Removal of aqueous Clopyralid by Photoctalytic-ozonation process on Activated carbon under solar radiation: Catalyst characterization and kinetic study

Solar photocatalytic ozonation has been used to oxidize the herbicide clopyralid in aqueous solution. Activated carbon used as a catalyst was characterized by Scanning Electron Microscopy (SEM) images showed the presence of irregular cavities and pores, which have different sizes and shapes, with a great surface area of BETand a high pHpzc. The adsorption kinetics was found to follow the pseudo-second-order kinetic mode. Catalyst stability was tested by means of consecutive reuse cycles. After five cycle of reuse the adsorption efficiency reached 75% of the clopyralid removal.Clopyralid elimination kinetic by direct ozonation has been studies. The system O3/AC/Daylight significantly improves clopyralid and mineralization rate abatement if compared to runs conducted in the absence of radiation and/ or activated carbon. Clopyralid total abatement was achieved in less than 30 min when 0.5 g/L of activated carbon and under solar radiation (300-800 nm) were used. Thus, TOC removal in 180 min treatments increased from 20 to about 90 % in O3 and O3/OSAC/Daylight, respectively, under similar operating conditions

CUTANEOUS MANIFESTATIONS OF PRIMARY IMMUNODEFICIENCY DISEASES IN TUNISIAN CHILDREN

Abstract. Skin manifestations are frequent among patients with primary immunodeficiency diseases (PIDs). Their prevalence varies according to the type of immunodeficiency. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of PIDs among Tunisian children. We conducted a prospective study on two hundred and ninety children with immune deficiency. Demographic details (including age, sex, and consanguinity) with personal and family history were recorded. Special attention was paid to cutaneous manifestations. Dermatological involvements were grouped according to the etiology of their most prominent sign. Cutaneous manifestations were found in 164 patients (56.5%). They revealed the diagnosis of PIDs in 71 patients (24.5 %). The mean age at presentation was 21 months. Overall the most prominent cutaneous alterations were infectious. They accounted for 106 cases (36.55%). The most prevalent causes of cutaneous infections were bacterial: 93 cases (32.06%). Immuno-allergic skin diseases were among the common findings in our study. These include eczematous dermatitis found in 62 cases (21.38%). Malignancy related PIDs was seen in a boy with Wiskott Aldrich syndrome. He developed Kaposi’s sarcoma at the age of 14 months. Cutaneous changes are common among children with PIDs. In pediatric patients with failure to thrive, chronic refractory systemic manifestations often present in other family members, recurrent cutaneous infections unresponsive to adequate therapy, atypical forms of eczematous dermatitis or unusual features should arouse the suspicion of PIDs and prompt specialized immunologic consultation should be made

Epidemiological, clinical, and therapeutic characteristics of Behçet's disease: a monocentric study in Tunisia

Introduction: to describe the epidemiological, clinical, therapeutic and evolving characteristics of Behçet´s disease and identify prognostic factors. Methods: we have realized a retrospective, single-center study, conducted over a period of 26 years and including 130 patients presenting Behçet´s disease and hospitalized in an Internal Medicine Department. Results: the mean age of the Behçet´s disease at onset was 30.3 ±8.8 years and that at diagnosis was 34.6 ±9.4 years. The sex ratio (male/female) was 2.5. The mean delay of diagnosis was 53.5 months. Oral aphthosis was constant. The frequency of the manifestations was: genital aphtosis 71.5%, pseudofolliculitis 84.6%, erythema nodosum 11.5%, positive pathergy test 50%, ocular disease 36.9%, venous thrombosis 30%, arterial disease 4.6%, joint damage 30.8%, neurological disease 19.2% and digestive disease 0.8%. The male gender was significantly associated with ocular involvement (p =0.02), venous disease (p =0.01) and occurrence of relapses (p =0.01). The mean follow up was 68.5 ± 77.3 months. The poor survival prognostic factors were male gender, ocular involvement, venous disease, cardiovascular disease, a duration of follow up ≤12 months and a diagnostic delay ≤ 24 months. Conclusion: improving the prognosis of Behçet´s disease requires a shortening of the time to diagnosis, multidisciplinary collaboration, intensive treatment of functional threats, regular monitoring, and patient adherence

Oral HPV infection and MHC class II deficiency (A study of two cases with atypical outcome)

<p>Abstract</p> <p>Background</p> <p>Major histocompatibility complex class II deficiency, also referred to as bare lymphocyte syndrome is a rare primary Immunodeficiency disorder characterized by a profondly deficient human leukocyte antigen class II expression and a lack of cellular and humoral immune responses to foreign antigens. Clinical manifestations include extreme susceptibility to viral, bacterial, and fungal infections. The infections begin in the first year of life and involve usually the respiratory system and the gastrointestinal tract. Severe malabsorption with failure to thrive ensues, often leading to death in early childhood. Bone marrow transplantation is the curative treatment.</p> <p>Case reports</p> <p>Here we report two cases with a late outcome MHC class II deficiency. They had a long term history of recurrent bronchopulmonary and gastrointestinal infections. Bone marrow transplantation could not be performed because no compatible donor had been identified. At the age of 12 years, they developed oral papillomatous lesions related to HPV (human papillomavirus). The diagnosis of HPV infection was done by histological examination. HPV typing performed on the tissue obtained at biopsy showed HPV type 6. The lesions were partially removed after two months of laser treatment.</p> <p>Conclusions</p> <p>Viral infections are common in patients with MHC class II and remain the main cause of death. Besides warts caused by HPV infection do not exhibit a propensity for malignant transformation; they can cause great psychosocial morbidity.</p

FANCA Gene Mutations in North African Fanconi Anemia Patients

Populations in North Africa (NA) are characterized by a high rate of consanguinity. Consequently, the proportion of founder mutations might be higher than expected and could be a major cause for the high prevalence of recessive genetic disorders like Fanconi anemia (FA). We report clinical, cytogenetic, and molecular characterization of FANCA in 29 North African FA patients from Tunisia, Libya, and Algeria. Cytogenetic tests revealed high rates of spontaneous chromosome breakages for all patients except two of them. FANCA molecular analysis was performed using three different molecular approaches which allowed us to identify causal mutations as homozygous or compound heterozygous forms. It included a nonsense mutation (c.2749C > T; p.Arg917Ter), one reported missense mutation (c.1304G > A; p.Arg435His), a novel missense variant (c.1258G > A; p.Asp409Glu), and the FANCA most common reported mutation (c.3788_3790delTCT; p.Phe1263del). Furthermore, three founder mutations were identified in 86.7% of the 22 Tunisian patients: (1) a deletion of exon 15, in 36.4% patients (8/22); (2), a deletion of exons 4 and 5 in 23% (5/22) and (3) an intronic mutation c.2222 + 166G > A, in 27.3% (6/22). Despite the relatively small number of patients studied, our results depict the mutational landscape of FA among NA populations and it should be taken into consideration for appropriate genetic counseling

X-linked agammaglobulinemia (XLA) : Phenotype, diagnosis, and therapeutic challenges around the world

Background: X-linked agammaglobulinemia is an inherited immunodeficiency recognized since 1952. In spite of seven decades of experience, there is still a limited understanding of regional differences in presentation and complications. This study was designed by the Primary Immunodeficiencies Committee of the World Allergy Organization to better understand regional needs, challenges and unique patient features. Methods: A survey instrument was designed by the Primary Immunodeficiencies Committee of the World Allergy Organization to collect both structured and semi-structured data on X-linked agammaglobulinemia. The survey was sent to 54 centers around the world chosen on the basis of World Allergy Organization participation and/or registration in the European Society for Immunodeficiencies. There were 40 centers that responded, comprising 32 countries. Results: This study reports on 783 patients from 40 centers around the world. Problems with diagnosis are highlighted by the reported delays in diagnosis>24 months in 34% of patients and the lack of genetic studies in 39% of centers Two infections exhibited regional variation. Vaccine-associated paralytic poliomyelitis was seen only in countries with live polio vaccination and two centers reported mycobacteria. High rates of morbidity were reported. Acute and chronic lung diseases accounted for 41% of the deaths. Unusual complications such as inflammatory bowel disease and large granular lymphocyte disease, among others were specifically enumerated, and while individually uncommon, they were collectively seen in 20.3% of patients. These data suggest that a broad range of both inflammatory, infectious, and autoimmune conditions can occur in patients. The breadth of complications and lack of data on management subsequently appeared as a significant challenge reported by centers. Survival above 20 years of age was lowest in Africa (22%) and reached above 70% in Australia, Europe and the Americas. Centers were asked to report their challenges and responses (n = 116) emphasized the difficulties in access to immunoglobulin products (16%) and reflected the ongoing need for education of both patients and referring physicians. Conclusions: This is the largest study of patients with X-linked agammaglobulinemia and emphasizes the continued morbidity and mortality of XLA despite progress in diagnosis and treatment. It presents a world view of the successes and challenges for patients and physicians alike. A pivotal finding is the need for education of physicians regarding typical symptoms suggesting a possible diagnosis of X-linked agammaglobulinemia and sharing of best practices for the less common complications.Peer reviewe

Surgical management of lifebuoy cataract

Lifebuoy cataract is a rare form of congenital cataract with anatomical characteristics. Herein, we present a case of an otherwise healthy 42-year-old female with a long-standing history of blurred vision. Examination showed the presence of esotropia and bilateral horizontal nystagmus. Visual acuity was limited to light perception in both eyes. Slit-lamp examination showed a calcified lens capsule with the absence of lens material in the right eye and the presence of an annular cataract in the left eye, corresponding to a unilateral lifebuoy cataract. She underwent cataract surgery with intraocular lens implantation. We report the clinical findings, anterior segment optical coherence tomography (AS-OCT), and surgical management tips. We noticed that during surgery, both anterior capsulorhexis and central membrane removal were the most challenging steps due to the absence of central nucleus and the strong adherence of central membrane to the anterior hyaloid

Removal of aqueous Clopyralid by Photoctalytic-ozonation process on Activated carbon under solar radiation: Catalyst characterization and kinetic study

Solar photocatalytic ozonation has been used to oxidize the herbicide clopyralid in aqueous solution. Activated carbon used as a catalyst was characterized by Scanning Electron Microscopy (SEM) images showed the presence of irregular cavities and pores, which have different sizes and shapes, with a great surface area of BETand a high pHpzc. The adsorption kinetics was found to follow the pseudo-second-order kinetic mode. Catalyst stability was tested by means of consecutive reuse cycles. After five cycle of reuse the adsorption efficiency reached 75% of the clopyralid removal.Clopyralid elimination kinetic by direct ozonation has been studies. The system O3/AC/Daylight significantly improves clopyralid and mineralization rate abatement if compared to runs conducted in the absence of radiation and/ or activated carbon. Clopyralid total abatement was achieved in less than 30 min when 0.5 g/L of activated carbon and under solar radiation (300-800 nm) were used. Thus, TOC removal in 180 min treatments increased from 20 to about 90 % in O3 and O3/OSAC/Daylight, respectively, under similar operating conditions