[Association of giant cell hepatitis and autoimmune hemolytic anemia in infancy]. / Óriássejtes hepatitis és autoimmun haemolyticus anaemia társulása kisdedkorban.

Giant cell hepatitis associated with autoimmune hemolytic anemia (GCH-AIHA) is a rare disorder with unfavorable prognosis, affecting infants and young children. The mortality rate is high, complications of acute liver failure, sepsis, or liver transplantation can be responsible for fatal outcomes. An 18-month-old child who was diagnosed previously with autoimmune hemolytic anemia, developed acute hepatitis and acute liver failure concomitant to the relapse of the disease. GCH-AIHA is characterized by Coombs positive hemolytic anemia and progressive liver injury, histologically defined by widespread giant cell transformation. Liver biopsy was performed to establish the diagnosis, histological examination confirmed the presence of multinuclear, giant cell hepatocytes. Corticosteroid and azathioprine treatment were started. As a result of subsequent rituximab treatment and intravenous immunoglobulin therapy, acute liver failure and anemia gradually resolved. The exact background of the association of the two entities is still unknown, an autoimmune mechanism is suspected. Conventional immunosuppressive treatment with corticosteroid and azathioprine seems to be ineffective in most cases, therefore second- and third-line therapies are required. Since the introduction of the anti-CD20 rituximab therapy, the prognosis of GCH-AIHA has improved significantly. Orv Hetil. 2023; 164(36): 1432-1436.

Body composition, physical activity, and quality of life in pediatric patients with inflammatory bowel disease on anti-TNF therapy-an observational follow-up study.

BACKGROUND: Poor outcome of inflammatory bowel disease (IBD) is associated with malnutrition. Our aim was to compare body composition (BC) and physical activity (PA) between patients with IBD and healthy controls, and to assess the changes in BC, PA and health related quality of life (HRQoL) in children with IBD during anti-TNF therapy. METHODS: 32 children with IBD (21 with Crohn's disease (CD), (age: 15.2 ± 2.6 years, 9 male) and 11 with ulcerative colitis (UC), (age: 16.4 ± 2.2 years, 5 male) participated in this prospective, observational follow up study conducted at Semmelweis University, Hungary. As control population, 307 children (age: 14.3 ± 2.1) (mean ± SD) were included. We assessed BC via bioelectric impedance, PA and HRQoL by questionnaires at initiation of anti-TNF therapy, and at two and six months later. The general linear model and Friedman test were applied to track changes in each variable. RESULTS: During follow-up, the fat-free mass Z score of children with CD increased significantly (-0.3 vs 0.1, p = 0.04), while the BC of patients with UC did not change. PA of CD patients was lower at baseline compared to healthy controls (1.1 vs. 2.4), but by the end of the follow up the difference disappeared. CONCLUSIONS: The fat-free mass as well as PA of CD patients increased during the first six months of anti-TNF treatment. As malnutrition and inactivity affects children with IBD during an important physical and mental developmental period, encouraging them to engage in more physical activity, and monitoring nutritional status should be an important goal in patient care.

The gastrointestinal effects of COVID­19 infection during childhood and among the children affected with inflammatory bowel disease / A COVID-19-fertozés gastrointestinalis hatásai gyermekkorban és a gyulladásos bélbeteg gyermekekben.

Összefoglaló. A SARS-CoV-2-infekció változatos kórlefolyású, a gyermekpopulációban növekvo incidenciát mutató fertozés. Ebben a korcsoportban a felnottekkel szemben sokkal gyakrabban tapasztalhatók gasztroenterológiai tünetek a betegség során, 18-32%-ban jelentkezik legalább egy szimptóma. Ezek nem specifikusak, gyakran megegyezhetnek a virális enteritisek, a gyulladásos bélbetegségek vagy a vakbélgyulladás tüneteivel. A gyermekkori SARS-CoV-2-infekciónak egy viszonylag ritkán megjeleno, de súlyos, akár életveszélyes szövodménye a gyermekkori sokszervi gyulladásos szindróma (multisystem inflammatory syndrome in children, MIS-C). Ilyenkor a gastrointestinalis tünetek gyakorisága 60-100%-ra no, sok esetben akut has benyomását keltve. A jelenlegi kutatások eredményei alapján a gyulladásos bélbeteg gyerekek az alapbetegségük miatt nincsenek nagyobb veszélynek kitéve az átlagpopulációhoz képest a COVID-19-fertozés szempontjából. A terápiájukban alkalmazott gyógyszereik közül a nagy dózisú szteroidkezelés okoz nagyobb kockázatot a megfertozodésre, illetve ebben az esetben a súlyosabb kórlefolyásra. Az éppen remisszióban lévo gyulladásos bélbetegek fenntartó terápiájának módosítások nélküli folytatása javasolt, kiemelt figyelmet fordítva a biológiai terápiák idoben történo, megszakítás nélküli alkalmazására. Törekedni kell a személyes vizitek számának csökkentésére a pandémia idején, ezek telemedicinával történo helyettesítése javasolt. A halasztható endoszkópos vizsgálatok noninvazív vizsgálómódszerekkel történo átmeneti kiváltása részesítendo elonyben a betegség aktivitásának, a terápia hatékonyságának megítélésére. A gyulladásos bélbetegségben szenvedo gyermekek COVID-19 elleni védooltása javasolt, jelenleg minden elérheto oltóanyag alkalmazható náluk (az élo ágenst tartalmazó vakcinák ellenjavalltak). Immunmoduláns, szteroid- vagy anti-tumornekrózisfaktor (TNF)-alfa-terápia esetén az oltás lehetséges csökkent hatékonyságával kell számolni. Orv Hetil. 2022; 163(6): 214-221. Summary. The SARS-CoV-2 infection is showing high variety in the disease course, with a constantly increasing incidence among the pediatric population. In this age group, at least one gastrointestinal symptom appears in 18-32% of the cases, showing a significant difference compared to the adult population. The gastrointestinal signs of COVID-19 are not specific, can mimic the symptoms of viral enteritis, inflammatory bowel diseases or acute appendicitis. The multisystem inflammatory syndrome in children (MIS-C) is a rather rare, but serious complication of the pediatric COVID-19 disease: in these cases, the incidence of the gastrointestinal symptoms is increased up to 60-100%, often observed as acute abdomen. Based on recent researches, patients with inflammatory bowel diseases (IBD) are shown to have the same risk in developing COVID-19 infection compared to the normal population: in their medications, the high dose steroid treatment is proved to increase the risk of infection or to make the disease course more serious. The treatment of patients with IBD should be continued without any changes (when the disease is in remission). The use of biologics should be done with special care, with more attention keeping the schedule and the continuity. It is advised to minimise the number of personal visits during the pandemic, they should be substituted with telemedicine. The postponable endoscopic examinations should be temporarily redeemed by non-invasive methods for screening the disease activity and the efficacy of the treatment. The vaccination against COVID-19 is advised in the population with IBD. All vaccines currently available are usable in this patient group (the use of vaccines containing live agents are contraindicated). In the case of patients treated with immunmodulators, steroids or anti-tumor necrosis factor (TNF) alpha, a possible lower efficacy can be expected after the vaccination. Orv Hetil. 2022; 163(6): 214-221.