Klinika Gastroenterologii

Pomnik, Poland

Klinika Gastroenterologii

Pomnik, Poland
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Lipinski P.,Klinika Gastroenterologii | Jankowska I.,Klinika Gastroenterologii | Grenda R.,Klinika Nefrologii
Pediatria Polska | Year: 2016

Ciliopathies constitute a group of disorders characterized by cilia abnormalities and an extremely heterogeneous clinical presentation. The liver and kidneys are the most commonly affected organs and the term hepatorenal fibrocystic disorders is used to describe ciliopathies with combined liver and kidney involvement. Liver disorders in ciliopathies can be grouped into three categories: congenital hepatic fibrosis, Caroli's disease and polycystic liver disease. Kidney disorders related to primary cilia abnormalities include autosomal dominant and recessive polycystic kidney diseases and nephronophthisis. © 2016 Polish Pediatric Society.


Lipinski P.,Klinika Gastroenterologii | Wiecek S.,Slaski University Medyczny | Jankowska I.,Klinika Gastroenterologii
Pediatria Polska | Year: 2017

Authors present the prevailing knowledge about bilirubin metabolism and its implications in Rotor and Dubin-Johnson syndromes. © 2017 Polish Pediatric Society.


Orlowska E.,Klinika Gastroenterologii | Czubkowski P.,Klinika Gastroenterologii | Socha P.,Klinika Gastroenterologii
Wiadomosci lekarskie (Warsaw, Poland : 1960) | Year: 2017

Biliary atresia is a chronic cholangiopathy leading to progressive fibrosis of both intra- and extrahepatic bile ducts. The cause of the condition is unknown. Fundamental management of biliary atresia is surgical intervention and the outcomes of the treatment depend on the child's age with best results when performed within the first 2 months of life. Thus, the main role of pediatric healthcare is an urgent differential diagnosis and prompt qualification for the surgery, optimal postoperative management and early qualification for the liver transplantation in patients with persistent cholestasis. The authors discuss the clinical presentation, diagnosis and management of biliary atresia.


Lipinski P.,Klinika Gastroenterologii | Pawlowska J.,Klinika Gastroenterologii | Jankowska I.,Klinika Gastroenterologii
Pediatria Polska | Year: 2017

The article constitutes an up-to-day overview on pathogenesis, clinical manifestation and treatment of benign recurrent intrahepatic cholestasis in children. © 2017 Polish Pediatric Society.


Lipinski P.,Klinika Gastroenterologii | Socha P.,Klinika Gastroenterologii | Jankowska I.,Klinika Gastroenterologii
Pediatria Polska | Year: 2017

Cholestatic liver diseases remain a major clinical issue for pediatricians. The article constitutes an up-to-day overview on the inborn errors of bile acids synthesis. © 2017 Polish Pediatric Society.


Lipinski P.,Klinika Gastroenterologii | Jankowska I.,Klinika Gastroenterologii
Pediatria Polska | Year: 2017

Cholestatic liver diseases remain a major clinical issue for pediatricians. The article constitutes an up-to-date overview on cholestasis with normal serum gamma-glutamyl transpeptidase activity. The authors presented the pathogenicity, clinical presentation, biochemical and molecular diagnostics of the following disorders: citrin deficiency, progressive familial intrahepatic cholestasis type 4, cholestasis associated with FXR mutations, familial hypercholanemia, cholestasis associated with MYO5B mutations (also cholestatic liver disorder in microvillus inclusion disease), ARC syndrome, giant cell hepatitis with autoimmune hemolytic anemia and cortisol deficiency. © 2017.


Lipinski P.,Klinika Gastroenterologii | Jankowska I.,Klinika Gastroenterologii
Pediatria Polska | Year: 2017

Cholestatic liver diseases remain a major clinical issue for pediatricians. The article constitutes an up-to-day overview on cholestasis with elevated serum gamma-glutamyl transpeptidase activity. Authors presented the pathogenicity, clinical presentation, biochemical and molecular diagnostics of the following disorders: progressive familial intrahepatic cholestasis type 3, adenosine kinase deficiency, neonatal ichthyosis - sclerosing cholangitis syndrome. © 2017 Polish Pediatric Society.


Daniluk U.,Klinika Pediatrii | Kierkus J.,Klinika Gastroenterologii | Lebensztejn D.,Klinika Pediatrii
Pediatria Polska | Year: 2017

It is known that certain patients with very early onset IBD (diagnosis before 6 years of age) have a higher possibility of immune deficiency with monogenetic aetiology. The growing prevalence of these types of disorders forces the gastroenterologist to gain some knowledge about the symptoms and further diagnostic management, which includes consultation with the clinical immunologist. The resemblance of the clinical manifestation of early-onset Crohn's disease with ulcerative colitis and high incidence rate of unclassified IBD suggests caution in IBD diagnosis in younger patients. © 2017.


Socha J.,Klinika Gastroenterologii | Socha P.,Klinika Gastroenterologii | Weker H.,Instytut Matki i Dziecka w Warszawie | Neuhoff-Murawska J.,Klinika Gastroenterologii
Pediatria Wspolczesna | Year: 2010

We present the influence of nutrition on health in concern of changing knowledge during last centuries and with future perspectives. © 2010 Almamedia Press.


Inflammatory bowel diseases (IBD) affect mainly the young population and therefore fertility and pregnancy-related issues are important clinical considerations. Generally, men and women with IBD do not have decreased fertility compared to the general population. Drugs used for IBD do not affect significantly fertility in humans, except sulfasalazine, which causes a temporary reduction in spermatogenesis, but does not reduce fertility itself. The disease course during pregnancy and the risk of pregnancy-related complications depend mainly on the disease activity at the time of conception, therefore, pregnancy should be planned during a phase of remission. Except for methotrexate, mycophenolate mofetil and thalidomide, which are strongly contraindicated, drugs used for IBD appear safe in pregnancy, if they are administered carefully. The highest degree of safety was proved for 5-ASA- -containing agents, thiopurines and corticosteroids. The use of TNFα agents remains disputable, especially in the third trimester of pregnancy, due to their high concentration in the infant`s blood and the lack of data concerning its long-term safety. Surgery, if necessary, should be delayed if possible, although pregnancy is not a contraindication for surgical procedures. The management of IBD in reproductive age and pregnant women remains still controversial, because literature data comes mostly from retrospective studies. The aim of this paper was to summarize and to present proper management of patients with IBD prior to conception, as well as pregnant women and breast-feeding mothers with IBD, based on current European Crohn's and Colitis Organisation (ECCO) guidelines and available literature.

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