell transplantation.     

Do different orthodontic pliers used in bracket debonding have different effects on pain and sensitivity? A prospective split-mouth study

Clinical Oral Investigations - The assessment of whether different orthodontic pliers used in bracket debonding have different effects on pain and sensitivity experience. Thirty-three patients (17...     

The effects of methylprednisolone and halofuginone on preventing esophageal and hypopharyngeal fibrosis in delivered radiotherapy

In this study, we assessed the effects of halofuginone and methylprednisolone on hypopharyngeal and esophageal stricture that can develop following radiation to the head and neck of rats. Rats were divided into four groups randomly and 18 Gy radiation was given to the head and neck regions of all rats except the control group. Group 1 (Control Group): No radiation or drugs were administered. Group 2 (Radiation Group): only radiation was applied without any drugs. Group 3 (Halofuginone Group): halofuginone 100 μg/kg per day was given intraperitoneally. Group 4 (Methylprednisolone Group): methylprednisolone 1 mg/kg per day was administered intramuscularly. In all groups, 90 days after application of radiation, sections of the proximal esophagus and hypopharynx were examined for fibrosis, fibroblast proliferation, vascularization, epithelial atypia, necrosis, polymorphonuclear leukocytes, mononuclear cells, and stenosis index by light microscope and the hydroxyproline levels were assessed biochemically. Fibrosis, epithelial atypia and hydroxyproline levels were found to be significantly higher in the radiation group compared to the control group (P < 0.05). We did not observe fibrosis in either the halofuginone or the control groups. Fibrosis was also significantly lower in the methylprednisolone group than the radiation group (P < 0.05). The differences of the stenosis index scores between the groups were not statistically significant (P < 0.05). Vascularization was similar in all groups. We think that especially halofuginone is a drug that can be used safely to prevent fibrosis due to radiotherapy, but further studies are needed.     

Mannose binding lectin levels are not related to radiographic damage in ankylosing spondylitis

In the current study we aimed to investigate the effect of MBL deficiency in radiographic damage of the spine in a large group of AS patients. One hundred and ninety-one AS patients and 85 healthy controls were studied. Disease activity, radiological scores, and demographic features were recorded. MBL levels were measured with standard ELISA kits. Results showed that median MBL levels in AS and healthy controls were 2,530 (range 0–5,861) ng/ml and 3,415 (0–7,950) ng/ml, respectively (p = 0.1). MBL deficiency (<500 ng/ml) was comparable in both groups (%21.5 in AS, % 17.6; p = 0.5). Disease activity, clinical picture, and therapies were not associated with MBL levels. Both BASRI and mSASSS scores were found similar in AS patients with or without MBL deficiency [BASRI: MBL < 500 ng/ml: 6(2–12), MBL ≥ 500 ng/ml: 6(2–12); p = 0.75], [mSASSS: MBL < 500 ng/ml: 3(0–72), MBL ≥ 500 ng/ml: 5(0–72); p = 0.81]. We conclude that MBL deficiency prevalence is not increased in AS patients and it is not a cause of a severe radiographic damage.     

Single or multiple perforations with varying locations as a complication of intestinal Behçet's disease: report of three cases

Intestinal ulcers in Beh?et's disease (BD) tend to cause perforation with significant morbidity. The optimal surgical procedure in such cases is controversial and the postoperative period can be eventful with an unpredictable course. We report three cases of perforations with varying locations in three patients with long-standing Beh?et's disease. Two patients required two and one patient required four operations. It is emphasized that the clinician must be alert in a patient with BD when abdominal symptoms accompany the clinical picture. As soon as the diagnosis is reached, surgical intervention with limited resection must be performed. Endoscopic examination and careful medication play major roles in the follow-up.     

A nomogram for measurement of mitral valve area by proximal isovelocity surface area method

INTRODUCTION: Although its accuracy has been documented in many studies, the proximal isovelocity surface area (PISA) method is not used widely for mitral valve area (MVA) measurement. In this study, we prepared a new nomogram and tested its use in MVA assessment. MATERIAL AND METHODS: The study included 23 patients (age: 27 +/- 5 years) with mitral stenosis, of whom 7 were in atrial fibrillation. The MVA was measured by four methods: planimetry (PL) (reference method), pressure-half time (PHT), conventional PISA (CP), and nomogram (Nomo) methods. The nomogram included two unknowns: (1) r; the radius of the first PISA section; (2) a; the length of the border opposite to the PISA angle in the triangle with both adjacent borders of 1 cm. The nomogram was also tested for its popularity potential by eight echocardiographers, none of whom were included in the author list. RESULTS: Mean MVA(PL) was 1.85 +/- 0.53 cm(2) (range: 0.72-2.99), mean MVA(PHT) was 1.72 +/- 0.56 cm(2) (range: 0.91-3.30), mean MVA(CP) was 1.69 +/- 0.45 cm(2) (range: 0.97-2.54), and MVA(Nomo) was 1.70 +/- 0.44 cm(2) (0.96-2.49). The nomogram correlated with planimetry (r = 0.87; P < 0.001), pressure half-time (r = 0.71; P < 0.001) and conventional PISA (r = 0.99; P = 0.000) methods. The nomogram method also correlated with planimetry in patients with atrial fibrillation (r = 0.81; P = 0.026). The echocardiographers found that the nomogram is superior to the planimetry and conventional PISA methods but inferior to the pressure half-time method in terms of simplicity. CONCLUSION: The new nomogram is potentially helpful in measurement of MVA. It may be used as an additional method in assessing severity of mitral stenosis.     

Does Coronary Artery Size Really Matter?

OBJECTIVE: To evaluate ischemia in right ventricle (RV) in patients with small caliber of right coronary artery (RCA). PATIENTS AND METHODS: The study population consisted of 60 consecutive patients undergoing coronary angiography within 3 months. The patients were divided into three different groups according to lumen diameter of RCA and coronary dominance. Group 1, 2, and 3 consisted of patients with small diameter of RCA, dominant RCA, and dominant circumflex artery, respectively. RV ischemia was assessed by using pulse-wave tissue Doppler sampling obtained from RV free wall close to the lateral tricuspid annulus at the apical four-chamber view during dobutamine stress echocardiography (DSE). RESULTS: When the mean systolic velocity percentages of increase from low to peak dose dobutamine in patients with small RCA were compared to those of other groups, statistically significant difference was found between group 1 and the other groups (P = 0.007 for group 1 vs group 2; P = 0.01 for group 1 vs group 3). The mean systolic velocity at peak dobutamine dose of patients with small caliber of right coronary artery was statistically lower than the other groups (P = 0.001 for group 1 vs group 2, P < 0.001 group 1 vs group 3). CONCLUSIONS: There are a group of patients with small diameter of RCA causing probable ischemia in RV and small caliber of RCA can really matter in these patients.     

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.