March 28, 2023


So here we are going to talk about the rails syndrome it is also known as rails johnson center so a i mean real syndrome and acute condition and that causes the swelling in the brain slowly in the brain and inflammation of the liver or swelling in the liver swelling the brain we call encephalopathy and swelling the liver we call hepatitis so courses by using

Aspirin to treat viral infections particularly blue or chickenpox so this is an acute encephalopathy that follows a viral illness and it is characterized pathologically by cerebral edema and fatigue change in the liver yeah definitive diagnosis is made by liver biopsy so real syndrome and non-inflammatory encephalopathy or fatigue change in the liver so maybe

Maybe diagnosed with a viral people or flu and you give baby like aspirin or explain continuously silent and patient develop swelling in the brain and swelling the liver that what are the pathology hepatic my mitochondria injured because of increase the ammonia level this is the pathology the accurate course is not clear the most commonly follow viral illness such

As influenza or varicella the administration of a spleen and aspirin containing solicit is not recommended for the children with the febrile illness or children with barisula or influenza because of its association with real syndrome acetaminophen or ibuprofen is usually prescribed for pediatrics clients so acetaminophen and ibuprofen no problem aspirin or aspen

Containing salicylate maybe problem this is good not to recommend them so what are the risk factors definitely recent viral infection specially influenza or use of aspirin is a risk factor early diagnosis and aggressive treatment is important the goal of treatment is to maintain effective cerebral perfusions and control increase intracranial pressure in this picture

We see the progressive encephalopathy with hepatic dysfunction so like virusala or influenza a and b and patient is treated or ticked salicylic salicylate or aspirin is the risk factor and patient develops after several days after recovery suddenly develops swelling in the liver and brain we called fulminant hepatitis so what is permanent hepatitis hepatitis is

Very uncommon syndrome of missive necrosis or liver parent climber and also a decrease the liver size it means acute yellow atropy that is usually occur after infection with certain hepatitis for hepatitis viruses exposed to the toxic agents or drug-induced injuries also cerebral because of several edema patient altered level of consciousness or behavior change

What are the other risk factors in bone error of metabolism medium chain acetylico enzyme dehydrogenase deficiency or other fatty acid oxidation disorder urea cycle disorder so this is another genetic risk factors so rice syndrome it is extremely serious serious pathological condition associated with swelling the liver and brain and in this picture we can say

The baby present with the skin rash so what are the other side symptoms sometimes patients preserve the vomiting confusion coma caesar and death so let me read it history of systemic viral illness four to seven days before the onset of symptom sometimes gi upset like nausea vomiting malaise also confusion sign of altered level hepatic functions such as lethargy

Progressive neurological deteriorate increased blood ammonia levels so altered level of consciousness hmm so what are the laboratory tests basically we allow the patient libert biopsy and increase and if we check the cbc we can see or liver function test increase a l t a l t bilirubin level ammonia level or also lumbar puncture or central csf allies is to rule

Out meningitis so it is not meningitis just for rule out so what are the treatment option so mainly supportive treatment also monitor for increase the intracranial pressure or intervention and mechanical ventilation is if needed so what are the nursing intervention we provide rest and decrease the stimuli in the environment monitor neurological status motor for

Or altered level of consciousness and sign of increase intracranial pressure monitor for sign of water altered hepatic function and result of liver function studies monitor intake and output and monitor for sign and bleeding a sign of bidding and sign of impaired coagulation such as prolonged bleeding time it is good to elevate head of the bad to 30 degree if

Patient has a cesar who need to scissor precursor because chance to develop cerebral edema can lead to caesar so monitor import and output and prevent any kind of hemorrhoids if present so due to the impaired liver function hemorrhage is not abnormal also avoid use of silly salad because silly salad or aspirin is a risk factor in case of children

Transcribed from video
Pediatrics Reye's Syndrome By Evolution Health Academy