TANGO-2 with Severe and Prolonged Rhabdomyolysis in a 2-year Old Male with Human metapneumovirus Infection. A case report and literature review.

Abstract

We report a unique case of prolonged acute rhabdomyolysis in a 2-year-old male with known TANGO-2 mutation with delayed peak in creatinine kinase levels secondary to human metapneumovirus infection. Creatinine kinase peaked at 424,760 U/l on day 9 of hospitalization. Resolution of rhabdomyolysis was achieved using aggressive management with intravenous fluids with optimal urine output and no kidney injury. TANGO-2 patients may develop severe and prolonged rhabdomyolysis with a delayed peak suggesting the need for prolonged inpatient hospitalization to prevent life-threatening complications.

Anesthetic Challenges in a Patient With TANGO2 Gene Deletion, DiGeorge Syndrome, and Tetralogy of Fallot: A Case Report

“…Though very little has been published on the perioperative care of these patients, the known inciting factors for acute metabolic crises (stress, dehydration, fever, infection, general anesthesia) are all frequently encountered in the operating room. 1,3,6 The potential for perioperative crises is therefore very high. In addition, while encephalopathy is a frequently encountered early sign of crisis, monitoring for signs of encephalopathy is very difficult in the intraoperative and early postoperative period given the effects of anesthesia.…”

“…Initial treatment includes ensuring adequate hydration, correction of metabolic acidosis, glycemic control, and management of end-organ complications including cardiac dysfunction, arrhythmias, and seizures (Table 2). [2][3][4]6 Of note, previous reports have documented cases of recalcitrant ventricular tachycardia which were unresponsive to medications and led to multiple patient deaths in this population. 3 This frightening reality emphasizes the need for early recognition and treatment of cardiac arrhythmias.…”

“…2 The resultant disease is characterized by long-term neurological decline, interspersed with acute metabolic crises during times of stress (Tables 1-2). 1-6…”

“…3 Reported triggers for crises include fasting, dehydration, infection, fever, and general anesthesia. 1,3,6 Metabolic crises are often a late finding, with a reported average age of first crisis of 2.8 years, preceded by chronic neurodevelopmental and motor delays. 1,8 However, this pattern is not universal, and crises have been reported as early as infancy.…”

“…2 The resultant disease is characterized by long-term neurological decline, interspersed with acute metabolic crises during times of stress (Tables 1-2). [1][2][3][4][5][6] Clinical sequelae during a metabolic crisis may include hypoglycemia, rhabdomyolysis, lactic acidosis, kidney injury, hypothyroidism, dysphagia, encephalopathy, seizures, and cardiac dysfunction. 1,3,7 Life-threatening arrhythmias are common during periods of metabolic stress, often starting with marked prolongation of the QT interval and progressing to torsades de pointe or ventricular tachycardia.…”