Final Diagnosis -- Disseminated Fungal Sepsis (Complete Heart Block)
- FINAL DIAGNOSES:
- History of Short Gut Syndrome
- Severe Jejunoileal Atresia, Treated with Total Parenteral Nutrition Since Birth
- Status Post Multiple Intestinal Resections
- Status Post Colostomy and Closure
- Status Post Gastrostomy Tube Placement
- Status Post Cholecystectomy Secondary to Cholelithiasis
- Cholestatic Liver Disease Consistent with Hyperalimentation Effect,
Diagnosed by Liver Biopsy
- Hepatomegaly with Bridging Fibrosis, Cholestasis, and Steatosis
- Status Post Small Intestinal Transplantation
- Multiple Episodes of Cellular Rejection
- Epstein-Barr Virus Infected Cells in Allograft Biopsies
- Extensive Fibrous Adhesions of Allograft Small Intestine
- Marked Thinning of Allograft Intestinal Wall with Mucosal Attenuation
- All Intestinal and Vascular Anastomoses Intact
- Polymorphous Posttransplant Lymphoproliferative Disease Involving
- Allograft Small Bowel Serosa and Mucosa
- Status Post Cadaveric Renal Transplantation
- History of Renal Insufficiency
- Vascular and Ureteroneocystostomy Anastomoses Intact
- Acute Tubular Necrosis, Allograft Kidney
- Small Native Kidneys (combined weight 75 grams, 165 expected)
- Glomerulosclerosis, Tubulointerstitial Inflammation, Fibrosis, and
Calcification, Native Kidneys
- Disseminated Fungal Sepsis (Aspergillus fumigatus Cultured in Life and
at Autopsy) Involving:
- Atrial and Ventricular Myocardium
- History of Cardiopulmonary Arrest 48 Hours Prior to Death
- Left Lower Lung with Focal Hemorrhagic Consolidation
- Allograft Small Bowel
- Native Kidneys
- Allograft Kidney
- Diffuse Hemorrhage of Urinary Bladder Mucosa
- Focal Hemorrhage of Gastric Mucosa
- Focal Hemorrhage of Diaphragm
- Splenomegaly (160 grams, 73 grams expected) with Lymphoid Depletion
- Serous Ascites, 300 ml
- Serosanguineous Pericardial Effusion, 35 ml
- Changes Consistent with Chronic Asthma, Lungs, Bilateral
- Autopsy Limited to Chest and Abdomen
Comments:
The findings of fungal sepsis and lymphoproliferative disease, and absence of
rejection, strongly suggest that the patient was overimmunosuppressed at the
time of death. It is significant that while lymphoproliferative disease was
not diagnosed in life, multiple allograft biopsies contained Epstein-Barr
virus infected lymphocytes, as demonstrated by EBER in situ hybridization. The
PTLD found at autopsy was predominantly serosal, and would not have been
detectable by mucosal biopsy; the involvement of the mucosa by PTLD was rather
spotty and would easily have been missed. At the time of the patient's
admission for heart disease, sepsis was suspected, and tracheal cultures taken
at that time were subsequently positive for Aspergillus fumigatus, the
organism grown from various organs at autopsy. However, the extent of the
patient's fungal infection and the extensive lymphoproliferative disease were
not suspected in life.
Contributed by Paul S. Dickman, M.D. and Charles A. Richert, M.D.