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The parents were not consanguineous and had no relevant medical history&#46; There had been a progressive decrease in the weight and length percentiles of the patient from birth to the time of the evaluation&#44; while his psychomotor development was normal&#46; The patient had been exclusively breastfed on demand in the first two months of life&#44; and was subsequently fed artificial formula&#44; with frequent feedings at two to three hour intervals with no breaks at night&#46; Physical examination revealed a weight of 8<span class="elsevierStyleHsp" style=""></span>kg &#40;3rd&#8211;10th percentile&#41;&#44; height of 67<span class="elsevierStyleHsp" style=""></span>cm &#40;3rd&#8211;10th percentile&#41;&#44; Waterlow height-for-age of 94&#46;3&#37;&#44; weight-for-height <span class="elsevierStyleItalic">z</span>-score of &#8722;0&#46;44 &#40;WHO standards&#41;&#44; and marked abdominal swelling with hepatomegaly of 9<span class="elsevierStyleHsp" style=""></span>cm&#46; These findings led to performance of an abdominal ultrasound scan&#44; which revealed uniform hepatomegaly&#44; and a chemistry panel with the following key findings&#58; AST&#44; 1071<span class="elsevierStyleHsp" style=""></span>U&#47;L &#40;normal range &#91;NR&#93;&#44; 0&#8211;34&#41;&#59; ALT&#44; 573<span class="elsevierStyleHsp" style=""></span>U&#47;L &#40;NR&#44; 10&#8211;49&#41;&#59; glucose 23<span class="elsevierStyleHsp" style=""></span>mg&#47;dL &#40;NR&#44; 70&#8211;100&#41;&#59; and triglycerides&#44; 772<span class="elsevierStyleHsp" style=""></span>mg&#47;dL &#40;NR&#44; &#60;110&#41;&#46; The patient did not respond to the glucagon stimulation test&#44; with a glucose level before stimulation of 38<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; followed by 40<span class="elsevierStyleHsp" style=""></span>mg&#47;dL 30<span class="elsevierStyleHsp" style=""></span>min after glucagon administration&#44; with no hyperlactacidaemia&#46; Genetic testing was performed due to suspicion of glycogen storage disease&#44; identifying a defect in the &#945; subunit of phosphorylase kinase&#44; which confirmed the diagnosis of glycogen storage disease type IXa&#46; The patient required continuous nocturnal gastric feeding until age 5 years&#44; when the family succeeded in achieving adequate metabolic control&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Clinical case 2</span><p id="par0020" class="elsevierStylePara elsevierViewall">Girl aged 19 months referred to the paediatrics clinic for failure to thrive between 15 and 18 months of life&#46; The parents were not consanguineous&#44; were originally from Bangladesh&#44; and had no relevant medical history&#46; The patient had a varied diet adequate for her age&#44; and she continued to breastfeed on demand&#46; Her psychomotor development was normal&#46; Physical examination revealed a weight of 9&#46;045<span class="elsevierStyleHsp" style=""></span>kg &#40;3rd&#8211;10th percentile&#41;&#44; height of 77<span class="elsevierStyleHsp" style=""></span>cm &#40;3rd&#8211;10th percentile&#41;&#44; Waterlow height-for-age of 95&#37;&#44; weight-for-height <span class="elsevierStyleItalic">z</span>-score of &#8722;0&#46;73 SDS &#40;WHO standards&#41;&#44; and marked abdominal swelling with hepatomegaly of 8<span class="elsevierStyleHsp" style=""></span>cm&#46; The abdominal ultrasound scan evinced a uniform hepatomegaly&#46; The salient findings of the chemistry panel were AST&#44; 1858<span class="elsevierStyleHsp" style=""></span>U&#47;L&#59; ALT&#44; 1029<span class="elsevierStyleHsp" style=""></span>U&#47;L&#59; glucose&#44; 56<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#59; and triglycerides&#44; 257<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#46; In the glucagon test&#44; the glucose level before administration of glucagon was 28<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; and at 30<span class="elsevierStyleHsp" style=""></span>min it was 40<span class="elsevierStyleHsp" style=""></span>mg&#47;dL without hyperlactacidaemia&#46; Genetic testing was requested for suspected glycogen storage disease&#44; which identified amylo-1&#44;6-glucosidase deficiency&#44; leading to diagnosis of type IIIa glycogen storage disease&#46; She required continuous nocturnal gastric feeding for a period of two weeks&#46; Subsequently&#44; thanks to extensive family involvement&#44; the patient achieved adequate metabolic control with feedings at 4<span class="elsevierStyleHsp" style=""></span>h intervals in the daytime&#44; and two cornstarch feedings during the night&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The presence of progressive abdominal swelling in an infant requires ruling out hepatomegaly&#44; among other conditions&#46; In some cases&#44; considerable liver enlargement that can reach as far as the iliac crest is difficult to feel with palpation and may go unnoticed&#44;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> as described in the two cases&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">A differential diagnosis of the multiple aetiologies of hepatomegaly can be performed by history taking&#44; physical examination and diagnostic tests&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> In the cases presented here&#44; in addition to hepatomegaly and the marked abdominal swelling&#44; the failure to thrive and the presence of hypertransaminasaemia&#44; hyperlipidaemia and hypoglycaemia with abnormal results in the glucagon test led us to suspect a diagnosis of glycogen storage disease&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Glycogen storage disease type III is caused by a deficiency of amylo-1&#44;6-glucosidase&#44; also known as glycogen debranching enzyme&#44; an enzyme involved in glycogenolysis whose deficiency leads to accumulation of limit dextrins&#46; In 85&#37; of cases&#44; this deficiency affects the liver and muscle tissues &#40;subtype IIIa&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Glycogen storage disease type IX results from a defect in the activation of phosphorylase kinase&#44; which is also involved in glycogenolysis&#46; Different mutations may occur in the genes of each of the subunits that compose the enzyme &#40;&#945;&#44; &#223;&#44; &#947;&#44; &#948;&#41; with variable presence in different tissues&#46; X-linked glycogen storage disease type IXb &#40;XLG&#41; is the most frequent form and only involves the liver&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The main goal of treatment is to prevent hypoglycaemia&#46; This requires avoiding prolonged fasting periods by the frequent intake of slow-release carbohydrates throughout the day&#44; and in some cases&#44; especially in infants&#44; nocturnal gastric feedings&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p></span></span>"
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Scientific Letter
Different glycogen storage diseases presenting as abdominal distention and growth and weight retardation
Distensión abdominal y estancamiento ponderoestatural como forma de presentación de diferentes tipos de glucogenosis
R. Sierra-Poyatosa, T. Gavela-Pérezb, M. Blanco-Rodríguezb, L. Soriano-Guillénb,
Corresponding author
a Servicio de Endocrinología y Nutrición, Instituto de Investigación Sanitaria Fundación Jiménez Díaz, Universidad Autónoma de Madrid, Madrid, Spain
b Servicio de Pediatría, Instituto de Investigación Sanitaria Fundación Jiménez Díaz, Universidad Autónoma de Madrid, Madrid, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Glycogen storage diseases comprise a heterogeneous group of rare hereditary disorders with an approximate incidence of one case per 20&#44;000 live births a year&#44; and are caused by genetic defects in proteins responsible for glycogen synthesis and storage&#46; Several types of glycogen storage diseases have been described that are caused by different genetic changes and whose clinical expression is diverse&#44; with predominance of hepatic and&#47;or muscular involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Despite the low prevalence of glycogen storage diseases in everyday paediatric practice&#44; we present two illustrative clinical cases of patients diagnosed with different forms of glycogen storage disease&#58;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Clinical case 1</span><p id="par0015" class="elsevierStylePara elsevierViewall">Boy aged 9 months referred to the paediatrics clinic for evaluation of progressive abdominal swelling&#46; The parents were not consanguineous and had no relevant medical history&#46; There had been a progressive decrease in the weight and length percentiles of the patient from birth to the time of the evaluation&#44; while his psychomotor development was normal&#46; The patient had been exclusively breastfed on demand in the first two months of life&#44; and was subsequently fed artificial formula&#44; with frequent feedings at two to three hour intervals with no breaks at night&#46; Physical examination revealed a weight of 8<span class="elsevierStyleHsp" style=""></span>kg &#40;3rd&#8211;10th percentile&#41;&#44; height of 67<span class="elsevierStyleHsp" style=""></span>cm &#40;3rd&#8211;10th percentile&#41;&#44; Waterlow height-for-age of 94&#46;3&#37;&#44; weight-for-height <span class="elsevierStyleItalic">z</span>-score of &#8722;0&#46;44 &#40;WHO standards&#41;&#44; and marked abdominal swelling with hepatomegaly of 9<span class="elsevierStyleHsp" style=""></span>cm&#46; These findings led to performance of an abdominal ultrasound scan&#44; which revealed uniform hepatomegaly&#44; and a chemistry panel with the following key findings&#58; AST&#44; 1071<span class="elsevierStyleHsp" style=""></span>U&#47;L &#40;normal range &#91;NR&#93;&#44; 0&#8211;34&#41;&#59; ALT&#44; 573<span class="elsevierStyleHsp" style=""></span>U&#47;L &#40;NR&#44; 10&#8211;49&#41;&#59; glucose 23<span class="elsevierStyleHsp" style=""></span>mg&#47;dL &#40;NR&#44; 70&#8211;100&#41;&#59; and triglycerides&#44; 772<span class="elsevierStyleHsp" style=""></span>mg&#47;dL &#40;NR&#44; &#60;110&#41;&#46; The patient did not respond to the glucagon stimulation test&#44; with a glucose level before stimulation of 38<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; followed by 40<span class="elsevierStyleHsp" style=""></span>mg&#47;dL 30<span class="elsevierStyleHsp" style=""></span>min after glucagon administration&#44; with no hyperlactacidaemia&#46; Genetic testing was performed due to suspicion of glycogen storage disease&#44; identifying a defect in the &#945; subunit of phosphorylase kinase&#44; which confirmed the diagnosis of glycogen storage disease type IXa&#46; The patient required continuous nocturnal gastric feeding until age 5 years&#44; when the family succeeded in achieving adequate metabolic control&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Clinical case 2</span><p id="par0020" class="elsevierStylePara elsevierViewall">Girl aged 19 months referred to the paediatrics clinic for failure to thrive between 15 and 18 months of life&#46; The parents were not consanguineous&#44; were originally from Bangladesh&#44; and had no relevant medical history&#46; The patient had a varied diet adequate for her age&#44; and she continued to breastfeed on demand&#46; Her psychomotor development was normal&#46; Physical examination revealed a weight of 9&#46;045<span class="elsevierStyleHsp" style=""></span>kg &#40;3rd&#8211;10th percentile&#41;&#44; height of 77<span class="elsevierStyleHsp" style=""></span>cm &#40;3rd&#8211;10th percentile&#41;&#44; Waterlow height-for-age of 95&#37;&#44; weight-for-height <span class="elsevierStyleItalic">z</span>-score of &#8722;0&#46;73 SDS &#40;WHO standards&#41;&#44; and marked abdominal swelling with hepatomegaly of 8<span class="elsevierStyleHsp" style=""></span>cm&#46; The abdominal ultrasound scan evinced a uniform hepatomegaly&#46; The salient findings of the chemistry panel were AST&#44; 1858<span class="elsevierStyleHsp" style=""></span>U&#47;L&#59; ALT&#44; 1029<span class="elsevierStyleHsp" style=""></span>U&#47;L&#59; glucose&#44; 56<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#59; and triglycerides&#44; 257<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#46; In the glucagon test&#44; the glucose level before administration of glucagon was 28<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; and at 30<span class="elsevierStyleHsp" style=""></span>min it was 40<span class="elsevierStyleHsp" style=""></span>mg&#47;dL without hyperlactacidaemia&#46; Genetic testing was requested for suspected glycogen storage disease&#44; which identified amylo-1&#44;6-glucosidase deficiency&#44; leading to diagnosis of type IIIa glycogen storage disease&#46; She required continuous nocturnal gastric feeding for a period of two weeks&#46; Subsequently&#44; thanks to extensive family involvement&#44; the patient achieved adequate metabolic control with feedings at 4<span class="elsevierStyleHsp" style=""></span>h intervals in the daytime&#44; and two cornstarch feedings during the night&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The presence of progressive abdominal swelling in an infant requires ruling out hepatomegaly&#44; among other conditions&#46; In some cases&#44; considerable liver enlargement that can reach as far as the iliac crest is difficult to feel with palpation and may go unnoticed&#44;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> as described in the two cases&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">A differential diagnosis of the multiple aetiologies of hepatomegaly can be performed by history taking&#44; physical examination and diagnostic tests&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> In the cases presented here&#44; in addition to hepatomegaly and the marked abdominal swelling&#44; the failure to thrive and the presence of hypertransaminasaemia&#44; hyperlipidaemia and hypoglycaemia with abnormal results in the glucagon test led us to suspect a diagnosis of glycogen storage disease&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Glycogen storage disease type III is caused by a deficiency of amylo-1&#44;6-glucosidase&#44; also known as glycogen debranching enzyme&#44; an enzyme involved in glycogenolysis whose deficiency leads to accumulation of limit dextrins&#46; In 85&#37; of cases&#44; this deficiency affects the liver and muscle tissues &#40;subtype IIIa&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Glycogen storage disease type IX results from a defect in the activation of phosphorylase kinase&#44; which is also involved in glycogenolysis&#46; Different mutations may occur in the genes of each of the subunits that compose the enzyme &#40;&#945;&#44; &#223;&#44; &#947;&#44; &#948;&#41; with variable presence in different tissues&#46; X-linked glycogen storage disease type IXb &#40;XLG&#41; is the most frequent form and only involves the liver&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The main goal of treatment is to prevent hypoglycaemia&#46; This requires avoiding prolonged fasting periods by the frequent intake of slow-release carbohydrates throughout the day&#44; and in some cases&#44; especially in infants&#44; nocturnal gastric feedings&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p></span></span>"
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