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It is not exclusive to children and has been diagnosed in persons of all ages. It is a poorly-recognized cause of dyslipidemia, associated with the development of atherosclerosis, cardiovascular disease, and progressive liver disease. It typically presents as hepatomegaly, elevated aminotransferases, and diffuse microvesicular steatosis in liver biopsy. LAL-D is a disease that can go unnoticed if not suspected, or confused with other entities, such as nonalcoholic fatty liver disease NAFLD , non-alcoholic steatohepatitis, heterozygote familial hypercholesterolemia, or familial combined hyperlipidemia, among others.
The aim of the present work was to propose a quick guide for the suspicion and identification of LAL-D through a proposed diagnostic algorithm.. Mexican experts carried out a systematized review of the literature in relation to the clinical manifestations and diagnosis of LAL-D. Fifteen specialists in the field, with knowledge of LAL-D, were summoned to develop the consensus.
Only articles in Spanish and English were evaluated that mentioned clinical manifestations and laboratory and pathology results of LAL-D. The articles were analyzed by the following 5 groups of consensus participants: The statements were formulated by the groups and then voted upon.
The structured quantitative method employed for reaching consensus was the nominal group technique. Voting was done in person, but anonymously, using an electronic device. Diagnostic algorithm for lysosomal acid lipase deficiency.. The systematic search produced articles, none of which corresponded to clinical practice guidelines or clinical trials. Repeated articles, letters to the editor, or case reports contemplated in other articles were eliminated, leaving a total of articles reporting on clinical manifestations and laboratory and imaging data related to LAL-D that were then reviewed by the consensus participants..
The reduced enzymatic activity manifests as a progressive accumulation of cholesteryl esters and triglycerides in the liver, spleen, and other organs. The degree of lysosomal acid lipase LAL enzyme deficiency is variable, but does not determine the heterogeneity of the clinical symptoms.
There are two phenotypes: All children with disease presentation in infancy Wolman disease that do not receive treatment, die within the first year of life.
The frequency of LAL-D in different populations is unknown, perhaps due to its under-diagnosis. Population studies that evaluated the most frequent CESD mutation produced a carrier frequency of 1 in 1, in Asian populations and 1 in in Caucasian and Hispanic populations, supporting the hypothesis that the disease is under-diagnosed.. LAL-D is frequently unnoticed. LAL is essential for lipid metabolism. It hydrolyzes cholesteryl esters and triglycerides in low-density lipoprotein LDL particles and produces cholesterol and free fatty acids that, in turn, participate in the regulation of cholesterol homeostasis.
When LAL enzyme activity is reduced or absent, cholesteryl esters and triglycerides accumulate in the liposomes, producing intracellular free cholesterol depletion, a reduced capacity for suppressing 3-hydroxymethylglutaryl-coenzyme A reductase activity, and increased endogenous production of cholesterol.
Altered regulation of that transporter causing a decrease in HDL particle formation has been found in studies on fibroblasts in LAL-D patients. Those mechanisms explain the dyslipidemia present in LAL-D patients: The diagnosis of LAL-D is made through the clinical history, intentionally looking for its signs and symptoms, and complementing it with the corresponding laboratory tests.
The early clinical manifestations of the disease are diverse and hyporexia, frequent vomiting, diarrhea, steatorrhea, malnutrition and failure to grow, and hepatosplenomegaly stand out among them.
Liver damage rapidly progresses to portal hypertension and severe liver dysfunction in the first months of life. Metabolic acidosis and adrenal gland calcification have been described in almost one third of the patients. Clinical presentation varies in children and adults. The mean age for symptom onset is 5 years, but some patients remain asymptomatic and are not diagnosed until adulthood. Abdominal distension from visceromegaly and short stature are common.
Clinical manifestations in late-presentation LAL-D can be nonspecific, such as abdominal distension and short stature. Abdominal visceromegaly is frequent in LAL-D, and when detected, the disease should be suspected. In addition to the clinical manifestations of chronic liver disease and its complications, such as portal hypertension, bleeding, advanced-stage encephalopathy, and hepatocellular carcinoma, LAL-D manifestations also include: Main clinical manifestations of late-presentation LAL-D..
Main laboratory data of late-presentation LAL-D.. Late-presentation LAL-D should be ruled out in patients with hepatosplenomegaly, persistent aminotransferase elevation, and dyslipidemia. The accidental finding of aminotransferase elevation requires a search for liver and spleen enlargement. The primary cardiovascular manifestations are: Dyslipidemia associated with late-presentation LAL-D is related to atherosclerosis and is a risk factor for early cardiovascular disease.
Some cases can present with hypertriglyceridemia. Biochemically, the majority of patients with LAL-D present with elevated levels of alanine aminotransferase and aspartate aminotransferase, mild hyperbilirubinemia, elevated levels of gamma-glutamyl transferase, prolonged prothrombin time, prolonged fasting hypoglycemia, and IIA or IIB dyslipidemia, with hypercholesterolemia, low levels of HDL-C, elevated levels of LDL-C, Apo B, and sometimes hypertriglyceridemia.
Anemia is identified in Elevated levels of alanine aminotransferase have been observed in A complete lipid profile and periodic monitoring of aminotransferases for at least six months are recommended in all patients with liver disease. Adrenal gland calcification is characteristic of LAL-D that presents in infancy.
Hepatomegaly and splenomegaly are corroborated through imaging studies and they also identify signs of liver steatosis. Ultrasound, computed axial tomography, spectroscopy magnetic resonance, and transition elastography or the fusion of elastography and magnetic resonance, are noninvasive diagnostic tools 25 for evaluating and staging fatty liver fig. A Ultrasound image of a normal liver..
Late-presentation LAL-D is a cause of liver steatosis, identifiable through ultrasound imaging. Ultrasound shows diffuse echogenicity and identifies hepatomegaly and splenomegaly fig. The Tominaga classification 26 for evaluating liver echogenicity includes three stages: A Ultrasound image showing conserved echogenicity in the liver and right kidney..
Non-contrasted computed axial tomography evaluates liver attenuation measured in Hounsfield units and attenuation values between the liver-spleen. Magnetic resonance and elastography optimization are also diagnostic methods, as is nuclear magnetic resonance spectroscopy.. Non-contrasted computed tomography scan with Hounsfield unit measurement in the liver and spleen.. A cholesterol moiety has five CH 3 groups with a strong resonance on the 1 H magnetic resonance spectrum compared with a fatty acid moiety that has one methyl group per molecule.
The histopathologic study of biopsies from patients with LAL-D offers at least three benefits: Histopathologic alterations in LAL-D are characteristic and vary in intensity according to age, length of progression, and disease expressivity. The liver and all organs with a predominance of macrophages are the most commonly affected. Diffuse microvesicular steatosis is the basic lesion in the liver.
The cytoplasm of the hepatocytes contains numerous lipid droplets that are intensely positive with the oil red O stain. Those vesicles measure less than one micron in diameter and do not displace the nucleus fig.
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C Semi-fine slices that show the microvesicular nature of the steatosis, seen in practically all the hepatocytes toluidine blue, original magnification x D Multiple neutral fat droplets are revealed in the entire hepatic lobule oleous red, original magnification x Diffuse microvesicular steatosis and cholesterol crystals in the cytoplasm of hepatocytes and macrophages are liver biopsy findings suggestive of LAL-D.
The monocyte-macrophage system lesion affects Kupffer cells and the portal macrophages. Both show small droplets and cholesterol crystals in their cytoplasm.
In addition, macrophages accumulate pigment similar to lipofuscin fig. B Silver birefringence characteristic of the numerous cholesterol crystals polarized light, original magnification x C Kupffer cells with microvesicular steatosis and cholesterol crystals clearly stand out PAS, original magnification x1, Two basic tools demonstrate the existence of lipids inside lysosomes: Antibodies can be used that recognize lysosomal components, such as lysosomal integral membrane protein-2 LIMP2 , a protein associated with lysosome associated membrane protein-1 LAMP1 , and the lysosomal luminal protein or cathepsin D fig.
A Immunohistochemical reaction showing lipid droplets inside lysosomes cathepsin D, original magnification x B Photomicrograph showing lipids surrounded by simple lysosomal membrane that reveals its lysosomal nature, as well as cholesterol crystals electron microscopy, original magnification x2, Through electron microscopy, lipids are viewed inside the lysosomes fig. Liver fibrosis is dependent on progression time and disease expressivity, which can range from mild disease to cirrhosis.
Late-presentation LAL-D should be ruled out through immunohistochemistry or electron microscopy in patients with biopsy-demonstrated steatosis that is predominantly microvesicular.
With that histopathologic data and the demonstration of lipid storage in the lysosomes, LAL-D diagnosis can be made, differentiating it from other entities that present with microvesicular steatosis, including other storage diseases, 29,30 as shown in Table Late-presentation LAL-D should be ruled out in non-obese adult patients with cryptogenic cirrhosis of the liver.
LAL E. The biochemical diagnosis of LAL-D in leukocytes, fibroblasts, and amniotic fluid has been in use for 50 years 36,37 and in , an inhibitor Lalistat 2 was incorporated in the technique that improved its specificity.
The dried blood spot DBS test on filter paper can be used on patients of any age and it enables LAL activity to be determined. It is useful for detecting affected patients 2 and its sensitivity and specificity is very high.
The blood is absorbed and left to dry at room temperature until the following day. The sample should not be exposed to heat and should be transported in double bags with desiccant, to maintain enzyme activity. An international expert consensus for LAL diagnosis determined the importance of analyzing LAL activity results based on the percentage of activity, with respect to the normal mean of the healthy reference population analyzed by each laboratory.
Enzymatic diagnosis of LAL-D can be made through the dried blood spot test on filter paper. It is located at 10q The test is carried out in peripheral blood leukocytes and involves the sequencing of the LIPA gene or of a gene panel containing it.
DNA is extracted from the sample leukocytes or DBS for sequencing the gene or for massive sequencing. When LAL-D diagnosis is not confirmed through gene or gene panel sequencing, then the genomic approach should be considered. In Mexico, the c. Molecular diagnosis should be considered as a support method, given that the mutation s responsible cannot always be identified, as well as its high cost.
Enzymatic activity determination on paper is an accessible, accurate, and less costly test..
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Because of its variability, LAL-D should be differentiated at its neonatal stage from the different causes of neonatal cholestasis. Metabolic diseases clearly stand out as such causes, especially those that present with rapid-progression liver disease, such as tyrosinemia, neonatal galactosemia, hemochromatosis, and Niemann-Pick disease type C. LAL-D should be considered in the differential diagnosis of neonatal cholestasis with liver failure.
Because type IIA or IIB hyperlipidemia has been observed in patients with LAL-D, heterozygous familial hypercholesterolemia, defective familial ApoB , combined familial hyperlipidemia, and polygenic hypercholesterolemia should be considered in the differential diagnosis. Familial hypercholesterolemia can be confused with LAL-D, given that LDL is elevated in both diseases, but there is no hepatosplenomegaly in the former. Familial hypercholesterolemia is an autosomal co-dominant inherited disease, whereas LAL-D is an autosomal recessive inherited disease.
Late-presentation LAL-D should be considered in the differential diagnosis in all children and adults suspected of having heterozygous familial hypercholesterolemia. Because patients with LAL-D present with hepatomegaly, elevated levels of liver enzymes, fatty liver, and dyslipidemia, the differential diagnosis should include metabolic syndrome, which has a greater risk for cerebrovascular disease and type 2 diabetes mellitus.
Late-presentation LAL-D should be considered in non-obese individuals with or without metabolic syndrome that present with hepatomegaly, elevated aminotransferase levels, fatty liver, and dyslipidemia. LAL-D is a little-known, and consequently unsuspected disease that can go unnoticed or be confused with other pathologies and consequently misdiagnosed.
The algorithm proposed herein provides a sequence of actions to be taken, based on the clinical manifestations collected from different studies to optimize the diagnostic process of patients with LAL-D..
Given the heterogeneous phenotype of LAL-D, a patient does not need to meet all the criteria previously described to consider the disease within the differential diagnosis. The criteria for LAL-D suspicion are put forward, taking two large groups into account: Both groups can be accompanied with or have a history of chronic diarrhea, short stature, anemia, gallstones, or bleeding..
In the group of patients with liver involvement with no clear etiology, LAL-D diagnosis should be considered and the dried blood spot test on filter paper should be performed to determine LAL in blood.. Therefore, aminotransferase determination is recommended and if they are high, LAL determination through the dried blood spot test on filter paper should be performed..
No financial support was received in relation to this article.. The authors declare that there is no conflict of interest.. Please cite this article as: Indexed in: Previous article Next article.
January - March Pages More article options. Mexican consensus on lysosomal acid lipase deficiency diagnosis. Download PDF. Valencia-Mayoral c , G. Castro-Narro d , P. Medina-Bravo e , Y.
Mehta h , C. Valadez-Reyes l , F. Corresponding author. This item has received. Under a Creative Commons license. Article information. Show more Show less. Table 1. Table 2. Introduction Lysosomal acid lipase deficiency LAL-D causes progressive cholesteryl ester and triglyceride accumulation in the lysosomes of hepatocytes and monocyte-macrophage system cells, resulting in a systemic disease with various manifestations that may go unnoticed.
The aim of the present review was to offer a guide for physicians in understanding the fundamental diagnostic aspects of LAL-D, to successfully aid in its identification. Methods The review was designed by a group of Mexican experts and is presented as an orienting algorithm for the pediatrician, internist, gastroenterologist, endocrinologist, geneticist, pathologist, radiologist, and other specialists that could come across this disease in their patients.
Results A practical algorithm of the diagnostic process in LAL-D patients was proposed, based on clinical and laboratory data indicative of the disease and in accordance with the consensus established for each recommendation. Conclusion The algorithm provides a sequence of clinical actions from different studies for optimizing the diagnostic process of patients suspected of having LAL-D. Lysosomal acid lipase. Palabras clave:. Introduction and aims Lysosomal acid lipase deficiency LAL-D is a recessive autosomal disease characterized by the progressive accumulation of cholesteryl esters and triglycerides in the lysosomes of hepatocytes and the monocyte-macrophage system.
Methodology Mexican experts carried out a systematized review of the literature in relation to the clinical manifestations and diagnosis of LAL-D. Diagnostic algorithm for lysosomal acid lipase deficiency. Figure 1.
Main clinical manifestations of late-presentation LAL-D. Main laboratory data of late-presentation LAL-D. A Ultrasound image of a normal liver. Figure 2. A Ultrasound image showing conserved echogenicity in the liver and right kidney. Figure 3.
Non-contrasted computed tomography scan with Hounsfield unit measurement in the liver and spleen. Figure 4. Figure 5. Figure 6. Figure 7.
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Bernstein, H. Bialer, et al. Cholesteryl ester storage disease: J Hepatol, 58 , pp. Guardamanga, D. Nair, et al. Lysosomal acid lipase deficiency --An under-recognized cause of dyslipidaemia and liver dysfunction.
Atherosclerosis, , pp. Clin Pediatr Phila , 34 , pp. Lysosomal acid lipase deficiency: Wolman disease and cholesteryl ester storage disease.
Jones, V. Valayannopoulos, E. Schneider, et al. Rapid progression and mortality of lysosomal acid lipase deficiency presenting in infants. Genet Med. Pullinger, E. Stock, I. Movsesyan, et al. Identification and metabolic profiling of patients with lysosomal acid lipase deficiency. J Clin Lipidol, 9 , pp. Scott, B. Liu, I. Nazarenko, et al. Hepatology, 58 , pp. Muntoni, H. Wiebusch, M. Jansen-Rust, et al.
Prevalence of cholesteryl ester storage disease. Arterioscler Thromb Vasc Biol, 27 , pp. Pisciotta, R. Fresa, A. Bellocchio, et al. Mol Genet Metab, 97 , pp. Meikle, J. Hopwood, A.
Clague, et al. J Am Med Assoc, , pp. Zhang, A. J Pediatr Gastroenterol Nutr, 56 , pp. Dubland, G. Proteger PDF. Largar PDF aqui. Category: Documents. Analysis of the effects of two techniques of manual therapy on the autonomic For data analysis, nursing technicians and aides were grouped into one category. I sized him up, faked a slap and applied a leg sweep that had him off balance. But he stood. PDF - docplayer. The file contains page s and is free to view, download or print.
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A cholesterol moiety has five CH 3 groups with a strong resonance on the 1 H magnetic resonance spectrum compared with a fatty acid moiety that has one methyl group per molecule.