Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cholestatic hepatitis is identified as one of the features of hypopituitarism in the newborn, but the exact etiology of cholestasis in these cases has not been well established yet. We report here two infants, one with isolated glucocorticoid deficiency and the other with multiple pituitary hormone deficiency, indicating primary and central adrenal insufficiency, respectively, who presented with recurrent hypoglycemic seizures and cholestatic hepatitis. Severe cortisol deficiency in these cases was suggested to be the cause of cholestatic hepatitis. Review of the literature and our cases showed that the cortisol deficiency in both primary and central adrenal insufficiency occurring only during neonatal and early infancy period cause cholestatic hepatitis. The severity and the age of onset of cortisol deficiency are suggested to be the important predictors of cholestatic hepatitis in childhood.
 ...
PMID:Cholestatic hepatitis as a result of severe cortisol deficiency in early infancy: report of two cases and review of literature. 1729 May 78

We report a 9-year-old girl with cereulide-producing Bacillus cereus food poisoning, who developed fulminant hepatitis, renal and pancreatic insufficiency, shock, and prolonged seizures. She was transferred to our institution for hepatic transplantation before her diagnosis was established. As a result of rapid identification of the microorganism and supportive care, liver transplantation was avoided, and she recovered fully.
 ...
PMID:Food poisoning as a cause of acute liver failure. 1866 29

A 5-year-old male, drowsy, jaundiced child presented with fulminant hepatitis and had HAV and HEV infection. He had hepatic encephalopathy grade 1, fever, pallor, hypotension, crepitations in his right lung base and hepatosplenomegaly with dyspnoea. He had highly raised liver enzymes and hypoalbuminemia (2.8 g/dl) but anemia (hemoglobin of 7.7 g/dl and 5.7 g/dl 2 days later), reticulocytopenia and severe thrombocytopenia (44 x 10(9)/l) were unexplained. Parvovirus B19-specific IgM antibodies and B19 DNA were found in the serum of the child. Chest X-ray showed pleural effusion and bronchopneumonia, while blood culture isolated coagulase-negative staphylococci (BACTEC 9120) and he had low oxygen saturation. Hence, he was treated with IV amoxicillin+ clavulinic acid and oxygen inhalation. He had seizures and cardiac arrest but was revived. On the third day his condition worsened and the child died despite intensive care. Hence it is concluded that his anemia and thrombocytopenia were B19 induced and this might have aggravated or caused fulminant hepatitis.
 ...
PMID:Parvovirus B19-induced thrombocytopenia and anemia in a child with fatal fulminant hepatic failure coinfected with hepatitis A and E viruses. 1920 89

Lamotrigine is a non-aromatic antiepileptic drug. Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a severe idiosyncratic reaction to drugs, especially anti-epileptic drugs. Associated clinical features include cutaneous eruption, fever, multiple peripheral lymphadenopathies, and potentially life-threatening damage of one or more organs. We report a case of DRESS syndrome induced by lamotrigine presenting with a hypersensitivity syndrome and fulminant hepatic failure requiring liver transplant. A 21-year old female patient presented an episode of seizure with loss of conscience. CT and EEG studies performed were normal. Treatment with lamotrigine was prescribed. In the course of 30 days, the patient developed skin lesions, pruritus, cholestatic hepatitis, and systemic symptoms -fever, lymphadenopathies, extensive exfoliative erythematous maculopapular rash, and jaundice. Serologic and laboratory tests showed no other causes responsible for the clinical spectrum. Hematologic tests revealed peripheral eosinophilia. Fulminant hepatic failure was diagnosed and an orthotopic liver transplant was performed. Histologic sections of the explanted liver demonstrated submassive hepatic necrosis, with the remnant portal spaces and lobules showing a mixed inflammatory infiltrate with lymphocytes and eosinophils. Lamotrigine treatment has been associated with multiorgan failure, DRESS syndrome, acute hepatic failure, and disseminated intravascular coagulation. In conclusion, we suggest that these potentially fatal side effects should be considered in any patient with clinical deterioration following administration of this drug.
 ...
PMID:Dress syndrome and fulminant hepatic failure induced by lamotrigine. 1922 40

Idiopathic hypereosinophilic syndrome (HES) is a rare syndrome for which Rottweilers appear to over-represent the canine cases. A 6-month-old female entire Rottweiler presented with seizures following a traumatic incident. The dog was identified as having a marked, sustained eosinophilia and investigations did not identify an underlying cause. Concurrently, the dog had chronic eosinophilic hepatitis with impaired liver function and mesenteric eosinophilic lymphadenitis. The dog went on to have spontaneous resolution of HES and normal liver function was subsequently documented. To date, the dog is still alive, more than 4 years after initial presentation. The diagnosis of idiopathic HES in Rottweilers may not carry a poor prognosis and the condition may spontaneously resolve, even in cases with documented organ damage.
 ...
PMID:Clinical remission of idiopathic hypereosinophilic syndrome in a Rottweiler. 1967 49

Liver transplantation is the only curative treatment in patients with end-stage liver disease. Neurological complications (NC) are increasingly reported to occur in patients after cadaveric liver transplantation. This retrospective cohort study aims to evaluate the incidence and causes of NC in living donor liver transplant (LDLT) patients in our transplant center. Between August 1998 and December 2005, 121 adult LDLT patients were recruited into our study. 17% of patients experienced NC, and it occurred significantly more frequently in patients with alcoholic cirrhosis (42%) and autoimmune hepatitis (43%) as compared with patients with hepatitis B or C (9/10%, P = 0.013). The most common NC was encephalopathy (47.6%) followed by seizures (9.5%). The choice of immunosuppression by calcineurin inhibitor (Tacrolimus or Cyclosporin A) showed no significant difference in the incidence of NC (19 vs. 17%). The occurrence of NC did not influence the clinical outcome, since mortality rate, median ICU stay and length of hospital stay were similar between the two groups. Most patients who survived showed a nearly complete recovery of their NC. NCs occur in approximately 1 in 6 patients after LDLT and seem to be predominantly transient in nature, without major impact on clinical outcome.
 ...
PMID:Neurologic complications in adult living donor liver transplant patients: an underestimated factor? 1972 99

Niemann-Pick C disease (NP-C) is a neurovisceral atypical lysosomal lipid storage disorder with an estimated minimal incidence of 1/120,000 live births. The broad clinical spectrum ranges from a neonatal rapidly fatal disorder to an adult-onset chronic neurodegenerative disease. The neurological involvement defines the disease severity in most patients but is typically preceded by systemic signs (cholestatic jaundice in the neonatal period or isolated spleno- or hepatosplenomegaly in infancy or childhood). The first neurological symptoms vary with age of onset: delay in developmental motor milestones (early infantile period), gait problems, falls, clumsiness, cataplexy, school problems (late infantile and juvenile period), and ataxia not unfrequently following initial psychiatric disturbances (adult form). The most characteristic sign is vertical supranuclear gaze palsy. The neurological disorder consists mainly of cerebellar ataxia, dysarthria, dysphagia, and progressive dementia. Cataplexy, seizures and dystonia are other common features. NP-C is transmitted in an autosomal recessive manner and is caused by mutations of either the NPC1 (95% of families) or the NPC2 genes. The exact functions of the NPC1 and NPC2 proteins are still unclear. NP-C is currently described as a cellular cholesterol trafficking defect but in the brain, the prominently stored lipids are gangliosides. Clinical examination should include comprehensive neurological and ophthalmological evaluations. The primary laboratory diagnosis requires living skin fibroblasts to demonstrate accumulation of unesterified cholesterol in perinuclear vesicles (lysosomes) after staining with filipin. Pronounced abnormalities are observed in about 80% of the cases, mild to moderate alterations in the remainder ("variant" biochemical phenotype). Genotyping of patients is useful to confirm the diagnosis in the latter patients and essential for future prenatal diagnosis. The differential diagnosis may include other lipidoses; idiopathic neonatal hepatitis and other causes of cholestatic icterus should be considered in neonates, and conditions with cerebellar ataxia, dystonia, cataplexy and supranuclear gaze palsy in older children and adults. Symptomatic management of patients is crucial. A first product, miglustat, has been granted marketing authorization in Europe and several other countries for specific treatment of the neurological manifestations. The prognosis largely correlates with the age at onset of the neurological manifestations.
 ...
PMID:Niemann-Pick disease type C. 2052 56

Bilateral thalamic lesions detected on magnetic resonance imaging have a wide differential diagnosis. This report describes a previously healthy young man who developed bilateral thalamic necrosis with seizures, vomiting, hepatitis, neutrophilic leukocytosis and metabolic acidosis following consumption of raw dried fruits of the ridge gourd plant (Luffa acutangula) prescribed by a traditional medicine practitioner. These fruits were subsequently shown to be infested with spores and conidiomata of Diplodia, a coelomycete fungus known to cause neurotoxicity in farm animals. The patient made a partial recovery with supportive care, and has persistent deficits consistent with bilateral medial thalamic damage. This is the first report of neurological toxicity attributable to Diplodia in humans, and this entity should be considered in the differential diagnosis of bilateral thalamic lesions in the appropriate clinical setting.
 ...
PMID:Bilateral thalamic necrosis following ingestion of ridge gourd infested with coelomycete fungi (Diplodia). 2056 34

Four children; all between 10-40 days of age, were admitted with multiple episodes of multifocal seizure. After admission blood investigation was carried out and they were found to have hypocalcemia, hyperphosphatemia; kidney function tests were normal. Serum parathyroid hormone was highly elevated and serum magnesium level was found to be normal in these patients. The infants did not have any other features of pseudohypoparathyroidism. The seizure was initially resistant to intravenous calcium therapy and later responded to oral calcitriol and calcium therapy. The diagnosis of pseudohypoparathyroidism was made according to biochemical investigations. The infants were symptom-free during follow-up and the biochemistry reports were also normalized. One of the infants was also suffering from neonatal hepatitis, due to CMV infection.
 ...
PMID:Case series of neonatal hypocalcemia due to pseudohypoparathyroidism. 2115 19

Depakote-induced hepatotoxicity has been well established as an adverse effect, and periodic monitoring of drug level is often required. Depakote-induced hepatotoxicity mostly occurs at supratherapeutic drug level. Rarely, an idiosyncratic response is triggered, and hepatotoxicity can occur at the therapeutic drug level mostly in chronic users. Here, we describe a rare case of idiosyncratic depakote-induced hepatotoxicity. A 25-year-old female with non-insulin-dependent diabetes mellitus, hypothyroidism, seizure disorder, and Dandy Walker Syndrome presented with an unwitnessed seizure and altered mental status. The patient's medication list included zonisamide, depakote, and synthroid. She was noted to be lethargic, disoriented, nonverbal, but awake. An arterial blood gas examination showed severe anion gap metabolic acidosis. Blood work was consistent with hepatitis, hyperammonemia, thrombocytopenia, and coagulopathy. The Depakote level was therapeutic. Head computed tomography and liver ultrasound results were not significant. After ruling out all other causes and seeing improvement of parameters after the drug was discontinued, idiosyncratic depakote toxicity was diagnosed. Based on the patient's rapid improvement; idiosyncratic valproate toxicity was confirmed. This case signifies the importance of recognizing, diagnosing, and treating depakote toxicity in chronic users who have no other explanation for their symptomatology.
 ...
PMID:Thinking beyond the obvious: hepatotoxicity secondary to idiosyncratic depakote toxicity. 2124 13


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>