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Raluca Vlad

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Articole semnate de acelasi autor in Revista Romana de Pediatrie:

Overlap and other autoimmune phenomena in pediatric chronic hepatic disease

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Revista Romana de PEDIATRIE | Volumul LXI, Nr. 2, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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Overlap and other autoimmune phenomena in pediatric chronic hepatic disease

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ABSTRACT

Patients with chronic liver disease can present serological immune associations or a real overlap syndrome. The hepatic overlap syndrome includes biochemical and histological features either for two autoimmune hepatic diseases or for one autoimmune hepatitis and another chronic hepatic disease (chronic viral hepatitis B or C, Wilson’s disease, fatty liver disease). In the absence of appropriate treatment, the natural course of autoimmune hepatitis and overlap syndromes is towards cirrhosis and liver failure. The diagnostic criteria, prognostic scores and predictive factors for overlap syndromes are not yet established. The serological immune reactions are also found in liver disease other than autoimmune hepatitis. In chronic viral hepatitis C different types of immunological manifestations are oftenly associated. In non-alcoholic fatty liver disease the high prevalence of self-antibodies indicates the presence of an autoimmune response; on the other hand these antibodies can help predict the severity of the disease. The medical therapy of either the autoimmune reaction or the underlying hepatic disease should use an individualized algorithm regarding drug associations, dosing and duration of treatment.

Key words: hepatitis, overlap syndrome

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FENOMENE AUTOIMUNE DE OVERLAP IN HEPATITELE CRONICE ALE COPILULUI

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Revista Romana de PEDIATRIE | Volumul LXI, Nr. 2, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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FENOMENE AUTOIMUNE DE OVERLAP IN HEPATITELE CRONICE ALE COPILULUI

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REZUMAT

Pacienţii cu hepatopatii cronice pot prezenta fenomene imunologice serologice sau chiar un veritabil sindrom de overlap. Aceste sindroame prezintă caractere comune biochimice şi histopatologice pentru cel puţin două dintre afecţiunile autoimune hepatice sau pentru o afecţiune autoimună hepatică şi o altă afecţiune hepatică cronică nonimună (infecţie hepatică VHC, VHB, boala Wilson, steatoza hepatică non-alcoolică). Evoluţia hepatitelor autoimune sau sindroamelor de overlap este adesea către ciroză şi insuficienţă hepatică fără terapie adecvată. Criteriile de diagnostic, scorurile de prognostic sau factorii de predicţie pentru apariţia sindroamelor de overlap nu sunt încă pe deplin stabilite. Fenomene de overlap serologic sunt descrise, şi în alte afecţiuni hepatice în afara celor imune recunoscute. În infecţia cronică cu virus hepatitic C pot fi identificate mai multe tipuri de reacţii imunologice. În steatoza hepatică nonalcoolică prevalenţa crescută a autoanticorpilor sugerează prezenţa fenomenului imun precum şi posibilitatea folosirii acestora ca factori de predicţie pentru severitate. Tratamentul medical al fenomenelor autoimune, dar şi al bolii de bază conduce la o terapie personalizată ca asociere terapeutică, doze, durată.

Cuvinte cheie: hepatită, hepatopatii, sindrom overlap

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Overlap syndromes in pediatric hepathology

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Revista Romana de PEDIATRIE | Volumul LXI, Nr. 3, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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Overlap syndromes in pediatric hepathology

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ABSTRACT

In hepathology, the term “overlap syndrome” describes different forms of hepatobiliary autoimmune disease: autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), autoimmune cholangitis (AIC). Patients with hepatic overlap syndrome present with both biochemical and histological features of at least two of AIH, PBC, PSC. In the absence of appropriate treatment, the course of the disease in autoimmune hepatitis and overlap syndromes is towards cirrhosis and liver failure. It remains unclear whether these overlap syndromes are distinct entities or variants of the major autoimmune hepatopathies. Standardization and diagnostic criteria for overlap syndromes are not yet established and the term “overlap syndrome” is common in clinical practice, but not always used correctly. Empiric medical treatment of overlap syndromes includes anticholestatic therapy with ursodeoxycholic acid and immunosuppressive therapy with corticosteroids and azathioprine in a case to case based therapy schedule. In end-stage disease, liver transplantation is the treatment of choice.

Key words: hepatitis, child, overlap

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SINDROAME DE OVERLAP IN HEPATOLOGIA PEDIATRICA

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Revista Romana de PEDIATRIE | Volumul LXI, Nr. 3, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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SINDROAME DE OVERLAP IN HEPATOLOGIA PEDIATRICA

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REZUMAT

Termenul „sindrom de overlap“ descrie, în hepatologie, forme diferite de afecţiuni hepatobiliare autoimune: hepatită autoimună (HAI), ciroză biliară primitivă (CBP), colangită sclerozantă primitivă (CSP), colangită autoimună. Pacienţii cu sindrom hepatic de overlap prezintă caractere comune biochimice şi histopatologice pentru cel puţin două din afecţiunile autoimune hepatice. Evoluţia hepatitelor autoimune sau sindroamelor de overlap este adesea către ciroză şi insuficienţă hepatică fără terapie adecvată. Rămâne încă neclar dacă aceste „sindroame de overlap“ sunt entităţi distincte sau doar variante ale hepatopatiilor majore autoimune. Stadializarea şi criteriile de diagnostic, scorurile de prognostic sau de predicţie pentru apariţia sindroamelor de overlap nu sunt încă pe deplin stabilite, ceea ce face a termenul să fie folosit frecvent dar, nu totdeauna corect în practica medicală. Sindroamele de overlap sunt afecţiuni hepatice rare, dar cu prognostic sever. Tratamentul medical empiric al sindroamelor de overlap hepatic include: terapie imunosupresivă cu corticosteroizi şi azathioprină, terapie anticolestatică cu acid ursodeoxicholic, strict personalizată ca asociere terapeutică, doze, durată. Stadiile finale necesită transplant hepatic.

Cuvinte cheie: hepatită, copil, overlap

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Chronic pancreatitis in children

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Revista Romana de PEDIATRIE | Volumul LXI, Nr. 3, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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Chronic pancreatitis in children

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ABSTRACT

Chronic pancreatitis (CP) is characterised by chronic inflammation, progressive fibrosis, pain and loss of endocrine and exocrine functions. In 1999 the hypothesis of sentinel acute pancreatitis event – SAPE was formulated. This suggests that the initial episode of acute pancreatitis generated by metabolic or oxidative stress is followed by recurrent or chronic inflammation which will finally lead to fibrosis. The pancreatic stellate cells have a key role in the progression of chronic pancreatitis and in the initiation of fibrinogenesis. CP is a heterogenous disese with a clinical picture that includes pain, endocrine and exocrine pancreatic dysfunction and various complications that might implicate adjacent organs. For the diagnosis of CP in adults histological, morphological criteria or a combination of clinical, functional and morphological arguments are needed. Recently the International Study Group Of Pediatric Pancreatitis proposed a set of diagnostic criteria for CP: (a) typical abdominal pain plus characteristic imagistic findings or (b) exocrine pancreatic failure plus imagistic findings or (c) endocrine pancreatic failure plus imagistic findings. The etiology of CP is different in children compared to adult patients. Lately remarkable progress was made in the knowledge of hereditary and autoimmune pancreatitis. Obstructive causes may be identified in CP in children. Chronic obstructive pancreatitis are determined by congenital or acquired mechanical factors. The therapy includes: conservative measures (analgesia, antiinflammatory medication, enzyme substitution), endoscopic interventions and surgical procedures. Endoscopic retrograde cholangiopancreatography is used as therapeutic procedure for biliopancreatic disease with recurrent or chronic pancreatitis. In patients with no response to any of this therapeutic measures total pancreatectomy and self transplantation of pancreatic islets may be tried.

Key words: pancreatitis, pancreatic stellate cells

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PANCREATITE CRONICE LA COPIL

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Revista Romana de PEDIATRIE | Volumul LXI, Nr. 3, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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Promoting Global Health

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PANCREATITE CRONICE LA COPIL

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REZUMAT

Pancreatita cronică (PC) se caracterizează prin inflamaţie cronică, fibroză progresivă, durere şi pierderea funcţiilor endocrină şi exocrină. În 1999 a fost formulată ipoteza episodului santinelă de pancreatită acută (sentinel acute pancreatitis event - SAPE), care sugerează că episodul iniţial de pancreatită generat de stres metabolic sau oxidativ este urmat de inflamaţie recurentă sau cronică care produce fibroză. Celulele stelate pancreatice au fost identificate ca având rol cheie în progresia pancreatitei cronice şi în dezvoltarea fibrinogenezei. PC este o afecţiune heterogenă cu un spectru clinic care include: durere, afectarea funcţiilor endocrine şi exocrine pancreatice şi variate complicaţii ce implică organele adiacente pancreasului. Pentru a stabili diagnosticul de PC la adult sunt necesare dovezi histologice, morfologice sau o combinaţie de argumente clinice, funcţionale şi morfologice. Recent, Grupul Internaţional de Studiu pentru Pancreatită la Copil (International Study Group Of Pediatric Pancreatitis) a propus următoarele criterii de diagnostic pentru PC: a) dureri abdominale tipice plus aspecte imagistice caracteristice sau (b) insuficienţă pancreatică exocrină plus aspecte imagistice sau (c) insuficienţă pancreatică endocrină plus aspecte imagistice. Etiologia PC este diferită la pacienţii pediatrici comparativ cu pacienţii adulţi. În ultimiii ani s-au realizat progrese remarcabile în cunoaşterea pancreatitei ereditare şi pancreatitei autoimune. În etiologia pancreatitelor cronice diagnosticate la copil pot fi identificate cauze obstructive. Pancreatitele cronice obstructive sunt determinate de factori mecanici congenitali sau dobîndiţi. Abordarea terapeutică include măsuri conservatoare (analgetice, antiinflamatorii, substituţie enzimatică), intervenţii endoscopice, precum şi proceduri chirurgicale. Colangiopancreatografia retrogradă endoscopică este utilizată ca procedură terapeutică pentru boala biliopancreatică ce generează pancreatita recurentă sau cronică. La cazurile de PC la care modalităţile terapeutice menţionate se dovedesc ineficiente se poate recurge la pancreatectomie totală cu autotransplant portal de insule pancreatice.

Cuvinte cheie: pancreatită, celule stelate pancreatice

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