Čibirkaitė, Agnė

Straipsnyje pateikiamas pacientės, kuriai kūdikystėje diagnozuotas atopinis dermatitas ir nustatytas raidos bei protinis atsilikimas, atvejis. Pacientei pasireiškė ypač intensyvus niežulys, kuris, lydimas nervinės kilmės draskymosi epizodų, išprovokavo vaikams retos odos būklės - mazginio niežulio - atsiradimą. Straipsnyje nagrinėjami bei aptariami ligos eigos, diagnostikos bei gydymo ypatumai.

Background More than 8.5% of children aged 6–7 suffer from allergic rhinitis, and it is even more common among teenagers, aged 13–14 (14%). Allergic rhinitis worsens the quality of life for children of all ages. It can cause sleep difficulties, reduce participation in daily activities, and negatively impact academic performance, physical activity, and social functioning. Studies show that sublingual immunotherapy has a positive effect on allergy symptoms and that systemic side effects are considered rare for this type of treatment.

Method A retrospective cohort study was performed using the data from the Department of Pediatrics of the Hospital of Lithuanian University of Health Sciences outpatient registry to acquire the data of patients using sublingual immunotherapy. The data of 44 patients was collected. Data was analyzed using IBM Statistics SPSS for frequencies, t and χ2 tests.

Results The mean age when starting sublingual immunotherapy was 6.89 years. 43% of patients immunotherapy duration was less than 1 year, almost 23% - more than 3 years. The most prevalent allergy risk factor was living in the city (59.1%) as shown in figure 1. More than 93% of patients had congested nose before starting immunotherapy. Sneezing and nose itching was a problem for 88.6% of children. Runny nose bothered 79% of patients. A positive effect was seen in 91%. Due to this treatment, 20.5% of patients had no longer allergy symptoms and did not need medication for them. 9% did not feel any positive effect. 90.9% of patients did not experience any side effects. However, out of those who did have them, the most common was stomach ache (6.8%) and nausea (2.3%).

Conclusion Most patients had a positive effect during the use of sublingual immunotherapy. Only a small number of patients did not feel any changes in their allergy symptoms. Furthermore, most patients did not encounter any side effects.

Purpose: To highlight a rare case of nodular prurigo (NP) in a child, emphasizing the importance of early diagnosis and tailored treatment to improve quality of life.

Clinical case: An 11-year-old. Month-long history of increasing itchy skin lesions (VAS: 10/10). Sleep disturbances and functional impairment. Firm, reddish-blue plaques with pale centers, hyperpigmented keratotic nodules, ulcers, and scabs (Pictures 1-3).

History: Atopic dermatitis (AD) from early childhood. Remission ages 3–8. Recurrence of AD at age 8, triggered by asthma and allergic rhinitis. Skin infections since age 10.

Investigations: Allergy Test (Alex2 macroarray test): Sensitization to 66 allergen extracts and molecular allergens (pollen, dust mites, food). Skin culture: Staphylococcus aureus and Malassezia fungus detected. Immune Function: Elevated IgE (983.8 kU/l; normal 0–150), other immune parameters normal. Biopsy: Perivascular dermatitis confirmed NP.

Diagnosis: Nodular prurigo. Atopic dermatitis.

Treatment: Skin Care: Oily emollients, antiseptics. Medications: Topical: Betamethasone + Fusidic Acid, switched to Clobetasol; Oral: Second-generation antihistamines, antifungals (Itraconazole), tricyclic antidepressant (Doxepin for itching control); Intravenous: Cefazolin. Supportive Measures: Socks/gloves at night to minimize scratching (Pictures 4-6).

Outcome: Improvement in skin condition and itching (VAS: 1–2) (Pictures 7-9).

Discussion: NP is a chronic, intensely itchy condition often linked to AD. NP is extremely rare in children, typically affecting older adults. Causes: genetic, immunological, and environmental factors. Diagnosis - clinical, with biopsy confirming uncertain cases. Treatment: For itching - antihistamines, tricyclic antidepressants, antiepileptics; Topical - corticosteroids, calcineurin inhibitors, vitamin D analogs; Advanced: phototherapy (age ≥16), immunosuppressants, biologicals.

Conclusion: NP should be considered in children with chronic skin conditions in order to provide early, individualized treatment and to improve outcomes.

Background and Objectives: Assess the quality of life of children aged 2–10 with mild to moderate bronchial asthma. To evaluate the general health condition of children with mild and moderate severity bronchial asthma. To determine health changes in children with mild- and moderate-severity bronchial asthma as they grow older. To evaluate the impact of mild- and moderate-severity bronchial asthma on children’s daily and social activities, physical health, emotional state, and general well-being. Materials and Methods: A comparative cross-sectional study was conducted in March–June 2020. Parents or guardians of 2–10-year-old children without bronchial asthma and children with mild to moderate bronchial asthma were interviewed after receiving their written informed consent. The questionnaire was based on the standardized quality-of-life quiz SF-36. A total of 248 questionnaires were collected—106 from the parents or guardians of children with bronchial asthma and 142 from parents/guardians of children without bronchial asthma. For further analysis, 106 children without bronchial asthma and with no chronic conditions were selected. Quantitative variables were compared using the Mann–Whitney U test and qualitative data using the chi-square (χ2) criteria. Quantitative data were described by giving means, medians, and standard deviations (SD); qualitative features by giving relative frequencies. Statistical data were analyzed using SPSS and Excel 2020. Results: Children with mild and moderate asthma exhibit poorer health compared to their healthy counterparts. Only 20.7% of respondents with asthma reported excellent or very good health, contrasting with 64.1% of healthy children (p < 0.001). As children with asthma age, their general condition improves, with 46.2% showing improvement in the past year, while 42.5% of healthy children had a stable condition (p < 0.05). In various activities, children with asthma face more constraints than healthy children (p < 0.05), including energetic activities (sick—59.5%; healthy—10.3%), moderate activities (sick—24.5%; healthy—4.7%), climbing stairs (sick—22.7%; healthy—3.8%), and walking over 100 m (sick—9.4%; healthy—0%). Children with asthma are more likely to experience exhaustion, anxiety, tiredness, lack of energy, and restraint in public activities (p < 0.05). Conclusions: Parents/caregivers of children with mild to moderate bronchial asthma rate their health worse than those of healthy children do. As children with mild to moderate bronchial asthma grow, the disease impact on their overall well-being decreases. Children with mild to moderate bronchial asthma, compared to healthy children, experience more limitations in vigorous or moderate activities; face more difficulties climbing stairs or walking more than 100 m; frequently feel exhaustion, anxiety, fatigue, or lack of energy; and encounter restrictions in social activities.

Introduction: Previously healthy 8-year-old boy presented to the emergency department with a itchy maculopapular rash which later developed into target lesions with progression predominantly in his face, limbs, palms and soles. Right leg was swollen. No rashes on the mouth and genitals. Patient had febrile fever for a few days every 12 hours. One week before he played golf on the artificial grass, probably he had an unknown sting bite on his right shin a day before the rash as well. He had no allergies up to now, no history of chronic diseases. Methods: –. Results: Lab. tests: CRP 28 - > 5 mg/L, (slightly elevated, then normal), blood count – eosinophilia (abs. count 3,7 x 10^9/l), ASO titer 559kU/l—(elevated), strep test—positive, Respiratory Viral Panel (DNA)—Parainfluenza A virus, Parainfluenza B virus, Metapneumovirus,Adenovirus, Coronavirus HKU-1, Rhinovirus, Bocavirus, Parainfluenza 1 virus, Parainfluenza 2 virus, Parainfluenza 3 virus, Parainfluenza 4 virus, Respiratory syncytial virus A/B, Coronavirus NL63, Coronavirus 229E, Coronavirus OC43, Enterovirus—notfound. Liver enzymes, electrolites levels without changes, HSVIgM, IgG—negative, CMV IgM, IgG – negative, EBV IgG, IgM negative, Imunoglobuline E—625 kU/l—elevated. Blood, urine and tonsills swab test—negative, SARS CoV-2 infection antibodies—notfound. Radiologic tests: Ultrasound of left knee—oedemous soft tissues, small streak of liquid. Ultrasound of heart—without changes.Ultrasound of internal organs—without changes.Diagnosis: Idiopathic erythema multiforme minor.Discussion. Erythema multiforme (EM) first described in literature in 1886. It is uncommon inflammatory skin disease which has a pathognomonic sign—target lesion, sometimes oral or other mucosal involvement. This disease affects all age groups, 20% patients are kids,males are a bit more common than females. Etiology—in most cases are unknown, also can be caused by viruses (HSV1, HSV2, CMV,etc.), atypical infections (Mycoplasma pneumonia, Streptococcus,etc.), medications. There are a few cases that EM was caused by a spider-bite. Conclusions: In this case the cause is unknown, but the main triggers could be – Streptococcal infection (Elevated ASO titer, positive Strep test), insect bite or some allergens due to artificial grass. Table of classification of erythema multiforme. [...].

Hereditary type 1 tyrosinemia (HT1) is a rare inherited autosomal recessive disorder of tyrosine metabolism, characterized by progressive liver damage, dysfunction of kidney tubules, and neurological crises. In the course of this disease, due to the deficiency of the enzyme fumarylacetoacetate hydrolase (FAH), toxic intermediate metabolites of tyrosine breakdown, such as fumarylacetoacetate (FAA), succinylacetoacetate (SAA), and succinylacetone (SA), accumulate in liver and kidney cells, causing cellular damage. Because of this, an increased SA concentration in the blood or urine is pathognomonic of HT1. In the year 2000, HT1 was diagnosed in Lithuania for the first time, and this was the first time when a specific treatment for HT1 was administered in the country. Over two decades, four cases of this disease have been diagnosed in Lithuania. In the first of these patients, the disease was diagnosed in infancy, manifesting as liver damage with liver failure. Treatment with nitisinone was initiated, which continues to be administered, maintaining normal liver function. Liver transplantation was performed on two subsequent patients due to complications of HT1. It is crucial to diagnose HT1 as early as possible in order to reduce or completely eliminate complications related to the disease, including progressive liver failure and kidney dysfunction, among others. This can only be achieved by conducting a universal newborn screening for tyrosinemia and by starting treatment with nitisinone (NTBC) before the age of 1 month in all cases of HT1. However, in those countries where this screening is not being carried out, physicians must be aware of and consider this highly rare disorder. They should be vigilant, paying attention to even minimal changes in a few specific laboratory test results—such as unexplained anemia alongside neutropenia and thrombocytopenia—and should conduct more detailed examinations to determine the causes of these changes. In this article, we present the latest clinical case of HT1 in Lithuania, diagnosed at the Children’s Diseases’ Clinic of the Lithuanian University of Health Sciences (LUHS) Hospital Kaunas Clinics. The case manifested as life-threatening acute liver failure in early childhood. This article explores and discusses the peculiarities of diagnosing this condition in the absence of universal newborn screening for tyrosinemia in the country, as well as the course, treatment, and ongoing monitoring of patients with this disorder.

Introduction. 5-Aminosalicylic acid (5-ASA) preparations are widely used in the treatment of inflammatory bowel diseases. The most commonly used medicine is mesalazine. Overall, it is a very safe drug with few side effects. A rare side effect of this drug is kidney damage (interstitial nephritis). In this case report, we present a clinical case of mesalazine induced interstitial nephritis. Case Description. In 2020 a 56-year-old patient was hospitalized in Lithuanian University of Health Sciences Kaunas Clinics because of renal failure, hyperkalemia (6.5 mmol / l), uremia (35 mmol / l), increased creatinine (1070 μmol/l / l) and anuria. It was known that this patient had ulcerative colitis for 20 years and used mesalizine for treatment. In 2019 the patient was diagnosed with mesalazine-induced interstitial nephritis which was confirmed by biopsy. Due to that, treatment with mesalazine was discontinued. In 2020 after the patient felt symptoms of acute colitis, he started using mesalazine on his own, resulting in worsening of his health condition and impaired renal function. The patient was treated with hemodialysis, corticosteroids-positive dynamics were noticed. The patient was released for further monitoring. Significant improvement was observed during the planned consultation. Therefore, biological therapy is planned for further treatment of ulcerative colitis. Summary. In this case we presented the patient with renal failure due to acute interstitial nephritis, confirmed by biopsy, after the use of mesalazine to treat ulcerative colitis. Improvement of renal function occurred after discontinuation of mesalazine and administration of corticoids. Conclusion. In conclusion, users of 5-ASA have an increased risk of renal disease. It is important to monitor creatinine levels regularly during treatment with 5-ASA to prevent irreversible renal impairment.

Evanso sindromas (ES) yra reta, gyvybei grėsmę kelianti autoimuninė būklė, kurios tikslus paplitimas pasaulyje nėra žinomas. Šis sindromas manifestuoja pasikartojančiais imuninės trombocitopenijos (ITP) ir autoimuninės hemolizinės anemijos (AIHA) epizodais. Kiek rečiau pacientams pasireiškia imuninė neutropenija. Ši būklė gydoma, skiriant intraveninį imunoglobuliną, sisteminius kortikosteroidus, mononuklearinius antikūnus (rituksimabą) ar kitus, rezervinius, gydymo metodus. ES prognozė priklauso nuo sindromo etiologijos bei atsako į gydymą. Šiame straipsnyje pristatomas ES klinikinis atvejis. Pacientei sindromas pasireiškė dviem ITP epizodais, po kurių trečiąjį kartą pacientė atvyko manifestavus AIHA epizodui. Diagnozuotas ES. Taikytas gydymas intraveniniu imunoglobulinu (IVIG), gliukokortikoidais bei rituksimabu. Pasiekta remisija.

Berdono sindromas arba didelės šlapimo pūslės – mažos gaubtinės žarnos – sumažėjusios žarnų peristaltikos sindromas (angl. megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS)) yra labai retas, įgimtas ir gyvybei pavojingas sindromas, kurio tikslus paplitimas nėra žinomas. Šiame straipsnyje pristatomas Berdono sindromo klinikinis atvejis. Šio sindromo metu pasireiškia pasikartojantys dinaminio žarnyno nepraeinamumo simptomai bei šlapimo pūslės tonuso sutrikimai, sukeliantys šlapimo susilaikymą, nesant jokios distalinės obstrukcijos, kliudančios nutekėti šlapimui. Mūsų aprašomam pacientui šis sindromas buvo patvirtintas molekuliniais genetiniais metodais Japonijos Keio universitete, nustačius de novo atsiradusį ACTG2 geno c.769C>T, pArg257Cys heterozigotinį patogeninį variantą. Berdono sindromas gydomas tik simptomiškai – taikoma parenterinė mityba bei didelės šlapimo pūslės kateterizavimas.

Atopinis dermatitas (AD) yra lėtinė, banguojančios eigos odos liga, dažniausiai sukelianti stiprų odos niežėjimą ir vientisumo pažeidimą. AD serga 20 proc. kūdikių ir vaikų iki 2 metų, iš kurių maždaug trečdalio simptomai siejami su alergija maistui. AD kartais pasireiškia su pirminiu imunodeficitu, o AD sergančių vaikų odą neretai kolonizuoja Staphylococcus aureus (S. aureus). AD gali būti sunki liga, kurią gali sukelti su IgE susijusios arba nesusijusios bei mišrios imuninės reakcijos. Nuo AD sukėlusio mechanizmo priklauso diagnostinių tyrimų pasirinkimas bei simptomų kontrolei naudojamų vaistų paskyrimas. Esant sunkiai AD formai su dauginės alergijos maistui sindromu ir pirminiu imunodeficitu, gyvenimo kokybę gerina tinkamas kompleksinis gydymas. Darbo tikslas – remiantis mokslinės literatūros šaltinių analize, išnagrinėti ir pristatyti sunkios formos atopinio dermatito atvejį, kuris pasireiškė kūdikiui kartu su dauginės alergijos maistui sindromu bei pirminiu imunodeficitu.