scholarly journals Pediatric Liver Disease Patients and Secondary Glycosylation Abnormalities

2021 ◽  
Vol 8 ◽  
Author(s):  
Anna Bogdańska ◽  
Patryk Lipiński ◽  
Paulina Szymańska-Rożek ◽  
Irena Jankowska ◽  
Piotr Socha ◽  
...  
Keyword(s):  
Liver Disease ◽  
Pediatric Patients ◽  
Acute Liver Injury ◽  
Liver Function Tests ◽  
Congenital Disorders Of Glycosylation ◽  
Adult Liver ◽  
Specific Serum ◽  
Pediatric Liver Disease ◽  
Abnormal Glycosylation

Background: Isoelectric focusing (IEF) of serum transferrin (Tf) is still the method of choice for diagnosis of congenital disorders of glycosylation (CDG). An abnormal glycosylation is also a known phenomenon in adult liver disease patients. The aim of this study was to characterize glycosylation disturbances in pediatric patients with primary liver disease. However, there are no reports of this phenomenon in children.Materials and Methods: Between 1995 and 2019, circa 2,000 serum Tf isoform analyses have been performed in children with primary liver diseases; some of them underwent subsequent analyses. We enrolled in this study 19 patients who developed an acute liver injury (ALI)/failure (ALF) or exhibited a chronic liver disease (CLD) and were evaluated and listed for liver transplantation (LTx) or had just undergone this procedure, and secondary abnormal serum Tf isoform profile.Results: Among 12 patients with ALI/ALF, 10 had an increased percentage of asialo-, monosialo-, and disialo-Tf isoforms. All patients with CLD had an increased percentage of asialo- and monosialo-Tf isoform. Two patients diagnosed with recurrent ALF had very specific serum Tf profile with a huge increase in the asialo- and monosialo-Tf isoform. On follow-up analyses (available in some patients), serum Tf IEF profile normalized in parallel to normalization of liver function tests, spontaneously or during treatment, including glucocorticosteroids in AIH, LTx in CLD.Conclusions: All pediatric patients with primary liver disease had increased asialo-Tf as well as monosialo-Tf isoforms. None of them had elevated percentage of trisialo-Tf isoform.

scholarly journals Cystic fibrosis-related liver disease: a single-center experience

2011 ◽  
Vol 3 (3) ◽  
pp. 21 ◽  
Author(s):  
Paula Catarino Costa ◽  
Celeste Canha Barreto ◽  
Luisa Pereira ◽  
Maria Luisa Lobo ◽  
Maria Adília Costa ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Disease ◽  
Liver Function ◽  
Pediatric Patients ◽  
Liver Function Tests ◽  
Liver Involvement ◽  
Clinical Expression ◽  
Function Tests ◽  
Related Liver Disease

Prospective studies concerning liver disease in pediatric cystic fibrosis patients are scarce. The present study aimed to describe the prevalence and clinical expression of cystic fibrosis - related liver disease, in a cohort of 62 pediatric patients. Descriptive study, resulting from the prospective evaluation, between 1994 and 2009, of 62 pediatric patients (age <18 years) with cystic fibrosis. The follow-up protocol included a clinical assessment every 2 months, liver function tests every 6 months and annual liver ultrasonography. The cumulative prevalence of liver disease was 11.2% (7/62 cases). All patients had ΔF508 mutation and pancreatic insufficiency, none had meconium ileus. The liver involvement became clinically evident at a mean age of 8 years (3-15 years), revealed by hepatomegaly or hepatosplenomegaly (3 cases) and/ or abnormalities of liver function tests (3 cases) changes of liver ultrasound (7 cases) with evidence of portal hypertension (2 cases). Four patients were submitted to liver biopsy; biliary fibrosis was documented in one case, focal biliary cirrhosis in 2 cases and multilobular cirrhosis in another case. Within a median 11.6 years follow-up period (all patients under UDCA therapy after liver disease diagnosis), progression of liver disease was observed in 2 patients; one patient developed refractory variceal bleeding and progressive hepatic failure, requiring liver transplant. The results of the present study agree with those of previous pediatric studies, further documenting clinical expression of liver disease in CF patients, which is usually detected in the first decade of life and emphasize the contribution of ultrasound to early diagnosis of liver involvement. Moreover, although advanced liver disease is a relatively rare event, early isolated liver transplantation may have to be considered at this age group.

scholarly journals Nonprogressive course of non-A, non-B chronic hepatitis in multitransfused hemophiliacs

Blood ◽  
1982 ◽  
Vol 60 (3) ◽  
pp. 655-658
Author(s):  
PM Mannucci ◽  
M Colombo ◽  
M Rizzetto
Keyword(s):  
Chronic Hepatitis ◽  
Liver Disease ◽  
Chronic Liver Disease ◽  
Liver Function Tests ◽  
Chronic Liver ◽  
Viral Marker ◽  
Spontaneous Improvement ◽  
Liver Biopsies ◽  
Piecemeal Necrosis

Eleven hemophiliacs with chronic liver disease were studied prospectively for 6 yr, with liver function tests and liver biopsies carried out at intervals of 3 yr. The second series of biopsies, compared with the first series, showed continuation of chronic persistent hepatitis in four patients, change to chronic lobular hepatitis in two, and spontaneous improvement of the disease in the four cases who had had chronic active hepatitis characterized by moderate piecemeal necrosis. One patient with active cirrhosis died of liver failure during the follow-up period. Study of the serum and intrahepatic markers for hepatitis B and delta viruses suggests that chronic liver disease is nonprogressive in hemophiliacs who have no intrahepatic viral marker.

scholarly journals Changes of liver transaminases levels during one year follow up of Deferasirox treatment in children with β-thalassemia major

2020 ◽  
Vol 19 (3) ◽  
pp. 453-457
Author(s):  
Nashwan M Al Hafidh ◽  
Mozahim S Younis
Keyword(s):  
Relative Risk ◽  
Pediatric Patients ◽  
Medical Science ◽  
Liver Function Tests ◽  
Thalassemia Major ◽  
Abnormal Liver Function ◽  
Weekly Interval ◽  
Liver Transaminases ◽  
One Year

Objectives: Abnormal liver function tests lead to interruptions of Deferasirox therapy. The aim of this study is to determine the changes in liver transaminases levels in pediatric patients with β -thalassemia major during one year follow up of Deferasirox treatment. Material and methods: This study was conducted at Ibn Al Atheer center of thalassemia, Mosul city, Iraq during the period from 3rd of February 2013 till 2nd of February 2014. Seventy one pediatric patients with β -thalassemia major were included in the study. Alanine aminotransferase (ALT) and aspartate aminotransferase (AST) were measured every 4 weeks after starting Deferasirox therapy dose of 30 mg /kg/day for one year. Results: In comparison to mean baseline ALT values, there were significant elevations of mean ALT values in each of the subsequent 4-weekly interval readingsafter Deferasirox therapy. There was nearly eleven times relative risk of having ALT ≥ 5 upper normal level (UNL) in patient with abnormal baseline ALT (Odd ratio 10.96,95% Confidence Interval: lower 2.05, upper 58.58). During a year of study, Deferasirox therapy was associated withALT readings of ≥ 5UNL in 22(31%) of pediatric β-thalassemia patients and that elevation lasted for 4 weeks in 95.5% of patients. Conclusions: Elevated ALT of ≥ 5UNL after Deferasirox therapy was short- lived, and lasted for 4 weeks in 95.5% of patients. It is advisable to start Deferasirox therapy at a dose of 30 mg /kg / day when baseline ALT level is normal. Bangladesh Journal of Medical Science Vol.19(3) 2020 p.453-457

scholarly journals Orthotopic Liver Transplantation in Children: Two-Year Experience with 47 Patients

PEDIATRICS ◽  
1984 ◽  
Vol 74 (1) ◽  
pp. 140-145
Author(s):  
J. Carlton Gartner ◽  
Basil J. Zitelli ◽  
J. Jeffrey Malatack ◽  
Byers W. Shaw ◽  
Shunzaburo Iwatsuki ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Biliary Atresia ◽  
Orthotopic Liver Transplantation ◽  
Surgical Complications ◽  
Pediatric Patients ◽  
Pediatric Liver Disease ◽  
Life Threatening ◽  
Catch Up ◽  
End Stage

During a 24-month period (May 1981 to May 1983), 47 pediatric patients (ranging in age from 7 months to 18 years) underwent orthotopic liver transplantation using cyclosporine and prednisone. Major indications were biliary atresia/hypoplasia, and metabolic liver disease. Thirty-two of 138 patients evaluated for the procedure died prior to transplantation. Thirty patients are alive from 6 to 29 months later including 7/15 patients who required retransplantation. Twenty-one of 32 patients are alive at 1 year following initial transplantation. All 30 survivors are clinically well and living at home; only one has an abnormal bilirubin level. Serious, life threatening medical and surgical complications were common during the early months following transplantation. With one exception, deaths and major rejection episodes occurred early (before 120 days). All survivors are relieved of the stigmata of chronic liver disease, and many have demonstrated catch-up growth. Liver transplantation is an effective treatment for end-stage pediatric liver disease.

scholarly journals Nonprogressive course of non-A, non-B chronic hepatitis in multitransfused hemophiliacs

Blood ◽  
1982 ◽  
Vol 60 (3) ◽  
pp. 655-658 ◽  
Author(s):  
PM Mannucci ◽  
M Colombo ◽  
M Rizzetto
Keyword(s):  
Chronic Hepatitis ◽  
Liver Disease ◽  
Chronic Liver Disease ◽  
Liver Function Tests ◽  
Chronic Liver ◽  
Viral Marker ◽  
Spontaneous Improvement ◽  
Liver Biopsies ◽  
Piecemeal Necrosis

Abstract Eleven hemophiliacs with chronic liver disease were studied prospectively for 6 yr, with liver function tests and liver biopsies carried out at intervals of 3 yr. The second series of biopsies, compared with the first series, showed continuation of chronic persistent hepatitis in four patients, change to chronic lobular hepatitis in two, and spontaneous improvement of the disease in the four cases who had had chronic active hepatitis characterized by moderate piecemeal necrosis. One patient with active cirrhosis died of liver failure during the follow-up period. Study of the serum and intrahepatic markers for hepatitis B and delta viruses suggests that chronic liver disease is nonprogressive in hemophiliacs who have no intrahepatic viral marker.

scholarly journals Screening for abnormal glycosylation in a cohort of adult liver disease patients

2020 ◽  
Vol 43 (6) ◽  
pp. 1310-1320
Author(s):  
Jos C. Jansen ◽  
Bart Hoek ◽  
Herold J. Metselaar ◽  
Aad P. Berg ◽  
Fokje Zijlstra ◽  
...  
Keyword(s):  
Liver Disease ◽  
Adult Liver ◽  
Abnormal Glycosylation

scholarly journals Prevalence and Natural History of Hepatitis C Infection in Patients Cured of Childhood Leukemia

Blood ◽  
1997 ◽  
Vol 90 (11) ◽  
pp. 4628-4633 ◽  
Author(s):  
Anna Locasciulli ◽  
Marina Testa ◽  
Patrizia Pontisso ◽  
Luisa Benvegnù ◽  
Donatella Fraschini ◽  
...  
Keyword(s):  
Liver Disease ◽  
Hepatitis C ◽  
Natural History ◽  
Childhood Leukemia ◽  
Liver Function Tests ◽  
Hcv Rna ◽  
Hepatitis C Infection ◽  
History Of ◽  
Long Term Survivors

Abstract The aim of this study was to ascertain prevalence and natural history of hepatitis C virus (HCV) infection in a large cohort of patients cured of childhood leukemia who had been followed prospectively for liver disease for at least 10 years since chemotherapy withdrawal: 114 consecutive patients entered the study. Liver function tests and ultrasonography were used to assess presence of liver disease. Patients were tested for antibody to HCV and for serum HCV-RNA at the end of chemotherapy and at the end of follow-up. At chemotherapy withdrawal, 56 patients (49%) were HCV-RNA positive, often without detectable anti-HCV, and in these cases, transaminase levels were more elevated during (P = .08) and after (P = .04) chemotherapy compared with HCV-RNA negative cases. Patients were then followed-up 13 to 27 years (mean, 17) after chemotherapy withdrawal. During this period, 38 initially anti-HCV negative patients seroconverted to anti-HCV and 17 initially anti-HCV positive cases lost reactivity. Forty patients were persistently HCV-RNA positive in serum, while 16 initially viremic patients became HCV-RNA negative during follow-up. At the end of the observation period, a persistent transaminase elevation was detected only in four HCV-RNA positive and anti-HCV positive cases, while no patient developed signs or symptoms of decompensated liver disease. Thus, hepatitis C was a frequent finding in long-term survivors after chemotherapy. It was associated with an atypical serologic profile and did not cause severe liver impairment over a period of 13 to 27 years.

Bleeding and Clotting Disorders in Pediatric Liver Disease

Hematology ◽  
2011 ◽  
Vol 2011 (1) ◽  
pp. 170-177 ◽  
Author(s):  
Brian M. Wicklund
Keyword(s):  
Liver Disease ◽  
Prothrombin Time ◽  
Partial Thromboplastin Time ◽  
Thrombin Generation ◽  
Pediatric Patients ◽  
Thromboembolic Disease ◽  
Activated Partial Thromboplastin Time ◽  
Bleeding Tendency ◽  
Clotting Factors ◽  
Pediatric Liver Disease

Abstract The coagulopathy of liver disease in pediatric patients presents an unusual set of challenges. Little pediatric data have been published, so this review is based largely on adult studies. There is a precarious balance between deficiencies of clotting factors and anticoagulation factors in liver disease that result in abnormal prothrombin time (PT) and activated partial thromboplastin time (aPTT) tests that would suggest a bleeding tendency, yet the patients can form a clot and are at risk of thromboembolic disease. Attention has centered on thromboelastography and thrombin-generation assays to clarify the patient's ability to control bleeding, but these tests are not routinely available to many treating physicians.

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