A child died of what he was treated for, but... Patient Victoria, 10 years old, was admitted to the Republican Children's Infectious Diseases Hospital Simferopol 09.10.2013 year with a diagnosis of acute intestinal infection, gastroenteritis, encephalopathy. Complaints on admission on the increased 

В статье описан нетипичный клинический случай синдрома Рейе у девочки 10 лет, возникший на фоне ОКИ, приведший к летальному исходу. На основании клинических и лабораторных данных, а также анамнеза жизни ребенка проведена краткая дифференциальная диагностика синдрома Рейе, эпилепсии и энцефалита, а также состояний, сопровождающихся нарушением функции нервной системы и печени: фульминантной печеночной недостаточности, панкреатической энцефалопатии, менингита, врожденных ошибок метаболизма.

The abdomen was soft, painful on palpation in the upper part, rumbling along the cecum.Liver palpable 2 cm below the costal margin, the edge of the liver and its surface is smooth, tight-elastic consistency.Spleen was not palpable.Stool at admission was not.Urinary diversion for urinary catheters.
Neurological status at admission: level of consciousness -stupor -delirium.
Periods of excitement replaced by lethargy, drowsiness expressed.Meningeal signs are negative.Hyperesthesia of the skin.Pupils D = S, photoreaction saved.Symmetrical face.Physiological muscle tone, tendon reflexes were brisk, uniform.

Conclusion: cryptogenic epilepsy? Encephalitis?
MRT a brain with i/v amplification: Conclusion: organic changes in the brain before and after I/v amplification is not revealed.Cyst in the pineal gland.
On the second day of the admitted to the hospital the patient's condition worsened due to the deepening of neurological symptoms to coma I degree with rapid progression to coma II, then III degree, absence of reaction to painful stimuli, hypotonia, hyporeflexia.
Flagrant violation of homeostasis: there -hematinics vomiting, then developed bleeding, not to stop medication.
Progressive inhibition of the function of respiration and cardiac activity.
Reanimation measures -without effect, 12.10.2013 in 20 hours 30 minutes pronounced biological death.

Pathological examination: autopsy:
-expressed brain edema with herniation of the cerebellum in the foramen magnum.
-enlarged liver, bright yellow, dense m 2340 gr (n 1100 gr) extend below the costal margin, the cut liver tissue stearic -yellow, with a greasy luster.
-kidneys on the cut yellow color, with a greasy luster.
At histological study.
-Liver: total fatty dystrophy of hepatocytes with impaired liver structure -Kidney lipophanerosis tubular epithelium.Disorders of intestine manifested only with vomiting, which in the beginning became repeted, and then -persistant.But a fully-developed gastroenteritis or gastroenterocolitis were absent as there were no diarrhea.In CBC there was no evidence of bacterial infection, and the stool test showed minimal changes of the intestine.
While neurological symptoms worsened in persistence and severity we suspected the beginning of cryptogenic epilepsy together with acute intestinal infection (vomiting leave unattended impossible), especially taking into account the data history of episodes of memory loss in 4 and 9 years old.
Summarizing the available data, including the results of post-mortem studies, despite some inconsistencies, we should accept the conclusion of "coincidences of diagnoses."After all.
A postmortem conclusion for the doctors is a positive proof of qualification.
But the final result of treatment, even in the most hopeless cases is unacceptable, like it was in this case.On the one hand the whole course of the disease and the development of pathological process (in general) logical, and its logical outcome (to date).The above discrepancy between history, clinical, laboratory and pathologic data bring the great professional dissatisfaction.
These discrepancies, which began back in "deep " maternal history (the first child because of status epilepticus without post-mortem examinations, the similar episodes of loss of consciousness in a child of 4 and 9 years), clinical and pathological inconsistencies make you think what is the core of it -that deep, as yet unknown mechanisms to us that in such situations lead to death.There is a possibility that the child's metabolic or genetic disorders of brain, liver and other organs, but the modest technical ability did not allow us to answer those questions.
But such mechanisms are to know is our top priority.

For the benefit of epilepsy testified:
1. Anamnesis data: the age of 4 and 9 years old were observed short loss of consciousness, at 6 age had cerebral paroxysm (syncope, transient loss of consciousness).
2. Burdened family history: the first child in the family died, according to parents, against status epilepticus (postmortem studies have been conducted).

Against the diagnosis of "epilepsy."
1. Infection history: the autumn period, acute onset, fever to febrile digits, repeated vomiting, violation of the stool.
2. Progressive deterioration: depression status until coma, impaired hemostasis, growth of liver failure.
However, the rapid dynamics of neurological picture when agitation or retardation changed stupor, coma and then, forced to think about encephalitis.

Against the diagnosis of "encephalitis" was:
1. Absence of clinical symptoms ARVI.b) an increase in the peripheral blood levels of ALT and AST, with a tendency to increase; c) the severity, speed of development and incurable DIC syndrome.
Thus, the direction and weighting the clinical picture in this child, who had early signs of AID, was not due to any epilepsy or encephalitis.
Further differential diagnostic search included pathology, accompanied by a disturbance of the nervous system and liver: fulminant hepatic failure, pancreatic encephalopathy, meningitis, inborn error of metabolism, Reye's syndrome.
Naturally, following the dictates of time, the child was excluded HIV infection and.As for the rest, the arguments were as follows.
Fulminant hepatic failure.It may be associated with infection (hepatitis), or intoxication with drugs (salicylates, acetaminophen) and toxic substances (isopropyl alcohol and valproate).However, neither the history nor the clinic or laboratory studies have not confirmed these thoughts.However, against Reye's syndrome showed the following data:
Absence of this child symptoms of ARVI or any other bacterial and viral diseases.

2.
Reye's syndrome usually develops after reception of salicylates, this child did not take them.7. Hepatomegaly modest rates determined during the life (2 cm below the costal margin, while in Reye's syndrome has been a significant increase in the liver in 60% of cases).At autopsy the liver was increased twice and stands out from under the rib 7 cm.
8. Relatively slow development of coma.At Reye syndrome coma develops within the first day, and that is a bad prognostic sign.In this case, coma is developed on day 4.
-family intestinal pathogenic bacteria were detected.7. Bacteriological sowing of CSF -single colonies Staphylococcus epiclermiclis.Inspection oculist: pupils wide, OD> OS, slow photoreaction, ocular fundus -discs pale pink, clear boundaries, narrowed arteries, veins moderately expanded retina without apparent pathology.Examination by a neurologist: level of consciousness -stupor, to inspect reacts cry.In the contact does not interfere.Meningeal signs are negative.S = D pupils dilated, photoreaction questionable.Nasolabial folds symmetrical.Distonichen muscle tone.Tendon reflexes S = D, live.Abdominal reflexes are absent.
Morphological manifestations are typical for gastroenterocolitis -Signs of DIC; stasis and sludge in the microvasculature, bleeding in parenchymal organs, lung, intestinal bleeding -Stomach, intestines -desquamative changes in mucosal lymph leukocyte infiltration of all layers, hyperplasia of lymphoid follicles.'s syndrome (toxic encephalopathy, total fatty liver, kidneys).I. 2 gastroenterocolitis (bak.sowing № 58-64 E. faecalis) Complications: Brain edema with herniation of the cerebellar tonsils into the foramen magnum, DIC -syndrome, stasis and sludge erythrocytes in the blood vessels of the microvasculature, lung, stomach bleeding, bleeding in the spleen, kidneys and lungs.Venous congestion and parenchymatous degeneration of internal organs.The coincidence of diagnoses.Histological examination of autopsy revealed: total fatty degeneration of hepatocytes, swelling, degenerative changes of neurons in the brain, fatty epithelium of the convoluted tubules of the kidneys, and also inflammatory infiltration in the wall of the stomach and intestines.On the basis of the clinic, autopsy and histological examination of autopsy material was exposed anatomicopathological final diagnosis, consisting of two the competing diseases.I.I.Sindrom Reyes.I.II.Gastroenterocolitis.Each of these conditions could lead to the death of the child.Direct cause of death were cerebral edema with herniation of the brain tonsils in the foramen magnum and DIC -syndrome.The coincidence of diagnoses.Discussion of the clinical diagnosis.Clinical manifestations of disease onset did not cause in doubt in the diagnosis -autumn season, acute onset, obvious symptoms of gastroenteritis surely testified about the development of acute intestinal infection.Neurological disorders affirmed the severity of the pathological process.Data about short-term episodes of loss of consciousness in 4 and 9 years old at the initial stage of observation did not influenced the diagnosis and was left for later discussions.However, in the first day in the ICU the diagnosis of acute intestinal infections was doubted.First of all it was because the clinical manifestations of intestinal infection did not the severity of neurological status.

2.
The presence (though such) clinical manifestations of AID (encephalitis thus happens only in severe forms, but rarely).3.Absence inthe clinical picture of focal neurological symptoms.4. No change in the analysis of CSF. 5. Absence on brain MRI signs of focal lesions of the brain substance.6.The rapid development of changes in the body, is not characteristic of encephalitis: a) a progressive increase in liver size and density as determined by palpation.
encephalopathy.It is a rare disease characterized by confusion during an attack of acute pancreatitis.Anamnesis of disease and clinical manifestations of the disease (absent of sharp pain in the upper abdomen and nausea, disorders of the stool) and normal indicators of amylase and lipase in the blood serum, allowed to exclude this pathology.Infectious lesions of CNS.Viral and bacterial meningitis or encephalitis not supported by clinical manifestations and laboratory investigations.Inborn error of metabolism.The possible presence of inborn errors of metabolism should be borne in mind in young children with Reye's syndrome and in family cases and relapses.Metabolic defects in the urea cycle disorders (enzyme deficiency), disorders of the organic acids (glutaric aciduria, and isovaleric acidemia) and systemic carnitine deficiency.In cases where clinical and laboratory data do not confidently make a differential diagnosis, is crucial liver biopsy.No family history or age of the child, the lack of full-length clinical manifestations and, of course, any inability to determine the level of organic acids, led us to abandon these arguments.But doubts remain.And, last but not the least, considering all available anatomical, clinical and laboratory data have suggested the presence of Reye's syndrome.For the benefit Reye's syndrome testified: 1.Early development of toxic encephalopathy, passed into a coma without increase (worsening) of the initial symptoms of AID, which began with the disease 2. During the lifetime of a clinically determined moderate enlargement of the liver (2 cm) having a dense texture (at necropsy the liver was increased in two times).3. Hypoglycemia -1.35 mmol glucose / liter.4. Resistant and repeated vomiting throughout the period of illness. 5. Excitement, alternating with inhibition, further transformed into a coma.6. Fatty liver.7. Fatty degeneration of tubular epithelial 8. Having a clear clinical picture of the disease (AID), able to "run" mechanism of the development of Reye's syndrome.
3.the absence In life of clinically definite of brain edema: a) no change the eye fundus: pale pink discs, clear boundaries, narrowed arteries, veins moderately dilated, the retina -for no apparent pathology.At Reye syndrome, cerebral edema when expressed on the 4th day of illness onset should be expressed in changes the eye fundus: dilated veins, artery spasms, retinal edema, blurred boundaries.b) MRI -the data for the presence of cerebral edema are absent.c) low pressure liquor.Lumbar puncture CSF was clear, flows slowly drops.At Reye syndrome always emphasized cerebral edema and intracranial pressure increased by 20% or more, the level of protein in normal, in our caseincreased.d) brain edema and DIC -syndrome defined posthumously.4. Absence seizures.5.No marked increase in transaminase levels: AST-1.0 mmol / L (normal -0.68), ALT -0.89 mmol / L (normal -0.68), with Reye's syndrome increases in transaminase levels 3 or more times.6.A history of episodes of loss of consciousness in 4 and 9 years old, similar to the clinic on the present state (only without the intestinal syndrome).