Living domino liver transplantation in an adult with congenital absence of portal vein.

Congenital absence of the portal vein (CAPV) is a rare malformation of the splanchnic venous system. Although CAPV is usually detected in the pediatric age group, our patient was a 35-year-old woman. She had been diagnosed with CAPV in 1996 when she was 27 years old. In 1998, she was placed on hemodialysis due to chronic renal failure. After several episodes of encephalopathy in 2002, liver transplantation (LT) was recommended to her and her family. Since there was no suitable living donor candidate, she was put on the waiting list for a deceased donor liver transplant in Japan. In 2004, her ammonia level increased to around 300 microg/dl, and she went into a coma lasting for three days. After recovering from this event, she underwent a living domino transplantation using a whole liver donated by a familial amyloid polyneuropathy (FAP) patient. Her portal vein, which had drained directly into the inferior vena cava (IVC), was transected together with a cuff of the IVC wall and anastomosed to the graft liver portal vein in an end-to-end fashion. In conclusion, liver transplantation proved to be a safe and effective way to save this patient and improve her quality of life.     

Radiation dose reduction during hysterosalpingography: an application of scanning-beam digital radiography

Hysterosalpingography was performed in 31 patients by means of a low-dose scanning-beam digital radiographic system. The technique permits adequate evaluation of gynecologic abnormalities while allowing significant reduction in radiation: 2.4-mR (6.1 X 10(-7) C/kg) exposure to the skin and 0.7-mrad (7 X 10(-6) Gy) mean dose to the ovaries per image obtained. Sixteen patients demonstrated readily recognizable and documented abnormalities, corroborated by laparoscopy, laparotomy, or other supportive evidence.     

Relationship Between Exhaled Carbon Monoxide and Airway Hyperresponsiveness in Asthmatic Patients

The study objectives were to analyze the changes in exhaled carbon monoxide (COex) induced by histamine provocation challenge in asthmatic patients and to evaluate the relationship between COex and airway sensitivity and reactivity. Levels of COex were measured in 105 nonsmoking mildly asthmatic subjects before and after histamine provocation challenge. Dose-response curves were characterized by their sensitivity (PD₂₀) and reactivity. Dose-response slope (DRS), continuous index of responsiveness (CIR), and bronchial reactivity index (BRI) were determined as reactivity indices. Bronchial challenge was positive for 47 subjects and negative for 58. The COex levels rose significantly after bronchial challenge in the positive response group (4.49 ± 0.4 vs. 5.74 ± 0.57 ppm, p = 0.025) and in the negative response group (2.84 ± 0.25 vs 4.00 ± 0.41 ppm, p = 0.000). An inverse relation between basal COex and PD₂₀ was found (r = - 0.318, p = 0.030). In all subjects, a proportional direct relationship between COex and DRS (r = 0.214, p = 0.015), CIR (r = 0.401, p = 0.000), and BRI (r = 0.208, p = 0.012) was observed. On stepwise multiple linear regression analysis, COex only significantly correlated with CIR (multiple r² = 0.174, p = 0.000). In conclusion, exhaled CO determination is a noninvasive inflammatory marker of the respiratory tract, which shows an acceptable association with airway hyperresponsiveness.     

Merkel Cell Carcinoma Arising from the Subcutaneous Fat of the Arm with Intact Skin

BACKGROUND: Merkel cell carcinoma is a rare malignant neuroendocrine neoplasm characteristically arising from the dermis of sunlight-exposed skin. It rarely arises outside the skin. OBJECTIVE: We present a patient with primary Merkel cell carcinoma arising from subcutaneous fat, with no involvement of the overlying skin. We describe the clinical manifestations and magnetic resonance imaging (MRI) findings. METHODS: We report a 63-year-old woman with a primary lesion of Merkel cell carcinoma that arose from the subcutaneous fat layer of the left arm. The lesion presented as a subcutaneous nodule with intact overlying skin. MRI showed that the nodular lesion was located entirely in the subcutaneous fat layer, with no involvement of the dermis. Peritumoral infiltration around the lesion and enlarged lymph nodes deep to the lesion were noted. The patient received wide excision of the lesion with dissection of the regional lymph nodes and adjuvant radiotherapy and chemotherapy. RESULTS: Histopathologic examination confirmed the diagnosis of Merkel cell carcinoma with local lymphatic metastasis, and the lesion was completely located in the subcutaneous fat, with no involvement of the dermis. These findings were well correlated with MRI findings. CONCLUSION: Primary Merkel cell carcinoma may arise from the subcutaneous fat and present as an entirely subcutaneous lesion with intact skin. MRI is helpful to evaluate the local extension of the lesion and regional lymphatic metastasis.     

Hypernephroma associated with multiple endocrine neoplasia type I: a case report

We report a case of multiple endocrine neoplasia type I and hypernephroma. Parathyroid hyperplasia, adrenocortical hyperplasia, a nodular goiter, multiple lipomas, a chromophobe adenoma of the pituitary and hypernephroma had all been diagnosed previously. All but the last are features consistent with the diagnosis of multiple endocrine neoplasia type I (Wermer's syndrome). The association of multiple endocrine neoplasia type I and hypernephroma may represent a new manifestation of this pleiotropic syndrome.     

Modulatory effects of progesterone upon dopamine release from the corpus striatum of ovariectomized estrogen-treated rats are stereo-specific

We have shown previously that progesterone linked with bovine serum albumin at position 3 of the progesterone molecule (P-3-BSA) was effective in modulating (both stimulating and inhibiting) amphetamine (AMPH)-evoked dopamine (DA) release in vitro from superfused corpus striatal (CS) tissue fragments of ovariectomized estrogen-primed rats. To examine the stereospecificity of this effect, progesterone linked with bovine serum albumin at position 11 (P-11-BSA) and deoxycorticosterone linked with bovine serum albumin at position 21 (DOC-BSA) were tested for their ability to modulate amphetamine-stimulated DA release in vitro when administered in either a pulsatile or a continuous infusion mode at different doses. The results demonstrate that a specific orientation of the P-BSA molecule is required for differential modulation of AMPH stimulated DA release from the CS of ovariectomized estrogen-treated rats, since DOC-BSA was ineffective and P-11-BSA showed only a stimulatory effect.     

Early and late results of orthotopic liver transplantation

Between 4/1986 to 1/1989, 74 orthotopic liver transplantation were performed in 62 patients (62 first liver transplants, 10 as second graft and two as a third graft); 57 in adults and 17 in children. The main indication for the operation was liver cirrhosis (61.4%) (the most frequent etiology was alcoholic cirrhosis, 28.5%). Six cirrhotic patients had a hepatocarcinoma (9.6%). Two received a liver and kidney transplant due to terminal renal insufficiency and hemodialysis. The most frequent indication in children was biliary atresia (33.3%). Six patients had a fulminal liver failure (9.6%). AB0 blood group compatibility was identical in 87.5%, compatible in six and incompatible in three patients. Total orthotopic liver transplantation was performed in 67 patients, and size-reduced liver was indicated in 7 patients. Extracorporeal veno-venous bypass was used in adults but never in children. In 93.1% of the transplants a single hepatic artery was anastomosed to the recipient and in 6.9% a double anastomosis was performed. In 62.5% of the patients a end-to-end choledocho-choledochostomy was performed and in 34.8% hepatico-jejunostomy was indicated. Three months postoperative mortality rate was 12.9%. Arterial stenosis and thrombosis were the most frequent complication.