46 research outputs found
Aktivnost eritrocitne glutation S-transferaze u djece oboljele od HenochSchƶnleinove purpure [Erythrocyte glutathione S-transferase activity in children with Henoch-Schƶnlein purpura]
Introduction: Henoch-Schƶnlein purpura (HSP) is the most common vasculitis of
the childhood. Among all possible symptoms / complications, nephritis (HSPN) is
the main and almost only cause of morbidity and mortality in HSP. The aim of this
study was to investigate the value of erythrocyte glutathione S-transferase (eGST) activity as an early predictor of nephritis in HSP. -----
Subjects and methods: Ninety-seven children with HSP were enrolled into the
study. The control group consisted of 52 children without clinical and laboratory
signs of inflammation. In all patients e-GST activity was determined
spectrometrically from the whole blood samples, after incubation with a
commercial GST assay at the time of enrolment and twice more in regular
intervals during follow up period of six months. In children from the control group
e-GST activity was determined at the time of enrolment. -----
Results: At the beginning of the disease the e-GST activity values were
significantly higher in the group of patients with HSPN compared to the group of
HSP patients without nephritis: median (interquartile range) 5,70 U/gHb (4,38-7,50
U/gHb) compared to 3,10 U/gHb (2,20-4,20 U/gHb); PĖ0,001. Similar results were
obtained after the comparison of the patients with HSPN and control group: 5,70
U/gHb (4,38-7,50 U/gHb) vs. 3,13 U/gHb (1,91-4,20 U/gHb); PĖ0,001. There were no
statistically significant differences between the group of HSP patients without
nephritis and a control group (P=0,837). During the follow up period of six months,
a significant decrease of e-GST activity was observed in the HSPN patients, but it was still significantly higher compared to the group of HSP patients without
nephritis (PĖ0,001 / PĖ0,001).
In the ROC analysis of the e-GST activity determination value in the prediction of
HSP nephritis, at the e-GST values >4,1 U/gHb a significant area under the curve
(AUC) of 91.1% (P < 0.001) and sensitivity of 90.5% and specificity of 72.7% was
found at the beginning of the study. The sensitivity of the nephritis detection tests
decreased, and the specificity increased during the follow up period. No significant
correlation between e-GST activity and severity of skin changes, or used therapy
was found. Among the routine laboratory tests, a consistent, statistically
significant, positive correlation was found only between e-GST activity and the
number of erythrocytes per mm3 in urine samples. -----
Conclusion: e-GST activity is a reliable, independent marker of early nephritis risk
assessment in children with HSP. As a sensitive and specific, feasible and
inexpensive laboratory test, it has potential practical utility in the diagnostic
algorithm and monitoring of the children with HSP
VASCULITIDES IN CHILDHOOD
Primarni sistemski vaskulitisi u djece relativno su rijetke bolesti, veÄinom nepoznate etiologije, kojima
je zajedniÄko obilježje upala u stijenci krvne žile. TeÅ”ko ih je dijagnosticirati jer su zahvaÄeni mnogi organi, a simptomi
su uglavnom nespecifi Äni. U posljednjih 10 godina postignut je znatan napredak u podruÄju vaskulitisa u djeÄjoj dobi:
defi nirani su i validirani klasifi kacijski kriteriji, razvijeni i validirani upitnici za procjenu aktivnosti bolesti i ishoda,
pedijatrijski bolesnici ukljuÄeni su u meÄunarodna multicentriÄna istraživanja vezana uz terapiju vaskulitisa, zapoÄeta
su kliniÄka istraživanja izuzetno rijetkih vaskulitisa i izdvojena je zasebna skupina rijetkih monogenskih vaskulitisa.
Ovim radom želimo upoznati Äitaoce s velikim iskorakom u podruÄju vaskulitisa u djeÄjoj dobi, nastalim na osnovi
mukotrpnog rada pedijatrijskih reumatologa u radnim skupinama za vaskulitise.Primary systemic vasculitides in children are relatively rare diseases. In most cases, they have an unknown
etiology and are defi ned as the presence of infl ammation in the blood vessel wall. Establishing the diagnosis of
vasculitis is oft en challenging, since the disorder is multisystem in nature with mostly nonspecifi c symptoms. Th e last
10 years have seen signifi cant advances in the fi eld of pediatric vasculitis: the development and validation of classifi cation
criteria as well as tools to assess clinical disease activity and disease outcome, the inclusion of pediatric patients in
international multicentre randomized controlled trial designs for therapies of vasculitis, clinical trials for very rare
pediatric vasculitides, and identifi cation of a special group of monogenic vasculitides. In this paper we want to introduce
readers to the giant leap in the fi eld of pediatric vasculitis as a result of the hard work of pediatric rheumatologists
in vasculitis work groups
Rituximab in Treatment of Children with Refractory Vasculitis and Systemic Lupus Erythematosus ā Single Center Experience in Croatia
The aim of this study was to present our experience
in rituximab therapy in patients with childhood-onset systemic
lupus erythematosus, lupus nephritis, and ANCA-associated vasculitis.
We conducted a retrospective clinical chart review of all
patients treated with rituximab in the time period from January
2009 to December 2015. Eight patients (3 boys and 5 girls)
aged 8 to 15 at the onset of disease were treated with rituximab.
Remission of disease was accomplished in 4 patients with childhood-
onset systemic lupus erythematosus and lupus nephritis, a
partial improvement was achieved in 1 patient with childhoodonset
systemic lupus erythematosus and lupus nephritis as well
as in 2 patients with vasculitis, while in one patient with vasculitis
treatment with rituximab showed no effect and the patient
died due to Candida sepsis. Reduction of corticosteroid doses
was enabled by rituximab treatment. Rituximab appeared to be
a safe and efficient therapeutic option in severe cases of childhood-
onset systemic lupus erythematosus or ANCA-associated
vasculitis that failed to respond to conventional therapy or as a
rescue therapy in life-threatening conditions
Kako lijeÄiti bolesnike nakon teÅ”kih neželjenih djelovanja uzrokovanih inhibitorima TNF
Biological agents are widely used in the treatment of autoimmune rheumatic disorders. We report on serious adverse events during treatment with anti-tumor necrosis factor antibody in two of our patients with juvenile idiopathic arthritis. One patient was treated with a biological agent due to juvenile idiopathic arthritis complicated by uveitis, developing miliary tuberculosis during treatment. After treatment with antituberculotics, she recovered completely. Her underlying disease is currently in remission. Another patient was treated for juvenile spondyloarthritis and developed an inflammatory process of the central nervous system with serious neurological deficits. He was treated with high-dose corticosteroids, followed by slowly tapering doses of corticosteroids. His neurological deficits improved, but are still present. Similar cases have been described previously, but there are no
recommendations how to treat arthritis afterwards in such patients. We would like to emphasize the need of developing guidelines for further treatment of arthritis after the occurrence of serious adverse effects during treatment with biological agents.BioloÅ”ki lijekovi se primjenjuju u lijeÄenju brojnih autoimunih reumatskih bolesti. U ovom Älanku prikazujemo dva sluÄaja ozbiljnih nuspojava lijeÄenja inhibitorima Äimbenika nekroze tumora (tumor necrosis factor, TNF) kod bolesnika s juvenilnim idiopatskim artritisom (JIA): bolesnice lijeÄene zbog JIA kompliciranog razvojem uveitisa, kod koje se javila milijarna tuberkuloza tijekom lijeÄenja. Nakon lijeÄenja antituberkuloticima doÅ”lo je do potpunog oporavka. Njena osnovna bolest je u remisiji. Drugi bolesnik je lijeÄen zbog juvenilnog spondiloartritisa te je razvio upalni proces srediÅ”njega živÄanog sustava
s ozbiljnim neuroloÅ”kim posljedicama. LijeÄen je visokim dozama kortikosteroida koje su potom postupno snižavane. NeuroloÅ”ki ispadi su se dijelom poboljÅ”ali, ali su ipak joÅ” uvijek prisutni. SliÄni sluÄajevi su opisivani i ranije, ali nema preporuka kako bi trebalo lijeÄiti artritis nakon Å”to nastupe takve nuspojave. Željeli bismo naglasiti potrebu stvaranja smjernica za daljnje lijeÄenje artritisa nakon pojave teÅ”kih nuspojava prilikom lijeÄenja bioloÅ”kim lijekom
Usporedba razliÄitih dijagnostiÄkih smjernica za dijagnozu sindroma aktivacije makrofaga koji komplicira sistemski tip juvenilnog idiopatskog artritisa: iskustvo jednog centra
Macrophage activation syndrome (MAS) is a potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA), caused by exaggerated but ineff ective immune response. The aim of the study was to compare the capacity of the HLH-2004 guidelines with the capacity of the MAS guidelines from 2005, and with the new set of classifi cation criteria from 2016 in diagnosing MAS complicating sJIA. Th e study included 35 children aged 1-18 diagnosed with sJIA according to ILAR criteria and treated at the Department of Pediatrics, Division of Immunology and Rheumatology, Zagreb University Hospital Centre, in the period from 2009 to 2015. Out of 35 patients diagnosed with sJIA, there were 12 girls and 23 boys, with the mean age at disease onset (Ā±SD) 5.51Ā±3.65 years. Eight patients had fl are of disease. With the guidelines from 2005, MAS was diagnosed in six (17.1%) patients with sJIA. With the new set of classifi cation criteria from 2016, MAS was diagnosed in four (11.4%) patients with sJIA. MAS was not diagnosed with the HLH-2004 guidelines. In our study, four out of six patients had MAS at the onset of sJIA, and in the rest two it occurred during relapse. Two patients with MAS developed full-blown clinical picture while another four had incomplete clinical features with minor laboratory alteration. Due to the use of diff erent diagnostic guidelines, we found diff erence in the prevalence of MAS. It was slightly higher in comparison to available studies, while other researched features, such as clinical characteristics, were similar.Sindrom aktivacije makrofaga (MAS) potencijalno je smrtonosna komplikacija sistemskog tipa juvenilnog idiopatskog artritisa (sJIA) uzrokovana prekomjernim, ali neuÄinkovitim imunim odgovorom. Cilj ovoga istraživanja bio je usporediti dijagnostiÄku moguÄnost smjernica HLH-2004 sa smjernicama za MAS iz 2005. godine, kao i s novim skupom klasifi kacijskih kriterija iz 2016. godine u dijagnostici MAS-a koji komplicira sJIA. U istraživanje je bilo ukljuÄeno 35 djece u dobi od 1 do 18 godina kojima je postavljena dijagnoza sJIA prema kriterijima ILAR-a i koja su lijeÄena u Klinici za pedijatriju, Zavodu za imunologiju i reumatologiju KliniÄkog bolniÄkog centra Zagreb u razdoblju od 2009. do 2015. godine. Od 35 bolesnika kojima je postavljena dijagnoza sJIA bilo je 12 djevojÄica i 23 djeÄaka koji su u vrijeme poÄetka bolesti bili prosjeÄne dobi (Ā±SD) 5,51Ā±3,65 godina. Osmero bolesnika imalo je recidiv bolesti. Prema smjernicama iz 2005. godine dijagnoza MAS-a postavljena je u Å”estero (17,1%) bolesnika sa sJIA. Prema novom skupu klasifi kacijskih kriterija iz 2016. godine dijagnoza MAS-a postavljena je u Äetvero (11,4%) bolesnika sa sJIA. Dijagnoza MAS-a nije postavljena ni u jednog bolesnika prema smjernicama HLH-2004. U naÅ”em istraživanju Äetvero od Å”estero bolesnika imalo je MAS na poÄetku sJIA, a u preostalih dvoje on se pojavio tijekom recidiva bolesti. Dvoje bolesnika s MAS-om razvilo je punu kliniÄku sliku bolesti, dok ih je preostalih Äetvoro imalo nepotpuna kliniÄka obilježja s manjim odstupanjem u laboratorijskim nalazima. Primjenom razliÄitih dijagnostiÄkih smjernica utvrdili smo razliku u uÄestalosti MAS-a. UÄestalost je bila neÅ”to viÅ”a u usporedbi s postojeÄim istraživanjima, dok su ostala istraživana obilježja poput kliniÄkih karakteristika bila sliÄna
Diagnosis and surgical treatment of intestinal malrotation in a patient with Cornelia de Lange syndrome
Prikazujemo žensko dojenÄe s fenotipskim karakteristikama sindroma Cornelia de Lange kod kojeg je dokazana i uspjeÅ”no kirurÅ”ki
lijeÄena pridružena malrotacija crijeva. Svrha je rada upozoriti na Äinjenicu da malrotacija crijeva, iako ne pripada skupini uÄestalih
simptoma sindroma Cornelia de Lange, ne smije biti izostavljena u diferencijalnoj dijagnostici gastrointestinalnih tegoba u navedenih
bolesnika.We report on a female infant with phenotypic characteristics of Cornelia de Lange syndrome and associated, successfully surgically
treated, intestinal malrotation. The purpose of this report is to point out that intestinal malrotation, as a rare element of Cornelia de
Lange syndrome, should not be left out on the diff erential diagnosis of gastrointestinal symptoms in these patients
Povezanost kožnih manifestacija i kliniÄkih znaÄajki IgA vaskulitisa
Introduction: IgA vasculitis (IgAV ) is the most common systemic vasculitis in childhood. Purpuric rash is a mandatory criterion for diagnosing IgAV , it is mostly localized on the lower extremities and gluteal region, although it can also appear atypically affecting the face, trunk and upper extremities. In the most severe cases, ulcerations, necrosis and bullae can be present. Objectives: To evaluate the characteristics of cutaneous manifestations in patients with IgAV and to examine its association with clinical features. Subjects and methods: Retrospective analysis of data from patients with IgAV diagnosed and treated at the Referral Centre for Paediatric and Adolescent Rheumatology of the Ministry of Health of the Republic of Croatia, in the period from January 2009 to December 2021. Results: IgAV was diagnosed in 234 patients, 124 boys and 110 girls with the median (range) age at the time of diagnosis of 6.5 (4.5ā8.2) years. All patients had a purpuric rash, and in 127 of them (54.3%) IgAV began with a rash. Cutaneous manifestations were most
often presented in the form of palpable purpura and/or petechiae (87.2%) and in all patients were localized on the lower extremities. In 103 patients (44%) purpuric rash spread further to the upper extremities, trunk and/or face. At least one skin relapse occurred in 47 patients (20.1%). The most severe cutaneous manifestations which included ulcerations and necrosis developed in 11 patients (4.7%). Patients with cutaneous manifestations spread above the waist had a more statistically significant gastrointestinal involvement compared to patients with cutaneous manifestations affecting the lower extremities and gluteal region (50.5% vs. 36.6%, p=0.033), higher incidence of IgA vasculitis nephritis (IgAVN ) (31.1% vs. 19.8%, p=0.048) and were more frequently treated with systemic glucocorticoids (68% vs. 52.7%, p=0.018) and angiotensin-converting enzyme inhibitors (14.5% vs. 5.3%, p=0.016). Almost all patients with ulcerations and necrosis required treatment with systemic glucocorticoids compared to the rest (90.9% vs. 57.8%,p=0.031). Conclusion: We observed that patients with purpuric rash spread above the waist have more frequently affected gastrointestinal system and a higher incidence of IgAVN . The prevalence of ulcerations and necrosis in IgAV is less common than the standard purpuric rash and this group of patients required systemic glucocorticoid therapy.Uvod: IgA vaskulitis (IgAV ) najÄeÅ”Äi je sistemski vaskulitis djeÄje dobi. PurpuriÄni osip kljuÄan je kriterij za dijagnozu IgAV -a, a najÄeÅ”Äe je rasprostranjen po donjim udovima i gluteusima, iako može biti proÅ”iren i na atipiÄnim mjestima poput lica, trupa i gornjih udova. U najtežim sluÄajevima mogu biti prisutne ulceracije, nekroze i bule. Cilj: Utvrditi osobitosti kožnih promjena u bolesnika s IgAV -om te ispitati njihovu povezanost s kliniÄkim znaÄajkama. Ispitanici i metode: Retrospektivna analiza podataka bolesnika s IgAV -om dijagnosticiranih i lijeÄenih u Referentnom centru za pedijatrijsku i adolescentnu reumatologiju Ministarstva zdravstva RH, u razdoblju od sijeÄnja 2009. do prosinca 2021. godine. Rezultati: IgAV je dijagnosticiran u 234 bolesnika, 124 djeÄaka i 110 djevojÄica s medijanom (rasponom) dobi u trenutku dijagnoze 6,5 (4,5 ā 8,2) godina. Svi su bolesnici imali kožni osip, a u njih 127 (54,3%) IgAV je i zapoÄeo osipom. Kožne promjene najÄeÅ”Äe su bile zastupljene u obliku palpabilne purpure i/ili petehija (87,2%) i u svih bolesnika bile su lokalizirane po donjim udovima. U 103 bolesnika (44%) kožni osip se dalje proÅ”irio na ruke, trup i/ili lice. U 47 bolesnika (20,1%) doÅ”lo je do barem jednog recidiva kožnih promjena. Najteže kožne promjene u vidu ulceracija i nekroza razvilo je 11 bolesnika (4,7%). Bolesnici s kožnim promjenama proÅ”irenim iznad donjih udova imali su statistiÄki znaÄajno ÄeÅ”Äe zahvaÄen gastrointestinalni sustav u odnosu na bolesnike s kožnim osipom ograniÄenim na donje udove i glutealno (50,5% u odnosu na 36,6%, p=0,033), veÄu pojavnost nefritisa (IgAVN) (31,1% u odnosu na 19,8%, p=0,048) te su ÄeÅ”Äe lijeÄeni sistemskim glukokortikoidima (68% u odnosu na 52,7%, p=0,018) i
inhibitorima angiotenzin konvertaze (14,5% u odnosu na 5,3%, p=0,016). Gotovi svi bolesnici s ulceracijama i nekrozama zahtjevali su lijeÄenje sistemskim glukokortikoidima u odnosu na sve preostale bolesnike (90,9% u odnosu na 57,8%, p=0,031). ZakljuÄak: UoÄili smo da bolesnici s kožnim osipom proÅ”irenim iznad donjih udova imaju ÄeÅ”Äe zahvaÄen gastrointestinalni sustav i ÄeÅ”Äu pojavu IgAVN -a. UÄestalost ulceracija i nekroza u IgAV -u rjeÄa je od klasiÄne slike kožnog osipa i takvi bolesnici zahtijevali su lijeÄenje sistemskim glukokortikoidima