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

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.

Cardiopulmonary effects of bilateral hemithorax ventilation and diagnostic thoracoscopy in dogs.

OBJECTIVE: To describe alterations in respiratory and cardiovascular variables during diagnostic thoracoscopy, using bilateral hemithorax ventilation with sustained pneumothorax. ANIMALS: 7 adult dogs. PROCEDURE: Each dog was anesthetized and instrumented for 2 episodes of cardiopulmonary monitoring that were performed at an interval of more than 14 days. The first anesthetic episode served as a control procedure for the thoracoscopy treatment performed during the second anesthetic episode. Multiple cardiopulmonary variables were evaluated by comparing changes from baseline values within treatments and between treatments. RESULTS: Arterial oxygen tension decreased significantly from baseline values during thoracoscopy but was unchanged during sham treatment. Arterial carbon dioxide tension, clinical shunt fraction, and systemic mean arterial pressure increased during thoracoscopy. In contrast, these variables were unaffected by the sham treatment. Heart rate and cardiac index increased during sham and thoracoscopy treatments; however, the increase was significantly greater during thoracoscopy. Total peripheral vascular resistance significantly decreased from baseline values for both treatments, but the decrease was greater during thoracoscopy. Significant changes were not observed for oxyhemoglobin saturation or pulmonary vascular resistance during either treatment. Dogs recovered without major clinical complications. CONCLUSIONS: Significant changes were found for several cardiopulmonary variables during bilateral hemithorax ventilation with sustained pneumothorax for diagnostic thoracoscopy of clinically normal dogs. CLINICAL RELEVANCE: Diagnostic thoracoscopy with bilateral hemithorax ventilation and sustained pneumothorax is well tolerated in clinically normal dogs and may provide a diagnostic modality enabling intrathoracic procedures with less morbidity than thoracotomy for dogs with intrathoracic disease.

Evaluation of biopsy specimens obtained during thoracoscopy from lungs of clinically normal dogs.

OBJECTIVE: To determine whether lung biopsy specimens obtained during thoracoscopy, using a commercially available ligature, can provide an adequate amount of tissue for histologic evaluation and to characterize changes in the lungs and thoracic cavity that result from the procedure. ANIMALS: 6 mixed-breed dogs. PROCEDURE: All dogs underwent 2 anesthetic episodes. The first anesthetic episode was a sham procedure. During the second anesthetic episode, each dog underwent a thoracoscopic procedure to obtain a lung biopsy specimen, using a commercially available ligature. Biopsy specimens were assessed subjectively by means of histologic evaluation. Samples for arterial blood gas analysis were obtained, and thoracic radiography was performed after surgery. Dogs were evaluated daily for 14 days after thoracoscopy and then were euthanatized. Tissues were evaluated grossly and histologically. RESULTS: Excellent intraoperative visibility and biopsy specimens adequate for histologic evaluation were obtained from all dogs. Significant differences were not found between arterial blood gas values of sham- and thoracoscopy-treated dogs for samples obtained 0.25, 2, and 24 hours after extubation. Examination of thoracic radiographs obtained 2 and 24 hours after thoracoscopy revealed minimal localized pathologic changes. All dogs were clinically normal 24 hours after thoracoscopy, and major postoperative complications were not detected. Gross and histologic findings of specimens obtained during necropsy revealed changes localized to biopsy and trocar sites. CONCLUSIONS: Thoracoscopic placement of ligatures allowed procurement of lung lobe biopsy specimens from clinically normal dogs without complications. CLINICAL RELEVANCE: This procedure may provide a safe and minimally invasive means of obtaining lung biopsy specimens from clinically affected dogs.

Isolated right-ventricular hypertrophy associated with severe pulmonary vascular apolipoprotein A1-derived amyloidosis in a dog.

A 12-year-old dachshund was referred for respiratory distress, coughing, and weight loss. Cyanosis, dyspnea, tachypnea, and harsh lung sounds were noted on physical examination. Polycythemia with an increased number of nucleated red blood cells; right atrial enlargement; severe interstitial-to-alveolar pattern in all lung fields; and peripheral, echogenic, pulmonary masses were observed. Cytological examination of pulmonary aspirates indicated possible pulmonary carcinoma. The dog was euthanized at the owner's request. Isolated right-ventricular hypertrophy and pulmonary arteriopathy with amyloid deposits of apolipoprotein A1 were identified upon necropsy and histopathology. Pulmonary vascular amyloidosis should be considered in the differential diagnoses of respiratory distress in aged dogs.