How could hypoglycemia-inducing glycogen storage disease lead to hyperglycemia-induced mucormycosis?

Larry Nichols,Diana Alejandra Rios

doi:10.4322/acr.2020.149

Abstract

Mucormycosis is an increasingly frequent, difficult to diagnose, difficult to treat, often fatal infection, especially in patients with hyperglycemia from uncontrolled diabetes. Type I (von Gierke) glycogen storage disease is due to inherited deficiency of enzymes in glycogen metabolism, which causes hypoglycemia. This report is the case of a patient with von Gierke disease and a missed diagnosis of pulmonary mucormycosis. This report illustrates the importance of having a high index of suspicion for mucormycosis in the appropriate clinical context.

Highlights

Mucormycosis is an increasingly frequent, difficult to diagnose, difficult to treat, often fatal infection, especially in patients with hyperglycemia from uncontrolled diabetes
The patient had received a living-related renal transplant at age 15 for renal failure attributed to her glycogen storage disease
Bleeding from left upper lobe lung, with blood pouring into the endotracheal tube, seems to have precipitating the patient’s terminal cardiopulmonary arrest. This pulmonary bleeding was related to angioinvasive pulmonary mucormycosis

Summary

Introduction

Mucormycosis is an increasingly frequent, difficult to diagnose, difficult to treat, often fatal infection, especially in patients with hyperglycemia from uncontrolled diabetes. This report illustrates the importance of having a high index of suspicion for mucormycosis in the appropriate clinical context This 20-year-old woman from the Northeast USA had type I (von Gierke) glycogen storage disease and was transferred to a referral hospital for liver transplant evaluation. The patient had increasing transaminases, prothrombin time, partial thromboplastin time and lactate An infusion of dopamine was started for blood pressure support She was given platelets, packed red blood cells, fresh frozen plasma, cryoprecipitate and desmopressin. She was transferred to a referral hospital on postoperative day 7 for liver transplant evaluation. Arterial line catheter tip culture from admission yielded Enterococcus faecium and Enterococcus faecalis

Results

Discussion

Conclusion