Zespół samotnego wrzodu odbytnicy i enterocele u 13-letniego chłopca

Zespół samotnego wrzodu odbytnicy (solitary rectal ulcer syndrome - SURS) jest rzadkim schorzeniem u dzieci i młodzieży. Zwykle objawia się zaburzeniami oddawania stolca i bólami brzucha. Powszechnie uważa się, że w patogenezie tego schorzenia odgrywają rolę zaburzenia defekacji. W niniejszej pracy przedstawiono opis przypadku 13-letniego chłopca z zespołem nadpobudliwości psychoruchowej i SURS. Głównymi objawami, które zgłaszał pacjent, były: zwiększona częstość oddawania stolca z obecnością śluzu, okresowe krwawienia z odbytnicy, uczucie parcia na stolec i niepełnego wypróżnienia oraz bóle brzucha. W badaniu kolonoskopowym stwierdzono okrężne owrzodzenie i uniesioną, nieregularną zmianę sięgającą esicy z typowym dla SURS obrazem mikroskopowym. Defekografia uwidoczniła typowy dla enterocele obraz wpuklenia się pętli jelita cienkiego w ścianę prostnicy. Chłopcu i jego matce wyjaśniono łagodny, chociaż przewlekły, charakter schorzenia. Leczenie zachowawcze obejmowało zalecenie unikania wysiłkowego oddawania stolca oraz stosowanie diety bogatobłonnikowej, aby zapobiegać zaparciom. W SURS obraz zmian makroskopowych jest bardziej różnorodny, niż powszechnie się uważa. Ustalenie właściwego rozpoznania zależy w dużej mierze od wnikliwości zarówno klinicysty, jak i patologa.Solitary rectal ulcer syndrome (SRUS) is a rare disorder of childhood, which usually presents with any of the manifestations of anorectal disease, bowel habit alteration and abdominal pain. It is commonly accepted that pathogenesis is associated with disturbances of defecation. We describe a 13-year-old boy with attention deficit hyperactivity disorder (ADHD) and SRUS. He complained of increased stool frequency associated with the passage of blood and mucus, tenesmus, sensation of incomplete defecation and abdominal pain. Circumferential ulcer and flat lesion extending up to the sigmoid colon with typical microscopic findings were revealed upon colonoscopy. Defecography showed an opacified small bowel invaginating into the rectal wall, the pictures consistent with enterocele. Conservative treatment, including reassuring the patient and his mother of the benign nature of the disease, recommendation to avoid straining defecation and use of a high-fiber diet to prevent constipation, was applied. The macroscopic appearance of rectal lesions in SRUS are much more variable than is usually realized. An early diagnosis requires a high index of suspicion both for clinicians and the pathologist

Ultrasound variants of autoimmune thyroiditis in children and adolescents and their clinical implication in relation to papillary thyroid carcinoma development

Background The prevalence of autoimmune thyroiditis (AIT) and papillary thyroid carcinoma (PTC) is rising in children and adolescents, and the coincidence of AIT and PTC is as high as 6.3-43%. Objective To investigate the ultrasound manifestation of AIT in relation to PTC development in paediatric patients. Patients 179 paediatric patients (133 females), mean (SD) age: 13.9 (3.03) years diagnosed with AIT and referred for ultrasound evaluation. Eight patients were diagnosed with PTC (6 females). Methods Retrospective analysis of thyroid ultrasound scans of patients diagnosed with AIT. Thyroid and autoimmune status was assessed based on TSH, fT4, fT3 and increased aTPO and/or aTG and/or TRAB levels. In patients with PTC, total thyroidectomy was performed. Results Analysis of thyroid US scans revealed that the following five ultrasound variants of AIT were observed in 179 patients: the most common in 35.2%-diffuse thyroiditis with hypoechogenic background and normoechogenic parenchyma, in 30.2%-diffuse thyroiditis with irregular background, in 18.9% nodular variant with normoechogenic background, in 11.7%-micronodulations and in 3.9%-diffuse hypoechogenic background. Eight cases of PTC were diagnosed in nodular variant of AIT with normoechogenic irregular background. Conclusion Patients with AIT and nodular variant with normoechogenic irregular background of the thyroid gland on US scans are in the risk group of developing PTC and should be followed up with regular neck US assessment

Wrodzona naczyniakowatość krwotoczna rozpoznana u 13-miesięcznego dziecka: opis przypadku i przegląd literatury

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant, rare and severe disease that results from vascular dysplasia. It is characterised by recurrent epistaxis, teleangiectasias of skin and mucosa and arteriovenous malformations in: lungs, brain, liver, gastrointestinal tract and spinal cord. Diagnosis of HHT is rarely done in early childhood, because of the gradual appearance of vascular malformations. Patients with HHT require complex, multidisciplinary management, because of heterogeneity of clinical manifestations, risk of potentially life-threatening complications and poor prognosis especially in case of multiple visceral teleangiectasia. Here we report a case of severe HTT with diffuse pulmonary arteriovenous malformations in 13-month-old child with negative family history, who presented with polyglobulia and development retardation. On the basis of literature review we present current recommendations for diagnosis and management in HHT, in particularly in the case of diffuse pulmonary arteriovenous malformations, disqualified from any interventional procedures and associated with a poor prognosis

Single-agent MOR208 salvage and maintenance therapy in a patient with refractory/relapsing diffuse large B-cell lymphoma : a case report

Background: Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin’s lymphoma. Standard first-line treatment for this aggressive subtype comprises the anti-CD20 antibody rituximab combined with cyclophosphamide, doxorubicin, vincristine, and prednisone. If patients receiving such treatment have an early relapse, or their disease is initially refractory to such treatment, standard salvage regimens may not be effective. There is therefore a high unmet clinical need for new targeted agents that might improve the outcome for such patients. CD19 is a B-lymphocyte lineage-specific cell surface antigen that is expressed by most B-cell non-Hodgkin's lymphomas. MOR208 is an fragment-crystallizable engineered humanized monoclonal antibody with enhanced antitumor activity that targets CD19 and that may consequently have clinical utility in this setting. Case presentation: We describe the case of a 33-year-old Caucasian man who presented with a 3-month history of general symptoms and who was admitted to our pulmonology ward with dyspnea due to pneumonia and severe anemia. A histopathological examination of an enlarged right suprasternal lymph node confirmed a diagnosis of T-cell/ histiocyte-rich large B-cell lymphoma, an uncommon morphological variant of diffuse large B-cell lymphoma. Our patient had a complete response to first-line rituximab combined with cyclophosphamide, doxorubicin, vincristine, and prednisone, but had an early relapse 5 months after the end of treatment. After intensive salvage therapy consolidated with an autologous stem-cell transplant, our patient again had an early relapse and was subsequently enrolled in a phase IIa trial of single-agent MOR208. Following a scheduled 3 months of weekly treatment, a partial response was confirmed and MOR208 was continued as maintenance therapy, with administration every second week. Positron emission tomography-computed tomography confirmed a complete response 9 months later. This response is ongoing, with a duration of 24 months. MOR208 was well-tolerated by our patient and his quality of life and performance status remain high. No hospitalizations were required and our patient engaged in full-time work and physical activities. Conclusion: Third-line single-agent therapy with the CD19 antibody MOR208 was highly effective in this patient, despite a history of early relapse after standard first-line and second-line treatment regimens. These data provide support for future randomized studies of MOR208

Follow-up of parenchymal changes in the thyroid gland with diffuse autoimmune thyroiditis in children prior to the development of papillary thyroid carcinoma

Purpose To present the outcomes of ultrasound (US) follow-ups in children with autoimmune thyroid disease who did not have a thyroid nodule on admission but developed papillary thyroid carcinoma (PTC) and to characterize the parenchymal changes in the thyroid gland prior to the development of PTC. Methods A retrospective thyroid US scan review of 327 patients diagnosed with AIT was performed. Forty patients (40/327, 12.2%) presented nodular AIT variant with a normoechogenic background. Eleven patients (11/327, 3.4%, 11/40, 27.5%) presenting this variant were diagnosed with PTC (nine females-mean age 15.3 years; two males aged 11 and 13 years). In five of 11 patients, the suspicious nodule that was later confirmed to be PTC was detected on the initial US at presentation. For the remaining six females (6/11) who developed PTC during the follow-up, we retrospectively analysed their US thyroid scans and these patients were selected for analysis in this study. Results On admission, the US evaluation revealed an enlarged normoechogenic thyroid gland in three patients and a hypoechogenic thyroid gland with fibrosis as indicated by irregular, chaotic hyperechogenic layers in three patients. No thyroid nodules were identified. Ultrasound monitoring revealed increasing echogenicity of the thyroid parenchyma during the follow-up. PTC developed in a mean time of 4.6 years (1 9/12-7 4/12 years) since referral to the outpatient thyroid clinic and 2.9 years (6/12-6 9/12) since the last nodule-free US thyroid scan. Conclusions Sonographic follow-up assessments warrant further exploration as a strategy to determine PTC susceptibility in the paediatric population

Economic Determinants of Low-Carbon Development in the Visegrad Group Countries

Low-carbon development is one of the more significant problems of the Visegrad Group countries (Czech Republic, Poland, Slovakia, and Hungary). It is related, among others, to the improvement of life quality in economic terms while taking into account activities for environmental protection. The aim of the article is to identify and explain the problems connected with low-carbon development. The purpose of the analyses is also to prove the negative impact of the emission of greenhouse gas emission (GHG) and other harmful substances into the air on the quality of human life and the natural environment. During the research, an assessment of the eco-efficiency of the used energy resources and technologies that negatively affect the environment was carried out. Moreover, the paper also presents methods to use greener energy sources and analyses the potential of implementing solutions supporting low-carbon development. The study recommends actions that may contribute to the reduction of greenhouse gas emissions. These include the limitation of the use of fossil fuels for the benefit of renewable energy and the development of distributed energy