Lesión hepática por déficit de alpha-1-antitripsina en la infancia. Revisión de 14 casos.

M. J. Mellado; P. Jara; F. Valverde; M. C. Díaz; E. Fuentes; J. Larrauri; C. Vázquez

Lesión hepática por déficit de alpha-1-antitripsina en la infancia. Revisión de 14 casos.

Translated title of the contribution: Hepatic lesions caused by alpha 1-antitrypsin deficiency in childhood. Review of 14 cases

M. J. Mellado
, P. Jara
, F. Valverde
, M. C. Díaz
, E. Fuentes
, J. Larrauri
, C. Vázquez

Research output: Contribution to journal › Article › peer-review

1 Scopus citations

Abstract

Fourteen cases of alpha-1-antitrypsin deficiency are presented. All of them had a PIZZ phenotype except two in which a PIMZ phenotype was found. It must be pointed out that histological findings show a great variability among the different patients most of which did not have intracellular PAS-positive amylase inclusions in liver biopsy specimens. Clinical course did not correlate with either the age of onset of the disease or the phenotype found, thus indicating that other additional factors are involved in determining prognosis. We insist on the importance of a careful study of all neonatal hepatitis syndromes in order to rule out a alpha-1-antitrypsin deficiency.

Translated title of the contribution	Hepatic lesions caused by alpha 1-antitrypsin deficiency in childhood. Review of 14 cases
Original language	Spanish
Pages (from-to)	5-12
Number of pages	8
Journal	Anales Espanoles de Pediatria
Volume	25
Issue number	1
State	Published - Jul 1986

Cite this

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title = "Lesi{\'o}n hep{\'a}tica por d{\'e}ficit de alpha-1-antitripsina en la infancia. Revisi{\'o}n de 14 casos.",

abstract = "Fourteen cases of alpha-1-antitrypsin deficiency are presented. All of them had a PIZZ phenotype except two in which a PIMZ phenotype was found. It must be pointed out that histological findings show a great variability among the different patients most of which did not have intracellular PAS-positive amylase inclusions in liver biopsy specimens. Clinical course did not correlate with either the age of onset of the disease or the phenotype found, thus indicating that other additional factors are involved in determining prognosis. We insist on the importance of a careful study of all neonatal hepatitis syndromes in order to rule out a alpha-1-antitrypsin deficiency.",

author = "Mellado, \{M. J.\} and P. Jara and F. Valverde and D{\'i}az, \{M. C.\} and E. Fuentes and J. Larrauri and C. V{\'a}zquez",

year = "1986",

month = jul,

language = "Spanish",

volume = "25",

pages = "5--12",

journal = "Anales Espanoles de Pediatria",

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publisher = "Ediciones Doyma, S.L.",

number = "1",

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TY - JOUR

T1 - Lesión hepática por déficit de alpha-1-antitripsina en la infancia. Revisión de 14 casos.

AU - Mellado, M. J.

AU - Jara, P.

AU - Valverde, F.

AU - Díaz, M. C.

AU - Fuentes, E.

AU - Larrauri, J.

AU - Vázquez, C.

PY - 1986/7

Y1 - 1986/7

N2 - Fourteen cases of alpha-1-antitrypsin deficiency are presented. All of them had a PIZZ phenotype except two in which a PIMZ phenotype was found. It must be pointed out that histological findings show a great variability among the different patients most of which did not have intracellular PAS-positive amylase inclusions in liver biopsy specimens. Clinical course did not correlate with either the age of onset of the disease or the phenotype found, thus indicating that other additional factors are involved in determining prognosis. We insist on the importance of a careful study of all neonatal hepatitis syndromes in order to rule out a alpha-1-antitrypsin deficiency.

AB - Fourteen cases of alpha-1-antitrypsin deficiency are presented. All of them had a PIZZ phenotype except two in which a PIMZ phenotype was found. It must be pointed out that histological findings show a great variability among the different patients most of which did not have intracellular PAS-positive amylase inclusions in liver biopsy specimens. Clinical course did not correlate with either the age of onset of the disease or the phenotype found, thus indicating that other additional factors are involved in determining prognosis. We insist on the importance of a careful study of all neonatal hepatitis syndromes in order to rule out a alpha-1-antitrypsin deficiency.

UR - https://www.scopus.com/pages/publications/0022744684

M3 - Article

C2 - 3489428

AN - SCOPUS:0022744684

SN - 0302-4342

VL - 25

SP - 5

EP - 12

JO - Anales Espanoles de Pediatria

JF - Anales Espanoles de Pediatria

IS - 1

ER -

Lesión hepática por déficit de alpha-1-antitripsina en la infancia. Revisión de 14 casos.

Abstract

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