CHRONIC ATYPICAL NEUTROPHILIC DERMATOSIS WITH LIPODYSTROPHY AND FEVER (CANDLE SYNDROME) IN PEDIATRIC PRACTICE

2021 ◽  
Vol 100 (2) ◽  
pp. 254-261
Author(s):  
O.G. Sukhovjova ◽  
◽  
I.A. Ivanova ◽  
N.A. Kalugina ◽  
E.S. Zholobova ◽  
...  
Keyword(s):  
Clinical Observation ◽  
Clinical Manifestations ◽  
Maculopapular Rash ◽  
The Body ◽  
Neutrophilic Dermatosis ◽  
Inherited Disease ◽  
Laboratory Activity ◽  
Ubiquitinated Proteins ◽  
Systemic Inflammatory Reaction ◽  
Long Time

The purpose of this publication is to describe the CANDLE syndrome (its etiology, pathogenesis, clinical manifestations) and present a clinical observation. CANDLE syndrome is a rare genetically inherited disease caused by impaired assembly by of the protease, which leads to the accumulation of abnormal (ubiquitinated) proteins in B-lymphocytes, fibroblasts, macrophages and some other cells of the body. As a result, an excessive activation of interferon synthesis occurs and a systemic inflammatory reaction develops with symptoms such as febrile fever, skin syndrome, represented by nodules and maculopapular rash, hepatosplenomegaly, lipodystrophy, amyotrophy, delayed physical development, as well as high laboratory activity. The article describes a clinical observation of a female patient with typical symptoms of CANDLE syndrome. However, due to the rarity of the disease and lack of publications describing it, it has not been possible to diagnose the child for a long time.

scholarly journals Gastric duplication cyst with localization in the pancreas in children

2020 ◽  
Vol 10 (1) ◽  
pp. 35-42
Author(s):  
Yurii Yu. Sokolov ◽  
Artem M. Efremenkov ◽  
Aleksandr P. Zykin ◽  
Elena L. Tumanova ◽  
Zhanna R. Omarova ◽  
...  
Keyword(s):  
Mucous Membrane ◽  
Clinical Observation ◽  
Abdominal Cavity ◽  
Clinical Manifestations ◽  
Duplication Cyst ◽  
The Body ◽  
Recurrent Bleeding ◽  
Gastric Duplication ◽  
Gastric Duplication Cyst ◽  
Gastric Type

Introduction. Gastric duplication cyst is a rare clinical observation. More often, these cysts are localized in the fundus or body of the stomach and have a common muscle layers and blood supply. Even more rarely, duplication cysts are not anatomically connected to the stomach and are located in other parts of the abdominal cavity or in the retroperitoneal space. Cystic duplication of the gastrointestinal tract, which are localized in the pancreas, is extremely rare. The aim of the study is to demonstrate the possibility of using laparoscopy for this disease. Materials and methods. The paper describes three rare clinical observations of gastric duplication cyst topographically associated with the pancreas. In the first clinical observation, gastric duplication cyst was combined with mediastinal duplication cysts, an esophageal bronchial fistula, and extralobar pulmonary sequestrations. In the second observation, a duplication cyst had communication with the pancreatic duct system and was clinically manifested by recurrent bleeding. In the third case, a duplication cyst is diagnosed behind the body and tail of the pancreas. Results. In all cases, surgical treatment was carried out by the laparoscopic method, leading to complete recovery. Histological examination in all the described observations confirmed the gastric type of epithelium of the mucous membrane of the cysts. The article provides a review of the literature. Conclusion. Thus, the efficiency of laparoscopic interventions in children with gastric duplications topographically associated with the pancreas was demonstrated. The described rare clinical manifestations, a combination of defects, as well as the possible presence of heterotopy of the mucous membrane of the duplication cyst confirm the need for surgical correction of the disease.

scholarly journals Neglected intrauterine fetal demise for more than two decades leading to the development of a lithopedion: a case report

2019 ◽  
Vol 13 (1) ◽  
Author(s):  
Fitsum Fikru Gebresenbet ◽  
Abdu Mengesha Mulat ◽  
Namus Muhajir Nur ◽  
Ferehiwot Bekele Getaneh
Keyword(s):  
Vesicovaginal Fistula ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
Clinical History ◽  
The Body ◽  
Obstructed Labor ◽  
Fetal Demise ◽  
Case Presentation ◽  
Intrauterine Fetal Demise ◽  
Long Time

Abstract Background Lithopedion is a word derived from the Greek words lithos, meaning stone, and paidion, meaning child, to describe a fetus that has become stony or petrified. Lithopedion is a rare complication of pregnancy which occurs when a fetus dies and becomes too large to be reabsorbed by the body. This entity in rare circumstances can be challenging for physicians to diagnose since it has a range of clinical manifestations. Case presentation We present a case of a 55-year-old, gravida IV para III, Ethiopian woman from Ethiopia with a retained fetus and vesicovaginal fistula after an obstructed labor and a neglected intrauterine fetal demise of approximately 22 years. The diagnosis was confirmed by suggestive clinical history, physical examination findings, and an abdominopelvic computed tomography scan. Laparotomy and removal of the lithopedion was done and our patient was sent to a fistula hospital for vesicovaginal fistula repair. Conclusion This case is a rare phenomenon in which the dead fetus remained in the uterus for a long time after a neglected obstructed labor and uterine rupture.

scholarly journals Correction of micronutrient deficiencies is one of the priority directions in the practical work of a pediatrician

2019 ◽  
pp. 24-35
Author(s):  
I. N. Zakharova ◽  
Т. M. Tvorogova
Keyword(s):  
Clinical Manifestations ◽  
Physiological Role ◽  
Total Content ◽  
Functional Condition ◽  
Negative Influence ◽  
The Body ◽  
Water Soluble ◽  
Micronutrient Deficiencies ◽  
Long Time ◽  
Group B

Micronutrients (vitamins, minerals, micronutrients) are essential for the normal functioning of the body. The problem of vitamin deficiency leads to the risk of alimentary-dependent diseases. The reasons of inadequate provision with vitamins and minerals are given: unbalanced irrational nutrition, the quality of the products themselves, the nutritional value of which is significantly reduced with the use of modern production technologies. It is noted that the biological availability, i.e. the degree of absorption of vitamins from different products, varies widely from 5 to 80% of their total content in the product. In order to concretize the diverse physiological role of micronutrients as an important component of various enzyme systems and metabolic catalysts, the authors summarize the literature reflecting the importance of vitamins of group B, ascorbic acid, retinol, vitamin D and their functional correlations, as well as the need for a balanced and harmonious approach to their dosing. The problem of negative influence of deficiency of microelements on the structural and functional condition of various organs and systems, on morbidity, linear growth, development of various skills and psycho-emotional sphere of the child is covered. Attention is paid to the danger of micronutrient deficiency, which often remains unrecognized for a long time, because its clinical manifestations are not specific and can be observed in various diseases. However, the excess of individual micronutrients also disrupts the physiological and biochemical processes in the body. The vitamin requirements of healthy infants correspond to the amount of vitamins consumed daily with the mother’s milk: From 4-6 months of age, the provision of vitamins to the child is significantly affected by the introduction of complementary foods, for children from 1.5 to 3 years of age, the daily dose of vitamins and minerals in the VMC should not exceed 50% of the daily physiological needs, and for children over 3 years of age should not exceed 100% of the daily physiological needs for vitamins A, D and micronutrients (selenium, zinc, iodine, copper, iron), and 200% for water-soluble vitamins. An example of balanced and complete composition of vitamin complexes Multi-Tabs is given.

scholarly journals Thresholds of Endoglin Expression in Endothelial Cells Explains Vascular Etiology in Hereditary Hemorrhagic Telangiectasia Type 1

2021 ◽  
Vol 22 (16) ◽  
pp. 8948
Author(s):  
Georgios Galaris ◽  
Kévin Montagne ◽  
Jérémy H. Thalgott ◽  
Geoffroy J. P. E. Goujon ◽  
Sander van den Driesche ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Growth Factor ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Transforming Growth Factor ◽  
Clinical Manifestations ◽  
The Body ◽  
Vascular Lesions ◽  
Vascular Endothelial ◽  
Inherited Disease

Hereditary Hemorrhagic Telangiectasia type 1 (HHT1) is an autosomal dominant inherited disease characterized by arteriovenous malformations and hemorrhage. HHT1 is caused by mutations in ENDOGLIN, which encodes an ancillary receptor for Transforming Growth Factor-β/Bone Morphogenetic Protein-9 expressed in all vascular endothelial cells. Haploinsufficiency is widely accepted as the underlying mechanism for HHT1. However, it remains intriguing that only some, but not all, vascular beds are affected, as these causal gene mutations are present in vasculature throughout the body. Here, we have examined the endoglin expression levels in the blood vessels of multiple organs in mice and in humans. We found a positive correlation between low basal levels of endoglin and the general prevalence of clinical manifestations in selected organs. Endoglin was found to be particularly low in the skin, the earliest site of vascular lesions in HHT1, and even undetectable in the arteries and capillaries of heterozygous endoglin mice. Endoglin levels did not appear to be associated with organ-specific vascular functions. Instead, our data revealed a critical endoglin threshold compatible with the haploinsufficiency model, below which endothelial cells independent of their tissue of origin exhibited abnormal responses to Vascular Endothelial Growth Factor. Our results support the development of drugs promoting endoglin expression as potentially protective.

Clinical, morphological and biochemical signs of polymycotoxicosis in highly productive cows

2020 ◽  
pp. 14-17
Author(s):  
Irina A. Shkuratova ◽  
◽  
Lyudmila I. Drozdova ◽  
Aleksander I. Belousov ◽  
Keyword(s):  
Metabolic Disorders ◽  
Hepatorenal Syndrome ◽  
Morphological Changes ◽  
Clinical Manifestations ◽  
The Body ◽  
Feed Mixture ◽  
Milk Productivity ◽  
Structural Disorders ◽  
Increased Sensitivity ◽  
Penicillium Spp

Mycotoxicological monitoring of forages shows that the problem of mycotoxicosis has been relevant for several decades. Minimal doses of mycotoxins in feed lead to a decrease in milk productivity, increased sensitivity to infectious and non-infectious diseases. When several mycotoxins enter the body simultaneously, a synergistic effect develops, causing a significant increase in toxicity. Feed contaminated with several types of fungi and their toxins is dangerous for dairy cattle. It was found that the feed mixture contained the types of associations of Aspergillus spp. fungi + Fusarium; Aspergillus spp. + Penicillium spp. + Mucor spp; Fusarium + Penicillium; Mucor spp. + Fusarium + Ustilaginales. Pathogenetic features of metabolic and morphological changes in highly productive cows with polymycotoxicosis were studied. Feeding food contaminated with various metabolites of mold fungi leads to the development of signs of chronic toxemia in animals. Clinical manifestations are the development of diarrhea and dehydration, with a decrease in milk productivity. Metabolic disorders feature the development of an inflammatory process, metabolic acidosis, hyperfermentonemia, with an increase in the amount of creatinine and urea in the blood serum. Metabolic signs indicate the development of hepatorenal syndrome due to structural disorders of the liver and kidneys. Histological signs of polymicotoxicosis are intracapillary and hemorrhagic glomerulonephritis, hepatocyte micronecrosis, and proliferation of connective tissue stroma cells, which leads to the development of atrophic cirrhosis in the interstitial and circular phases.

HISTOLOGICAL CHANGES IN THE INTESTINES OF POULTRY DURING FODDER INTOXICATION

2020 ◽  
Author(s):  
E.P. Dolgov ◽  
◽  
A.A. Abramov ◽  
E.V. Kuzminova ◽  
E.V. Rogaleva ◽  
...  
Keyword(s):  
Body Weight ◽  
Histological Examination ◽  
Aflatoxin B1 ◽  
Structural Changes ◽  
Clinical Manifestations ◽  
Feed Additives ◽  
The Body ◽  
Histological Changes ◽  
Beet Pulp

The article presents the data on the study of the influence of mycotoxins combination (T-2 toxin at the concentration of 0.095 mg/kg and aflatoxin B1 in the concentration of 0.019 mg/kg) on the body of quails and the results of pharmacocorrection of toxicosis with a complex consisting of beet pulp and lecithin. Structural changes in the intestines of quais at fodder mycotoxicosis are described. The use of antitoxic feed additives in poultry led to a weakening of the action of xenobiotics, which was confirmed by an increase in the safety of poultry and increase in body weight of quails, a decrease in the clinical manifestations of intoxication, as well as in positive changes in the structure of the intestine of the poultry during histological examination.

Respiratory tract damage with bocavirus infection in children

2020 ◽  
Vol 19 (2) ◽  
pp. 14-18
Author(s):  
E. V. Sharipova ◽  
I. V. Babachenko ◽  
M. A. Shcherbatyh
Keyword(s):  
Respiratory Tract ◽  
Lower Respiratory Tract ◽  
Clinical Manifestations ◽  
Respiratory Viruses ◽  
Community Acquired Pneumonia ◽  
Human Bocavirus ◽  
Long Time ◽  
Clinically Significant ◽  
Tract Infections ◽  
Tract Damage

Long time the main pathogens associated with the development of community-acquired pneumonia were bacteria. However, in recent years in the Russian Federation, like all over the world, the view of the damage of lower respiratory tract changed, including a unique approach to community-acquired pneumonia as a bacterial infection, and respiratory viruses have become seen as a direct cause of lower respiratory tract damage, or as part of a viral-bacterial co-infection. These studies became possible since the widespread introduction of PCR techniques in the clinical setting, identification of respiratory viruses has increased and new microorganisms such, one as human bocavirus have been discovered. Objective: to study the features of respiratory tract damage in acute bocavirus infection in children of different ages. Materials and methods: A retrospective analysis of 97 medical hospital documentation of children with acute bocavirus infection, detected confirmed by PCR in nasopharyngeal aspirate. Results: In this work, it was shown that human bocavirus spread throughout the year with an increase in the incidence of clinically significant forms in the autumnwinter period, including during the period of an increase in the incidence of influenza. HBoV infection requiring hospitals is most significant in the first three years of life. In 74.2% of hospitalized children, bocavirus infection occurs with lower respiratory tract infections in the form of bronchitis — 77.8%, pneumonia — 28.9% and rarely bronchiolitis and is complicated by the development of respiratory failure in 28.9% of cases. Changes in the blood test are non-specific, and the level of C-reactive protein in children with various clinical manifestations of HBoV infection generally does not exceed 50 mg / l. An x-ray of the chest organs does not objectively reflect the existing volume and nature of the inflammatory process in the lungs.

scholarly journals Development of Novel Oral Formulations of Disulfide Antioxidants Based on Porous Silica for Controlled Release of the Drugs

Materials ◽  
2021 ◽  
Vol 14 (4) ◽  
pp. 963
Author(s):  
Ekaterina S. Dolinina ◽  
Elena V. Parfenyuk
Keyword(s):  
Antioxidant Activity ◽  
Surface Chemistry ◽  
Sol Gel ◽  
Porous Silica ◽  
The Body ◽  
Oral Drug ◽  
Therapeutic Concentration ◽  
Release Process ◽  
Zero Order Kinetics ◽  
Long Time

Powerful antioxidant α-lipoic acid (LA) exhibits limited therapeutic efficiency due to its pharmacokinetic properties. Therefore, the purpose of this work was to evaluate the ability of silica-based composites of LA as well as its amide (lipoamide, LM), as new oral drug formulations, to control their release and maintain their therapeutic concentration and antioxidant activity in the body over a long time. The composites synthesized at different sol–gel synthesis pH and based on silica matrixes with various surface chemistry were investigated. The release behavior of the composites in media mimicking pH of digestive fluids (pH 1.6, 6.8, and 7.4) was revealed. The effects of chemical structure of the antioxidants, synthesis pH, surface chemistry of the silica matrixes in the composites as well as the pH of release medium on kinetic parameters of the drug release and mechanisms of the process were discussed. The comparative analysis of the obtained data allowed the determination of the most promising composites. Using these composites, modeling of the release process of the antioxidants in accordance with transit conditions of the drugs in stomach, proximal, and distal parts of small intestine and colon was carried out. The composites exhibited the release close to the zero order kinetics and maintained the therapeutic concentration of the drugs and antioxidant effect in all parts of the intestine for up to 24 h. The obtained results showed that encapsulation of LA and LM in the silica matrixes is a promising way to improve their bioavailability and antioxidant activity.

Acute exudative paraneoplastic polymorphous vitelliform maculopathy in a patient with thymoma, myasthenia gravis, and polymyositis

2021 ◽  
pp. 112067212199404
Author(s):  
He Yu ◽  
Xinyu Ma ◽  
Nianting Tong ◽  
Zhanyu Zhou ◽  
Yu Zhang
Keyword(s):  
Myasthenia Gravis ◽  
Medical Center ◽  
Postoperative Radiotherapy ◽  
Clinical Manifestations ◽  
Subretinal Fluid ◽  
Metastatic Tumor ◽  
The Body ◽  
Pleural Thickening ◽  
History Of ◽  
Clinically Significant

Importance: This is the first reported case of acute exudative paraneoplastic polymorphous vitelliform maculopathy (AEPPVM) in a patient with thymoma, accompanied by myasthenia gravis (MG) and polymyositis. Objective: To examine the pathogenesis of ocular disease in a patient with yolk-like fundus lesions and thymoma, MG, and polymyositis throughout the body based on clinical manifestations, diagnosis, differential diagnosis, and genetic testing to determine the appropriate treatment course. Design, setting, and participants: We describe a 63-year-old woman who presented to our tertiary medical center with a 3-month history of reduced visual acuity in both eyes. Concurrent fundoscopy revealed a 2.0 × 1.7-mm, unifocal, yellow, round vitelliform lesion in the macular region, surrounded by multifocal, shallow, yellow-white pockets of subretinal fluid. The patient’s medical history included thymoma with thymectomy treatment, combined with pericardiectomy and postoperative radiotherapy (20 years prior), followed by a diagnosis of MG with suspect thymic association (15 years prior). Three years prior, the patient had been diagnosed with polymyositis related to paraneoplastic syndrome; 1 year prior, she had been examined for pleural thickening due to suspected metastatic tumor. Results: On her most recent follow-up visit at 3 months after initial diagnosis, the patient was stable with no clinically significant progression in ocular or systemic conditions.

scholarly journals Mangiferin as New Potential Anti-Cancer Agent and Mangiferin-Integrated Polymer Systems—A Novel Research Direction

Biomolecules ◽  
2021 ◽  
Vol 11 (1) ◽  
pp. 79
Author(s):  
Svetlana N. Morozkina ◽  
Thi Hong Nhung Vu ◽  
Yuliya E. Generalova ◽  
Petr P. Snetkov ◽  
Mayya V. Uspenskaya
Keyword(s):  
Molecular Mechanisms ◽  
Biological Activities ◽  
Research Direction ◽  
Biological Action ◽  
The Body ◽  
Biologically Active ◽  
Polymer Systems ◽  
Anti Cancer ◽  
Long Time ◽  
Cancer Agent

For a long time, the pharmaceutical industry focused on natural biologically active molecules due to their unique properties, availability and significantly less side-effects. Mangiferin is a naturally occurring C-glucosylxantone that has substantial potential for the treatment of various diseases thanks to its numerous biological activities. Many research studies have proven that mangiferin possesses antioxidant, anti-infection, anti-cancer, anti-diabetic, cardiovascular, neuroprotective properties and it also increases immunity. It is especially important that it has no toxicity. However, mangiferin is not being currently applied to clinical use because its oral bioavailability as well as its absorption in the body are too low. To improve the solubility, enhance the biological action and bioavailability, mangiferin integrated polymer systems have been developed. In this paper, we review molecular mechanisms of anti-cancer action as well as a number of designed polymer-mangiferin systems. Taking together, mangiferin is a very promising anti-cancer molecule with excellent properties and the absence of toxicity.

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