Transient Elastography to Represent Hepatic Copper Accumulation in Wilson Disease
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degradation products, calcium and other minerals. These products decrease as blood glucose levels are controlled and hence the recovery is explainable. Hyperglycemia causes hyperviscosity which leads to local tissue hypoperfusion, and depletion of gamma-aminobutyric acid secondary to a nonketotic state. Hyaline degeneration, vascular proliferation and arteriolar thickening can be seen in striatal biopsy [5]. Astrocyte ballooning and neuronal degeneration can explain the hyper-intensities on imaging [1]. Similar hyperintensities in the basal nuclei can be seen in hepatic encephalopathy, toxic exposure to manganese, Wilson disease, intracerebral hemorrhage, carbon monoxide poisoning and methanol toxicity.
1. Das L, Pal R, Dutta P, Bhansali A. Diabetic striatopathy and ketoacidosis: Report of two cases and review of literature. Diabetes Research and Clinical Practice. 2017;128:1 2. Faundez T, Klee P, Hanquinet S, Schwitzgebel V, Burkhard PR, Korff CM. Diabetic striatopathy in childhood: A case report. Pediatrics. 2016;137:e20143761. 3. Expert Committee on the Diagnosis and Classification of Diabetes Mellitus. Report of the Expert Committee on the Diagnosis and Classification of Diabetes Mellitus. Diabetes Care. 2003;26:S5-20. 4. Postuma RB, Lang AE. Hemiballism: Revisiting a classic disorder. Lancet Neurol. 2003;2:661-8. 5. Abe Y, Yamamoto T, Soeda T, Kumagai T, Tanno Y, Kubo J, et al. Diabetic striatal disease: Clinical presentation, neuroimaging, and pathology. Intern Med. 2009;48: 1135-41.
More than 90% patients show complete resolution between 2 to 28 days with or without radiological improvement [1]. Recovery was seen at 21 days with adequate glycemic control in the index case. In conclusion, diabetic striatopathy is a rare entity in children, more so with bilateral presentation. This case highlights the need to suspect diabetic striatopathy in any child with uncontrolled diabetes and acute onset of choreaballism, irrespective of its cause.
Transient Elastography to Represent Hepatic Copper Accumulation in Wilson Disease
copper 0.42 pm; zinc 0.296 pm; PT 31.5 sec, INR 2.68; ceruloplasmin 9 mg/dL; and 24-hour urine copper 115.2 µg. Liver biopsy was omitted due to uncorrectable coagulopathy. TE (FibroScan; Echosens, Paris, France) was measured in preprandial state by a trained operator which showed a value of 50.6 kilopascals (kPa). The patient was diagnosed with Wilson disease with 6 marks from the scoring system and the Wilson index score (WI) of 7, which implies good outcome without liver transplantation. Hence, D-penicillamine and zinc were initiated, and child closely followed-up for deterioration, and worsening coagulopathy. Six weeks later, while her clinical features and laboratory parameters did not improve, the TE value dramatically decreased (36.8 kPa). After one year, she rejoined school and performed well academically; her attention and memory were improved as per feedback from parents and teachers. However, KF rings were still present, even though tests of liver function were normal. The TE value
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