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Indian Pediatr 2017;54:331 -332 |
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Incidentally Detected
Elevated Liver Enzymes: From Liver to Muscle
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*Padma Balaji #Srinivas
Sankaranarayanan, Viswanathan Venkataraman and
#VS Sankaranarayanan
From Department of Pediatric Neurology and #Pediatric
Gastroenterology, CHILDS Trust Medical Research foundation and Kanchi
Kamakoti CHILDS Trust Hospital, Chennai, India.
Email: *[email protected]
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We describe 8 children – with
incidentally detected isolated elevation of liver enzymes aspartate
aminotansferase and alanine aminotransferase – who were extensively
evaluated for hepatic causes before finally being diagnosed to have
muscular dystrophy. Serum creatinine phosphokinase levels, if performed
early during the work-up, may help in diagnosis of muscle disease and
avoid unnecessary investigations for liver disease.
Keywords: Anicteric hepatitis, Creatinine
phosphokinase, Muscular dystrophy.
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Elevated levels of serum aspartate aminotransferase
(AST) and serum alanine aminotransferase (ALT) usually indicates
hepatocyte injury. However, due to their widespread distribution in the
body, serum levels of these enzymes can be elevated in other conditions
as well [1,2]. Muscle disorders like muscular dystrophies, inflammatory
myopathies and metabolic myopathies can lead to elevated blood levels of
creatine phosphokinase (CPK), lactate dehydrogenase (LDH), ALT and AST
[1] .
Over a period of 6 years, eight boys with a median
(range) age of 4.5 (0.5,13) year were referred to us for evaluation of
persistently abnormal liver function test (LFT). Abnormal LFTs were
detected incidentally and the indications for ordering LFT were
antituberculous drug therapy (n=3), anticonvulsant therapy (n=1),
evaluation of poor weight gain (n=3) and abdominal pain (n=1).
On detailed evaluation, there was no history or clinical evidence of
icterus, organomegaly or other signs to suggest chronic liver disease.
The only abnormality in all the serial LFTs was elevated serum
transaminases (ALT and AST) in the presence of normal bilirubin,
albumin, prothrombin time and other liver enzymes. Mean (range) AST was
320 (134, 550) IU/L and ALT was 342 (154, 560) IU/ L. Ultrasonography of
abdomen, HbsAg and anti-HCV antibodies were performed in all patients.
Five patients had been worked-up for Wilson’s disease and autoimmune
hepatitis. Serum ammonia and lactate was done in three patients and
celiac serology in one patient. Liver biopsy was performed in three
children. All these tests were reported normal.
One child (13-year-old) had a waddling gait. The
other seven children had a normal gait but a positive early Gower’s
sign. In two of these children, a positive Gower’s sign was seen only on
asking the child to get up from the supine position (need to turn on
their sides to get up because of weakness of neck flexors - initial
component of Gower’s). Mean (range) serum CPK levels were 18,250 (7340,
53617) IU/L. Genetic analysis confirmed a diagnosis of Duchenne muscular
dystrophy in seven patients, and one had sarcoglycanopathy on muscle
biopsy.
Though many conditions such as viral hepatitis,
Wilson’s disease, autoimmune disease, non-alcoholic steatic hepatitis
(NASH) and celiac disease can cause anicteric hepatitis, muscle disease
should also be considered early in the differential diagnosis of a child
with isolated elevation of AST and ALT. Recognition of muscle disease in
children may sometimes be difficult, especially in the pre-symptomatic
period [3], and failure to recognize it as a cause of abnormal LFT may
lead to unnecessary and invasive investigations like liver biopsy [1,4].
We recommend that serum CPK levels should be performed early in
evaluation of children with isolated elevated liver enzymes with no
clinical signs/symptoms of liver disease.
Contributors: All authors were involved in
acquisition, analysis and interpretation of data, drafting the work and
final approval; and are accountable for all aspects of the work
Funding: None; Competing interest: None
stated.
References
1. McMillan HJ, Gregas M, Darras BT, Kang PB. Serum
transaminase levels in boys with Duchenne and Becker muscular dystrophy.
Pediatrics. 2011;127:e132-6.
2. Vajro P, Maddaluno S, Veropalumbo C. Persistent
hyper-transaminasemia in asymptomatic children: a stepwise approach.
World J Gastroenterol. 2013;19:2740-51.
3. Swaiman KF, Ashwal S, Ferriero DM, Schor NF.
Swaimans Pediatric Neurology, 5th ed, Elsevier Saunders; 2012,
1578-81.
4. Veropalumbo C, Giudice ED, Esposito G, Maddaluno
S, Ruggiero L, Vajro P. Aminotransferases and muscular diseases: a
disregarded lesson. Case reports and review of the literature. J Pediatr
Child Health. 2010;48:886-90.
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