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Reye syndrome, a reversible metabolic encephalopathy and hepatopathy, offers a unique opportunity to investigate the pharmacologic mechanisms by which a toxic-metabolic insult to mitochondria is translated into neurochemical and neurologic dysfunction. Similarity of some clinical and metabolic abnormalities between certain inborn errors of organic acid, ammonia, and carbohydrate metabolism and Reye syndrome suggests a common pathophysiologic mechanism at some level. The primary metabolic aberration in Reye syndrome is unknown. Viral, drug, and toxic precipitants in a conductive host alter glial and neuronal function, possibly by direct toxic effects or by altered transmitter metabolism and signal transduction. These events translate into a rather stereotyped progression of the clinical syndrome. Increased ICP, which is a life-threatening epiphenomenon, is the focus of conventional therapy. Investigational treatments, still in preliminary stages, are aimed at early correction of instigating metabolic abnormalities or correction of their consequences on central neurotransmission. Our fragmentary knowledge of neurotransmitter abnormalities in this disorder, which have suggested disparate interpretations, does not allow a cohesive pharmacologic theory of Reye syndrome. The greatest difficulties in interpretation of possible central mechanisms from existing data, which derive largely from peripheral tissues, is in the differentiation of primary from compensatory changes. The unitarian notion that a single pharmacologic disturbance is the source of the encephalopathy is perhaps too simplistic. It is hoped that future studies of disorders such as Reye syndrome will elucidate the intricate relationships between biochemical pathways and neurotransmitter metabolism.
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PMID:Pharmacology of Reye syndrome. 284 66

Thirty-six of 68 consecutive patients with fulminant hepatic failure (FHF) progressing to grade 4 encephalopathy who had extradural ICP monitors inserted were reviewed to determine the safety and the value of ICP monitoring. Only minor complications were encountered. These included local wound bleeding at the burrhole site in four patients and a small cerebral hemorrhage in relation to the monitor in one other patient. No significant long-term sequelae were related to the operative procedure. ICP monitoring identified rises in ICP unaccompanied by clinical signs and as a consequence treatment was given to the monitored patients more often than the non-monitored group (median 6 vs. 2 treatments, P < 0.01). The duration of survival from the onset of grade 4 encephalopathy was significantly greater in the ICP monitored group (median 60 vs. 10 h, P < 0.01) although overall survival was unchanged. Monitoring also provided important prognostic information since the peak ICP was higher in non-survivors than in survivors (median 45 vs. 35 mmHg, P = 0.051). The pattern of clinical signs accompanying episodes of intracranial hypertension differed between survivors and non-survivors. Pupillary abnormalities were detected more often in non-survivors while systolic hypertension occurred more frequently amongst survivors with the peak systolic blood pressure being significantly higher. ICP monitoring proved safe and effective, provided valuable information regarding subclinical intracranial hypertension and prognosis and should be regarded as part of the routine management of intracranial hypertension complicating FHF.
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PMID:The safety and value of extradural intracranial pressure monitors in fulminant hepatic failure. 840 36

Viral hepatitis is the commonest cause of fulminant hepatic failure (FHF) in developing countries. We evaluated the early indicators of prognosis in these patients by multivariate analysis. The records of 204 consecutive patients with acute liver failure admitted with hepatic encephalopathy over five years were studied. The etiology of these patients included virus related in 186 (91.1%), drug induced in 15 (7.4%), Wilson's disease in one (0.5%), acute Budd-Chiari syndrome in one (0.5%), and malignant infiltration in one (0.5%). Patients with FHF complicating viral hepatitis were analyzed by univariate and multivariate analysis. These patients were further subclassified depending upon the interval between the onset of jaundice and the onset of encephalopathy into hyperacute (HALF; interval 0-7 days), acute (ALF; interval 8-28 days) and subacute liver failure (SALF; interval 4-12 weeks). Sixty (32.3%) patients with viral hepatitis survived. Univariate analysis showed that the interval between onset of encephalopathy and onset of jaundice, grade of encephalopathy, raised intracranial pressure, prothrombin time, and serum bilirubin levels on admission were related to outcome in these patients. Multivariate logistic regression analysis showed that the presence of raised intracranial pressure at the time of admission, prothrombin time >100 sec on admission, age (>50 yr), and onset of encephalopathy seven days after onset of jaundice were associated with poor prognosis. Forty seven (37.0%) of 129 patients with HALF survived compared with 9 (22.5%) of 40 with ALF and 4 (21.1%) of 19 with SALF (P = NS). Raised intracranial pressure was more frequent in patients with HALF (48.8%) than in patients with ALF (32.5%) and SALF (15.8%; P = 0.01), while clinically detectable ascites was more frequent in patients with SALF (78.9%) compared with HALF (19.7%) and ALF (37.5%; P < 0.0001). The factors adversely affecting the outcome in our patients with FHF complicating viral hepatitis include presence of overt clinical features of raised ICP at the time of hospitalization, prothrombin time (>100 sec) on admission, age (>50 yr), and onset of encephalopathy seven days after onset of jaundice.
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PMID:Prognostic evaluation of early indicators in fulminant hepatic failure by multivariate analysis. 963 24

On the basis of an experience of more than 400 recordings, we demonstrate the usefulness of the anterior fontanelle pressure monitoring (AFP) in several clinical conditions. Main indications for AFP monitoring are the evaluation and the differential diagnosis of neonatal encephalopathy and the assessment of infants with enlarged ventricular spaces, ventriculo-peritoneal derivation or increased head growth rate. Further technical progress is needed to permit AFP recordings in infants with small anterior fontanelle and to reduce the time necessary for the AFP measurement and interpretation procedure. We conclude that it is technically possible and clinically helpful to obtain accurate information about ICP and changes in cerebral compliance in a wide range of clinical conditions without the use of invasive techniques.
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PMID:Non-invasive measurement of intracranial pressure in neonates and infants: experience with the Rotterdam teletransducer. 977 48

In the management of severe pediatric brain injury, attention has previously been paid to brain edema, ICP elevation and low cerebral perfusion pressure (CPP). However, in the acute stage within 3-6 hours after trauma, brain hypoxia and hyperglycemia associated with diffuse brain injury are often observed. We have pointed out brain thermo-pooling (elevation of brain tissue temperature) and brain hypoxia caused by defective release of oxygen from hemoglobin (due to decrease in red blood cell enzyme (DPG)) as a new mechanism of brain injury. To treat these pathologic changes, we have developed a brain hypothermia treatment, the major purpose of which is to prevent brain hypoxia, brain thermo-pooling, neurohormonal changes causing cytokine encephalopathy, and a selective, radical-mediated damage of the dopamine A10 nervous system. The brain tissue temperature is initially adjusted to 35 degrees C with adequate cerebral oxygenation, followed by brain hypothermia at 34 degrees C for 1 weeks to prevent brain hypoxia, free radical reactions, brain edema and ICP elevation. What is most difficult in the pediatric brain hypothermia treatment is to maintain metabolic balance in the injured brain tissue and pulmonary infections associated with an immune crisis. When a rapid elevation of serum glucose is noted it is critical to lower the value because glucose quickly penetrates the blood-brain barrier and increases pyruvate and lactate by inhibiting the TCA cycle metabolism. Thus, hyperglycemia during brain hypothermia treatment is one of the major target of management. Another problem is immune crisis associated with secondary pulmonary infections. To prevent them, early enteral nutrition and replacement of L-arginine were most useful, as well as preconditioning for rewarming as follows: serum albumin > 3.0 g/dl; lymphocyte > 1500/mm3; T-H (CD4) lymphocytes > 55%; serum glucose, 120-140 mg/dl; vitamin A > 50 mg/dl; Hb > 12 g/dl and 2,3 DPG, 10-15 mumol/gHb; O2 ER, 23-25% and AT-III, > 100%. The clinical benefit of this therapy is still controversial.
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PMID:[Brain hypothermia treatment for the management of severe pediatric brain injury]. 1072 86

Abnormal liver tests occur in 3%-5% of pregnancies, with many potential causes, including coincidental liver disease (most commonly viral hepatitis or gallstones) and underlying chronic liver disease. However, most liver dysfunction in pregnancy is pregnancy-related and caused by 1 of the 5 liver diseases unique to the pregnant state: these fall into 2 main categories depending on their association with or without preeclampsia. The preeclampsia-associated liver diseases are preeclampsia itself, the hemolysis (H), elevated liver tests (EL), and low platelet count (LP) (HELLP) syndrome, and acute fatty liver of pregnancy. Hyperemesis gravidarum and intrahepatic cholestasis of pregnancy have no relationship to preeclampsia. Although still enigmatic, there have been recent interesting advances in understanding of these unique pregnancy-related liver diseases. Hyperemesis gravidarum is intractable, dehydrating vomiting in the first trimester of pregnancy; 50% of patients with this condition have liver dysfunction. Intrahepatic cholestasis of pregnancy is pruritus and elevated bile acids in the second half of pregnancy, accompanied by high levels of aminotransferases and mild jaundice. Maternal management is symptomatic with ursodeoxycholic acid; for the fetus, however, this is a high-risk pregnancy requiring close fetal monitoring and early delivery. Severe preeclampsia itself is the commonest cause of hepatic tenderness and liver dysfunction in pregnancy, and 2%-12% of cases are further complicated by hemolysis (H), elevated liver tests (EL), and low platelet count (LP)-the HELLP syndrome. Immediate delivery is the only definitive therapy, but many maternal complications can occur, including abruptio placentae, renal failure, subcapsular hematomas, and hepatic rupture. Acute fatty liver of pregnancy is a sudden catastrophic illness occurring almost exclusively in the third trimester; microvesicular fatty infiltration of hepatocytes causes acute liver failure with coagulopathy and encephalopathy. Early diagnosis and immediate delivery are essential for maternal and fetal survival.
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PMID:Liver disease in pregnancy. 1826 10

Abnormal liver function tests occur in 3 - 5% of pregnancies for different reasons. Apart from pre-existing liver diseases liver diseases occurring during pregnancy, such as gall stones or viral hepatitis, most liver dysfunctions in pregnancy are caused by one of the five pregnancy-related liver diseases. The five known pregnancy-related liver diseases can be classified in two main categories depending on their association with or without preeclampsia. The preeclampsia-associated liver diseases are the preeclampsia itself, the HELLP-syndrome ("Hemolysis" (H), "Elevated Liver Tests" (EL), "Low Platelet Count" (LP)) and the acute fatty liver of pregnancy. Hyperemesis gravidarum and intrahepatic cholestasis of pregnancy are not associated with preeclampsia. Hyperemesis gravidarum is characterised by intractable vomiting in the first trimester of pregnancy. 50% of patients with this condition have liver dysfunction. Intrahepatic cholestasis of pregnancy presents with pruritus and elevated bile acids in the second half of pregnancy. Patients have often mild jaundice and highly elevated liver enzymes. Treatment of choice is ursodeoxycholic acid to relieve the mother's symptoms. With this condition mainly the fetus is at risk. Severe preeclampsia is the most common cause of liver dysfunction in pregnancy, and is in some cases further complicated by HELLP syndrome. The prompt delivery of the baby is the only definitive therapy. However, many life-threatening maternal complications like liver hematoma or rupture and abruptio placentae can occur. Acute fatty liver of pregnancy is also a severe illness occuring mostly in the third trimester; microvesicular fat deposition in the liver can cause liver failure with coagulopathy and encephalopathy. Only the immediate delivery of the fetus can save mother and child.
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PMID:[Liver diseases in pregnancy]. 1894 56

Clinical neurophysiology is both an extension of clinical examination and an integration of neuroimaging. It plays a role in diagnosis, prognosis and monitoring in the Intensive Care Unit (ICU). Electroencephalography (EEG) and somatosensory evoked potentials (SEPs) are the most informative neurophysiological tests. Both have a major prognostic role in the hypoxic-ischemic encephalopathy and traumatic brain injury (TBI). In the former the absence of bilateral cortical SEPs has an unfavorable prognostic significance of 100%, whereas bilateral normal SEPs has uncertain prognostic value. In TBI these SEP patterns have high early prognostic value for both bad and good outcome. Continuous EEG monitoring is indicated for diagnosis and treatment of non convulsive seizures and status epilepticus (NCSE), whereas SEPs are more able to indicate the occurrence of neurological deterioration. In our opinion EEG-SEP monitoring is also valuable for interpretation and management of ICP trends, contributing to optimise treatment in a single patient. The EEG seems to have the same prognostic utility in pediatric as in adult ICU. Recent reviews supported the use of SEPs in the integrated process of outcome prediction after acute brain injury in children. However differences in interpretation are needed and the issue is whether it is possible to establish an age limit over which the prediction of SEPs is similar to that in adults. There are only a few studies of seizure prevalence in pediatric ICU. The variability of frequency of NCSE in comatose children is high as in adults and, similar to the adult, remains unclear the impact on outcome.
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PMID:Neurophysiological monitoring in adult and pediatric intensive care. 2267 30

Posterior reversible encephalopathy syndrome (PRES) is a rare and poorly understood neurologic condition that has been described in some patients with systemic lupus erythematosus (SLE). Intracerebral hemorrhage is a unique and atypical presentation of PRES and has been described only in a small number of patients with SLE. We present the case of a 33-year-old female, diagnosed with SLE and active nephritis, who was admitted for seizures. She had acute-onset headache, confusion, and bilateral vision loss associated with severe hypertension. CT scan revealed right occipital and parietal lobe hemorrhage. MRI showed vasogenic edema and hyperintense foci in bilateral cortical and subcortical regions of the occipital and posterior parietal lobes which are consistent with posterior reversible encephalopathy syndrome (PRES). Strict blood pressure control and medical ICP-lowering treatment were immediately instituted, while maintaining her on anticonvulsants, high-dose steroids, and mycophenolate mofetil. The patient was discharged with improvement in vision and resolution of headache. On follow-up, she had gained her premorbid visual acuity and reported no recurrence of headache or seizures. Despite its name, reversibility remains to be conditional in PRES. A high index of suspicion is important, especially among those who present with seizure, headache, and visual loss. Early diagnosis and timely initiation of therapy is recommended, as clinical symptoms are potentially reversible and delayed therapy may result in life-threatening complications, such as coma or death.
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PMID:Seizure and Acute Vision Loss in a Filipino Lupus Patient: A Case of Posterior Reversible Encephalopathy Syndrome with Intraparenchymal Hemorrhage. 3063 68