Age: 9.0
Sex: F
Vaccine: VARCEL
Symptoms: Ascites, Drug ineffective, Glomerulonephritis, Hepatic necrosis, Hepatitis
Clinical narrative: Information has been received from a physician concerning a 9 year old female who in approximately 1998 at the approximate age of 12-15 months was vaccinated with a dose of varicella virus vaccine live. It was noted that the child had been healthy until about two to three years ago (approximately 2001 to 2002) when she developed chronic glomerulonephritis. It was also noted that she had chronic renal insufficiency. Her condition was relatively stable, however, "shortly after Christmas in early January," she had an exacerbation of her glomerulonephritis. Her physician was concerned about her decreasing renal function adn treated her with a bolus of steroids (also reported as "was placed on very high dose of steroids"). About one week prior to receiving the steroids, she had been exposed to a child who had chickenpox. Subsequently, the pt developed severe varicella, although clinically had few skin lesions (also reported as a case of breakthrough chickenpox). About one week after receiving the steroids, the child was hospitalized with a rapidly progressive severe hepatitis that progressed to hepatic necrosis. It was also reported that the pt developed pneumonia. In approximately February 2004, "about a month ago," the pt died of a disseminated varicella infection with the primary mode of death being from the hepatitis (hepatic necrosis). Post mortum evaluation revealed that the liver had intra nuclear inclusion bodies in the hepatic cells. A VZV virus was identified in her liver and kidneys. Upon internal medical review chronic renal insufficiency, chronic glomerulonephritis, hepatic necrosis and pneumonia were considered to be other important medical events (OMIC). Additional info has been requested.   Follow-up from physician on 6/22/04: "There is no documentation of varicella immunization and therefore no reasonable evidence that a vaccine failure has occurred." Follow up on 07/19/04 states: "Follow-up information from the physician indicated that the pt died at the hospital on 01/12/04 following an acute, severe illness characterized by a progressive papulovesicular rash that first appeared on 01/05/04, hepatitic failure and diffuse coagulopathy. The pt had an underlying ANA positive glomerulonephritis that was first diagnosed in Feb 2002 and had been maintained in good control with low doses of immunosuppressive therapy. On 12/25/03 she developed an acute exacerbation characterized by gross hematuria, periorbital edema and rising creatinine. She had been generally well prior to this time except for a history of a persistent cough for about two months and a recent febrile illness diagnosed as mononucleosis on the basis of a positive monospot test on 12/13/03.  She received a brief course of prednisone in a dose of 60mg/day for 4 days as treatment for mononucleosis. Subsequently she had significant indoor exposure to chickenpox on 12/22/03.  On 12/28/08 she was admitted to the hospital and started on a three day course of IV methylprednisolone sodium soccunate (solu-medrol) at a dose of 500mg per day. Despite a rocky course requiring admission to the PICU for management of hypertension, electrolyte abnormalities, and a diminishing renal function, she slowly improved and was discharged on 01/04/04. She remained relatively stable at home until readmitted on 01/08/04 with her terminal illness. The DFA stain obtained on 01/08/04 from one of the vesicular lesions was negative for VZV and HSV antigen. However her progressive illness was characteristic of disseminated varicella and this diagnosis was confirmed postmortem by demonstration of VZV DNA in the kidney and other tissues. Throughout her chart is was recorded by several physicians that her immunizations were "up to date" and it was noted that she had receoved varicella vaccine at some point in the past. The pt's primary care physican indicated that she did not receive the varicella vaccine at his practice where she was a pt a since birth. Her hospital medical record contains an immunization record with no enteries. The pt's father and mother thought that the pt received the chickenpox vaccine but they could not recall which provider or what medical institution and they did not have an immunization record at home. Follow-up from physician states: "There is no documentation of varicella immunization and therefore no reasonable evidence that a vaccine failure has occurred."Additional follow up information indicated that neither the physician nor the parents were interested in pursuing any additional specimen analysis. The physician reported that the pt had a known exposure to wild type varicella and that most certainly this was a case of wild type varicella in a child who had not been vaccinated. No further information is expected." 7/7/05 Autopsy report received which revealed COD to be glomerulonephritis, varicalla infection, septic shock, DIC, pulmonary edema & effusions, LV ischemia & infarct, ascites, HTN and cardiac hypertrophy.