| This study was conducted to identify bilirubin in deciduous teeth obtained from two patients with a history of severe liver dysfunction. Teeth were histologically analyzed and bilirubin was extracted and quantified spectrophotometrically. Histological analysis revealed a green line in the dentine running parallel to the incremental lines. A chloroform/methanol/acetic acid (30:10:0.5, v/v) extract of the teeth was evaporated and the residue dissolved in chloroform. absorption spectra were prepared before and after the diazo reaction. The absorption maximum shifted from 450 nm before to 540 nm after the diazo reaction and was higher than that of normal deciduous teeth. These results indicate that the discolouration of teeth in patients with severe liver dysfunction is due to bilirubin deposition. ( info) |
2/172. A patient with hypertrophic cardiomyopathy accompanied by right ventricular dilation of unknown cause. Hypertrophic cardiomyopathy (HCM) is a disease characterized by an unknown cause of hypertrophy in the left or right ventricle. The dilated phase of HCM shows disease conditions resembling dilated cardiomyopathy, such as ventricular dilation, thin ventricular wall, and reduction of the ejection fraction. A patient presented with left ventricular concentric hypertrophy accompanied by right ventricular dilatation of unknown cause. Right ventricular endomyocardial biopsy specimens showed characteristic myocardial disarray. Therefore, there is the possibility that the patient had right and left ventricular HCM in the process toward the dilated phase, in which dilatation first occurred in the right ventricle. ( info) |
3/172. Hemoglobin S/O(Arab): thirteen new cases and review of the literature. Hemoglobin S/O(Arab) (Hb S/O(Arab)) is a rare compound heterozygous hemoglobinopathy characterized by the presence of two variant beta-globin chains: beta6Glu --> Val (Hb S) and beta121Glu --> Lys (Hb O(Arab)). The diagnosis of Hb S/O(Arab) requires electrophoresis on both cellulose acetate and citrate agar, since Hb O(Arab) co-migrates with Hb C at alkaline pH and close to Hb S at acidic pH. To date only case reports and small series of patients with Hb S/O(Arab) have been described. To better characterize the clinical and laboratory aspects of this unusual disorder, we reviewed the Duke University Medical Center experience. We identified 13 African-American children and adults with Hb S/O(Arab) ranging in age from 2.7 to 62.5 years. All patients had hemolytic anemia with a median Hb of 8.7 gm/dL (range 6.1-9.9 gm/dL), and a median reticulocyte count of 5.8% (range 1.2-10.3%). The peripheral blood smear typically showed sickled erythrocytes, target cells, polychromasia, and nucleated red blood cells. All 13 patients have had significant clinical sickling events including acute chest syndrome (11), recurrent vasoocclusive painful events (10), dactylitis (7), gallstones (5), nephropathy (4), aplastic crises (2), avascular necrosis (2), leg ulcers (2), cerebrovascular accident (CVA) (1), osteomyelitis (1), and retinopathy (1). Four patients have died, including two from pneumococcal sepsis/meningitis at ages 5 and 10 years, one of acute chest syndrome at age 14 years, and one of multiorgan failure at age 35 years. We conclude that Hb S/O(Arab) disease is a severe sickling hemoglobinopathy with laboratory and clinical manifestations similar to those of homozygous sickle cell anemia. ( info) |
4/172. Appearance of severe jaundice after radiometabolical treatment of thyrotoxicosis. The appearance of moderate jaundice with mildly raised levels of plasma bilirubin is an uncommon complication of thyrotoxicosis and is usually accompanied by signs of right heart failure. Some described cases were actually related, at least in part, to autoimmune chronic hepatitis. In this paper we describe a case of thyrotoxicosis accompanied by deep jaundice with very high levels of bilirubin occuring in the absence of cardiac failure and with no signs of hepatitis. jaundice disappeared shortly after the start of thyrostatic drug treatment, supporting a possible detrimental effect of hyperthyroidism on the hepatic bilirubin metabolism. ( info) |
5/172. Long-term extracorporeal bilirubin elimination: A case report on cascade resin plasmaperfusion. Acute hepatic failure develops as a disease entity of rather diverse origin. With disease progression, toxic bilirubin levels may cause severe complications which include AV-nodal blockage, cardiac arrhythmia, impaired consciousness, generalized seizures, and status epilepticus. Treatment choices to prevent clinical deterioration comprise of costly and limited available orthotopic liver transplantation, utilization of extracorporeal bioartificial liver support devices and haemoperfusion/plasmaperfusion treatment with activated charcoal/anion exchange filters. Here, we present a patient with acute drug-induced cholestatic hepatitis. Excessively elevated bilirubin levels were accompanied by cardiac and cerebral complications. Extracorporeal resin perfusion treatment (Plasorba, BR-350) was successfully performed over a 50-day period without activation of the coagulation system or side effects. Bilirubin levels were lowered to a minimum of 225 micromol/l, with concurrent clinical improvement. In conclusion, extracorporeal anion exchange plasmaperfusion may be a viable long-term treatment for hyperbilirubinaemic side effects in overt cholestatic hepatitis. ( info) |
6/172. Successful pregnancy in a woman with secondary biliary cirrhosis with portal hypertension from recurrent pyogenic cholangitis. A case report. BACKGROUND: pregnancy in women with secondary biliary cirrhosis due to recurrent pyogenic cholangitis is extremely rare. Little information is available on the effect of pregnancy on the disease and vice versa. CASE: A patient who had secondary biliary cirrhosis due to recurrent pyogenic cholangitis complicated by splenomegaly and portal hypertension had a successful pregnancy. Although she had a history of esophageal variceal bleeding before this pregnancy, there was no such bleeding during pregnancy. She had an uneventful antenatal course except that her liver enzyme level fluctuated slightly. The serum bilirubin level increased during the third trimester of pregnancy but returned to the prepregnant level after delivery. CONCLUSION: Termination of pregnancy may not be the only option for management. The management protocol for patients with primary biliary cirrhosis complicating pregnancy, which includes regular fetal surveillance and monitoring of maternal liver function, should be considered for pregnant women with secondary biliary cirrhosis. ( info) |
7/172. Clinical findings for a group of infants and young children with auditory neuropathy. OBJECTIVE: To examine the prevalence of auditory neuropathy in a group of infants at risk for hearing impairment and to present an overview of the clinical findings for affected children. DESIGN: Results for 20 subjects who showed repeatable cochlear microphonic potentials in the absence of click-evoked auditory brain stem responses are included in this study. Behavioral and steady state evoked potential thresholds were established in each case. Where possible, otoacoustic emission and speech perception results (unaided and aided) also were obtained. RESULTS: One in 433 (0.23%) of the children in our series had evidence of auditory neuropathy. The audiometric findings for these subjects varied significantly, with behavioral thresholds ranging from normal to profound levels. Discrimination skills were also variable. Approximately half of the subjects showed little understanding, or even awareness, of speech inputs in both the unaided and aided conditions. There were, however, a number of children who could score at significant levels on speech discrimination tasks and who benefited from the provision of amplification. CONCLUSION: The results suggest that auditory neuropathy is more common in the infant population than previously suspected. The effects of neuropathy on auditory function appear to be idiosyncratic, producing significant variations in both the detection and discrimination of auditory signals. As such, the management of children with this disorder must allow for individual differences. ( info) |
8/172. Orthotopic liver transplantation in a patient with severe haemophilia A and with advanced liver cirrhosis. A patient with severe haemophilia A underwent orthotopic liver transplantation because of changes correlated to end-stage liver cirrhosis due to hepatitis b, C and D infection. Replacement therapy was carried out for 4 days and the clinical course was uneventful. At the time of reporting the patient has a normal working life. FVIII plasma concentration is normal. The indirect hyperbilirubinaemia may be related to the Gilbert's anomaly of the donor. ( info) |
9/172. An adverse interaction between warfarin and 5-fluorouracil: A case report and review of the literature. Adverse interactions between warfarin and 5-fluorouracil (5-FU) have been reported. Such an interaction occurred in a patient with lung cancer receiving vinblastine and 5-FU. This case is the first involving a patient taking minidose warfarin for prophylaxis of catheter-associated thrombosis. Although the mechanism of the interaction is unclear, it has been postulated that 5-FU interferes with the synthesis of hepatic cytochrome P-450 2C9. Because warfarin and 5-FU are regularly coadministered, this adverse interaction might be occurring more frequently than is realized. Clinicians should be aware of this interaction and should regularly monitor the prothrombin time of patients receiving warfarin and 5-FU. ( info) |
10/172. manganese deposition in the brain following parenteral manganese administration in association with radical operation for esophageal cancer: report of a case. We report herein the case of a patient in whom manganese (Mn) deposition in the basal ganglia was detected by magnetic resonance imaging (MRI) subsequent to thoracic esophagectomy, performed following perioperative parenteral nutrition. A multi-trace-element supplement solution which included 20 micromol of Mn per day had been parenterally administered for 7 days preoperatively and 21 days postoperatively. The serum level of total bilirubin reached a maximum value of 5.1 mg/dl postoperatively. The T1-weighted MRI on the 32nd postoperative day demonstrated bilateral and symmetrical hyperintense lesions in the globus pallidus and the whole-blood Mn level on the 34th postoperative day was 4.9 microg/l, the normal range being 0.8-2.5 microg/l. This hyperintensity on T1-weighted MRI was gradually improved following normalization of the blood Mn level. This case report serves to demonstrate that even short-term perioperative parenteral nutrition may result in Mn deposition in the brain following radical surgery for esophageal cancer, especially in patients with hyperbilirubinemia. ( info) |