PubMed Health⌕ Search

Biomedical subjects

Anna Liberek

Publications and source records attributed to Anna Liberek.

At least 19 recordsLinked to original sources
PubMed2006

Lamivudine therapy for children with chronic hepatitis B.

AIM: To assess the effectiveness and side-effects of lamivudine therapy for children with chronic hepatitis B (CHB) who fail to respond to or have contraindications to interferon-alpha (IFN-alpha) therapy. METHODS: Fifty-nine children with CHB were treated with 100 mg lamivudine tablets given orally once daily for 12 mo. Alanine aminotransferase (ALT) activity was evaluated monthly during the therapy and every 3 mo after its discontinuation. HBe antigen, anti-HBe antibodies, HBV DNA level in serum were evaluated at baseline and every six months during and after the lamivudine therapy. Sustained viral response (SVR) to lamivudine therapy was defined as permanent (not shorter than 6 mo after the end of the therapy), namely ALT activity normalization, seroconversion of HBeAg to anti-HBe antibodies, and undetectable viral HBV-DNA in serum (lower than 200 copies per mL). The analysis of the side-effects of the lamivudine treatment was based upon interviews with the patients and their parents using a questionnaire concerning subjective and objective symptoms, clinical examinations, and laboratory tests performed during clinical visits monthly during the therapy, and every 3 mo after the therapy. RESULTS: ALT normalisation occurred in 47 (79.7%) patients between the first and 11(th) mo of treatment (mean 4.4+/-2.95 mo, median 4.0 mo), and in 18 (30.5%) of them after 2 mo of the therapy. There was no correlation between the time of ALT normalization and the children's age, the age of HBV infection, the duration of HBV infection, inflammation activity score (grading), staging, ALT activity before treatment, serum HBV DNA level, and lamivudine dose per kg of body weight. HBeAg/anti HBe seroconversion was achieved in 27.1% of cases. The higher rate of seroconversion was connected with lower serum HBV DNA level and longer duration of HBV infection. There was no connection between HBeAg/anti HBeAb seroconversion and the children's age, age of HBV infection, grading, staging, ALT activity before treatment, and lamivudnie dose per kg of body weight. No complaints or clinical symptoms were observed during lamivudine therapy. Impairment of renal function or myelotoxic effect was noted in none of the patients. CONCLUSION: One year lamivudine therapy for children with chronic hepatitis B is effective and well tolerated. Seroconversion of HBeAg/HBeAb and SVR are connected with lower pre-treatment serum HBV DNA level.

Adolescent↗
PubMed2006

The "sugar" clear cell tumor of the lung-clinical presentation and diagnostic difficulties of an unusual lung tumor in youth.

A case of the "sugar" clear cell tumor of the lung in a 16-year-old boy is presented. The course of the disease with general symptoms, never reported before, highlights diagnostic difficulties of an extremely rare lung tumor in youth. The boy presented with daily spikes of unexplained high fever of 6 weeks' duration with features of hypochromic microcytic anemia, elevated erythrocyte sedimentation rate, C-reactive protein, alpha(2)- and beta-globulins, and elevated platelet count. The lung tumor was a yellow, circumscribed mass confined to the sixth segment of the left lung. Histological examination revealed the tumor composed of cells with clear cytoplasm with large content of glycogen, with no signs of necrosis, and immunoreactive for HMB-45, but not for cytokeratin, LCA, CD34, and CD68. The performed thoracotomy and segmentectomy were both diagnostic and curative.

Adolescent↗
PubMed2005

Changes in hair morphology of mucopolysaccharidosis I patients treated with recombinant human alpha-L-iduronidase (laronidase, Aldurazyme).

Mucopolysaccharidoses (MPS) are heritable, metabolic diseases caused by accumulation of mucopolysaccharides (glycosaminoglycans, GAGs) in lysosomes. This accumulation is due to a deficiency in one of several specific enzymes involved in the degradation of GAGs. MPS type I (MPS I) is caused by low or undetectable activity of alpha-L-iduronidase, an enzyme involved in removing the terminal iduronic acid residues from heparan and dermatan sulfate. Recently, an enzyme replacement therapy (ERT) for MPS I, based on administration of recombinant human alpha-L-iduronidase (laronidase, Aldurazyme), became available. The assessment of efficacy of ERT is especially important because MPS I is a highly variable and very rare disease, and the clinical trials involved relatively low number of patients. Among various significant clinical improvements during ERT, remarkable changes in hair morphology were noted. Detailed studies of hair samples from one patient, who did not have a hair cut from the beginning of ERT to the end of this study, and supported by results obtained for two other patients, revealed hair shaft structural abnormalities in MPS I hair. These hair abnormalities disappeared upon treatment with Aldurazyme. Although hair morphology is of limited clinical importance, the data suggest that changes in this parameter could be a useful, additional tool for a rapid, non-invasive, preliminary assessment of ERT efficacy.

Adolescent↗
PubMed2005

Atypical microbial infections of digestive tract may contribute to diarrhea in mucopolysaccharidosis patients: a MPS I case study.

BACKGROUND: Mucopolysaccharidoses are heritable, metabolic diseases caused by deficiency in an activity of one of specific lysosomal enzymes involved in degradation of mucoplysaccharides (glycosaminoglycans). Among many medical problems of patients with mucopolysaccharidoses, there are frequent episodes of diarrhea of unknown etiology. CASE PRESENTATION: A girl, diagnosed enzymatically for mucopolysaccharidosis type I (deficiency of alpha-L-iduronidase) at the age of 3 years and 9 months, was investigated until the age of 5 years and 4 months. Frequent loose stools and episodes of diarrhea, often accompanied by vomiting, were encountered. Detailed microbiological analyses were performed and atypical microbial infections (most often enetropathogenic Escherichia coli, but also other species, like Pseudomonas aeruginosa or Staphylococcus aureus, as well as adenoviruses) of the digestive tract were found in most severe diarrhea episodes. Often, isolations of pathogenic bacterial strains from stools of the investigated patient suffering from diarrhea were not obvious during the first screening, and only detailed microbiological studies, including re-isolation of colonies, gave the results of isolation of particular pathogenic strains (especially in the case of enetropathogenic E. coli). CONCLUSION: We conclude that atypical microbial infections of digestive tract may contribute significantly to diarrhea in mucopolysaccaridosis patients. Since isolated strains were not typical and their isolation was often possible only after detailed investigation (not during a standard screening), such atypical microbial infections of digestive tract of mucopolysaccharidosis patients could be usually overlooked to date. Importantly, these atypical infections could be effectively treated with antimicrobial agents.

Adenoviridae Infections↗
PubMed2002

TGF-beta (transforming growth factor-beta) in chronic inflammatory conditions - a new diagnostic and prognostic marker?

TGF-beta is a cytokine with varied properties and pleiotropic activity. It is released in an inactive form. To exhibit its biological activity, it requires binding to extracellular matrix proteins and, after that, proteolytic elimination of LAP (Latent Associated Protein) and LTBP (Latent TGF-beta Binding Protein). The process involves, among others, tissue transglutaminase, thrombin and plasmin. By stimulation of specific receptors, it influences transcription of some genes and translation of formed mRNA. Locally, it demonstrates proinflammatory properties whereas systemically, it has primarily a potent immunosuppressive effect. TGF-beta, by affecting proliferation, differentiation and migration of cells, as well as stimulation of extracellular matrix protein production, plays an important role in tissue regeneration and remodeling, but also in fibrosis. TGF-beta is also indispensable to maintain immune homeostasis of the organism. Reduced TGF-beta activity is considered to be responsible for development of autoimmune disorders in the course of several pathologic conditions. This cytokine plays an important role in the pathogenesis of chronic inflammatory processes taking place, among others, in inflammatory bowel diseases (IBD) and chronic hepatitis B and C. The paper presents a review of literature concerning diagnostic and prognostic value of TGF-beta level determinations in blood and tissue bioptates of patients with chronic non-specific enteritis and chronic hepatitis B and C.

Adult↗
PubMed2002

Cytomegalovirus disease in neonates and infants--clinical presentation, diagnostic and therapeutic problems--own experience.

BACKGROUND: The aim of the study was to present diagnostic problems, different clinical presentations and results of treatment of cytomegalovirus infections in neonates and infants. MATERIAL/METHODS: The study was carried out in a group of 31 children from 10 days to 12 months of age (17 boys, 14 girls). The diagnosis was based on serological investigations (presence of specific IgM antibodies and/or increasing titer of IgG antibodies), presence of intermediate or early CMV antigen in peripheral blood leukocytes or positive blood or urine PCR results. The treatment of 25 cases involved intravenous administration of gancyclovir at 5-7 mg/kg daily doses for 14-21 days. In 1 case, hyperimmunized anti-CMV serum - Cytotect was used. RESULTS: The most common clinical symptoms were jaundice, hepato- and splenomegaly. Clinical investigations demonstrated increased aminotransferese activity and the signs of cholestasis. Other frequent findings included anemia, leukocytosis with atypical lymphocyte forms present, as well as thrombocytopenia. In majority treated patients, rapid regression of the clinical symptoms and normalization of transaminases activity was observed. Good outcome of the therapy was confirmed by immunological investigations. The effects of the terapy were similar irrespective of the dose and duration of gancyclovir treatment. The tolerance of the drug was good - no indications for discontinuation of the treatment were observed in any of the patients. CONCLUSIONS: The clinical presentations of cytomegalovirus disease in infants are varied, the diagnosis of the disease should be considered in children with persistent jaundice, especially if it is accompanied by hepatomegaly and increased aminotransferase activity or signs of cholestasis. Despite relatively good tolerance of the drug by children, the patients should be monitored for possible side effects, especially myelo- and nephrotoxic.

Antibodies, Viral↗
PubMedDate not supplied

Serum interleukin 6 and interleukin 12 levels in children with chronic hepatitis HBV treated with interferon alpha.

INTRODUCTION: The course of HBV infection and the outcome of interferon alpha (IFNalpha) therapy of patients with chronic hepatitis B, is determined by the antiviral immune response of the host. The aim of the study was to investigate 1) the correlation between IL-6 and IL-12 serum levels and biochemical and histopathological changes in children with chronic hepatitis B, 2) predictive value of pre-treatment serum levels of these cytokines in patients treated with interferon alpha and 3) changes in serum levels of these cytokines after interferon alpha treatment. METHODS: Serum levels of IL-6, IL-12 (heterodimer p70) and IL-12 (heterodimer p70 & p40 subunit) were determined by specific ELISA in 39 children with chronic hepatitis B on the first and the last day of IFNalpha therapy. RESULTS: Serum levels of IL-6, IL-12 (p70) and IL-12 (p70&p40) were respectively within the following ranges of values: 0-1.7 pg/mL, 3.0-85.1pg/mL, 93.7-442.7 pg/mL and they showed no correlation with biochemical and histopathological changes. The pre-treatment cytokines levels in patients who responded and those who did not respond to IFNa therapy did not differ statistically. There was no statistical difference between the end and pre-treatment cytokines levels in both groups. CONCLUSIONS: Serum levels of IL-6 and IL-12 do not reflect the inflammatory activity of hepatitis and have no predictive value of positive response to the IFNalpha therapy in children with chronic hepatitis B. Serum IL-6 and IL-12 levels at the end of INFalpha treatment do not inform of their role in immunological changes which take place while inhibition of HBV replication or virus clearance.

Adolescent↗
PubMedDate not supplied

[Therapeutic difficulties in a patient with ulcerative colitis and concomitant HBV and HCV infection].

The aim of the paper was to describe therapeutic difficulties in a patient with ulcerative colitis. In the long-term clinical course of the disease a concomitant infection with hepatotropic viruses (HBV, HCV) was diagnosed. Due to the different treatment of autoimmunological diseases and chronic viral hepatitis the necessity of further search of therapeutic schedules for this group of patients is still needed.

Adult↗
PubMedDate not supplied

[Autoimmunity in children with chronic hepatitis C treated with interferon alpha and ribavirin].

The role of interferon alpha or the virus itself in the pathogenesis and the risk of autoimmunological disorders in patients infected with HCV, still remain unknown, especially in children. The aim of the study was to evaluate the incidence of autoantibodies and the risk of autoimmunological disorders in children with chronic hepatitis C, treated with interferon alpha and ribavirin in the Department of Paediatrics, Paediatric Gastroenterology and Oncology in Gdansk. In the studied group of 12 patients, in 4 cases autoantibodies were present in low titers prior to the treatment and they had no prognostic value for the response to the therapy or the risk of autoimmunological disorders. Positive response for the treatment was achieved in 4 cases; in 3 cases indications for discontinuation of the therapy were established. During the therapy with interferon alpha and ribavirin, in 2 children elevation of serum titers of antibodies to liver-kidney microsome type 1 (anti-LKM1) (> 1:640) with normal gammaglobulin levels was noted. In none of the children autoimmunological disorders were observed.

Adolescent↗
PubMedDate not supplied

[Free radical reactions and activity of antioxidant barrier in children with chronic hepatitis B].

UNLABELLED: Recent research has shown that highly reactive oxygen species may take part in pathogenesis of many diseases, in particular chronic inflammatory processes. It was demonstrated that the activation of the inflammatory process is linked with generation of significant quantities of free radicals. This may lead to uncontrolled free radical reactions, particularly in case of antioxidant barrier insufficiency. THE AIM OF THE STUDY: To assess the biochemical parameters of oxidative stress, activation of glutathione peroxidase (GPX) and superoxide dismutase (SOD1) in erythrocytes of children with chronic hepatitis B (CBH). MATERIALS AND METHODS: Group of 47 children aged from 7 to 18 years with histopathologically confirmed CBH was studied. The content of carbonyl groups in plasma proteins and activity of SOD1 and GPX in the erythrocytes was evaluated. The control group consisted of 61 healthy children aged from 7 to 18 years. RESULTS: Verified statistically with Mann-Whitney U test. Significantly higher content of plasma proteins' carbonyl groups was found in the study group (1.07 +/- 0.33 nmol/mg protein) than in the control group (0.86 +/- 0.2, p < 0,001). The activity of GPX [U/gHb] was lower in group of children with CBH (18.7 +/- 9.7) than in healthy children (26.1 +/- 0.2, p =0.005). A significantly higher activity or SOD1 [U/gHb] was found in children with CBH (2470+/- 714) as compared with healthy controls (1857+/- 782, p =0.006). CONCLUSIONS: In patients with CBH the intensification of free radicals' reactions can be observed, which confirms the role of ROS in its pathogenesis. One of the reasons for this phenomenon may be due to the inappropriate activity of antioxidant barrier enzymes. The elevation of SOD activity seems to be a response to increased oxidative stress.

Adolescent↗
PubMedDate not supplied

[Molecular analysis of uropathogenic strains of Escherichia coli and the clinical course of urinary tract infection in children].

UNLABELLED: The most frequent etiological factor of urinary tract infections are virulent Escherichia coli strains. Identification of uropathogenic Escherichia coli strains is possible using biomolecular techniques. THE PURPOSE OF THIS STUDY was to determine the prevalence of Escherichia coli strains encoding papG adhesins and adhesins of Dr family in children with urinary tract infections and to establish the relationship between the bacterial genotype and clinical course of a sickness. MATERIAL AND METHODS: In 163 children with urinary tract infection caused by Escherichia coli strains, after taking history and physical examination, inflammatory parameters were determined. In all patients abdominal ultrasonography and in chosen patients -- miction cystourethrography and urodynamic examinations were performed. In order to identify Escherichia coli strains possessing pap gene cluster class I, II and III (encoding P fimbriae) and genes encoding Dr family of adhesins, the Polymerase Chain Reaction was used. RESULTS AND CONCLUSIONS: From all isolated Escherichia coli strains, pap gene cluster was identified in 41.1% and dra gene cluster in 42.3%. Escherichia coli strains encoding papG gene class II frequently caused upper urinary tract injections, while strains encoding papG gene class III and genes encoding adhesins of Dr family were mostly responsible for lower urinary tract infections and their recurrences. Occurrence of virulent Escherichia coli strains was not more common in children with pyelonephritis and with disorders of urinary tract in comparison with the group of children without urinary tract anomalies. Determination of bacterial virulence factors causing urinary tract infections in children may be helpful in the prognosis of the course of the disease.

Adhesins, Escherichia coli↗
PubMedDate not supplied

[Congenital extrahepatic biliary atresia as a cause of cholestasis in newborns and infants].

UNLABELLED: Congenital extrahepatic biliary atresia (CEBA) is one of the most common causes of cholestasis in newborns and infants. THE AIM OF THE STUDY: was the analysis of clinical presentation, results of laboratory and imaging investigations as well as clinical outcome of children with extrahepatic cholestasis caused by CEBA. MATERIALS AND METHODS: the analyzed group included 15 children aged from 2 weeks to 4 months. Data concerning: pregnancy and delivery, newborn's health condition, faeces color; jaundice onset, manifestation of coagulation disorders coexisting malformations and disorders of other systems were obtained. The following investigations were performed: biochemical tests evaluating the function of the liver and cholestasis (serum bilirubin concentration and fractions, bile acids in serum, AlAT, AspAT, GGTP, FALK activities, serum electrophoresis, prothrombin index). We also performed tests focusing on hepatotropic infections, - metabolic disorders tests and in all children - ultrasound of the abdomen, scintigraphy of the bile ducts - HEPIDA. 14 children had undergone hepatoportoenterostomy, modo Kasai. RESULTS: jaundice, acholic stools and hepatomegaly were present in all children. The serum concentration of bilirubin and its conjugated fraction and bile acids in all children were increased. GGTP and FALK activities were markedly elevated in all children. Aminotransferases activities elevations were observed, more distinctively for AST. Active cytomagalovirus infection was detected in 3 children. Abdominal ultrasound revealed gallbladder in 7 children, whereas intrahepatic bile ducts were described as normal in 12 cases. In all cases the HEPIDA scintigraphy showed no passage of the tracer to the GI tract even after 24 hours delay. Hepatoportoenterostomy was performed in 14 children, 5 of them had liver transplantation. CONCLUSIONS: 1. There is still not one effective and specific diagnostic method in differentiating between the causes of cholestasis in the newborns and infants. Thus many investigation methods should be run simultaneously. 2. Congenital atresia must be definitely excluded before cholestasis with other background is finally diagnosed. 3. The hepatoportoenterostomy should be considered as the first line treatment in children with CEBA. Most cases will need liver transplantation in the future.

Bile Acids and Salts↗
PubMedDate not supplied

[Focal nodular hyperplasia - own experiences].

INTRODUCTION: focal nodular hyperplasia (FNH) is a benign tumour of the liver, often discovered incidentally. It occurs mainly in women between 25-44 years old. Though it is rarefy reported in children the evidence has been growing in the recent years. THE AIM: of our work was to show our experience of focal nodular hyperplasia in pediatric cases. MATERIAL AND METHODS: 10 children, aged 6-17 years, were hospitalized in the Children's Memorial Health Institute between 2000 and 2005, with provisional diagnosis of focal nodular hyperplasia. In all children radiological examination was performed. In some cases laparotomy and operative liver biopsy was carried out. RESULTS: only in 3 children diagnosis of FNH was made from radiological examination. 6 children needed laparotomy and operative liver biopsy. Although operative resection of FNH lesions was carried out in some patients, the authors observed recurrence of hepatic lesions. CONCLUSIONS: our experiences confirm that nowadays the conservative approach should to be standard procedure in children with FNH. Only in doubtful cases laparotomy is recommended.

Adolescent↗
PubMedDate not supplied

[Mesenteric lymphadenopathy - a valid health problem in children].

THE AIM: of the study is to evaluate the reasons of mesenteric lymphadenopathy and its clinical picture in hospitalized children. MATERIAL AND METHODS: the study was performed on 127 children (49 girls and 78 boys age of 8 months to 18 years; mean age 9 years and 3 months) hospitalized in the Department of Paediatrics, and Paediatric Gastroenterology and Oncology, Medical University of Gdansk. Ultrasonography showed enlarged abdominal lymph nodes in all children. According to definition of mesenteric lymphadenopathy, the clinical course of disease was analyzed in children, in whom there were at least three lymph nodes in ultrasonography with the peroneal diameter of 5 mm or more. Inflammatory parameters were examined in all children. In selected cases culture, viral and parasitic, as well as endoscopic examination, were also performed. RESULTS: analyzing accompanying clinical symptoms, it was found, that abdominal pain was the most dominant complaint in children with mesenteric lymphadenopathy; it was observed in 63 children (49.6%). In 33 (26%) of them the pain was the sole complaint, while in the rest vomiting and fever were present. 8 children (6.3%) with generalized lymphadenopathy were diagnosed. Ultrasonographic evaluation demonstrated that numerous enlarged lymph nodes were present the most frequently, in 65 (51.2%), less numerous, in 42 (33.1%), while sparse lymph nodes were seen only in 20 (15.7%) children. In 85 patients (66.9%) long axis of the lymph nodes reached min. 10 mm, in 39 (30.1%) was smaller than 10 mm, in 3 (2.4%) exceed 20 mm. Conglomerates of lymph nodes were described in 9 (7.1%) patients with various diagnosis (acute diarrhea - 3 children, ulcerative colitis - 3 children, celiac disease, cytomegaly, lambliosis). Tendency to invagination was observed in 5 (3.9%) children. In 4 of them acute infection (acute diarrhea, pneumonia) with high inflammatory parameters was diagnosed. Elevated inflammatory parameters were present in 42 (33.1%) patients. Examining the reasons of the abdominal lymph nodes enlargement, it was found that primary mesenteric lymphadenopathy was the most frequent diagnosis; it was recognized in 27 (21.3%) children. In 20 (15.7%) lymphadenopathy was caused by acute diarrhea, in 19 (14.9%) patients - by respiratory tract infection. Cytomegaly was recognized in 4 (3.1%), toxoplasmosis in 3 (2.3%), lambliosis in 9 (7.0%) patients. Both gastritis and colitis were diagnosed in 12 (9.4%) children. In 7 (5.5%) patients generalized lymphadenopathy with unknown etiology was described. In single cases other diseases were diagnosed. CONCLUSIONS: the enlargement of mesenteric lymph nodes frequently causes abdominal pain in children, being an indication for laboratory investigations. Vomiting and fever are the most common other symptoms in these patients. Ultrasonographic examination usually shows large enlargement of lymph nodes, sometimes in conglomerates, with tendency to invagination. Acute diarrhea and respiratory tract infection are the most frequent reasons of the enlargement of abdominal lymph nodes. In about 20% of the children primary mesenteric lymphadenopathy is diagnosed.

Abdominal Pain↗
PubMedDate not supplied

[Etiology of community - acquired acute diarrhea in children].

THE AIM: of the study was to analyse the etiology of home acute diarrhea in children. MATERIAL AND METHODS: 2636 children with community-acquired acute diarrhea were included in the study. Stool samples were analysed for the presence of Salmonella spp, Shigella spp, rotaviruses and adenoviruses. RESULTS: etiology of acute diarrhea was established in 1149 out of 2636 children (43.6%). Among the children with known etiology - rotaviral diarrhea was most frequently diagnosed (24.67%), whereas salmonellosis - in 7.06% of cases, EPEC C - 6.37%, EPEC B - 2.92%, EPEC A - 1.89%, adenoviral diarrhea in 0.68%. Mean age of children with bacterial acute diarrhea (Salmonella, EPEC A, B, C) was higher as compared to the mean age of children with rotaviral acute diarrhea (p < 0.002). Rotaviral acute diarrhea was more frequently diagnosed in girls (p < 0.025), whereas bacterial - in boys (p < 0.044). The highest prevalence of acute rotaviral diarrhea was found in winter/spring, with peak in April. CONCLUSIONS: commonly used diagnostic procedure does not allow to establish the etiology of acute home diarrhea in children in most cases. Rotavirus is found in the majority of diagnosed cases. It seems reasonable to expand the spectrum of diagnostic tests in cases of acute diarrhea in children.

Acute Disease↗