TY - JOUR
T1 - Lysosomal acid lipase deficiency
T2 - A form of non-obese fatty liver disease (NOFLD)
AU - A-Kader, Hassan H.
N1 - Funding Information:
The author is deeply appreciative of Dr Achyut Bhaattacharrya, Professor and chief of Pathology at the University of Arizona for his kind help with the pathology slides. He would also like to thank Dr William Balistreri, Professor of Pediatrics and Medicine at the University of Cincinnati for suggesting the term of NOFLD (Non-Obese Fatty Liver Disease).
Publisher Copyright:
© 2017 Informa UK Limited, trading as Taylor & Francis Group.
PY - 2017
Y1 - 2017
N2 - Introduction: With the growing obesity epidemic, nonalcoholic fatty liver disease (NAFLD) is rapidly becoming one of the leading causes of liver disease worldwide. Although obesity is a main risk factor for the development of NAFLD, it can also develop in lean subjects and can be encountered in different clinical setting and in association with an array of genetic, metabolic, nutritional, infectious and drug-induced disorders. Areas covered: This article discusses causes of fatty liver in non-obese subjects focusing on Lysosomal acid lipase deficiency (LAL-D), a commonly overlooked disorder reviewing its prevalence, genetics, pathogenesis, clinical features, diagnosis and treatment. It will also review other causes of non-alcoholic fatty liver disease, which can be encountered in the absence of obesity and metabolic syndrome. Expert commentary: Although the prevalence of LAL-D has been estimated in the range of 1 in 40,000 and 1 in 300,000, this estimate is much more than the identified cases reported in the literature, which suggests that that the disease may be considerably under-diagnosed. There is a pressing need to educate clinicians about the disease, especially with the development of new promising therapeutic modalities.
AB - Introduction: With the growing obesity epidemic, nonalcoholic fatty liver disease (NAFLD) is rapidly becoming one of the leading causes of liver disease worldwide. Although obesity is a main risk factor for the development of NAFLD, it can also develop in lean subjects and can be encountered in different clinical setting and in association with an array of genetic, metabolic, nutritional, infectious and drug-induced disorders. Areas covered: This article discusses causes of fatty liver in non-obese subjects focusing on Lysosomal acid lipase deficiency (LAL-D), a commonly overlooked disorder reviewing its prevalence, genetics, pathogenesis, clinical features, diagnosis and treatment. It will also review other causes of non-alcoholic fatty liver disease, which can be encountered in the absence of obesity and metabolic syndrome. Expert commentary: Although the prevalence of LAL-D has been estimated in the range of 1 in 40,000 and 1 in 300,000, this estimate is much more than the identified cases reported in the literature, which suggests that that the disease may be considerably under-diagnosed. There is a pressing need to educate clinicians about the disease, especially with the development of new promising therapeutic modalities.
KW - LAL-D
KW - Lysosomal acid lipase deficiency
KW - Metabolic syndrome
KW - NAFLD
KW - NASH
KW - NOFLD
KW - Nonalcoholic fatty liver disease
KW - Obesity
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U2 - 10.1080/17474124.2017.1343144
DO - 10.1080/17474124.2017.1343144
M3 - Review article
C2 - 28612634
AN - SCOPUS:85054426863
SN - 1747-4124
VL - 11
SP - 911
EP - 924
JO - Expert Review of Gastroenterology and Hepatology
JF - Expert Review of Gastroenterology and Hepatology
IS - 10
ER -