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

PURPOSE: To identify the relationship between hemoglobin (Hgb) or hematocrit (Hct) level and dural sinus density using unenhanced computed tomography (UECT). MATERIALS AND METHODS: Patients who were performed UECT and had records of a complete blood count within 24 hours from UECT were included (n=122). We measured the Hounsfield unit (HU) of the dural sinus at the right sigmoid sinus, left sigmoid sinus and 2 points of the superior sagittal sinus. Quantitative measurement of dural sinus density using the circle regions of interest (ROI) method was calculated as average ROI values at 3 or 4 points. Simple regression analysis was used to evaluate the correlation between mean HU and Hgb or mean HU and Hct. RESULTS: The mean densities of the dural sinuses ranged from 24.67 to 53.67 HU (mean, 43.28 HU). There was a strong correlation between mean density and Hgb level (r=0.832) and between mean density and Hct level (r=0.840). CONCLUSION: Dural sinus density on UECT is closely related to Hgb and Hct levels. Therefore, the Hgb or Hct levels can be used to determine whether the dural sinus density is within the normal range or pathological conditions such as venous thrombosis.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Cranial Sinuses/pathology/*radiography ; Female ; *Hematocrit ; Hemoglobins/*analysis ; Hepatolenticular Degeneration/complications ; Humans ; Male ; Middle Aged ; Pregnancy ; Pregnancy Complications ; Radiographic Image Interpretation, Computer-Assisted ; Reference Values ; Regression Analysis ; Superior Sagittal Sinus/pathology/*radiography ; Tomography, X-Ray Computed/*methods ; Young Adult

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

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

OBJECTIVETo probe into indication of living-related liver transplantation (LRLT) for Wilson's Disease.

METHODSFrom January 2001 to February 2007, thirty-seven living-related liver transplants were performed. A retrospective analysis was carried on outcome of those patients. The indications for LRLT were acute hepatic failure in 3 patients and chronic advanced liver disease in 32 patients including 13 patients with Wilsonian neurological manifestations. Two patients presented with severe Wilsonian neurological manifestations even though their liver functions were stable. According to the scoring system for evaluation of the neurological impairment in Wilson disease based on neurological signs and functions (total score was 30), the pre-transplantation score of those patients with neurological manifestations was 15.9 +/- 4.3 (n = 15).

RESULTSThirty-seven patients were followed up for 20 - 93 months. The survival rates of post-transplant patients and grafts at 1, 3, and 5 year were 91.9%, 83.8%, 75.7%, and 86.5%, 78.4%, 75.7%, respectively. Postoperative surgical complications occurred in 2 donors with bile leakage required drainage, in 2 recipients with hepatic thrombosis underwent retransplantation of cadaveric liver and in 1 recipient with hepatic stenosis required balloon dilatation. Neurological function was improved in all recipients and the score of posttransplantation at 6, 12, 18, 24, and 30 month was 17.5 +/- 3.7 (n = 13); 21.0 +/- 4.3 (n = 12); 23.9 +/- 3.9 (n = 10); 26.6 +/- 2.2 (n = 10) and 28.1 +/- 1.9 (n = 7) respectively.

CONCLUSIONSPatients with acute hepatic failure or patients with severe liver disease unresponsive to chelation treatment should be treated with LRLT. Early transplantation in patients with an unsatisfactory response medical treatment may prevent irreversible neurological deterioration even though their liver function is stable.

Adolescent ; Adult ; Child ; Female ; Follow-Up Studies ; Hepatolenticular Degeneration ; complications ; surgery ; Humans ; Liver Failure ; etiology ; surgery ; Liver Transplantation ; Living Donors ; Male ; Nervous System Diseases ; etiology ; Retrospective Studies ; Transplantation, Homologous ; Treatment Outcome ; Young Adult

OBJECTIVETo observe the clinical manifestation of 155 patients with hepatolenticular degeneration (HLD) complicated with epilepsy and the therapeutic effect of integrative Chinese and Western medicine treatment on them.

METHODSClinical manifestation of patients and its relationship with abnormalities in cranial CT and/or MRI were observed. Patients were treated by combined treatment of copper repellent with sodium dimercaptosulfonate 20 mg/kg per day by intravenous dripping, and modified Gandou Decoction (GDD) by oral intake and antiepileptics as well, after treatment for 8-10 courses, the clinical effect, copper levels in urine and serum were compared between groups.

RESULTSIn the 155 HLD patients, 96 were complicated with petit mal and 59 with grand mal. In the CT and/or MRI conducted in 72 patients, all showed abnormal images, besides such frequently met images as bilateral symmetrical basal ganglia focal lesion in 65 case-episode (90.3%) and brain atrophy of various degrees in 61 case-episode (84.7%), the massive lesions in cerebral white matter as principal, with the cortex involved, were also found in 54 patients (74%), which were mostly bilateral and symmetric or located in 2 adjecent lobes of brain, the sites of damage, in sequence of occurrence, were frontal lobe, parietal lobe, temporal lobe and callosal gyrus. Brain atrophy was found in all the remained patients without above-mentioned lesions. Abnormal EEG was shown in 29 patients (40.2%), which mainly manifested as theta wave of moderate to high potential and/or short paroxysmal spike-slow or sharp-slow complex wave evoked. The urinary copper level in patients after treatment was 34.5 +/- 21.6 micromol/24 hrs, significantly higher than that before treatment, 4.49 +/- 1.93 micromol/24 hrs (P < 0.01). And the serum copper level in patients also lowered significantly (P< 0.01). Epileptic seizure was controlled completely along with the gradually improving of extrapyramidal symptoms.

CONCLUSIONPartial seizure was the most common type of seizure of HLD patient complicated with epilepsy, the next is systemic seizure. Cerebral damage lesion and obvious brain atrophy could be the main etiological factors of HLD complicated with epilepsy, combined copper repellent therapy of integrative Chinese and Western medicine, and antiepileptics produced good clinical effect on the patients.

Adolescent ; Adult ; Anticonvulsants ; therapeutic use ; Chelating Agents ; therapeutic use ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Epilepsy ; complications ; drug therapy ; Female ; Hepatolenticular Degeneration ; complications ; drug therapy ; Humans ; Male ; Phytotherapy ; Unithiol ; therapeutic use

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

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