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Peer-reviewed veterinary case report

Seizures and low blood sugar in Maltese dogs with malonic aciduria

By O'Brien, D P et al.·Published in Journal of inherited metabolic disease·1999·Department of Veterinary Medicine and Surgery, United States·View original on PubMed

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Original publication title: Malonic aciduria in Maltese dogs: normal methylmalonic acid concentrations and malonyl-CoA decarboxylase activity in fibroblasts.

Species:
dog

Plain-English summary

A 3-year-old Maltese dog was brought in for seizures and confusion, along with low blood sugar and other metabolic issues. Tests revealed high levels of malonic acid in the urine, but normal levels of another substance called methylmalonic acid. The vet recommended a special diet that was low in fat but high in medium-chain triglycerides, which helped the dog recover and eliminated the malonic acid in the urine. Unfortunately, two of the dog's siblings had died young from similar undiagnosed issues, highlighting potential genetic concerns in this family of dogs.

People also search for: Maltese dog seizures treatment · malonic aciduria in dogs · low-fat diet for dog seizures

Abstract

A family of Maltese dogs with malonic aciduria is reported. The propositus presented at 3 years of age with episodes of seizures and stupor with hypoglycaemia, acidosis, and ketonuria. Urinary organic acid assays showed elevated malonic acid without elevation of methylmalonic acid. Cultured fibroblasts had normal malonyl-CoA decarboxylase activity. Treatment with frequent feedings of a low-fat diet high in medium-chain triglycerides resulted in normalization of clinical signs and a resolution of the malonic aciduria. Two full siblings of the propositus had died at a young age of undiagnosed metabolic and neurological disease. Urine organic acid assays were performed on other family members. A half-sister showed mild malonic aciduria and other organic acid changes similar to the propositus, while the mother and half-brother showed mildly elevated ketone bodies. This family suggests further genetic and clinical heterogeneity in the malonic acidurias.

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Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/10604140/