2013
DOI: 10.1155/2013/721871
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Acute Pancreatitis with Rapid Clinical Improvement in a Child with Isovaleric Acidemia

Abstract: Isovaleric acidemia is a rare branched-chain organic acidemia. The authors describe a 3.5-year-old girl with isovaleric acidemia and acute abdominal pain associated with bilious emesis. Elevated serum amylase and abdominal ultrasonography demonstrating an enlarged and edematous pancreas, along with the presence of peripancreatic exudates, confirmed the presence of acute pancreatitis. The patient recovered quickly with intravenous hydration, pancreatic rest, and administration of intravenous L-carnitine. Pancre… Show more

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Cited by 6 publications
(6 citation statements)
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References 14 publications
(11 reference statements)
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“…The acute form appears with findings of lethargy, vomiting, dehydration, convulsion and coma in the first week of life. The chronic intermittent form appears at subsequent periods of childhood following conditions of stress, such as viral or bacterial infections, prolonged fasting or high protein intake [6]. In our patient, clinical presentation and advanced age were compatible with the chronic intermittent form.…”
Section: Discussionsupporting
confidence: 50%
See 1 more Smart Citation
“…The acute form appears with findings of lethargy, vomiting, dehydration, convulsion and coma in the first week of life. The chronic intermittent form appears at subsequent periods of childhood following conditions of stress, such as viral or bacterial infections, prolonged fasting or high protein intake [6]. In our patient, clinical presentation and advanced age were compatible with the chronic intermittent form.…”
Section: Discussionsupporting
confidence: 50%
“…Chronic intermittent or asymptomatic forms have been reported in addition to the acute form. Prolonged fasting, infections and a protein-rich diet may trigger acute attacks in chronic form [6]. …”
Section: Introductionmentioning
confidence: 99%
“…Neurological manifestations of IVA relate to EEG abnormalities or seizures and motor dysfunction [71,72]. Similar to findings in other organic acidurias [73], there have been single reports of patients with pancreatitis [39,[74][75][76]. Liver fibrosis [28] and, most recently, optic nerve atrophy [71] have also been associated with IVA.…”
Section: Clinical Presentationmentioning
confidence: 61%
“…The mechanism underlying the development of pancreatitis has been elucidated, although the most common biochemical changes in the disease include hypoglycemia, lactic acidosis, and high levels of uric acid and triglycerides-any of which may contribute to the onset of pancreatitis. Previous studies have reported the incidence of pancreatitis in organic acidurias; the first ever report discussed pancreatitis in 9 out of 108 patients with branched-chain organic aciduria (BCOA) [2,7], while pancreatitis was observed in 10 patients with isovaleric acidemia [19,20], 2 with propionic academia [4,6], 1 with MMA [6], 2 with maple syrup urine disease (MSUD) [21], 1 with glutaric academia [5], and 2 with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency [22,23]. Case 5, who had a fatty acid oxidation metabolic disorder, showed elevated transaminase levels and hepatomegaly after prolonged fasting or inadequate diet, which led to pancreatitis.…”
Section: Discussionmentioning
confidence: 99%
“…Lack of energy supply could be [29][30][31]. Hyperlipidemia interferes with amylase measurements, leading to false-negative results [20,21,26,27,32,33]. This may explain the normal or mildly elevated amylase levels in patients with severe necrotizing pancreatitis.…”
Section: Discussionmentioning
confidence: 99%