Comprehensive comparison between 222 CTLA-4 haploinsufficiency and 212 LRBA deficiency patients: a systematic review.

Cytotoxic T lymphocyte antigen 4 (CTLA-4) haploinsufficiency (CHAI) and lipopolysaccharide-responsive beige-like anchor (LRBA) deficiency (LATAIE) are newly identified inborn errors of immunity with shared molecular pathomechanisms and clinical manifestations. In this review, we aimed to provide differential comparisons regarding demographic, clinical, immunological and molecular characteristics between these two similar conditions. A literature search was conducted in PubMed, Web of Science and Scopus databases and included studies were systematically evaluated. Overall, 434 (222 CHAI and 212 LATAIE) patients were found in 101 eligible studies. The CHAI patients were mainly reported from North America and western Europe, while LATAIE patients were predominantly from Asian countries. In CHAI, positive familial history (P < 0·001) and in LATAIE, consanguineous parents (P < 0·001) were more common. In CHAI patients the rates of granulomas (P < 0·001), malignancies (P = 0·001), atopy (P = 0·001), cutaneous disorders (P < 0·001) and neurological (P = 0·002) disorders were higher, while LATAIE patients were more commonly complicated with life-threatening infections (P = 0·002), pneumonia (P = 0·006), ear, nose and throat disorders (P < 0·001), organomegaly (P = 0·023), autoimmune enteropathy (P = 0·038) and growth failure (P < 0·001). Normal lymphocyte subsets and immunoglobulins except low serum levels of CD9+ B cells (14·0 versus 38·4%, P < 0·001), natural killer (NK) cells (21 versus 41·1%, P < 0·001), immunoglobulin (Ig)G (46·9 versus 41·1%, P = 0·291) and IgA (54·5 versus 44·7%, P = 0·076) were found in the majority of CHAI and LATAIE patients, respectively. The most frequent biological immunosuppressive agents prescribed for CHAI and LATAIE patients were rituximab and abatacept, respectively. Further investigations into the best conditioning and treatment regimens pre- and post-transplantation are required to improve the survival rate of transplanted CHAI and LATAIE patients.

Evaluating the relation between the elongated styloid process and the ponticulus posticus using cone-beam computed tomography.

BACKGROUND: Ponticlus posticus (PP) as a one of the cervical vertebra variations brings about symptoms similar to Eagle syndrome. This study aimed to determine the relationship between elongated styloid process (ESP) and PP in a group of Iranian patients using cone-beam computed tomography (CBCT) images. MATERIALS AND METHODS: The CBCT images of 349 patients (118 males and 231 females; mean age: 32.53 ± 14.143) were involved in this study. The atlas vertebra was investigated for the presence and classification of PP (partial or complete) in sagittal views. Also, the styloid process was evaluated for the presence of ESP in reconstructed panoramic and three-dimensional images. Data were analysed using Mann-Whitney test, Fisher's exact test, and Chi-square test to assess the relationship between the presence of PP and ESP with regard to age and gender. RESULTS: Ponticulus posticus was observed in 24.5% of patients with ESP and 31.98% of patients without ESP. There was no significant relationship between the presence of PP and ESP (p = 0.198). Twenty-five patients with ESP showed PP; cases of ESP with either side and opposite side PP were 7.84% and 1.96%, respectively. Cases of bilateral ESP and PP were predominant (14.70%). The mean age of patients with bilateral ESP and PP was higher than others. There was no significant difference between males and females (p = 0.456). CONCLUSIONS: Considering the prevalence and characteristics of PP in the case and control groups, there was no significant relationship between PP and ESP.

Mandibular Trabecular Bone Analysis Using Local Binary Pattern for Osteoporosis Diagnosis.

BACKGROUND: Osteoporosis is a systemic skeletal disease characterized by low bone mineral density (BMD) and micro-architectural deterioration of bone tissue, leading to bone fragility and increased fracture risk. Since Panoramic image is a feasible and relatively routine imaging technique in dentistry; it could provide an opportunistic chance for screening osteoporosis. In this regard, numerous panoramic derived indices have been developed and suggested for osteoporosis screening. Jaw trabecular pattern is one of the main bone strength factors and trabecular bone pattern assessment is important factor in bone quality analysis. Texture analysis applied to trabecular bone images offers an ability to exploit the information present on conventional radiographs. OBJECTIVE: The purpose of this study was to evaluate the relationship between Jaw trabecular pattern in panoramic image and osteoporosis based on image texture analyzing using local binary pattern. MATERIAL AND METHODS: An experiment is evaluated in this paper based on a real hand-captured database of panoramic radiograph images from osteoporosis and non-osteoporosis person in Namazi Hospital, Shiraz, Iran .An approach is proposed for osteoporosis diagnosis consisting of two steps. First, modified version of local binary patterns is used to extract discriminative features from jaw panoramic radiograph images. Then, classification is done using different classifiers. RESULTS: Comparative results show that the proposed approach provides classification accuracy about 99.6%, which is higher than many state-of-the-art methods. CONCLUSION: High classification accuracy, low computational complexity, multi-resolution and rotation invariant are among advantages of our proposed approach.

Intramyocardial implantation of CD133+ stem cells improved cardiac function without bypass surgery.

INTRODUCTION: Cell transplantation for myocardial regeneration has been shown to have beneficial effects on cardiac function after myocardial infarction. Most clinical studies of intramyocardial cell transplantation were performed in combination with coronary artery bypass grafting (CABG). The contribution of implanted stem cells could yet not be clearly distinguished from the effect of the CABG surgery. Our current phase 1 clinical study has focused on the safety and feasibility of CD133+-enriched stem cell transplantation without CABG and its potential beneficial effect on cardiac function. METHOD AND RESULTS: Ten patients with end-stage chronic ischemic cardiomyopathy (ejection fraction <22%) were enrolled in the study. Bone marrow (up to 380 mL) was harvested from the iliac crest. CD133+ cells were purified from bone marrow cells using the CliniMACS device with purities up to 99%. Autologous bone marrow CD133+ cells (1.5-9.7 X 106 cells) were injected into predefined regions. Cardiac functions prior to and 3, 6, and 9 months after cell transplantation were assessed by cardiac magnetic resonance imaging. Stem cell transplantation typically improved the heart function stage from New York Heart Association/Canadian Cardiovascular Society class III-IV to I-II. The mean preoperative and postoperative ventricular ejection fractions were 15.8 +/- 5% and 24.8 +/- 5%, respectively. CONCLUSION: CD133+ injection into ischemic myocardium was feasible and safe. Stem cell transplantation alone improved cardiac function in all patients. This technique might hold promise as an alternative to medical management in patients with severe ischemic heart failure who are ineligible for conventional revascularization.

Non-specialist management of acute renal failure.

In a 12-month prospective study of the initial management of patients with acute renal failure (ARF) in East Kent (population 593 000), we evaluated the initial management of ARF and assessed what proportion of ARF may have been preventable. Patients were seen and assessed on a daily basis, and were followed until discharge from hospital or death; survivors were subsequently followed for 3 years. Overall, 288 patients developed ARF (486 per million population/year). Mean age at presentation was 73 years (range 14-96). Initial assessment was often suboptimal, and key features in investigation and initial management were often lacking. In 108 cases, ARF was iatrogenic and/or potentially preventable (53 preventable, 99 iatrogenic, 44 both). Overall survival was 56% at discharge from hospital, 35% at 1-year follow-up, 31% at 2 years, and 28% at 3 years. In discharged patients, recovery of function was complete in 69%. A significant proportion of ARF is preventable. Clear guidelines, easily accessible at the point of care, could aid the diagnostic evaluation of the patient with ARF and indicate where referral for a specialist opinion is appropriate.

Prevalence of hepatitis B, hepatitis delta, and human immunodeficiency virus infections in Omani patients with renal diseases.

PIP: Patients treated at the Royal Hospital in Oman during January-June 1991 were divided in 3 groups. The 1st group included 103 patients (49 males, 54 females, with a mean of 39 years) who attended the Nephrology Clinic and none of whom were on dialysis. In the 2nd group there were 102 patients (46 males, 56 females, with a mean age of 42 years) on regular hemodialysis (with a mean duration of 35 months) because of end-stage renal failure. The 3rd group comprised 82 kidney transplant patients (44 males, 38 females, with a mean age of 33 years) with a mean duration of prior hemodialysis of 9 months in 80 patients. Blood serum samples from all patients as well as from 134 medical students and 564 blood donors were tested for hepatitis B surface antigen (HBsAg) and hepatitis B core antibody (anti-HBc) by enzyme-linked immunosorbent assay. HBsAg-positive samples were tested for antigen and antibody to hepatitis delta virus (HDV). The prevalence of HBsAg was significantly higher in hemodialysis and renal transplant patients than in nephrology clinic patients (P .05). Previous exposure to HBV was found in 48 of 103 (46.6%) nephrology clinic patients, in 53 of 102 (52%) hemodialysis patients, and in 43 of 82 (52.4%) renal transplant patients. Anti-HBc prevalence rates were significantly lower in medical students (23.1%) and blood donors (27%) than in the patient groups (P .001). In HBsAg-positive subjects HDV infection was found in 1 of 13 (7.7%) patients on dialysis and 2 of 9 (22.2%) kidney transplant recipients who had been transfused in the past. A double infection of HBV and HCV was found only in 4 hemodialysis and 2 transplant patients among 287 patients and 698 healthy subjects tested. Among 5 HIV-infected patients 3 transplant patients seroconverted between 3 and 7 months after kidney transplantation abroad; and 2 hemodialysis patients seroconverted after repeated dialysis and multiple blood transfusions used for kidney transplantation abroad.^ieng

Continuous ambulatory peritoneal dialysis. Experience in a single renal unit in the UK.

The outcome of 106 patients started on continuous ambulatory peritoneal dialysis (CAPD) in a single renal unit over a 3-year period was reviewed in order to compare our experience with that of other centres. The incidence of peritonitis was not different in diabetics compared with non-diabetics, in the elderly compared with younger patients, and in non-compliant compared with compliant patients. The patients' level of education had a significant effect on the number of days required to train for CAPD. Diabetics and non-compliant patients required more hospitalization. The need for community support increased the incidence of peritonitis.

Hepatitis B, delta and human immunodeficiency virus infections among Omani patients with renal diseases: A seroprevalence study.

The prevalence of hepatitis B virus (HBV), hepatitis delta virus (HDV), and human immunodeficiency virus (HIV) infections were determined in 102 patients on regular hemodialysis, 82 kidney recipients and 1030 nondialyzed, nontransplanted patients with various renal diseases. The prevalence rates of hepatitis B surface antigen (HBsAg) in dialysis and renal transplant patients (12.7% and 11.0% respectively) were significantly higher than the rate in a control group of patients who had never been dialyzed nor transplanted (2.9%, P<0.05). In patients who were HBsAg positive, evidence of HDV infection was found in one dialysis and two transplant patients only. HIV infection was confirmed in only two of 102 (2.0%) and three of 82 (3.7%) hemodialysis and kidney recipients respectively. These data indicate hepatitis B, delta and HIV infections are major health problems among hemodialysis and renal transplant patients in the Sultanate of Oman.

Association of risk factors with acute myocardial infarction in Middle Eastern countries: the INTERHEART Middle East study.

BACKGROUND AND OBJECTIVES: Mortality from cardiovascular disease in the Middle East (ME) is projected to increase substantially by 2020. There are no large studies on the impact of risk factors for acute myocardial infarction (AMI) in the region. This is a report on the association of nine risk factors with AMI in the ME. METHODS AND RESULTS: As part of the INTERHEART (IH) study, we enrolled 1364 cases of first AMI and 1525 matching controls from eight ME countries. The age at first AMI was 51.2 ± 10.3 years, which is the youngest, and with the largest proportion of patients <40 years in the entire IH population. The overall population attributable risk (PAR) of the nine risk factors to AMI was higher in the ME (97.5%) than worldwide (90.4%). Elevated apolipoprotein (Apo)B/ApoA1 had the strongest association with AMI, with odds ratio (OR) of 3.43 and PAR of 57.1%, followed by smoking (OR 3.63 and PAR 45.6%). ApoB/ApoA1 had greater association than the conventional low-density lipoprotein (LDL)/high-density lipoprotein (HDL) cholesterol ratio. Both diabetes (OR 3.42, PAR 16.4%) and hypertension (OR 1.89, PAR 10.7%) had greater association with AMI in women than men. Abdominal obesity (OR 2.12, PAR 26.1%) and depression (OR 1.97, PAR 45.3%), but not conventional BMI, were significantly associated with AMI (p < 0.0001). CONCLUSION: This is the largest prospective population study of risk factors associated with AMI in the ME. AMI occurs at younger age in the ME than all other regions. The PAR for the nine risk factors was higher in the ME (97.5%) than the rest of the world. These findings should guide serious prevention strategies.

OS035. Blood pressure measurement by health professionals, comparison with American Heart Association Technique.

INTRODUCTION: It seems measurement of the blood pressure is always easy, although it is important and should be done by applying standard method. Even a few mistake can make difference between being prescribed medication or having the blood pressure monitored. OBJECTIVES: To compare the rate and accuracy of measuring blood pressure. METHODS: In this clinical trail study five hundred health professional consist of 179 nurses, 58 physicians, 99 medical and 118 nursing students and 46 association nurses were studied in 10 hospitals (3th of them were mother and child health centers) and health centers and five nursing and medical instructions when working with their students observed them. Two questioners were use for the data, demographic information and American Heart Association Standard questioner. RESULTS: The mean systolic-diastolic blood pressure for research samples in this study was 126±20.82/78.55±12.70mmHg and for the researcher's measurement was 120.15±20.56/72.08±12.24. The differences were-5.85mmHg for systolic and -6.47mmHg for diastolic BP. The most change of blood pressure before and after taking blood pressure were about pregnant women.Nurses and nursing students aided by 63% of the recommended procedures for taking blood pressure and other health professionals done by approximately 40%. CONCLUSION: Results indicated that health professional failed comply with the BP measurement guidelines, this may lead to a wrong diagnose. The importance of performing certain variables should be taken into account when teaching and assessing students in the future. Because hypertension is one of the leading causes of cardiovascular morbidity and mortality, the simple and accurate measurement of BP can be life saving.

H-point standard addition method for simultaneous determination of Fe(II), Co(II) and Cu(II) in micellar media with simultaneous addition of three analytes.

H-point standard addition method, HPSAM, with simultaneous addition of three analytes is proposed for the resolution of ternary mixtures. It is a modification of the previously described H-point standard addition method that permits the resolution of three species from a unique calibration set by making the simultaneous addition of the three analytes. The method calculates the analyte concentration from spectral data at two wavelengths where the two species selected as interferents present the same absorbance relationship. These wavelength pairs are easily found, and can be selected to give the most precise results. Diethyldithiocarbomate (DDC) in a cationic micellar solution of cetyltrimethylammonium bromide (CTAB) was used for determination of Fe(II), Co(II) and Cu(II) at pH 5.50. The results showed that simultaneous determination of Fe(II), Co(II) and Cu(II) could be preformed in the range of 0.0-6.0, 0.0-8.0 and 0.0-12.0mugml(-1), respectively. The proposed method was successfully applied to the simultaneous determination of Fe(II), Co(II) and Cu(II) in several synthetic mixtures containing different concentration of Fe(II), Co(II) and Cu(II).

Autologous transplantation of CD133+ BM-derived stem cells as a therapeutic option for dilatative cardiomyopathy.

We report the case of a 58-year-old man with end-stage non-ischemic cardiomyopathy. Baseline transthoracic echocardiography (TTE) and cardiac magnetic resonance (cMRI) revealed a markedly depressed left ventricle systolic function. He underwent autologous CD133+ BM-derived cell transplantation through a minimally invasive approach. During surgery 19 x 10(6) BM-derived stem cells were injected by the transepimyocardial route. Six months after the operation TTE and cMRI showed a clear improvement in left ventricular contractility.