Revisiting human IL-12Rβ1 deficiency: a survey of 141 patients from 30 countries

Interleukin-12 receptor β1 (IL-12Rβ1) deficiency is the most common form of Mendelian susceptibility to mycobacterial disease (MSMD). We undertook an international survey of 141 patients from 102 kindreds in 30 countries. Among 102 probands, the first infection occurred at a mean age of 2.4 years. In 78 patients, this infection was caused by Bacille Calmette-Guérin (BCG; n = 65), environmental mycobacteria (EM; also known as atypical or nontuberculous mycobacteria) (n = 9) or Mycobacterium tuberculosis (n = 4). Twenty-two of the remaining 24 probands initially presented with nontyphoidal, extraintestinal salmonellosis. Twenty of the 29 genetically affected sibs displayed clinical signs (69%); however 8 remained asymptomatic (27%). Nine nongenotyped sibs with symptoms died. Recurrent BCG infection was diagnosed in 15 cases, recurrent EM in 3 cases, recurrent salmonellosis in 22 patients. Ninety of the 132 symptomatic patients had infections with a single microorganism. Multiple infections were diagnosed in 40 cases, with combined mycobacteriosis and salmonellosis in 36 individuals. BCG disease strongly protected against subsequent EM disease (p = 0.00008). Various other infectious diseases occurred, albeit each rarely, yet candidiasis was reported in 33 of the patients (23%). Ninety-nine patients (70%) survived, with a mean age at last follow-up visit of 12.7 years ± 9.8 years (range, 0.5-46.4 yr). IL-12Rβ1 deficiency is characterized by childhood-onset mycobacteriosis and salmonellosis, rare recurrences of mycobacterial disease, and more frequent recurrence of salmonellosis. The condition has higher clinical penetrance, broader susceptibility to infections, and less favorable outcome than previously thought.     

Comparison between Alpha and silver sulfadiazine ointments in treatment of Pseudomonas infections in 3rd degree burns

The goal of this study was to evaluate the efficacy of Alpha ointment in the treatment of burn wounds and compare its results with silver sulfadiazine (SS). Similar burn ulcers were produced on anterior surface of thigh of 60 rats. The wounds were infected with Pseudomonas aeruginosa and dressing and debridement was performed daily. The first group of rats received topical SS, the second group received Alpha ointment and the third (control group) received no medication. Wound healing, contraction, culture, and scar formation were evaluated at the end of the second and 10th week. Alpha ointment was equally effective as SS, considering wound healing and contraction. Wound infection was significantly less common in Alpha ointment group compared to the other two groups (p<0.05). Alpha ointment is a less expensive drug with an acceptable result compared to SS. Therefore, we recommend it as an alternative to SS, especially in patients with low economical backgrounds or in those who show adverse reactions to SS.     

An unusual case of preaxial polydactyly of the hand (triplication of the thumbs)

Preaxial polydactyly is the most common duplication pattern in white and Asian populations (1). It is a congenital anomaly with a wide range of manifestations. Current classification do not have the capacity to classify all different types of radial polydactyly. We describe here a very rare and unusual case of bilateral preaxial polydactyly (triplication) in a woman and report the operations results. We have not found similar case in the literature. Our case is unique and did not fit into the classification systems described for thumb polydactyly.     

<Emphasis Type="Italic">Leishmania major</Emphasis> infection in a patient with visceral leishmaniasis: treatment with Amphotericin B

We have reported an acute form of visceral leishmaniasis, which was caused by Leishmania major, in Southern Iran. The parasite was isolated from a 30-year-old man living in Bushehr Province, Southern Iran, and an endemic region of kala-azar in south of Iran. The patient had typical clinical signs of Mediterranean kala-azar, and besides parasitological, biochemical, and immunological findings confirming diagnosis of visceral leishmaniasis, the agent was identified as L. major using Nested polymerase chain reaction. The patient was treated successfully with Amphotericin B during a 1-month period.     

Parameter optimization and degradation mechanism for electrocatalytic degradation of 2,4-diclorophenoxyacetic acid (2,4-D) herbicide by lead dioxide electrodes

2,4-Dichlorophenoxyacetic acid (2,4-D) is one of the most commonly used herbicides in the world. In this work, the electro-catalytic degradation of 2,4-D herbicide from aqueous solutions was evaluated using three anode electrodes, i.e., lead dioxide coated on stainless steel 316 (SS316/β-PbO₂), lead dioxide coated on a lead bed (Pb/β-PbO₂), and lead dioxide coated on graphite (G/β-PbO₂). The structure and morphology of the prepared electrodes were studied by X-ray diffraction (XRD), scanning electron microscopy (SEM) and energy dispersive X-ray spectroscopy (EDX). The process of herbicide degradation was monitored during constant current electrolysis using cyclic voltammetry (CV). In this study, the experiments were designed based on the central composite design (CCD) and were analyzed and modeled by response surface methodology (RSM) to demonstrate the operational variables and the interactive effect of three independent variables on 3 responses. The effects of parameters including pH (3–11), current density (j = 1–5 mA cm⁻²) and electrolysis time (20–80 min) were studied. The results showed that, at j = 5 mA cm⁻², by increasing the reaction time from 20 to 80 min and decreasing the pH from 11 to 3, the 2,4-D herbicide degradation efficiency using SS316/β-PbO₂, Pb/β-PbO₂ and G/β-PbO₂ anode electrodes was observed to be 60.4, 75.9 and 89.8%, respectively. Moreover, the results showed that the highest COD and TOC removal efficiencies using the G/β-PbO₂ electrode were 83.7 and 78.5%, under the conditions pH = 3, electrolysis time = 80 min and j = 5 mA cm⁻², respectively. It was also found that G/β-PbO₂ has lower energy consumption (EC) (5.67 kW h m⁻³) compared to the two other studied electrodes (SS316/β-PbO₂ and Pb/β-PbO₂). The results showed a good correlation between the experimental values and the predicted values of the quadratic model (P < 0.05). Results revealed that the electrochemical process using the G/β-PbO₂ anode electrode has an acceptable efficiency in the degradation of 2,4-D herbicide and can be used as a proper pretreatment technique to treat wastewater containing resistant pollutants, e.g., phenoxy group herbicides (2,4-D).

Optimization of process parameters by the CCD method and electrocatalytic degradation and the electrochemical degradation mechanism of 2,4-D using modified electrode anodes were investigated.     

Cytomegalovirus Localization in Atherosclerotic Plaques is Associated with Acute Coronary Syndromes: Report of 105 Patients

It has been shown that cytomegalovirus (CMV) is present in coronary atherosclerotic plaques, but the clinical rele-vance of this presence remains to be elucidated. In this study we sought to examine CMV infection in atherosclerosis patients defined by different methods and to identify the clinical significance of CMV replication in the atherosclerotic plaques. The study included 105 consecutive patients who were admitted to our department and underwent coronary artery bypass grafting (CABG) surgical interventions. Coronary atherosclerotic specimens as well as 53 specimens from the mamillary artery of these same patients were analyzed. Enzyme-linked immunosorbent assay (ELISA) and poly-merase chain reaction (PCR) methods were used for evaluations. The CMV PCR test result was positive for 28 (26.7%) of patients with coronary artery atherosclerosis. After adjusting for other risk factors, coronary artery disease patients with a history of acute coronary syndrome were more likely to be positive for CMV PCR test (P=0.027; odds ratio: 4.2; 95% CI: 1.18-15.0). They were also more likely to have a positive family history for cardiovascular diseases (CVD). This study confirms previous evidence about the replication of CMV virus in the atherosclerotic plaques of coronary arteries and brings clinical significance to this observation by showing a higher prevalence of acute coronary syndromes in those patients with CMV-infected plaques. Our study also suggests a familial vulnerability to CMV replication in the coronary artery walls.