Acute Infectious Hepatitis with Fulminant Hepatic Failure – A Study of 289 Cases
Deshpande, R.S., M.B., B.S., D.P.H. Medical Superintendent In-charge, Kasturba Hospital for Infectious Diseases, Sane Guruji Marg, Bombay, India
The neurotoxic factor may be responsible for the neurological symptoms. There may be deficiency or absence of one or more substances necessary for cerebral metabolism and synthesised by the normal liver or the presence in excess of one or more substances toxic to the brain which are detoxified or excreted by the normal liver but not destroyed by the liver with fulminant hepatic necrosis. It is not known which of these mechanisms is at work (Geiger and Yamasaki, 1956)18. The neurotoxic factor normally destroyed by the liver is ammonia (Benhamou et al., 1961)2. Proponents of the neurochemical theory of hepatic coma postulate that there is either a depletion of neurochemical transmitter or norepinephrine and dopamide in certain vital areas of the central nervous system - there are false neurochemical transmitters which displace biologically active neuro-transmitters from neuronal receptor sites (Fischer 1972)15. Some studies reveal the potential reversibility of the coma of hepatic failure. One important observation relates to the reversibility of the neurological disorder. However, it is wise to treat all attacks as potentially fatal and to insist upon rest in bed, but allow bathroom facilities.
If deep coma is reached, the prognosis is very poor. It may be that the virulence of the virus in these cases may be greater or the severity of the disease is dependent upon an abnormal reaction of the host. We also do not exactly know the role of immune reactions in the development of this fulminant illness. We also need to know the factors that promote regeneration of the liver and also whether sufficient regeneration of hepatic parenchyma is ever possible after a certain stage of hepatic necrosis has been reached (Sherlock and Parbhoo, 1971)39 (Willams, 1972)47.
Clinical Features
Hepatic failure may manifest itself in a number of ways. Hepatic encephalopathy is defined as a neuro-psychiatric syndrome, consisting of intellectual deterioration, altered state of consciousness and abnormal neurologic function in a patient with advanced liver disease (Raskin, 1964)34. In the beginning there may be inappropriate behaviour and agitation followed by progressive lethargy, stupor and coma. Hypoglycaemia, sepsis, electrolyte imbalance, central nervous system trauma and drug intoxication may precipitate precoma or coma. There may be hyperventilation, metabolic encephalopathy, hypoglycaemia and uraemia. In these cases, the immediate problem is widespread and continued loss of liver cells and the resultant encephalopathy is a preterminal event. Drowsiness and coma may be due to failure of the respiratory and vasomotor centres. Unless effective respiration and circulation are maintained further anoxia and cerebral damage are inevitable. Recognition that the illness is progressing toward the severe fulminant stage of massive hepatic necrosis is an indication for immediate intensive care.
The following grades in the development of precoma and coma are described:
Grade I : Minor disorder of consciousness (motor system).
Grade II : Gross disorder of consciousness and disorientation in time and space.
Grade III : Coma.
The stages of the development of hepatic coma can be used as a clinical index of the degree of the liver cell failure present and of the likely prognosis. Changes in mood, confusion or drowsiness are usually the earlier detectable signs. The characteristic-flapping tremor, which is present in the early stages, disappears when the patient sinks into coma. Signs of encephalopathy usually appear with-in a few days of the onset. The whole illness, from the first symptoms to death, may be less than a week. Less frequently the patients have jaundice for a long time–days or weeks–before the appearance of neurological signs.
The clinical picture is a complex one and affects all parts of the brain. The disorder is organic, mental-reaction associated, with a neurological disturbance. Variability is a marked feature. The features differ in activeness and in symptomatology depending on the nature and intensity of etiological and precipitating factors. Children show a particularly acute reaction often with mania. Headache, dizziness, nightmares are inaugural non-specific symptoms, but even before this, the patient may show episodes of antisocial behaviour or character disturbances. Unco-operative behaviour often continues with consciousness clouded. The delirium is of the noisy, restless variety and attacks of screaming occur spontaneously or may be induced by slight stimuli.
Violent behaviour is common in children who may also have fits. Insight into such anomalities of behaviour is frequently present. Speech is slow and slurred the voice monotonous and often faint. In deep stupor dysphasia becomes marked and is always combined with perseveration. Deep tendon reflexes are usually exaggerated although patients during coma become flaccid and lose these reflexes. Plantar response is usually flexor becoming extensor in deep stupor or coma. The clinical course is fluctuant and frequent observation of the patient is necessary. Fetor hepaticus may be detected in the breath. Hyperventilation and hyperpyrexia may be terminal.
Hypersomnia appears early and progresses to inversion of sleep rhythm. Reduction of spontaneous movement, a fixed stare, apathy, slowness and brevity of response are early signs. Further deterioration results in reaction only to intense or noxious stimuli.
Coma at first resembles normal sleep but progresses to complete unresponsiveness. Deterioration may be arrested at any level with either regression or persistence of symptoms. Rapid changes in the level of consciousness are accompanied by delirium.
Personality changes are most conspicuous in association with chronic liver disease. Intermittent changes include childishness, irritability and loss of concern for the family. Intellectual deterioration varies from slight impairment of organic mental function to gross confusion. Daily fluctuations may be observed. Focal defects appearing in a setting of clear consciousness relate to disturbances in visual spatial gnosis. These are most easily elicited in the motor sphere as constructional apraxia shown by inability to reproduce simple designs–size, shape, structural function – Astereognosis. Changes in the pattern of handwriting are a good check of progress.
The psychiatric syndrome is an acute organic reaction with delirium, disorientation and visual hallucinosis and clouding of consciousness rapidly progressing to coma within hours or days. Mental abnormalities may develop before jaundice or the syndrome may appear when the patient is deeply jaundiced. Tremor may be transient or overlooked, if not carefully looked for.
If deep coma is reached, the prognosis is very poor. It may be that the virulence of the virus in these cases may be greater or the severity of the disease is dependent upon an abnormal reaction of the host. We also do not exactly know the role of immune reactions in the development of this fulminant illness. We also need to know the factors that promote regeneration of the liver and also whether sufficient regeneration of hepatic parenchyma is ever possible after a certain stage of hepatic necrosis has been reached (Sherlock and Parbhoo, 1971)39 (Willams, 1972)47.
Clinical Features
Hepatic failure may manifest itself in a number of ways. Hepatic encephalopathy is defined as a neuro-psychiatric syndrome, consisting of intellectual deterioration, altered state of consciousness and abnormal neurologic function in a patient with advanced liver disease (Raskin, 1964)34. In the beginning there may be inappropriate behaviour and agitation followed by progressive lethargy, stupor and coma. Hypoglycaemia, sepsis, electrolyte imbalance, central nervous system trauma and drug intoxication may precipitate precoma or coma. There may be hyperventilation, metabolic encephalopathy, hypoglycaemia and uraemia. In these cases, the immediate problem is widespread and continued loss of liver cells and the resultant encephalopathy is a preterminal event. Drowsiness and coma may be due to failure of the respiratory and vasomotor centres. Unless effective respiration and circulation are maintained further anoxia and cerebral damage are inevitable. Recognition that the illness is progressing toward the severe fulminant stage of massive hepatic necrosis is an indication for immediate intensive care.
The following grades in the development of precoma and coma are described:
Grade I : Minor disorder of consciousness (motor system).
Grade II : Gross disorder of consciousness and disorientation in time and space.
Grade III : Coma.
The stages of the development of hepatic coma can be used as a clinical index of the degree of the liver cell failure present and of the likely prognosis. Changes in mood, confusion or drowsiness are usually the earlier detectable signs. The characteristic-flapping tremor, which is present in the early stages, disappears when the patient sinks into coma. Signs of encephalopathy usually appear with-in a few days of the onset. The whole illness, from the first symptoms to death, may be less than a week. Less frequently the patients have jaundice for a long time–days or weeks–before the appearance of neurological signs.
The clinical picture is a complex one and affects all parts of the brain. The disorder is organic, mental-reaction associated, with a neurological disturbance. Variability is a marked feature. The features differ in activeness and in symptomatology depending on the nature and intensity of etiological and precipitating factors. Children show a particularly acute reaction often with mania. Headache, dizziness, nightmares are inaugural non-specific symptoms, but even before this, the patient may show episodes of antisocial behaviour or character disturbances. Unco-operative behaviour often continues with consciousness clouded. The delirium is of the noisy, restless variety and attacks of screaming occur spontaneously or may be induced by slight stimuli.
Violent behaviour is common in children who may also have fits. Insight into such anomalities of behaviour is frequently present. Speech is slow and slurred the voice monotonous and often faint. In deep stupor dysphasia becomes marked and is always combined with perseveration. Deep tendon reflexes are usually exaggerated although patients during coma become flaccid and lose these reflexes. Plantar response is usually flexor becoming extensor in deep stupor or coma. The clinical course is fluctuant and frequent observation of the patient is necessary. Fetor hepaticus may be detected in the breath. Hyperventilation and hyperpyrexia may be terminal.
Hypersomnia appears early and progresses to inversion of sleep rhythm. Reduction of spontaneous movement, a fixed stare, apathy, slowness and brevity of response are early signs. Further deterioration results in reaction only to intense or noxious stimuli.
Coma at first resembles normal sleep but progresses to complete unresponsiveness. Deterioration may be arrested at any level with either regression or persistence of symptoms. Rapid changes in the level of consciousness are accompanied by delirium.
Personality changes are most conspicuous in association with chronic liver disease. Intermittent changes include childishness, irritability and loss of concern for the family. Intellectual deterioration varies from slight impairment of organic mental function to gross confusion. Daily fluctuations may be observed. Focal defects appearing in a setting of clear consciousness relate to disturbances in visual spatial gnosis. These are most easily elicited in the motor sphere as constructional apraxia shown by inability to reproduce simple designs–size, shape, structural function – Astereognosis. Changes in the pattern of handwriting are a good check of progress.
The psychiatric syndrome is an acute organic reaction with delirium, disorientation and visual hallucinosis and clouding of consciousness rapidly progressing to coma within hours or days. Mental abnormalities may develop before jaundice or the syndrome may appear when the patient is deeply jaundiced. Tremor may be transient or overlooked, if not carefully looked for.
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