Primary biliary cirrhosis associated with idiopathic thrombocytopenic purpura

A case of primary biliary cirrhosis (PBC) associated with idiopathic thrombocytopenic purpura (ITP) is reported. The patient is a 59-year-old man. When he was 49 years old, he was diagnosed with ITP and received steroid therapy that successfully increased platelet numbers. However, the steroid therapy failed to normalize the elevated gamma-glutamyl transpeptidase. Ten years after this episode, he suffered from general itching and malaise and exhibited a gradual increase of serum biliary enzyme levels. Immunologically, IgM was increased and anti-mitochondrial antibody was positive. Histological findings of liver needle biopsy showed chronic non-suppurative destructive cholangitis, confirming the diagnosis of PBC. To date, very few PBC cases associated with ITP have been reported. Our case is the second one in Japan. PBC and ITP in our patient seemed to develop simultaneously, but the effect of steroid therapy on the two conditions was different. This result suggests that the autoimmune process may have been different in PBC and ITP in the present patient.     

A case with subcapsular hematoma of the liver following laparoscopic cholecystectomy

The occurrence of intracerebral hemorrhage during an acute attack of migraine remains questionable. A normotensive migrainous woman experienced multiple intracerebral hemorrhages. No drug abuse was recorded, but bleeding occurred after the use of several antimigrainous drugs. Angiography revealed severe vasospasm of both anterior cerebral arteries. Subsequent brain MRI and MRI angiography failed to show any vascular abnormalities. Echocardiography and an exhaustive biological evaluation ruled out an alternative condition favoring intracerebral hemorrhage. Multiple intracerebral hemorrhages in migraine may exceptionally be related to vasospasm leading to arterial wall injury. Intracranial arterial vasospasm could be precipitated by excessive vasoactive therapy.     

A case of systemic lupus erythematosus associated with spinal epidural hematoma

A 47 year-old Japanese female who showed transverse myelopathy (TM) due to spinal epidural hematoma diagnosed by MRI in the course of systemic lupus erythematosus (SLE) was reported. She was admitted to Keio University Hospital due to paraplegia, anesthesia of lower extremity, urinary disturbance. Neurological examination revealed transverse disturbance of Th 10. Lumbar spinal cord MRI showed irregular mass that located at epidural region of 9th-11th thoracic vertebrae. When the laminectomy of 9th-11th thoracic vertebrae was performed, hematoma (4.5 cm x 1.5 cm in size) was confirmed and removed completely. Post operative condition was stable and symptoms had been improving gradually. It has been reported that TM associated with SLE was closely related to myelitis. In this case, epidural hematoma was a major cause of TM and MRI was very useful for her diagnosis and treatment. This is the rare case of SLE associated with spinal epidural hematoma and was thought as a important case to consider the cause of neurological complication of SLE.     

A case of idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia associated with chronic nephritis

A 48-year-old female who had general fatigue was admitted to our hospital. She had swelling of the axillary, inguinal, and paraaortic lymph nodes and mediastinal lesions. Laboratory examinations showed anemia, polyclonal hyperimmunoglobulinemia with IgG 5570 mg/dl, renal dysfunction and interstitial changes of the lungs. Microscopic findings of hematoxylin-eosin staining in biopsy specimens of the left inguinal and axillary lymph nodes revealed increased levels of infiltration of mature plasma cells without evidence of malignancy. Immunoperoxidase staining showed intracytoplasmic polyclonal immunoglobulin. These findings were identical to those of idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia (IPL) described by Mori et al. (1980). The specimens also showed evidence of chronic nephritis with infiltration of lymph cells and a slight invasion of plasma cells. Accordingly this case was diagnosed as IPL with renal involvement, which is associated with chronic nephritis. Recently, five cases of IPL with renal dysfunction have been reported. In particular, two cases of IPL with renal dysfunction, which included our case, revealed an increased level of IL6. These findings suggest that the occurrence of renal involvement with IPL may be related to changes in IL6, which is an important factor in the pathogenesis of IPL.     

An autopsy case of primary squamous cell carcinoma of the liver associated with hepatitis C virus antibody positive liver cirrhosis

An increase in potassium (K) intake may lower blood pressure (BP), but inconsistent results have been obtained in clinical trials. We studied the effects of K supplementation in hypertensive patients with monitoring of home and ambulatory BP. Fifty-five patients with essential hypertension (26 men, 29 women, 36-77 years old) participated in this study. A 4-week K supplementation period and 4-week control period were assigned in a randomized crossover manner. During the K period, the subjects were given 64 mmol/day of K as slow-release KCl tablets. Office, home, and 24-h BP, as well as serum and urinary electrolytes, were measured at the end of each period. In the control period, office, home, and 24-h BP were 151 +/- 2/88 +/- 1 (mean +/- SE), 138 +/- 1/83 +/- 1, and 137 +/- 1/81 +/- 1 mm Hg, respectively. Serum K increased from 4.15 +/- 0.04 to 4.42 +/- 0.05 mmol/L, and urinary K increased from 54 +/- 2 to 96 +/- 3 mmol/day with the K supplementation. Office, home, and 24-h BP were significantly lower in the K period than in the control period, although the differences were small (2.7 +/- 1.1/1.4 +/- 0.6, 3.6 +/- 0.9/1.7 +/- 0.5, 3.4 +/- 1.0/1.2 +/- 0.5 mm Hg, respectively). Changes in home and 24-h systolic BP with K supplementation were highly significant (P < .001), compared with office BP (P < .05). The change in 24-h systolic BP was correlated negatively with baseline BP and urinary Na/K ratio, and positively with baseline urinary K excretion. The changes in daytime and nighttime BP were comparable. These results indicate that increasing K intake lowers BP in hypertensive subjects, especially in those with higher BP and lower K intake. Our study supports the usefulness of K supplementation in the treatment of hypertension, although its antihypertensive effect may be small.     

A case of adult idiopathic thrombocytopenic purpura associated with atypical mumps virus infection

A 30-year-old male admitted to out hospital complaing of petechiae. He had attack of fever 2 days before admission, without parotitis and orchitis. Laboratory data showed marked thrombocytopenia. Bone marrow showed normocellularity with an increase of megakaryocytes. Antimumps IgM was positive by the EIA. He was diagnosed as idiopathic thrombocytopenic purpura (ITP) associated with mumps virus infection. After the administration of oral prednisolone and 5 days-infusion of gamma-globulin, platelet count increased rapidly. Prednisolone discontinued within 35 days, as in acute ITP, and he maintained remission. In conclusion, the test for antiviral antibodies is indispensable to exclude acute ITP in adult ITP patients.     

Morbidity after video-laparoscopic cholecystectomy in cholelithiasis associated with liver cirrhosis. A case-control study

At present laparoscopic cholecystectomy represents the treatment of choice for symptomatic cholelithiasis. Authors performed a retrospective case-control study to evaluate whether cirrhosis associated with cholelithiasis increases the risk for morbidity of laparoscopic cholecystectomy. Twenty-one patients with cholelithiasis and cirrhosis (Child-Pugh class A or B) (group A) and 21 controls with cholelithiasis without cirrhosis (group B) entered the study. Controls were paired with cases for age, sex, and indication for cholecystectomy (simple cholelithiasis, acute cholecystitis). The two groups were compared for rate of conversion to open cholecystectomy (19% group A vs 9.5% group B; p = 0.31), morbidity (29.5% group A vs 5.3% group B; p = 0.17), median length of surgery (80 m in the two groups), and median time of postoperative hospitalization (5 days group A vs 3 days group B; p = 0.21). No difference among variables resulted to be statistically significant. Besides, neither common bile duct injuries nor intra or postoperative hemorrhages occurred in patients with cirrhosis. Authors conclude that the laparoscopic cholecystectomy can be considered a safe and effective surgical procedure also for patients with cholelithiasis associated with cirrhosis with a good residual hepatic function.     

Treatment of hepatocellular carcinoma associated with cirrhosis in the era of liver transplantation

PURPOSE: To review the treatment of cirrhotic patients with hepatocellular carcinoma in the era of liver transplantation and to determine the most appropriate approach to the treatment of patients at different stages of disease. DATA SOURCES: A MEDLINE search of English-language articles published between 1981 and 1997 and the clinical experience of the Mount Sinai Liver Transplant Program. STUDY SELECTION: Selected studies were 1) original articles reporting results of resection and transplantation in the treatment of hepatocellular carcinoma in cirrhotic patients and 2) initial reports from major transplantation centers of multimethod therapies combining chemotherapy with transplantation. DATA EXTRACTION: Study designs were assessed with careful attention to methods and aims. Relevant data on patient population, tumor stage distribution, treatment, survival, and rate of recurrent disease were extracted and analyzed. DATA SYNTHESIS: Options for the treatment of hepatocellular carcinoma in cirrhotic patients vary according to tumor stage and severity of underlying liver disease. Resection remains an important method primarily in eastern countries, where the screening of high-risk populations has been associated with early detection of small asymptomatic lesions. Long-term survival after resection, however, is low. In western countries, liver transplantation is becoming the treatment of choice in patients with advanced cirrhosis and small, unresectable lesions; resection is reserved for cirrhotic patients with small, peripheral lesions and preserved hepatic function. Minimally invasive procedures (such as percutaneous ethanol injection and transarterial chemoembolization) have been developed to treat unresectable tumors. Transarterial chemoembolization may also be effective in patients with advanced cirrhosis and unresectable lesions who are awaiting transplantation. CONCLUSIONS: The efficacy of liver transplantation for hepatocellular carcinoma has been proven mainly in patients with advanced cirrhosis and small lesions. Future studies may clarify the role of approaches combining neoadjuvant chemotherapy with transplantation for large (stage III) tumors.     

Subdural hematoma associated with arachnoid cyst

We describe 3 young patients with history of mild cranial trauma who presented the rare association of subdural hematoma and arachnoid cyst. Clinical and radiological signs are reviewed.     

Chordoid meningioma associated with chronic subdural hematoma

A 50-year-old man with 'presyncope' is presented. He was found to have an aneurysm of the right coronary sinus of Valsalva and an aneurysm of the noncoronary sinus. Neither aneurysm had ruptured. It is postulated that the patient's symptoms were related to partial obstruction of the right ventricle. Other potential complications of an unruptured aneurysm of the sinus of Valsalva are discussed.     

Focal myoclonus associated with posterior thalamic hematoma

PURPOSE: We report a new incision for repair of penile fracture. MATERIALS AND METHODS: We describe 2 cases in which the inguinal scrotal incision was used for repair of penile fracture. The preoperative evaluation as well as the technical case and rationale for use of this incision are discussed. RESULTS: Preoperative cavernosogram delineated the site of the fracture. Immediate repair of the fracture using the inguinal scrotal incision was successful. CONCLUSIONS: The inguinal scrotal incision should be entertained for cases of penile fracture. It avoids incision into markedly edematous penile skin and allows for excellent visualization of the fracture site.     

A 10-year-old case with idiopathic oculomotor nerve palsy

Raman spectroscopy has been used to determine the isotopic distribution of oxygen during the isomerization of pernitrous acid to nitrate in 18OH2. Decomposition of unlabeled pernitrous acid in carbon-dioxide-free phosphate-buffered solutions of 18OH2 at pH 6.8 results in the incorporation of 13 +/- 1% 18O into the nitrate product with no detectable double-isotopic incorporation and 83% of the product having complete retention of its oxygen atoms. The mechanistic implications of this are contrasted for three frequently considered pathways for peroxide bond cleavage and it is concluded that the results are most consistent with the formation of reactive intermediate which can either rearrange or undergo hydrolytic O-atom exchange with water.     

A case of veno-occlusive disease of the liver in Britain associated with herbal tea consumption

Veno-occlusive disease of the liver with clinical and pathological features similar to those of the condition occurring in the Caribbean is described in a young woman resident in Britain. The diagnosis was made from liver biopsies and hepatic venography and was confirmed at necropsy. Small amounts of pyrroliziding alkaloids were recovered from a sample of mate (Paraguay) tea, owned by the patient, to which she was addicted. It seems probable that the consumption of large amounts of this tea over a period of years was the cause of the hepatic disease.