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107 records – page 1 of 11.

[A case of cirrhosis of the liver in an infant]

https://arctichealth.org/en/permalink/ahliterature61264
Source
Pediatr Akus Ginekol. 1966 May-Jun;3:31
Publication Type
Article
Author
S M Gol'dis
Source
Pediatr Akus Ginekol. 1966 May-Jun;3:31
Language
Ukrainian
Publication Type
Article
Keywords
Female
Humans
Infant, Newborn
Liver Cirrhosis - pathology - surgery
PubMed ID
5990364 View in PubMed
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[A case of rupture of the liver in a newborn]

https://arctichealth.org/en/permalink/ahliterature61205
Source
Pediatr Akus Ginekol. 1968;3:26
Publication Type
Article
Date
1968
Author
R I Magolina
V P Brei
Source
Pediatr Akus Ginekol. 1968;3:26
Date
1968
Language
Ukrainian
Publication Type
Article
Keywords
Humans
Infant, Newborn
Infant, Newborn, Diseases
Liver - injuries - surgery
Male
Rupture
PubMed ID
5717822 View in PubMed
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Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group.

https://arctichealth.org/en/permalink/ahliterature169060
Source
J Pediatr. 2006 May;148(5):652-658
Publication Type
Article
Date
May-2006
Author
Robert H Squires
Benjamin L Shneider
John Bucuvalas
Estella Alonso
Ronald J Sokol
Michael R Narkewicz
Anil Dhawan
Philip Rosenthal
Norberto Rodriguez-Baez
Karen F Murray
Simon Horslen
Martin G Martin
M James Lopez
Humberto Soriano
Brendan M McGuire
Maureen M Jonas
Nada Yazigi
Ross W Shepherd
Kathleen Schwarz
Steven Lobritto
Daniel W Thomas
Joel E Lavine
Saul Karpen
Vicky Ng
Deirdre Kelly
Nancy Simonds
Linda S Hynan
Author Affiliation
University of Pittsburgh, Children's Hospital of Pittsburgh, PA 15213, USA. Robert.squires@chp.edu
Source
J Pediatr. 2006 May;148(5):652-658
Date
May-2006
Language
English
Publication Type
Article
Keywords
Adolescent
Canada - epidemiology
Child, Preschool
Cohort Studies
Databases, Factual
Female
Great Britain
Health status
Humans
Infant
Infant, Newborn
Liver Failure, Acute - diagnosis - epidemiology - therapy
Liver Transplantation
Male
Needs Assessment
Predictive value of tests
Prognosis
United States - epidemiology
Abstract
To determine short-term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to determine prognostic factors.
A prospective, multicenter case study collecting demographic, clinical, laboratory, and short-term outcome data on children from birth to 18 years with ALF. Patients without encephalopathy were included if the prothrombin time and international normalized ratio remained > or = 20 seconds and/or >2, respectively, despite vitamin K. Primary outcome measures 3 weeks after study entry were death, death after transplantation, alive with native liver, and alive with transplanted organ.
The cause of ALF in 348 children included acute acetaminophen toxicity (14%), metabolic disease (10%), autoimmune liver disease (6%), non-acetaminophen drug-related hepatotoxicity (5%), infections (6%), other diagnosed conditions (10%); 49% were indeterminate. Outcome varied between patient sub-groups; 20% with non-acetaminophen ALF died or underwent liver transplantation and never had clinical encephalopathy.
Causes of ALF in children differ from in adults. Clinical encephalopathy may not be present in children. The high percentage of indeterminate cases provides an opportunity for investigation.
Notes
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PubMed ID
16737880 View in PubMed
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Adverse outcomes of pregnancy in women with non-alcoholic fatty liver disease.

https://arctichealth.org/en/permalink/ahliterature277352
Source
Liver Int. 2016 Feb;36(2):268-74
Publication Type
Article
Date
Feb-2016
Author
Hannes Hagström
Jonas Höijer
Jonas F Ludvigsson
Matteo Bottai
Anders Ekbom
Rolf Hultcrantz
Olof Stephansson
Knut Stokkeland
Source
Liver Int. 2016 Feb;36(2):268-74
Date
Feb-2016
Language
English
Publication Type
Article
Keywords
Adult
Cesarean Section - statistics & numerical data
Cohort Studies
Diabetes, Gestational - epidemiology - etiology
Female
Humans
Infant, Low Birth Weight
Infant, Newborn
Non-alcoholic Fatty Liver Disease - complications - epidemiology
Pre-Eclampsia - epidemiology - etiology
Pregnancy
Pregnancy Complications - epidemiology
Pregnancy Outcome - epidemiology
Premature Birth - epidemiology - etiology
Sweden - epidemiology
Abstract
Non-alcoholic fatty liver disease (NAFLD) is considered the most common liver disease in the world, but little is known about its potential association with pregnancy outcomes. We aimed to investigate pregnancy outcomes in NAFLD.
The Swedish Medical Birth Register (MBR) was used to identify births between 1992 and 2011 (N = 1 960 416). By linkage with the National Patient Register, we identified women with a diagnosis of NAFLD. The MBR was then used to identify outcomes: gestational diabetes, pre-eclampsia, Caesarean section, Apgar score
PubMed ID
26114995 View in PubMed
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Age at diagnosis and disease progression of cystic fibrosis in an area without newborn screening.

https://arctichealth.org/en/permalink/ahliterature101753
Source
Paediatr Perinat Epidemiol. 2011 May;25(3):298-305
Publication Type
Article
Date
May-2011
Author
Isabelle de Monestrol
Asa Klint
Pär Sparén
Lena Hjelte
Author Affiliation
Stockholm CF Centre, Karolinska University Hospital Huddinge, Stockholm, Sweden. isabelle.demonestrol@ki.se
Source
Paediatr Perinat Epidemiol. 2011 May;25(3):298-305
Date
May-2011
Language
English
Publication Type
Article
Keywords
Age Distribution
Age Factors
Child, Preschool
Cystic Fibrosis - diagnosis - epidemiology
Disease Progression
Female
Humans
Infant
Infant, Newborn
Liver Diseases - diagnosis - epidemiology
Lung Diseases - diagnosis - epidemiology
Male
Morbidity
Nutrition Disorders - diagnosis - epidemiology
Risk factors
Sweden - epidemiology
Abstract
We studied age at diagnosis and disease progression of cystic fibrosis (CF) patients with a new study design, using data of 119 patients extracted from Stockholm CF Centre registry. Risk factors for overall morbidity and for lung, liver and nutritional morbidity were investigated separately using time to event methodology (Kaplan-Meier curves, proportional hazards regression). The patients were followed from: (i) healthy at diagnosis to morbidity, (ii) diagnosis with symptoms of morbidity to being free of morbidity, and (iii) free of morbidity to relapse of morbidity. Median age at diagnosis was 5.0 months. Of the patients with overall morbidity at diagnosis 50% became free of morbidity after 4.8 years; however, the patients above the age of 24 months at diagnosis had a reduced chance of becoming free of morbidity (crude hazard ratio 0.14 [95 % confidence interval 0.04, 0.45]) compared with those with diagnosis between the ages of 2 and 12 months (P
PubMed ID
21470269 View in PubMed
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Alpha1-antitrypsin deficiency-associated liver disease progresses slowly in some children.

https://arctichealth.org/en/permalink/ahliterature32577
Source
J Pediatr Gastroenterol Nutr. 2000 Sep;31(3):258-63
Publication Type
Article
Date
Sep-2000
Author
D. Volpert
J P Molleston
D H Perlmutter
Author Affiliation
Department of Pediatrics, Washington University School of Medicine and St. Louis Children's Hospital, Missouri 63110, USA.
Source
J Pediatr Gastroenterol Nutr. 2000 Sep;31(3):258-63
Date
Sep-2000
Language
English
Publication Type
Article
Keywords
Child
Child, Preschool
Databases, Factual
Disease Progression
Female
Follow-Up Studies
Humans
Hypertension, Portal - enzymology - etiology - therapy
Infant
Infant, Newborn
Liver Cirrhosis - enzymology - etiology - therapy
Liver Transplantation
Male
Metabolism, Inborn Errors - complications
Phenotype
Prognosis
Quality of Life
Retrospective Studies
alpha 1-Antitrypsin Deficiency - complications
Abstract
BACKGROUND: A prospective nationwide screening study initiated more than 20 years ago in Sweden has shown that clinically significant liver disease develops in only 10% to 15% of alpha1-antitrypsin (AT)-deficient children. This study provides information about 85% to 90% of those children, many of whom had elevated serum transaminases in infancy but have no evidence of liver injury by age 18 years. However, there is relatively limited information about the course of alpha1-AT-deficient children who have cirrhosis or portal hypertension. Based on several anecdotal experiences, we have been impressed by the relatively slow progression and stable course of the liver disease in some of these children. METHODS: We reviewed the course of patients with homozygous PIZZ alpha1-antitrypsin deficiency seen at this institution since establishing a patient database 16 years ago. RESULTS: Of 44 patients with alpha1-AT deficiency, 17 had cirrhosis, portal hypertension, or both. Nine of the 17 patients with cirrhosis or portal hypertension had a prolonged, relatively uneventful course for at least 4 years after the diagnosis of cirrhosis or portal hypertension. Two of these patients eventually underwent liver transplantation, but seven are leading relatively healthy lives for up to 23 years while carrying a diagnosis of severe alpha1-AT deficiency-associated liver disease. Patients with the prolonged stable course could be distinguished from those with a rapidly progressive course on the basis of overall life functioning but not on the basis of any other more conventional clinical or biochemical criteria. CONCLUSIONS: These data provide further evidence for the variable severity of liver disease associated with alpha1-AT deficiency and indicate that some patients have chronic, slowly progressing or nonprogressing cirrhosis.
Notes
Comment In: J Pediatr Gastroenterol Nutr. 2001 Apr;32(4):504-511396827
PubMed ID
10997369 View in PubMed
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Antenatal diagnosis of hereditary fetal growth retardation with aminoaciduria, cholestasis, iron overload, and lactic acidosis in the newborn infant.

https://arctichealth.org/en/permalink/ahliterature190069
Source
Acta Obstet Gynecol Scand. 2002 May;81(5):398-402
Publication Type
Article
Date
May-2002
Author
Vineta Fellman
Ilona Visapää
Mihailo Vujic
Ulla-Britt Wennerholm
Leena Peltonen
Author Affiliation
Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland. vineta.fellman@hus.fi
Source
Acta Obstet Gynecol Scand. 2002 May;81(5):398-402
Date
May-2002
Language
English
Publication Type
Article
Keywords
Abortion, Induced
Acidosis, Lactic - complications
Cholestasis - complications
Chorionic Villi Sampling
European Continental Ancestry Group - genetics
Female
Fetal Growth Retardation - complications - diagnosis - genetics
Finland
Humans
Infant, Newborn
Iron Overload - complications
Liver - pathology
Male
Pedigree
Pregnancy
Prenatal Diagnosis
Renal Aminoacidurias - complications
Syndrome
Abstract
A prenatal diagnosis of the fetus for a mother of two previously deceased infants who died from the recently described autosomal recessive disease (OMIM 603358). The infants presented with intrauterine growth retardation, aminoaciduria, cholestasis, iron overload, severe lactic acidosis, and early death (GRACILE syndrome).
DNA was extracted from the fibroblasts and tissue samples of the deceased infants, parental leukocytes, and from a chorion villus biopsy in the next pregnancy. Haplotypes were determined using the relevant markers flanking the disease-associated region of chromosome 2.
Both deceased infants were homozygous for the four critical markers. The fetal haploptypes were identical to those of the siblings and the pregnancy was terminated. The iron content of the fetal liver was increased (5000 microg/g) compared with the controls, with a marked iron accumulation in the Kupffer cells.
Antenatal diagnosis can be performed based on linkage analysis in families with at least one affected child because the disease locus has been assigned to a restricted chromosomal region. Typical histological abnormalities may be present in early fetal life.
PubMed ID
12027811 View in PubMed
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Assignment of the locus for a new lethal neonatal metabolic syndrome to 2q33-37.

https://arctichealth.org/en/permalink/ahliterature204110
Source
Am J Hum Genet. 1998 Nov;63(5):1396-403
Publication Type
Article
Date
Nov-1998
Author
I. Visapää
V. Fellman
T. Varilo
A. Palotie
K O Raivio
L. Peltonen
Author Affiliation
Department of Human Molecular Genetics, National Public Health Institute, Helsinki, Finland.
Source
Am J Hum Genet. 1998 Nov;63(5):1396-403
Date
Nov-1998
Language
English
Publication Type
Article
Keywords
Acidosis, Lactic - genetics - mortality
Amino Acids - urine
Chromosome Mapping
Chromosomes, Human, Pair 2
Family
Female
Fetal Growth Retardation - genetics - mortality
Finland
Genes, Lethal
Genetic Linkage
Genetic markers
Hemosiderosis - genetics - mortality
Humans
Infant, Newborn
Linkage Disequilibrium
Liver Diseases - genetics - mortality
Male
Metabolism, Inborn Errors - genetics - mortality
Pedigree
Syndrome
Abstract
A new neonatal syndrome characterized by intrauterine growth retardation, lactic acidosis, aminoaciduria, liver hemosiderosis, and early death was recently described. The pathogenesis of this disease is unknown. The mode of inheritance is autosomal recessive, and so far only 17 cases have been reported in 12 Finnish families. Here we report the assignment of the locus for this new disease to a restricted region on chromosome 2q33-37. We mapped the disease locus in a family material insufficient for traditional linkage analysis by using linkage disequilibrium, a possibility available in genetic isolates such as Finland. The primary screening of the genome was performed with samples from nine affected individuals in five families. In the next step, conventional linkage analysis was performed in eight families, with a total of 12 affected infants, and finally the locus assignment was proved by demonstrating linkage disequilibrium to the regional markers in 20 disease chromosomes. Linkage analysis restricted the disease locus to a 3-cM region between markers D2S164 and D2S2359, and linkage disequilibrium with the ancestral haplotype restricted the disease locus further to the immediate vicinity of marker D2S2250.
Notes
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PubMed ID
9792866 View in PubMed
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Basic and clinical aspects of the alpha1-antitrypsin.

https://arctichealth.org/en/permalink/ahliterature42622
Source
Pediatrics. 1975 Jul;56(1):91-9
Publication Type
Article
Date
Jul-1975
Author
R C Talamo
Source
Pediatrics. 1975 Jul;56(1):91-9
Date
Jul-1975
Language
English
Publication Type
Article
Keywords
Blood Protein Disorders - complications - diagnosis - therapy
Child
Emphysema - genetics
Genetics
Genotype
Humans
Infant
Infant, Newborn
Liver Cirrhosis - genetics - pathology
Molecular Weight
Research Support, U.S. Gov't, P.H.S.
Respiratory Distress Syndrome, Newborn - metabolism
alpha 1-Antitrypsin - analysis - metabolism - physiology
alpha 1-Antitrypsin Deficiency
Abstract
A deficiency of the major serum alpha1-globulin, the alpha1-antitrypsin, was first described in five patients by Laurell and Eriksson in Sweden in 1963. It soon became obvious that severe alpha1-antitrypsin deficiency was familial, and highly associated with chronic lung disease, having its onset in the third or fourth decade of life. Since the early descriptions of this common deficiency state, it has become clearly associated with familial emphysema in some families, familial infantile cirrhosis in others, and occasionally with a combination of childhood lung and liver disease in siblings. For the pediatrician, severe alpha1-antitrypsin deficiency now enters into the differential diagnosis of both chronic pulmonary disease in childhood and obstructive jaundice in the newborn period; In addition, low levels of alpha1-antitrypsin in serum are characteristic of respiratory distress syndrome, and elevations of this protein may be found in a variety of clinical situations. The, alpha1-antitrypsin probably functions as a major control protein against the tissue-damaging effects of both endogenous and exogenous enzymes. This review will cover several basic and clinical features of this protein with respect to its importance in pediatrics.
PubMed ID
1099521 View in PubMed
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107 records – page 1 of 11.