Patients with systemic lupus erythematosus (SLE) have a chance of developing liver involvement in their lifetime. The main cause of liver involvement in SLE patients is previous treatment with hepatotoxic drugs or hepatotropic viral hepatitis. Wilson's disease is a hereditary disorder and is usually diagnosed in patients presenting either neuropsychiatric disorders or manifestations related to chronic liver disease. Fulminant hepatic failure as the initial manifestation of Wilson's disease is rare. The relationship between systemic lupus erythematosus and Wilson's disease has not been established. We report a case of a 12-year-old girl with SLE who presented fulminant hepatic failure as an initial manifestation of Wilson's disease. The diagnosis was established with decreased serum ceruloplasmin level and the presence of Kayser-Fleischer ring. We treated with repeated plasma exchange. Despite repeated plasma exchange she died of multi-organ failure on the 16th hospital day. Considering this case, Wilson's disease should be considered as a cause of fulminant hepatic failure, especially in juvenile age cases.
Child ; English Abstract ; Female ; Hepatolenticular Degeneration/*complications ; Human ; Liver Failure/*etiology ; Lupus Erythematosus, Systemic/*complications

Child ; Cholecystitis, Acute ; complications ; diagnosis ; Comorbidity ; Hepatolenticular Degeneration ; complications ; diagnosis ; Humans

Liver transplantation has become a successful treatment modality for serious hepatic disease and it has been reported that there are successful pregnanies after liver transplantation. Previous reports regarding pregnancy after liver transplantation have described an increased risk of pregnancy complications, such as hypertension, preeclampsia, preterm delivery, and anemia. Hence, it is suggested that pregnancy after liver transplantation should be managed as high-risk pregnancy. We have experienced a case of successful full-term delivery after liver transplantation in patient with Wilson's disease and presented with brief review of literatures.
Anemia ; Hepatolenticular Degeneration* ; Humans ; Hypertension ; Liver Transplantation* ; Liver* ; Pre-Eclampsia ; Pregnancy ; Pregnancy Complications ; Pregnancy, High-Risk

Liver transplantation has become a successful treatment modality for serious hepatic disease and it has been reported that there are successful pregnanies after liver transplantation. Previous reports regarding pregnancy after liver transplantation have described an increased risk of pregnancy complications, such as hypertension, preeclampsia, preterm delivery, and anemia. Hence, it is suggested that pregnancy after liver transplantation should be managed as high-risk pregnancy. We have experienced a case of successful full-term delivery after liver transplantation in patient with Wilson's disease and presented with brief review of literatures.
Anemia ; Hepatolenticular Degeneration* ; Humans ; Hypertension ; Liver Transplantation* ; Liver* ; Pre-Eclampsia ; Pregnancy ; Pregnancy Complications ; Pregnancy, High-Risk

Anemia, Hemolytic ; etiology ; Child ; Diagnosis, Differential ; Female ; Hepatitis ; etiology ; Hepatolenticular Degeneration ; complications ; diagnosis ; Humans

Adolescent ; Anemia, Hemolytic ; etiology ; Female ; Hemolysis ; Hepatic Encephalopathy ; etiology ; Hepatolenticular Degeneration ; complications ; Humans

OBJECTIVETo analyze the etiology, clinical and laboratory characteristics of hepatic failure in 105 children.

METHODSThe clinical data of 105 children with hepatic failure treated in our hospital from January 1986 to June 2003 were retrospectively analyzed by EXCELL 2000 and t test.

RESULTS(1)Of the 105 children with hepatic failure, 9 were cases with fulminant hepatic failure, 38 with subacute hepatic failure and 58 with chronic hepatic failure. (2)Morbidity was the highest in 7-12 years old children (43/105, 41.0%) followed by infants (30/105, 28.6%). (3)CMV infection could be confirmed in 9 infants (30.0%), etiological diagnosis was not possible in 13 infants (43.3%). Etiological diagnosis could be confirmed in children over 1 year of age, which included hepatitis B (n=22, 29.3%), Wilson's disease (n=15, 20.0%), hepatitis A (n=10,13.3%). Etiology in 21 cases (28.0%) could not be confirmed. (4)Seventy-one cases (67.6%) had ascites, 34 of them (47.9%) had spontaneous peritonitis. Thirty-five cases were complicated with other infections. The commonest complication was pulmonary infection and sepsis was the next. Fifty-one cases (48.6%) had hydroelectrolyte imbalance. Forty-eight cases (46.2%) had hepatic encephalopathy, which may be subclinical in children under three years of age. (5)The incidence of hypoglycemia was 77.2%(71/92).

CONCLUSIONThe etiology of liver failure was related to age. CMV infection was the commonest in infants. HBV, HAV infection was the commonest in children over 1 year of age and Wilson?s disease was the next. It is necessary to prevent and manage the associated complications as early as possible such as spontaneous peritonitis, hepatic encephalopathy, hydroelectrolyte imbalance and hypoglycemia etc.

Adolescent ; Age Factors ; Child ; Child, Preschool ; Cytomegalovirus Infections ; complications ; Female ; Hepatic Encephalopathy ; etiology ; Hepatitis A ; complications ; Hepatitis B ; complications ; Hepatolenticular Degeneration ; complications ; Humans ; Hypoglycemia ; complications ; Infant ; Liver Failure ; etiology ; Liver Failure, Acute ; etiology ; Male ; Peritonitis ; complications

Laribacter hongkongensis is an emerging pathogen in patients with community-acquired gastroenteritis and traveler's diarrhea. We herein report a case of L. hongkongensis infection in a 24-yr-old male with liver cirrhosis complicated by Wilson's disease. He was admitted to a hospital with only abdominal distension. On day 6 following admission, he complained of abdominal pain and his body temperature reached 38.6degrees C. The results of peritoneal fluid evaluation revealed a leukocyte count of 1,180/microL (polymorphonuclear leukocyte 74%). Growth on blood culture was identified as a gram-negative bacillus. The isolate was initially identified as Acinetobacter lwoffii by conventional identification methods in the clinical microbiology laboratory, but was later identified as L. hongkongensis on the basis of molecular identification. The patient was successfully treated with cefotaxime. To the best of our knowledge, this case is the first report of hospital-acquired L. hongkongensis bacteremia with neutrophilic ascites.
Acinetobacter/isolation & purification ; Acinetobacter Infections/complications/diagnosis/microbiology ; Bacteremia/complications/*microbiology ; Cefotaxime/therapeutic use ; Diagnosis, Differential ; Gastroenteritis/complications/*diagnosis/*microbiology ; Hepatolenticular Degeneration/complications/microbiology ; Humans ; Liver Cirrhosis/complications/microbiology ; Male ; Neisseriaceae/*isolation & purification ; Phylogeny ; Republic of Korea ; Young Adult

Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.
Adrenal Insufficiency/diagnosis/etiology ; Adult ; Brain/diagnostic imaging ; Depression/etiology ; Female ; Hepatolenticular Degeneration/*complications ; Humans ; Hypopituitarism/complications/*diagnosis/drug therapy ; Hypothyroidism/diagnosis/etiology ; Liver Cirrhosis/complications/diagnostic imaging ; Magnetic Resonance Imaging ; Steroids/therapeutic use ; Thyrotropin-Releasing Hormone/therapeutic use

Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.
Adrenal Insufficiency/diagnosis/etiology ; Adult ; Brain/diagnostic imaging ; Depression/etiology ; Female ; Hepatolenticular Degeneration/*complications ; Humans ; Hypopituitarism/complications/*diagnosis/drug therapy ; Hypothyroidism/diagnosis/etiology ; Liver Cirrhosis/complications/diagnostic imaging ; Magnetic Resonance Imaging ; Steroids/therapeutic use ; Thyrotropin-Releasing Hormone/therapeutic use