Internal Medicine

0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:16

1004 Mucopolysaccharidoses Mucormycosis

■atlanto-axial subluxation

■aspiration pneumonia

■heart failure

■obstructive apnea

■graft-versus-host disease and autoimmune hemolytic anemia after

BM transplant

■difficulty with surgery (25% difficult intubation, 8% fail intubation

in general; 54% difficult intubation, 23% fail intubation in MPS

IH)

Prognosis

■progressive impairment

■slowly progressive forms (MPS III) may survive into adulthood, but

most have early demise (<10 years)

■peak of intellectual function 2–4 years in most patients, after which

there is regression

■death secondary to pneumonia, cardiac dysfunction

MUCORMYCOSIS

RICHARD A. JACOBS, MD, PhD

history & physical

History

■Name given to several opportunistic infections caused by fungi of the

Mucorales order (Rhizopus, Mucor,AbsidiaandCunninghamella)

■Think of the risk factors to assess susceptibility: diabetes mellitus

➣particularly diabetic ketoacidosis, chronic renal failure, steroids,

other immunosuppressive therapy

■Ubquitous fungi; exposure is common

Signs & Symptoms

■Can be classified into several different presentations based on

anatomy: rhinocerebral, pulmonary, cutaneous, GI, CNS, other sites

➣Disseminated disease is also possible

➣Black eschars and discharge are clues to diagnosis

■Rhinocerebral:

➣Usually found in diabetic patients, neutropenic cancer patients

➣Pts complain of headache, facial pain, fever.

➣If eye involved, may see proptosis, orbital cellulitis, conjunctival

swelling