CC BY
3MEDICAL SCIENCES
UDC 61
Tarnavska S.I., Shakhova O. O., Hrytsiuk M. O., Melnyk K. V. Senyshyn Kh.V., Sokalska A. Y.
Department of Pediatrics and Children's Infectious Diseases Bukovinian State Medical University, Chernivtsi, Ukraine DOI: 10.24412/2520-6990-2023-8167-15-17 FEATURES OF THE COURSE OF SYSTEMIC SCLERODERMA IN CHILDREN (CLINICAL CASE)
Introduction.
Juvenile systemic scleroderma (JSSD) is a chronic autoimmune disease with onset before the age of 16, manifested by progressive fibrosing sclerotic lesions of the skin, musculoskeletal system, internal organs (lungs, kidneys, digestive tract, heart, kidneys, and digestive tract) and widespread vasospastic disorders (Raynaud's syndrome), which are based on damage to connective tissue with predominance of fibrosis and vessels such as obliterating endarteritis [1-4].
It is known that scleroderma with systemic manifestations is 5-10 times less common than the limited form (when only the skin is affected). SCD is a fairly rare disease, more often debuting in preschool and primary school age and equally often in boys and girls [58]. According to studies conducted in the countries of Northern Europe, the incidence of USSD ranges from 0.05% per 100 thousand people to 0.27% per 100 thousand people.
Despite the fact that there are many articles published in the medical literature on JSDS and the patient's characteristic skin syndrome, it is not always possible to diagnose Juvenile Scleroderma in a timely manner. Diagnostics with systemic manifestations of the disease is quite lengthy and includes a wide range of laboratory and instrumental examination methods, and also requires the involvement of narrow specialists (ophthalmologist, neurologist, nephrologist, surgeon, etc. [9-11]).
Timely diagnosis of JSSD means timely initiation of long-term anti-inflammatory and immunosuppres-sive therapy, which increases the duration of remission and improves the patient's quality of life.
The aim of the study is to show the variety of clinical symptoms, a wide range of diagnostic search, as well as difficulties in therapy in an adolescent patient with systemic manifestations of juvenile scleroderma.
Material and Methods. The patient was followed for 15 years at the regional municipal non-profit enterprise "Chernivtsi Regional Children's Clinical Hospital". A range of laboratory tests (complete blood count, urinalysis, biochemical analysis and acute-phase blood counts, coagulogram, immunoblotting method for detection of antinuclear antibodies to PM-Scl antigen), instrumental tests (capillaroscopy, CT scan, ultrasound of the abdomen, electrocardiography, echocardiography, and EGD), as well as consultations of narrow specialists were used.
Results and discussion. Patient V., born in 2008, was first hospitalized at the CHODKL at the age of 8 in November 2016 with complaints of dense, dry and thin foci on the skin of the forearms, pain, numbness and dysfunction in the interphalangeal joints of the hands, prolonged dry cough. From the medical history, it is known that the skin syndrome first appeared in 2015, and she was seen by a dermatologist with a diagnosis
of atopic dermatitis. She received topical therapy and antihistamines. Against this background, the skin syndrome progressed, ulcers appeared with subsequent scarring in the area of the interphalangeal joints of the hands. The joint syndrome in the form of pain and limitation of movement in the interphalangeal joints of the hands was added. Periodically, he had a dry cough without visible signs of acute respiratory disease (fever, catarrhal phenomena).
On objective examination, the girl's physical development was average, harmonious. The patient is of normosthenic build. The skin syndrome is represented by typical scleroderma foci of irregular shape, with different stages of damage (edema, induration and atrophy). The lesions were localized on the skin of the shoulders and forearms. The skin in these lesions is dense, fused to the underlying tissues, immobile, doughy, white-yellow in color or hyperpigmented, shiny. The skin of the dorsal surface of the hands is thin, taut, shiny. In the area of interphalangeal joints of the hands, there are scarring changes in the skin. Hands and feet are cold to the touch.
Joint lesions were noted in the form of pseudoarthritis (caused by fibrosing sclerotic changes in periar-ticular tissues) of the interphalangeal joints of the hands.
Respiratory system: Respiratory rate - 21/min, clear lung sound during percussion over the lungs, during lung auscultation - breathing is stiff over all fields, no local symptoms were noted. Cardiovascular system: Blood pressure - 100/50 mm Hg, heart rate - 88/min, heart limits within the age range, during auscultation, heart sounds are loud and rhythmic.
No pathology was detected in the gastrointestinal tract and genitourinary system.
Data of laboratory and instrumental methods of examination (November 2016) complete blood count -mild anemia, leukocytosis, eosinophilia (erythrocytes -4.05*1012/l, hemoglobin - 110 g/l, platelets -242*109/l, leukocytes - 12.4*109/l, neutrophils -53.7%, eosinophils - 15.8%, lymphocytes - 22%, monocytes - 8.5%, ESR - 12 mm/h); blood biochemical parameters and coagulogram - within normal limits; general urinalysis - without pathology.
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To make the final diagnosis, the patient underwent a number of other tests: 1) blood test by immunoblot-ting - the result was strongly positive (+++) - antinuclear antibodies to the PM-Scl antigen were detected; 2) capillaroscopy - the shape of the capillaries was narrow, moderately and sharply tortuous with poorly differentiated and undifferentiated arterial and venous sections. The blood flow is slow. The color of the capillaries is from pale pink to deep pink. The phenomena of capillary dystonia of the fingers are pronounced; 3) CT of the lungs in full volume, a decrease in the transparency of both lungs by the type of "frosted glass" is determined, more pronounced in the peripheral parts, with the formation of subcortical reticular changes. Bronchial patency is not impaired. The sinuses are free. No enlarged lymph nodes in the mediastinum were found. Conclusion - CT signs of interstitial lung disease (by type of fibrosing alveolitis); 4) ECHO-CG - during ECHO-CG, no data on cavity enlargement, valvular pathology and septal defects were found. Myocardial contractility was not changed. Systolic pressure in the LA = 18 mm Hg; 5) ECG, ultrasound of the abdominal cavity, kidneys and bladder, EGD - no pathological changes were detected.
Based on the complaints, anamnesis, objective examination, results of laboratory and instrumental research methods, the patient was given a final clinical diagnosis: Juvenile systemic scleroderma (scleroderma skin lesions, hyperpigmentation, pseudoarthritis, interstitial lung disease - fibrosing alveolitis, Raynaud's syndrome), chronic course, generalized stage, activity grade I.
Immediately after the diagnosis was verified, the patient was prescribed the following treatment: methypred - 250 mg IV drip once daily #3, then methypred - 16 mg/day per os, cyclophosphamide - 300 mg IV drip once a month. To normalize microcirculation and other vascular disorders, the patient received amlodipine, actovegin, pentoxifylline. Local therapy (heparin, troxevasin ointments) and physiotherapy were prescribed for the affected skin areas. Calcium and vitamin D3 preparations were prescribed for the prevention of hypocalcemia and osteoporosis (due to glucocorticoid therapy).
After 2.5 months (in January 2017), against the background of this therapy, positive dynamics was noted in the patient's condition. New sclerodermic foci on the skin stopped appearing, the low-productive cough and pain in the interphalangeal joints of the hands disappeared. In addition, the signs of interstitial lesions significantly decreased on the CT scan of the OHC. In this regard, the dose of methypred per os was reduced, and the patient was transferred to a cyclophos-phamide regimen once every 3 months.
Subsequently (throughout 2017), the patient was hospitalized every three months in the Rheumatology Department of the Cherkasy Oblast Children's Hospital for evaluation, follow-up examination and administration of cyclophosphamide, and since March 2017, she has been receiving methypred per os 8 mg/day. The condition is noted as stable: the skin syndrome does not progress, the amplitude of movement in the interpha-langeal joints of the hands increases, there are no signs
of humoral activity in blood tests (ESR 2-8 mm/h, CRP negative), CT of the OHC (June 2017) with positive dynamics: a decrease in the number of interphalangeal joints. ) with positive dynamics: no decrease in transparency of both lungs by the type of "frosted glass", in the peripheral parts of both lungs, violations of the architectonics of the lung pattern in the form of zones of increased air permeability against the background of a porous parenchyma structure are determined.
Since January 2018 (14 months after the start of therapy), the patient has been receiving cyclophospha-mide once every 6 months, methopred per os at the same dose of 8 mg/day. Throughout 2018, the patient's condition remained stable. There were no signs of respiratory failure: Respiratory rate 18/min, chest CT scan showed no progression of lung damage. In July 2018, a slow dose reduction of methypred per os was started. The patient remains under the supervision of a rheuma-tologist at the CHODKL.
Conclusions. Thus, we have presented a clinical case of debut JSSD with rapidly progressive, widespread skin lesions, musculoskeletal involvement, and the development of fibrosing alveolitis. Thanks to intensive and long-term pathogenetic therapy (glucocor-ticoids, cyclophosphamide), we managed to stabilize the patient's condition and stop the progression of interstitial lung damage (which results in pulmonary fibro-sis).
List of references
1. Atzeni F., Bardoni A., Cutolo M. Localized and systemic forms of scleroder-ma in adults and children. Clin Exper Rheumatol.2018;24(1 Suppl 40):36-45.
2. Foeldvari I. Juvenile systemic sclerosis. In: Varga J., Denton C.P, Wigley F.M., editors. Sclero-derma: from pathogenesis to comprehensive management. New York: Springer Science+Business Media, LLC; 2017. 93 p.
3. Pelkonen P.M., Jalanco H.J., Lantto R.K. Incidence of systemic connective tissue disease in children: a nationwide prospective study in Finland. J Rheu-matol.2021; 21(11): 2143-2146.
4. Herrick A.L, Ennis H., Bhushan M. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Irelands. Arthritis Care Res (Hoboken). 2019; 62(2): 213-218. DOI: 10.1002/ acr.20070.
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7. Panda S. Scleroderma in children : emerging management issues / S. Panda // Indian J. Dermatol. Venereol. Leprol. -2020. - Vol. 76 (4) . - P. 348-356.
8. Rosenkranz M. E. Systemic and localized scle-roderma in children: current and future treatment options / M. E. Rosenkranz, L. M. Agle, P. Efthimiou, T. J. Lehman // Paediatr. Drugs. - 2017. - Vol. 8 (2). - P. 85-97.
9. Zandman-Goddard G. New therapeutic strategies for systemic sclerosis--a critical analysis of the literature / G. ZandmanGoddard, N. Tweezer-Zaks, Y. Shoenfeld // Clin. Dev. Immunol. - 2018. - Vol. 12 (3). - P. 165-173.
10. Zulian F. The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classi-
fication criteria for juvenile systemic sclerosis / F. Zulian, P. Woo, B. H. Athreya [et al.] // Arthritis Rheum. - 2019 - Vol. 57 (2). - D. 203-212.
11. Zeglaoui H, Landolsi H, Mankai A, et al. Type 1 diabetes mellitus, celiac disease, systemic lupus erythematosus and systemic scleroderma in a 15-year-old girl. Rheumatol Int. 2022;30(6):793-795.doi: 10.1007/s00296-009-0988-2
UDC: 616.36-002-02-036.22-092
Melenko Svitlana Romanivna,
PhD, Associate Professor of the Department of Infectious Diseases and Epidemiology
Bukovinian State Medical University Bezhnar Vitalina-Olena Ruslanivna,
student
Bukovinian State Medical University Tkachynska Yuliana Olexandrivna
student
Bukovinian State Medical University
EVALUATION OF THE EPIDEMIOLOGY OF HEPATITIS B VIRUS INFECTION:
RESEARCH DATASET
Abstract.
One of the biggest causes of liver cirrhosis and hepatocellular carcinoma is the hepatitis B virus (HBV). Although an HBV vaccine is available, the incidence rate is still high. HBV is a serious global problem. The course of this disease is quite different, it varies from a disease with acute symptoms to an asymptomatic course that can be detected in a patient during screening.
In this article we want to show up a review of modern literature and scientific sources describing the prevalence of HBV in the world in modern conditions.
Keywords: Hepatitis B virus; Cirrhosis; Epidemiology; Prevalence; Systematic review; Risk factors.
Introduction
Hepatitis B infection can be acute or chronic. Healthy adults infected with HBV are usually asymptomatic and can recover without any problems. However, some people infected with hepatitis B are suffering for only a few weeks, indicating an acute infection, while others may progress from acute to chronic Hepatitis B, which lasts a lifetime [1].
In accordance with the World Health Organization (WHO) statistics, nearly 0.257 billion people whole of the world are infected with the Hepatitis B virus (HBV), and finally, 0.88 million deaths annually, which causes a huge medical and economic burden [24]. Significant efforts have been made worldwide to provide practical and standardized guidelines and recommendations for the prevention, diagnosis, and treatment of HBV infection. It is essential for all the patients from the HBV-infected population [2].
Results
According to research of statistical data and scanning references of key publications, were made the following inferences the prevalence of HBV infection was much lower among patients with cirrhosis in Europe, the USA, and Oceania (3-14%) than in African and Asian countries (8-61%) [5]. In the same study, it was described that among patients with liver cirrhosis, 42% have hepatitis B viral infection, which is significantly higher than the prevalence of hepatitis C in this cohort. Also was created statistical table, where research and observations of various countries, including Ukraine, were analyzed. There was only one study from Ukraine in which 36 patients with liver cirrhosis were examined, 6% of which had hepatitis B virus.
