2. Objectives
• Case report-problem
• Defining the clinical condition
• Pathogenesis
• Diagnosis
• Clinical staging of RS
• Case report-solving the problem
3.
4. Undiagnosed death till…
• Off and on fever-antipyretics taken
• Progressive pallor and weakness developed
• epistaxsis
• Recommended to PIMS
• Enlarged liver
• APTT prolonged ( activated partial thromboplastin time)
• LDH increased
Keeping in view of possibility of tuberculosis and other infections, she was started on
.
injectable steroids, transamine, zantac and Leflox She was transfused three packs
of red cell(against pancytopenia) concentrates but still her condition did not improve.
• Urinary incontinence( involuntary leakage of urine) developed;
• Conditions deteriorated further and oxidative stress developed, transferred to ICU
5. CONTND…
However her condition continued to
decline with persistent low blood pressure
and weak pulse. Her right pupil became
fixed and dilated while left was semi
dilated and reactive. The patient went into
cardiac arrest and died on the same day
of being transferred To ICU.
Liver biopsy taken and sent for fungal and
tuberculosis culture as well as for
histopathology
• surprisingly both were negative.
• histopathological findings and other lab
work rendered the diagnosis as
compatible with Reye’s syndrome.
6. •Reye’s syndrome is a very rare condition that
causes serious liver and brain damage. If it is not
treated promptly it may result in permanent brain
injury or death.
•Reye’s syndrome is characterized by acut non-
inflammatory encephalopathy and fatty degeneration of
viscera was first described as a distinct entity in 1963 by
R. D. Reye
7. Pathogenesis
•Precise reason still not known BUT links with use of aspirin in children after the viral
infection has been linked with it (biphasic disease )
• It is now not recommended to give ASA to kids 18 and under without doctor’s
approval
However 4 concepts are prominent
1. An intrinsic toxin that affects mitochondrial metabolism
2. An extrinsic toxin that alters the host's response during recovery from a viral illness.
3.A genetic susceptibility to Reye's syndrome following exposure to a specific viral or
viral-like disease.
4. A primary defect in lipid and ammonia metabolism that results in cerebral edema and
coma following viral or viral-like exposure.
Current studies have demonstrated the transient disturbances in mitochondrial function
in Reye's syndrome.(mitochondrial permeability transition )
The pathogenesis is unclear, but it appears to involve
mitochondrial dysfunction that inhibits oxidative phosphorylation and fatty-acid beta-
oxidation in a virus-infected, genetically sensitized host.
8. Diagnosis
• Diagnosis can’t be established w/o a liver biopsy
• Centers for Disease Control and Prevention (CDC) have developed
criteria for diagnosis of Reye’s syndrome and include
Firstly, the presence of an acute non inflammatory encephalopathy
with an altered level of consciousness with no signs in CSF or in
brain histology to indicate infection or inflammation
. The second criteria is hepatic dysfunction with a liver biopsy
showing fatty change or a more than 3-fold increase in alanine
aminotransferase (ALT), aspartate aminotransferase (AST), and/or
ammonia levels
Thirdly there should be no other explanation for the condition
9.
10.
11. The answers ???
Case report
Off and on fever-antipyretics taken
13. The answers ???
Case report
•Enlarged liver ??
• APTT prolonged ( activated partial
thromboplastin time)
•LDH increased??
14. The answers??
Case report
• Oxidative stress ??
Hint
cellular respiration…
15. Reye’s result of PIMS’ patient: mystery solved
• Suspected clinical diagnosis of T.B could not be proven on
thorough investigations
• The fact that favored the diagnosis of Reye’s syndrome is the
sudden onset of the symptom complex that lead to death within a
week of presentation
• It can be speculated that the child had suffered from a viral
infection which was probably superimposed on an inborn metabolic
defect that was never suspected and hence never investigated as
is usually the case.
• Also the child had off and on history of intake of antipyretics and as
everyone has an easy access to aspirin, this may have been the
triggering event.
• Finally, the liver biopsy showed marked micro vesicular fatty
change that further supported this diagnosis.
• As mentioned earlier, the Reye’s syndrome is a diagnosis of
exclusion and as no other explainable cause of hepatic dysfunction
was found.
16.
17. References
• Reyc's syndrome: a clinical review John F.S. Crocker,
Md, Frci4c]; Philip C. Bagnell, Md, Frcp[c]
• Reye’s Syndrome: Diagnosed on a PostmortemNeedle
Liver Biopsy in an Unexplained Death Ambreen
Moatasim, Anwar Ul Haque and Humeira
RizwanDepartment of Pathology, Pakistan Institute of
Medical Sciences, Islamabad.
• Reye RDK, MorganG, Baral J. Encephalopathy and fatty
degeneration of the viscera, a disease entity in
childhood. Lancet 1963; ii: 749-52.
• Reye Syndrome and Reye-Like Syndrome Jayaprakash
A. Gosalakkal, MD and Vishwanath Kamoji, MD
Notas del editor
Reye RDK, MorganG, Baral J. Encephalopathy and fatty degeneration of the viscera, a disease entity in childhood. Lancet 1963; ii: 749-52.
Reyc's syndrome: a clinical review John F.S. Crocker, Md, Frci4c]; Philip C. Bagnell, Md, Frcp[c] ASA: acetylsalicylic acid
Reye’s Syndrome: Diagnosed on a PostmortemNeedle Liver Biopsy in an Unexplained Death Ambreen Moatasim, Anwar Ul Haque and Humeira RizwanDepartment of Pathology, Pakistan Institute of Medical Sciences, Islamabad.