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The goal of integrated explosion safety is a concept that was developed to prevent the formation of explosive atmospheres as well as sources of ignition and, in the event that an explosion does take place, to immediately stop to it and/or to limit the damage it causes. Ignition of explosive atmospheres by non-electrical equipment occurs when energy supplied by equipment used for processing or conveying of material is converted into heat, usually as the result of a mechanical failure of the equipment or associated systems. In light of this fact, the manufacturer has a responsibility to take precautions with regard to the potential ignition sources. In addition, the non-electrical equipment must be designed and manufactured after a thorough analysis of the possible operating faults. This is done in order to prevent potentially dangerous situations as much as possible by taking into account any misuse that can be anticipated in a reasonable manner. The most important aspect of the ignition hazard assessment for the non-electrical equipment is the identification of the probable ignition sources. The paper shows the methodology of the ignition hazard assessment for the non-electrical equipment intended for use in potentially explosive atmospheres.

In order to improve the safety and health protection of workers who may be exposed to a potential risk due to explosive atmospheres, in every industrial area where such atmospheres may occur, the employer shall ensure the development and updating of a document called Explosion Protection Document. The main chapter of this document is the Explosion Risk Assessment. Risk assessment is a complex process that involves identifying the risk, analyzing the risk and estimating the risk, the main purpose of assessing the level of risk being to establish the necessary protection measures to reduce it to acceptable levels. This paper identifies the factors that may influence the level of risk of explosions in the workplace in order to establish the protection measures required to reduce the risk to acceptable levels. Explosion risk assessment must be performed by competent persons with knowledge of technical, electrical and mechanical engineering and who understand the general principles of explosion protection.

The equipment of installations for the processing, transport or storage of flammable substances which may generate an explosive atmosphere, in or around them, must be assessed in terms of the risk of explosions, both when they are put into operation or when the installation is modified and periodically for verify that the initial level of protection is maintained. The purpose of the assessment is to establish appropriate protective measures to prevent sources of ignition that could initiate explosive atmospheres. Explosion risk assessment is an obligation of the employer who must draw up an explosion protection document, as regulated in GD 1058/2006 transposing European Directive 1999/92/EC. The explosion risk assessment process focuses primarily on the formation of explosive atmospheres and then on the presence and activation of ignition sources. The principle of explosion protection is to reduce the probability of an ignition source occurring at the same time as the explosive atmosphere, to a minimum acceptable level according to the applicable norms and standards. The paper presents some aspects regarding the evaluation of the risk of explosions to already installed equipment, assembled in an installation, depending on the specific conditions.

For any type of combustible dust, several important explosive parameters must be taken into account when designing and using protection systems: namely the ease with which dust clouds ignite and their burning rate, maximum explosion pressure, maximum pressure rise speed. of explosion. The accuracy of the results obtained necessary for the design and use of protection systems depends on a number of factors but also on the accuracy of the application of the test method (s) by the personnel involved. Test quality assurance is a requirement of EN ISO / IEC 17025 for the accreditation of testing laboratories. The standard requires laboratories to have a procedure in place to monitor the validity of test results, which involves participation in interlaboratory comparisons. This paper presents some specific issues highlighted during the successful participation of INSEMEX-GLI in several rounds of interlaboratory comparisons for the tests to determine explosive parameters of the combustible dust-air mixtures.

Combustible dusts which are present in workplaces are a significant hazard which cannot be ignored by the plant owners, managers and workers. Combustible dust deflagrations and explosions have caused large numbers of deaths and catastrophic property damages in various industries, ranging from pharmaceutical plants to sugar factories. One may say that dust explosions in process industries always start inside process equipment such as mills, dryers, filters. Such events may occur in any process in which a combustible dust is handled, produced or stored, and can be triggered by any energy source, including static electricity, friction and hot surfaces. For any combustible dust type, several important parameters have to be taken into account when designing and using protective systems: i.e. the ease with which dust clouds ignite and their burning rates, maximum explosion pressure, maximum rate of explosion pressure rise. These parameters vary considerably depending on the dust type, their knowledge being a first step for carrying out a proper explosion risk assessment in installations which circulate combustible dusts. The paper presents the main aspects concerning explosion protection which have to be taken into account when designing protective systems intended to be used in explosive atmospheres generated by combustible dusts and the importance of selecting the proper explosion protection technique.

Spontaneous combustion is a phenomenon that results from the heating of combustible organic powders by slow oxidation and which occurs through the air passage (created by an air depression) through the mass of dust. The oxidation phenomenon of combustible powders represents their reaction with atmospheric oxygen resulting in products of carbon dioxide, carbon oxide, water and other gases whose content depends on the temperature at which the oxidation takes place. The self-ignition of combustible dusts depends on their chemical composition, the properties of component substances, on the particle size and geometry of the material mass and, last but not least, on the temperature of the environment. Due to global worries of sustainability in construction engineering the trend is to use ecofriendly organic waste to various purposes as in construction materials. The challenge is that by using this kind of materials one should ensure the safety related to the process of such organic materials which are known to have combustible properties. The purpose of this work is to present the self-ignition behavior of combustible dusts such as sunflower and wood by means of drying tests under constant temperature conditions.

INSEMEX-OEC is a Conformity Assessment Body for certification activities in accordance with ATEX Directive 2014/34/EU, being accredited by RENAR and notified to Brussels (NB1809). INSEMEXGLI (Group of Testing Laboratories) is the main provider of laboratory testing services for the product certification body INSEMEX-OEC. The test laboratories meet the requirements of the standard SR EN ISO / IEC 17025. This standard requires laboratories to have a procedure in place to monitor the validity of the test results. This monitoring must be planned and, if possible, should include participation in inter-laboratory comparisons or proficiency testing programs. This paper presents some specific issues highlighted during the successful participation of INSEMEX-GLI in a round of inter-laboratory comparisons organized by the Physikalisch-Technische Bundesanstalt from Germany to determine International Protection Code (IP Code) for equipment enclosures.

During the technological processes of processing, production, handling and storage of combustible dusts, complex explosive mixtures may occur, the characteristics of which, in most cases, cannot be assimilated with the existing data in the specialized literature. If these combustible dusts are mixed with air in appropriate proportions and are initiated by an efficient source of ignition, they can burn rapidly and with considerable explosive force. One of the most common sources of ignition of potentially explosive atmospheres generated by the dust / air mixture is static electricity, materialized by electrostatic discharges. In order to assess the risk of ignition of an explosive mixture of air / dust, it is necessary to know the sensitivity of the explosive atmosphere to ignition, ie the value of the minimum ignition energy of the explosive mixture, which is then compared with the energy resulting from an electrostatic discharge. The paper presents a comparative analysis regarding the methods of determining the minimum ignition energy for air / fuel dust mixtures, using different devices, on the same type of dust.

According to the ATEX directive, any personal protective equipment or work equipment intended to be used in a potentially explosive area must be made of materials that cannot be the source of mechanical or static electricity-related sparks. The carbon steel is one of the most widely used metallic materials, but the possibility of using it in the manufacture of equipment used in explosive environments is low. Therefore, the purpose of this article is to present an alternative solution to use metal equipment in potentially explosive atmospheres, by depositing a phosphate layer on the surface of the carbon steel. The metal was coated by a simple process, the metal being immersed in a phosphating solution based on zinc. Due to the properties of zinc phosphate deposited on the steel surface, especially electrical insulating, the steel coated with this type of layer can be used in explosive atmosphere.

Non-electrical equipment has been used for over 150 years in industries with potentially explosive atmospheres and great experience has been gained with regard to the application of protective measures to reduce the risk of ignition down to an acceptable safety level. The use of non-electrical equipment in explosive atmospheres required the development of specific requirements with regard to the concept of protection against the ignition of explosive atmospheres, which to clearly define protection measures and to include the experience gained and extended over the years. The practical studies, laboratory research and methods for assessing and testing the hazard of ignition by hot surfaces presented within the paper have as main purpose the improvement of ignition hazard assessment in different operating conditions.

Carcasses of electrical or non-electrical equipment operating in potentially explosive atmospheres must provide a certain degree of protection against access to hazardous parts, penetration of solid bodies and/or water penetration, which must be tested according to the specifications of the Ex protection types. Normal protection classes are indicated by the international IP code followed by two characteristic digits referring to the protection against penetration of solid foreign bodies and water protection, followed by optional letters if required by the beneficiary according to the imposed conditions of use. Within the ENExEMEIP laboratory, have been developed laboratory tests to verify the first characteristic figure of the standard degree of protection according to the standardized European method. In the paper is presented the test stand of the large gauge equipment, a stand made with state-of-the-art equipment that provides the necessary performance for the accreditation of the test according to the standardized requirements. Checking dustproof protection to equipment used in areas where explosive atmospheres generated by combustion dust may occur is particularly important for assessing compliance with the requirements of the ATEX Directive [5], as this protection is a basic requirement for explosion protection.

Skeletal dysplasia (SD), also called osteochondrodysplasia (OCD), is a large group of skeletal disorders (over 400 distinct entities) caused by abnormalities in bone development and growth. SDs varies according to different natural histories, prognoses, hereditary patterns to etiopathogenetic mechanisms. At birth, the incidence is low, reported at the level of each entity, but taken collectively; the incidence is estimated at 1:5000 births. Nosology is a branch of medical science. It deals with the systematic classification of diseases and disorders. Thus, combining information about the catalogue of clinically distinct disorders, pending molecular explanations, and genotype-phenotype correlations, the classification of SDs will be more accurate. This is extremely useful for diagnosing patients with genetic skeletal diseases, especially given the expected flow of information with new sequencing technologies. Over the years, various terms and classifications of SD have been used and have attempted to order and classify this group of genetic diseases according to clinical, radiological, and molecular criteria. In 2019, the Nosology Committee of the International Skeletal Dysplasia Society (ISDS) updated the classification of SD. This new classification divides SD into 42 large groups that include 461 entities. Advances in next-generation sequencing techniques have revolutionized the entire field of genetics, with 437 different genes are currently identified in 426 (92.4%) of SDs. Nosology is a real help for the clinician in establishing a diagnosis as accurately as possible, for the recognition of new diseases while serving as a guide for the interpretation of new genetic variants.

Cardiofaciocutaneous (CFC) syndrome [Online Mendelian Inheritance in Man (OMIM) #115150] is characterized by craniofacial dysmorphism, heart malformation, ectodermal abnormalities, neuromotor delay and intellectual disability. It is not a frequent disease, about 300 cases have been reported in the medical literature. We describe the case of a 34-year-old patient presenting with CFC syndrome phenotype, monitored since the age of 1 1∕2 years. Clinical findings included craniofacial dysmorphism, development delay, heart malformation and severe intellectual disability. The evolution was with progressive intellectual disability, hypogonadism, hypertrophic cardiomyopathy, wrinkled palms and soles. Molecular analysis showed a heterozygous variant in the B-Raf proto-oncogene, serine∕threonine kinase (BRAF) gene (7q34): NM_001354609.2:c.1502A>G, with pathogenic significance. We report this case, observed along a period of 33 years, for illustration of clinical evolutive particularities, and for difficulties in establishing the positive diagnosis.

When we discuss the genetics of tumors, we cannot fail to remember that in the second decade of the twentieth century, more precisely in 1914, Theodore Boveri defined for the first time the chromosomal bases of cancer. In the last 30 years, progresses in genetics have only confirmed Boveri's remarkable predictions made more than 80 years ago. Before the cloning of the retinoblastoma 1 (RB1) gene, the existence of a genetic component in most, if not all, solid childhood tumors were well known. The existence of familial tumor aggregations has been found much more frequently than researchers expected to find at random. Sometimes, the demonstration of this family predisposition was very difficult, because the survival of children diagnosed as having a certain tumor, up to an age at which reproduction and procreation is possible, was very rare. In recent years, advances in the diagnosis and treatment of these diseases have made it possible for these children to survive until the age when they were able to start their own families, including the ability to procreate. Four distinct groups of so-called cancer genes have been identified: oncogenes, which promote tumor cell proliferation; tumor suppressor genes, which inhibit this growth/proliferation; anti-mutational genes, with a role in deoxyribonucleic acid (DNA) stability; and micro-ribonucleic acid (miRNA) genes, with a role in the posttranscriptional process.

Uveal melanoma is the most common intraocular tumor characterized by increased metastatic potential. The tumor develops from uveal melanocytes that, from an embryological point of view, derive from the cells of the anterior neural crest. The risk factors associated with melanoma development are close related to patient phenotype: light-colored hair and iris, fair skin. Recent studies have shown the link between choroidal melanoma and choroidal pigmentation of white-haired and light-colored eyes individuals. Increased pigmentation of the choroid is associated to marked increase in melanocyte density, which from a histological point of view can be a starting point for choroidal carcinogenesis. This case report is about a 36-year-old patient who presented in an outpatient ophthalmological examination for intermittent vision blurring. The ophthalmological examination revealed a view of 0.8 and nasally from the optic nerve head, a prominent tumor-shaped formation was detected during the fundus examination. The presence of a choroidal melanoma was suspected and eye ultrasound, angio-fluorography, optical coherence tomography (OCT) were performed and confirmed the diagnosis. Due to the small size, a team of three ophthalmologists decided to make brachytherapy. The procedure consisting of brachytherapy was temporarily applied to the scleral wall and was done at Debrecen University Hospital, Hungary, the follow-up monitoring being done at the Clinic in Oradea, Romania. Although the initial prognosis was optimistic, after four years of brachytherapy the tumor recurred, the dimensions found were over 14 mm, which is why enucleation was decided. Prior to enucleation, no computed tomography (CT) metastases were detected. Five months after enucleation, hepatic metastases occurred and after another two months, death occurred.

Introduction: Congenital anomalies of digits (CAD) can occur as isolated malformations, in combination with other malformation of the limbs, or as part of a genetic syndrome. The purpose of this work is to provide an overview of CAD, on morphological, genetic and epidemiological basis., Patients and Methods: We conducted a retrospective analysis of a cohort of 301 patients with CAD. Following the Swanson classification, the list of anomalies under study included: adactyly and oligodactyly, syndactyly and symphalangism, polydactyly, macrodactyly, amniotic bands syndrome, and generalized skeletal anomalies., Results: In Bihor County, Romania, the Department of Medical Genetics recorded 4916 patients with congenital anomalies (2.03% out of 241 601 live newborns) between 1984 and 2018. Of these, 301 (6.1%) patients had CAD. The prevalence of CAD was 1:800 living newborns. The most common CAD were polydactyly, followed by syndactyly, brachydactyly, adactyly and oligodactyly. Upper extremities were four times more frequently affected than lower extremities, while both upper and lower extremities were affected in a quarter of all cases. CAD were isolated in 64% of patients, while 14% were associated with other anomalies of the extremities and 22% were associated with recognized genetic syndromes., Conclusions: Our study, by its size and the long period of clinical observation, provides opportunities to generalize and compare our data with similar studies, offering the possibility for improved knowledge of the epidemiology of CAD and potential improvements in genetic counseling.

Empty sella means the absence of the pituitary gland on cranial computed tomography or magnetic resonance imaging. Empty sella syndrome is the pathological variant of the imaging-described empty sella. We present the case of a male Caucasian child, aged four years and two months, for short stature and diagnosed by imaging procedures as empty sella. The cause of short stature was isolated growth hormone (GH) deficiency. Associated he presented left hand postaxial polydactyly. In connection with this particular case, we propose a review of current knowledge in empty sella syndrome. The particularity of reported case consists of association empty sella with GH deficiency and polydactyly. The association of empty sella with polydactyly is not reported yet in the medical literature and is probably coincidental.

The co-occurrence in the same individual of two numerical chromosomal abnormalities (double aneuploidy) is a very rare condition, especially for autosomes. Clinical presentations are variable depending on the predominating aneuploidy. The authors present a rare case of a male infant with multiple congenital anomalies: craniofacial dysmorphism, short neck, agenesis of the corpus callosum, ventricular septal defect, bilateral broad hallux, large first interdigital space of the toes, plantar furrows, prominent calcaneus and right kidney agenesis. The karyotype identified 82% of mitosis with trisomy 8 (47,XY,+8) and 18% with trisomy 21 (47,XY,+21). The evolution was fatal because of eating difficulties, severe growth retardation and recurrent respiratory infections. He died at the age of five months. We report this case as a very rare double autosomal mosaicism, with a complete clinical and morphological description, as the first documented case in Romania.

Congenital hyperammonemia (HA) due to inborn errors of metabolism is a rare condition with a high rate of mortality. The main effects occur at the central nervous system (CNS) level, being neurotoxic by alteration of the neurotransmitter function. HA can be triggered by an inappropriate diet, infection or stress, but can also occur without a precise cause. In cases of metabolic crises, patients require immediately intensive care. In the last seven years (2011-2017), we cared in the Department of Genetics, "Dr. Gavril Curteanu" Municipal Clinical Hospital, Oradea, Romania, six patients with different causes of congenital HA: one case with argininosuccinate lyase deficiency, two cases (brothers) with argininosuccinate synthase deficiency, one case with non-ketotic hyperglycinemia, one case hyperglycinemia and one case with HA with unknown etiology. The medical surveillance and care of these children over a long period of time raise serious problems for the family and society. These patients are dependent on medical services: qualified medical staff (pediatrician, geneticist, radiologist, biochemist, nutritionist, and psychologist), expensive and repeated medical investigations, prolonged and costly medication. Most of these costs could be avoided by early diagnosis and treatment, rigorous monitoring of HA, ensuring proper diet and medication. Our experience regarding the clinical and genetic particularities of patients with congenital HA could be an opportunity for the better knowledge of special needs of these patients, especially regarding the psychological and social aspects.

Dandy-Walker complex (DWC) is a malformative association of the central nervous system. DWC includes four different types: Dandy-Walker malformation (vermis agenesis or hypoplasia, cystic dilatation of the fourth ventricle and a large posterior fossa); Dandy-Walker variant (vermis hypoplasia, cystic dilatation of the fourth ventricle, normal posterior fossa); mega cysterna magna (large posterior fossa, normal vermis and fourth ventricle) and posterior fossa arachnoid cyst. We present and discuss four cases with different morphological and clinical forms of the Dandy-Walker complex. In all four cases, diagnosis was reached by incorporation of clinical (macrocephaly, seizures) and imaging [X-ray, computed tomography (CT), magnetic resonance imaging (MRI)] data. Two patients were diagnosed with Dandy-Walker complex, one patient was diagnosed with Dandy-Walker variant in a rare association with neurofibromatosis and one patient was diagnosed with a posterior fossa arachnoid cyst associated with left-sided Claude Bernard-Horner syndrome, congenital heart disease (coarctation of the aorta, mitral stenosis) and gastroesophageal reflux. In all forms of DWC, the clinical, radiological and functional manifestations are variable and require adequate diagnostic and therapeutic measures.

Jacobsen syndrome (JS) is a contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. The syndrome is rare and there are very few observations regarding the pubertal period of the affected individuals. We report the case of a 22-year-old female, with JS, monitored since the age of three months. She presented intrauterine growth retardation, failure to thrive and feeding difficulties from the first year of the life, and she learned to walk at the age of four years. Phenotypically, the case is characterized by distinctive facial and limb abnormalities. She shows spasticity and profound delay in gross and fine motor skills. Additionally, she has severe learning difficulties, non-verbally communicates, and displays hetero-aggressive and auto-aggressive behavior. The evolution of puberty was characterized by hypogenitalism and primary amenorrhea. Thrombocytopenia and IgM deficiency became apparent also at puberty. Array comparative genomic hybridization (aCGH) analysis confirmed a deletion of 16.3 Mb on 11q23.3-q23.4. We report this case as the first documented case of JS in Romania, as well as for clinical particularities (long period of survival and late appearance of hematological and immunological disorders).