Atypical Course Research Articles

EXTH-81. A RARE CASE OF RELENTLESSLY PROGRESSIVE METASTATIC PINEOCYTOMA MANAGED WITH SEQUENTIAL MULTIMODAL THERAPY

Abstract BACKGROUND Pineocytoma is a rare pineal parenchymal WHO grade 1 tumor, typically well-managed with surgical resection alone. With gross total resection (GTR), 91% 1-year and 84% 5-year survival rates have been reported. Recurrent pineocytoma is rare and optimal management in that setting is unknown, in part owing to the rarity of the condition. METHODS We report an atypical course of pineocytoma in a 58-year-old male involving metastatic disease. RESULTS A pineal mass was identified upon MRI of the brain obtained for vision changes. Patient underwent GTR, pathology was consistent with pineocytoma, WHO grade 1. Ki-67 labeling index was low at 1-2%. Despite low-grade pathology, two years later, surveillance imaging demonstrated recurrence in the pineal region and a second lesion in the right thalamus. The patient underwent gamma knife surgery (GKRS) to both lesions. Three years later, he developed three new lesions in the right cerebellum and again underwent GKRS. Subsequent surveillance imaging identified new lesions in the right cerebellum and along ventricular walls, treated with further GKRS. Nine years after initial diagnosis, focal nodular leptomeningeal metastases were discovered, along with progression at the primary tumor site. Patient was treated with GKRS to the nodular deposits followed by pembrolizumab. Further disease progression as noted despite immunotherapy. Patient was treated with 6 cycles of temozolomide chemotherapy and at further progression 3 cycles of lomustine. Upon additional progression, further radiation therapy was administered with bevacizumab to reduce risk of radiation necrosis. Patient remains on surveillance 13 years after initial diagnosis with visual changes and mild headache representing only clinical issues. CONCLUSIONS Although exceedingly rare, pineocytoma is capable of metastasizing to brain parenchyma and the leptomeninges, as demonstrated in our patient. However, our case demonstrates prolonged survival is possible even in the setting of relentlessly progressive metastatic disease with utilization of multimodality treatment.

Clinical observations of skin Kaposi’s Sarcoma

Kaposi's sarcoma (KS) is an angioproliferative disease of endothelial origin associated with human herpes virus-8 (HHV-8), or Kaposi's sarcoma herpesvirus (KSHV). The disease was first described in 1872 by Moritz Kaposi as a pigmented cutaneous sarcoma localized on the lower extremities. The disease is considered quite rare, but at present there is an increase in the incidence worldwide, due to which there is a growing interest in this problem. Today, it remains the leading skin pathology among HIV-positive patients, occurring in 20%. However, the assessment of the pathological process can be difficult, and the diagnosis may not be made at all, due to atypical clinical manifestations (especially in HIV-negative patients), so the problem of KS diagnostics remains relevant for doctors of any specialty, especially dermatovenerologists and oncologists. Kaposi's sarcoma can affect almost any organ, including the skin. The most common localization is on the skin of the extremities and face. Clinical manifestations are rashes in the form of spots, nodules, plaques, nodes. Kaposi's sarcoma is classified according to clinical and epidemiological forms: classical, endemic, epidemic and iatrogenic. Recently, a fifth form has been described, called non-epidemic KS, which is observed in HIV-negative MSM (men who have sex with other men). These clinical cases demonstrate an atypical clinical course of Kaposi's sarcoma of the skin with the absence of HIV-1, HIV-2 and HHV-8 in the blood of the presented patients.

Persistent renal dysfunction post-chemotherapy: a diagnostic conundrum in pediatric cancer survivorship – a case report

BackgroundLate-onset type II Bartter syndrome is an exceedingly rare condition, with only six documented cases presenting symptoms and signs beyond infancy. We report a unique case of late-onset type II Bartter syndrome with an atypical presentation and clinical course following chemotherapy treatment during childhood.Case presentationA 10-year-old boy, diagnosed with hepatoblastoma at age 2 and treated with cisplatin and epirubicin, presented with polyuria, polydipsia, failure to thrive, and electrolyte imbalances. He exhibited hypokalemia, metabolic alkalosis, and elevated urinary excretion of sodium, chloride, calcium, and magnesium. Whole exome sequencing and Sanger sequencing identified compound heterozygous variants in the KCNJ1 gene, confirming the diagnosis of type II Bartter syndrome. The patient’s clinical presentation was distinct from previously reported cases, with an absence of nephrocalcinosis, unusually small and hyperechoic kidneys, and a substantial decline in kidney function. Treatment included oral potassium supplementation, spironolactone, and angiotensin-converting enzyme inhibitors.ConclusionsThis case highlights the importance of considering late-onset Bartter syndrome in patients with a history of chemotherapy presenting with persistent electrolyte imbalances and ongoing renal dysfunction. The atypical features and rapid progression of chronic kidney disease in this patient may be attributed to the deleterious nature of the identified variants and the potential impact of previous chemotherapy on kidney susceptibility to damage. Careful monitoring and management of electrolyte imbalances and renal function are crucial in such cases.

Modern approaches in the diagnosis, treatment and prevention from obesity

Today, from 1.0 to 1.9 billion adults are overweight, including 312-650 million of them, and 124 million children and adolescents are obese. In Russia and Europe, about 60% of residents are obese and overweight. According to WHO forecasts, by 2025 the number of obese patients will double and will account for 30-50% of the population of economically developed countries. Morbid obesity is usually a multifactorial disease that is acquired and progresses under the influence of many factors (geography, level of economic development, cultural values, lifestyle, and others) as well as hereditary predisposition. Among people suffering from comorbid obesity, there is an atypical course of concomitant comorbid diseases. Against the background of morbid obesity, the likelihood of acquiring other diseases of organs and systems increases: the cardiovascular system, hormonal system, digestive tract, erosive esophagitis, fatty liver dystrophy, cirrhosis of the liver, obstructive sleep apnea, osteoarthritis, etc. To confirm the presence of morbid obesity in humans, the following indicators are evaluated: the number of kilograms per square meter of human body area (BMI calculation), waist circumference, triglyceride index, as well as the use of instrumental diagnostics: radiation and ultrasound methods to study the level of obesity of internal organs. To date, orlistat, sibutramine and liraglutide have been registered in Russia for the conservative therapy of morbid obesity, i.e. the choice of drugs is very limited. Surgical methods and electrical stimulation of the stomach are also used. Obesity ranked 5th in the world ranking of risk factors for premature death. A targeted policy and an integrated approach aimed at improving the system of medical care for overweight and obese patients should become key factors in achieving the national goal of improving people's health and well-being. The expansion of preventive measures at the local, regional and federal levels will help significantly reduce the spread of this pathology and prevent the development of an "obesity pandemic".

7962 Transformation of Poorly Differentiated Papillary Thyroid Carcinoma to Anaplastic Thyroid Carcinoma, an Atypical Course!

Abstract Disclosure: T. Altaweel: None. S. Makadsi: None. M. Hussein: None. J. Samantray: None. Introduction: Anaplastic thyroid cancers are undifferentiated, rare and aggressive. The transformation of papillary thyroid cancer to anaplastic thyroid cancer is documented in literature, however, rare. In this report, we present a case of Anaplastic thyroid carcinoma likely arising from a co-existing poorly differentiated Papillary carcinoma. Case Presentation: A 74-year-old male with past medical history of tobacco smoking who presented with the onset of voice hoarseness for 6 months. He has also noticed a left neck mass that is in the mid supraclavicular region. A Neck CT neck was performed at the time of presentation and was significant for a 4.6x3.4x5.9 cm left thyroid mass with displacement of the trachea. FNA biopsy showed papillary carcinoma with focal features of Tall Cell Variant. Poorly differentiated components with fragments of stroma and muscle were seen and suggested invasion. These findings raised the possibility of anaplastic thyroid carcinoma in the setting of papillary carcinoma and poorly differentiated carcinoma. BRAF V600E pathogenic variant mutation along with TERT mutation were found. Patient underwent direct laryngoscopy, bronchoscopy with biopsies, and tracheostomy with surgical pathology confirming papillary thyroid cancer with muscles invasion. Combination dabrafenib and trametinib were started. No surgical resection of thyroid tissue was planned. A follow up CT showed a significant decrease in the low-density soft tissue involving the medial thyroid gland bilaterally, consistent with interim positive response to therapy. Three months later, CT neck showed near complete resolution of low-density soft tissue involving the medial thyroid and parathyroid glands and subglottic trachea consistent with further interim positive response to therapy. Six months post initiation of therapy, CT Neck showed focal low density in the mid and inferior right thyroid gland which may represent residual and/or recurrent thyroid carcinoma. That density was further demonstrated on a repeat CT neck as a 1.8 cm hypodense nodule in the inferior right thyroid gland and concerning for recurrent thyroid tumor with possible infiltration of the trachea. Conclusion: ATC prognosis is affected by many factors such as metastasis, age, treatment strategies, socioeconomic status and the presence of BRAF V600E and TERT mutations. Diagnosis can be challenging. Our patient presented months after his symptoms started and this is an atypical course of Anaplastic Thyroid Carcinoma as it usually progresses aggressively and rapidly. Given the pathology reports and the more slow presentation of this patient’s carcinoma, it can be suggested that this is a case of papillary carcinoma with a later transformation to ATC. In these cases, surgical resection Presentation: 6/2/2024

Traumatic Middle Meningeal Artery Aneurysm: A Rare Cause of Recurrent Acute Epidural Hematoma. A Case Report

AbstractTraumatic middle meningeal artery aneurysm (TMMA) is a rare condition and a known cause of several different bleeding patterns after head injury. Once detected, they need to be treated as an emergency due to their potential for morbidity and mortality. Generally, recurrence does not occur in surgery for acute epidural hematoma if adequate hemostasis is achieved. Here, we report a case of atypical postoperative recurrence of an acute epidural hematoma, possibly due to the development and rupture of a TMMA. A 41-year-old man with left acute epidural hematoma after a head injury was referred to our hospital. Emergency craniotomy was performed immediately, and the hematoma was removed. The source of the bleeding was near the fracture site in the middle cranial fossa, and sufficient hemostasis was confirmed. However, a head computed tomography (CT) scan the next day revealed a recurrence of the acute epidural hematoma. Magnetic resonance (MR) angiogram showed an aneurysm with a diameter of approximately 4 mm in the left middle meningeal artery. The recurrence of the acute epidural hematoma appeared to be related to the formation and a rupture of a middle meningeal artery aneurysm, and to prevent subsequent rebleeding, the patient underwent reoperation, and the hematoma and aneurysm were removed. In surgery for acute epidural hematoma, recurrence can be prevented by removing the hematoma and ensuring hemostasis. Although conventional surgery was performed in this case, a repeat of epidural hematoma occurred. A postoperative middle meningeal artery aneurysm had been thought to have developed, ruptured, and caused a repeat epidural hematoma.In treating acute epidural hematoma, a TMMA development should be considered when an atypical clinical course occurs, such as a recurrence of postoperative bleeding.

Self-limited giant cell arteritis: The dilemma associated with its diagnosis and treatment.

Giant cell arteritis (GCA) can result in visual loss and other sequelae. An 81-year-old man presented with a one-week history of fever. He had bilateral temporal headache, jaw claudication, tenderness of the temporal arteries and a recent skin rash. A temporal artery biopsy showed typical GCA, but the symptoms were self-limiting. We continued close observation, without administering prednisolone treatment. Five months later, the symptoms did not recur, and prednisolone again was not administered. Our patient presented with an atypical course of GCA that created a clinical dilemma. The final diagnosis was self-limiting GCA.

Hematologic mask of infective endocarditis as a cause of fever of unknown origin: a case report

Fever of unknown origin (FUO), despite the wide diagnostic potential of modern medicine, remains a difficult problem for clinicians. Often, making a correct diagnosis requires a comprehensive examination of the patient, as well as the joint work of doctors from different specialties.A 57-year-old male patient was hospitalized due to an episode of loss of consciousness, unmotivated weight loss and daily evening temperature rises to 38-39о C, accompanied by chills and increased sweating. Previously, he was examined by a general practitioner, a cardiologist, and repeatedly by a hematologist. There was no evidence of an infectious disease, arrhythmias, multiple myeloma, lymphoproliferative disorders. Monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. Blood tests revealed neutrophilia (9800 cells/ μl) and moderate anemia. The patient was hospitalized to determine the FUO cause. Heart auscultation revealed a systolic murmur in the mitral valve, which was previously absent. Echocardiography revealed a mobile masses on the mitral valve, severe mitral regurgitation, and therefore infective endocarditis was diagnosed. Empirical antibiotic therapy with ceftriaxone and levofloxacin was administered. Further blood culture revealed growth of a HACEK representative Aggregatibacter actinomycetemcomitans, sensitive to both drugs. Examination established the odontogenic nature of endocarditis. During treatment, stable normothermia and significant blood count improvement were achieved. However, fluoroquinolone therapy led to tendinopathy. Due to persistent grade 3 mitral regurgitation, the patient underwent routine mitral valve replacement. Further follow-up revealed satisfactory condition of the patient.A feature of this case is the atypical course of infective endocarditis, occurring under a hematological mask, which made its diagnosis difficult. When examining a FUO patient, infective endocarditis should be included in the range of possible causes, taking into account not only the typical clinical picture, but also the numerous masks of this disease.

Diagnosis of an atypical fever in a 55-year-old man.

The paper describes a diagnostically challenging case of recurrent fever with an atypical course in a 55-year-old man. The authors highlight the significant difficulty in reaching a correct diagnosis, as well as the doubts and problems associated with the diagnostics. They suggest considering brucellosis as a diagnosis based on the patient's history and the course of the disease characterized by fluctuating, intermittent, and nocturnal fever. The aim of the study was to present an atypical and diagnostically challenging case of recurrent febrile episodes in a 55-year-old man. A comprehensive diagnostic workup, including laboratory tests, imaging, and specialist consultations, was conducted. The patient's medical history, physical examination, and various diagnostic tests were analyzed. In a patient with recurrent episodes of fever, an infection with Brucella spp. was detected, which was effectively treated with long-term antibiotic therapy. Imaging and laboratory diagnostics, along with specialist consultations, helped rule out other potential causes of the symptoms. The patient achieved lasting improvement following the treatment. There is a high probability that the patient was infected with Brucella bacteria. Diagnostic challenges and the rarity of the disease, considered non-existent in Poland, hinder prompt confirmation or exclusion of infection. The authors aimed to highlight the possibility of a higher frequency of brucellosis in Poland. They suggest that Brucella bacteria may still be present, causing chronic, unrecognized, recurrent fevers. Based on the case analysis, the authors indicate that the frequent occurrence of this disease in Poland might be higher than reported by statistics.

Clinical and Etiology Presentations of Keratitis Caused by Mixed Infectious Agents (Bacteria and Fungi)

Introduction: Microbiological studies following the culture of corneal infiltrates are the gold standard for determining the etiology of infectious keratitis caused by bacteria or fungi; however, even if a culture of the corneal ulcer is obtained. Purpose: To investigate the predisposing factors and clinical and Etiology presentations of keratitis caused by mixed infectious agents (bacteria and fungi). Methods: This was a prospective study of cases with mixed bacterial and fungal keratitis, done between July to December 2022 in Department of Ophthalmology, Netraloy Eye Care Center, Thanthania, Bogura Sadar, Bogura, Bangladesh. Sixty (60) cases of mixed bacterial and fungal keratitis were identified. Samples (corneal swabs and scrapings) were collected aseptically from corneal ulcer patients. Isolation and identification of the microbial agents and antimicrobial susceptibility testing were done in the Microbiology and Pathology departments. Results: 38 cases (63.3%) were men, and the mean age was 54.2±9.3 years. The affected people were mostly (50; 83.3%) residing in the rural areas. 34 patients (56.7%) were involved in agricultural activities. The people of 41- 60 years of age were particularly prone to this disease (40; 66.7%). The most common predisposing factor for mixed keratitis was a history of ocular trauma (42; 70%) and 28 patients (46.7%) had a history of trauma with vegetative matter. The incidence of the disease was highest in the monsoon season, between June to September (32; 53.3%). The most common causative bacterial organisms was Staphylococcus aureus (26;43.3%) followed by Pseudomonas secies (10;16.7%) and among fungal organisms was Aspervgillus fumigates (22;36.7%) followed by Fusarium species (12;20%). Conclusion: The identification of the etiology and the predisposing factors of corneal ulcers in this region are important for the prevention and early treatment of the disease. Usually, patients with mixed bacterial and fungal keratitis have poor prognosis. Thus, when the infectious keratitis is running an atypical course or found unresponsive to the initial medical treatment, the possibility of a mixed infection by bacterial and fungal organisms should be considered.

Onset of Rheumatoid Arthritis with eye manifestations (clinical case report)

Objective. Rheumatoid Arthritis may onset with ocular manifestations. The purpose of our work was presentation of clinical case of the onset of rheumatoid arthritis with an eye lesion.Materials and methods. A clinical case of rheumatoid arthritis with an atypical onset was analyzed. As well, a search and review of relevant literature was performed.Results. Patient with rheumatoid arthritis got bilateral ulcerous keratitis, refractory to conventional treatment and complicated by bilateral corneal perforation with iris prolapse. Due to the atypical clinical course of ophthalmic lesions being insusceptible of medical treatment, the patient was examined further. Laboratory immunological workup revealed positive rheumatoid factor, anti-CCP and anti-MCV antibodies. The typical symmetric presentation of arthritis developed 8 months after the onset of ophthalmic disorders. Baseline therapy of rheumatoid arthritis has demonstrated efficacy both in controlling the joint manifestations and preventing relapse of keratitis.Conclusion. The development of eye syndrome may precede articular manifestation of rheumatoid arthritis. While managing patients with recurrent bilateral keratitis, rheumatologic pathology should be suspected.

A clinical case of acute polyneuropathy, with the onset of bilateral facial nerve palsy in an early childhood

BACKGROUND: The differential diagnosis of peripheral nervous system damage depends on a thorough collection of history, comprehensive assessment of clinical manifestations, and dynamic examination of the patient. This article describes a clinical case of acute polyneuropathy in a young child, which began with gastrointestinal symptoms and bilateral facial neuropathy. CLINICAL CASE DESCRIPTION: The article presents a case of a child with an atypical course of acute polyneuropathy, which began with bilateral facial neuropathy combined with gastrointestinal complaints and pain syndrome. Features of the clinical presentation of the disease required extended laboratory and instrumental testing. Protocols for instrumental verification of diagnosis included nerve conduction studies examining neural conduction and axonal excitability of the affected facial nerve and the healthy side. Additionally, the functional status of the peripheral nerves in the upper and lower limbs was assessed. Polyneuropathic alterations revealed through nerve conduction studies confirmed diagnostic lumbar puncture to determine the presence of neuroinfectious processes. Magnetic resonance imaging of the brain, brachial, and lumbosacral plexuses using intravenous contrast showed the nature and prevalence of a lesion. Based on the overall clinical, laboratory, and instrumental examination data of the patient, “acute inflammatory demyelinating polyneuropathy” was diagnosed; timely etiopathogenetic treatment was initiated. CONCLUSION: In children, the course of a neuroinfection may be atypical. Sometimes, when dissociating the clinical manifestations of the disease from the results of a standard laboratory and instrumental test, expert diagnostic techniques should be used for differential diagnosis purposes.

Atypical Course of the Habenulo-Interpeduncular Tract in Chick Embryos.

Classical studies of the avian diencephalon hardly mention the habenulo-interpeduncular tract (a.k.a. retroflex tract), although both the habenula (HB) (its origin) and the interpeduncular nuclear complex (its target) are present. Retroflex tract fibers were described at early embryonic stages but seem absent in the adult in routine stains. However, this tract is a salient diencephalic landmark in all other vertebrate lineages. It typically emerges out of the caudal HB, courses dorsoventrally across thalamic alar and basal plates just in front of the thalamo-pretectal boundary, and then sharply bends 90° caudalwards at paramedian basal plate levels (this is the "retroflexion"), to approach longitudinally via paramedian pretectum and midbrain the rostralmost hindbrain, specifically the prepontine median interpeduncular complex across isthmus and rhombomere 1. We systematize this habenulo-interpeduncular course into four parts named subhabenular, retrothalamic, tegmental, and interpeduncular. We reexamined the chicken habenulo-interpeduncular fibers at stages HH30 and HH35 (6.5- and 9-day incubation) by mapping them specifically with immunoreaction for BEN protein, a well-known marker. We found that only a small fraction of the stained retroflex tract fibers approaches the basal plate by coursing along the standard dorsoventral pathway in front of the thalamo-pretectal boundary. Many other habenular fibers instead diverge into atypical dispersed courses across the thalamic cell mass (implying alteration of the first subhabenular part of the standard course) before reaching the basal plate; this dispersion explains their invisibility. A significant number of such transthalamic habenular fibers cross orthogonally the zona limitans (ZLI) (the rostral thalamic boundary) and invade the caudal alar prethalamus. Here, they immediately descend dorsoventrally, just rostrally to the ZLI, until reaching the prethalamic basal plate, where they bend (retroflex) caudalwards, entering the thalamic basal paramedian area. These atypical fibers gradually fasciculate with the other groups of habenular efferent fibers in their final longitudinal approach to the hindbrain interpeduncular complex. We conclude that the poor visibility of this tract in birds is due to its dispersion into a diversity of atypical alternative routes, though all components eventually reach the interpeduncular complex. This case merits further analysis of the diverse permissive versus nonpermissive guidance mechanisms called into action, which partially correlate distinctly with successive diencephalic, mesencephalic, and hindbrain neuromeric fields and their boundaries.

Human Inborn Errors of Immunity in Pyoderma Gangrenosum: A Systematic Review.

Pyoderma gangrenosum (PG) is a rare ulcerative neutrophilic dermatosis that can be associated with primary immunodeficiency. The pathogenesis of PG has not yet been elucidated, although contributions from dysregulation of the immune system in patients with apparent genetic predispositions have been postulated. We conducted a Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-guided systematic review with the objective of identifying inborn errors of immunity in the presence of PG as well as their clinical characteristics of severity including number of PG lesions and anatomic areas affected, and treatment outcomes. A literature search was performed using PubMed/MEDLINE, Embase, Cochrane Library, and Web of Science through August 24, 2023, for studies published in English using the search terms: "pyoderma gangrenosum," "inborn error of immunity," "immune defect*," and a list of genetic mutations potentially associated with PG. Seventy-four cases of PG associated with inborn errors of immunity were identified. The results demonstrate an association of PG with a variety of inborn errors of immunity, including genetic mutations not classically associated with the condition. Genetic mutations such as BTK, IL1RN, ITGB2, LPIN2, MEFV, NFkB1, NLRP3, NLRP12, NOD2, PSMB8, PLCG2, PSTPIP1, RAG1, TTC37, and WDR1, as well as complement component 2/complement component 4 (C2/C4) and complement component 7 (C7) deficiencies were identified in the presence of either idiopathic or syndromic PG. Of note, mutations in genes such as PSMB8, NLRP3, and IL1RN were found to be associated with a more severe and atypical course of PG, whereas mutations in RAG1 as well as those causing a C2/C4 deficiency were associated with the mildest clinical presentations of PG. Mutations in NFkB1, ITGB2, and PSTPIP1 were associated with the most heterogeneous clinical presentations. Human inborn errors of immunity may be implicated in the genetic predisposition to PG and may influence the clinical presentation. Due to the rarity of these diseases, further work must be done to describe the association between inborn errors of immunity and PG. Identifying inborn errors of immunity that may contribute to the development of PG may assist in further elucidating the mechanism of PG, guiding targeted treatment, and improving clinical outcomes for these patients.

САРКОИДОЗ, ТРУДНОСТИ ДИАГНОСТИКИ

The incidence of sarcoidosis in Russia has not been studied enough. The clinical signs of sarcoidosis are diverse, and the lack of specific diagnostic tests makes noninvasive diagnosis difficult. In general therapeutic practice, in the case of an atypical course of the disease, diagnosis is complicated by the need for a differential diagnosis with many syndrome-like diseases. The etiology of sarcoidosis is still unknown. According to recent concepts, the cause of its occurrence cannot be considered a single factor, it is a combination of genetic, environmental, infectious and immunological causes. In this article, the author examines the main difficulties faced by doctors in the diagnosis of sarcoidosis. The clinical manifestations of the disease are analyzed, as well as methods of its detection, such as bronchoscopy, biopsy and laboratory tests. Possible diagnostic errors and how to avoid them are also considered. Understanding these difficulties will help improve the diagnosis and treatment of sarcoidosis, leading to more effective disease management.

Характеристика перебігу епідемічного процесу скарлатини у сучасних умовах

S carlet fever is one of the most common nosological forms of group A streptococcal infection (SGA). In recent years, there has been an increase in the incidence of this infection in the countries of Europe and Asia, the USA and Canada. The analysis of professional publications of different countries of the world regarding the manifestations of the epidemic process of scarlet fever was carried out, as well as information on epidemiological studies conducted by the authors was provided. It has been established that children remain the leading risk group for scarlet fever, men are more often affected among adults, but the role of young women who care for children as a source of infection is increasing. The aerosol-aspiration mechanism of transmission of the causative agent of scarlet fever is realized by airborne and contact-household ways of its transmission. There is an intensification of the contact-household way of transmission of the pathogen. An atypical course of scarlet fever is observed with manifestations of co-infection, in particular, gastroenterocolitis, chicken pox and respiratory infections. A significant number of publications are devoted to the influence of environmental factors on the course of the epidemic process of scarlet fever. The authors note a direct correlation between the incidence of scarlet fever and atmospheric air pollutants, meteorological factors, as well as the influence of social and economic factors on the epidemic situation of this infection. Long-term use of antibiotics has contributed to the development of antibiotic resistance to the most common drugs, which prompts scientists to develop vaccines against iSGA. So, scarlet fever is characterized by widespread distribution, impression of mainly children, co-infection, atypical clinical course, especially in immunocompromised persons, and implementation of the contact-household way of transmission of the pathogen. The influence of social, natural and environmental factors on the course of the epidemic process of scarlet fever has been established, which is important to consider when conducting epidemiological surveillance of this infection. The introduction of vaccination against iSGA will contribute to the reduction of antibiotic resistance, which is currently a global problem for humanity. We consider it necessary to include scarlet fever and other iSGA in the list of infectious diseases subject to registration in Ukraine. Key words: scarlet fever, incidence, risk groups, ways of pathogen transmission, atypical course, co-infection, environmental factors, antibiotic resistance.

Atypical course of severe myoclonic epilepsy of infancy (Dravet syndrome)

Atypical course of severe myoclonic epilepsy of infancy (Dravet syndrome)

Hyponatremia as acute adrenal insufficiency in patients with type 1 diabetes mellitus and kidney transplantation cause end-stage chronic kidney disease

Diabetes mellitus type 1 (DM1) and chronic adrenal insufficiency (CAF) are among the most common autoimmune endocrine diseases that develop both isolated and in combination with each other and with other diseases of autoimmune origin, as part of various syndromes. At the same time, type 1 diabetes is quite often the first component of a systemic autoimmune lesion and acts as a predictor of the development of congenital disorder, which, in turn, against the background of type 1 diabetes, acquires a mild, sometimes atypical course, which complicates the diagnosis and prescription of therapy. The clinical case describes a patient with type 1 diabetes and end-stage chronic kidney disease (CKD), kidney allotransplantation (ART), who was on triple immunosuppressive therapy, who developed CHN, which was manifested by severe hyponatremia and the occurrence of frequent hypoglycemic conditions.

Examples of surgical treatment of pulmonary echinococosis in adults and children

ABSTRACT. The article presents a modern view of the diagnosis and treatment of pulmonary echinococcosis. The etiology, pathogenesis, clinical picture, ways of diagnosis and available methods of treatment of this pathology are highlighted. The purpose of the work was to acquaint doctors of various specialties with clinical cases of pulmonary echinococcosis. Two clinical cases of pulmonary echinococcosis became the object of the work. The first clinical example concerned a 17-year-old boy who had a typical course of echinococcosis and who underwent left lower lobectomy. The second clinical example concerned a young woman in whom pulmonary echinococcosis had an atypical course and was diagnosed only intraoperatively. It was concluded that the most effective method of treatment of echinococcal lung cysts is surgical operation. The volume of surgical intervention depends on many factors and is decided individually in each specific case. In the postoperative period, preventive anthelmintic therapy is mandatory.

РАЗВИТИЕ ХРОНИЧЕСКОГО МУЛЬТИОЧАГОВОГО НЕБАКТЕРИАЛЬНОГО ОСТЕОМИЕЛИТА У ПАЦИЕНТА С ИСХОДНО ДИАГНОСТИРОВАННЫМ СИСТЕМНЫМ ЮВЕНИЛЬНЫМ АРТРИТОМ: КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ

Systemic juvenile arthritis (sJA) is a special variant of chronic arthritis in children belonging to the spectrum of polygenic autoinflammatory diseases whose main features are persistent febrile fever, skin rashes, other systemic manifestations (serositis, lymphadenopathy, hepatosplenomegaly) as well as the presence of joint damage with the frequent development of destructive polyarthritis and osteonecrosis, high laboratory indicators of systemic inflammation either at the disease onset or delayed. The list of differentiated conditions includes many diseases such as infections, cancer, primary immunodeficiencies, monogenic autoinflammatory diseases etc. Authors represent a clinical case of a patient with sJA manifestations who was diagnosed with chronic multifocal nonbacterial osteomyelitis (CMNO), that demonstrates the great difficulties in diagnosis and the variability of the course of rheumatic diseases in childhood, which in its turn causes a delay in the timely prescription of effective therapy. Thus, patients with manifestations of a systemic inflammatory process, joint changes, especially those with an atypical course of the disease, pain in the bones and joints of a nocturnal nature, as well as an insufficient response to the therapy even long-term, are in need of conducting of a differential diagnostic search with the inclusion of CMNO, the ‘whole body’ mode magnetic-resonance tomography prescribed by a rheumatologist practitioner.