Estimating pulmonary capillary wedge pressures using Doppler variables of early diastolic left ventricular inflow

The present study was performed to determine whether a multilineal regression model based on the early diastolic transmitral flow peak velocity (E) and the propagation velocity of early diastolic inflow (PV) could estimate the pulmonary capillary wedge pressure (PCWP). PCWP and Doppler variables were simultaneously recorded in 30 patients. PCWP was estimated by multilinear regression analysis using E and PV. The predictive accuracy of the equation obtained from the analysis was tested prospectively in a separate group of 65 patients divided into 3 groups: left ventricular (LV) systolic dysfunction (Group A), LV hypertrophy (Group B), and preserved systolic function without hypertrophy (Group C). The initial results obtained in groups B and C, respectively, were: r=0.77; r=0.81. These results indicate that a multilinear regression model based on E and PV is a noninvasive method of accurately estimating PCWP in a variety of cardiac disease states.     

Muir-Torre syndrome caused by exonic deletion of <Emphasis Type="Italic">MLH1</Emphasis> due to homologous recombination

Background

Muir-Torre syndrome (MTS) is characterized by sebaceous neoplasms with internal malignancies and regarded as a variant of hereditary nonpolyposis colorectal cancer (HNPCC). Pathogenic variations of MTS have been identified in the MSH2, MLH1, and MSH6 genes, with the majority of variations located in MSH2.

Objectives

To present an MTS patient who was the only individual with skin malignancies within a cancer-prone pedigree and to showthe usefulness ofRNA-based genetic analysis in the investigation of MTS.

Materials & methods

A 77-year-old man who had operated X-ray equipment at his workplace in his twenties was clinically diagnosed with MTS and investigated by RNA-based analysis, multiplex ligation-dependent probe amplification, and genomic DNA sequencing.

Results

The patient had suffered from sebaceous tumours, squamous cell carcinomas of the skin, and colon cancer. The patient’s family history was remarkable for visceral malignant diseases. Genetic analysis revealed homologous recombination between two Alu elements within intron 4 and 5 of the MLH1 gene. The rearrangement caused a 1,222-bp deletion, including the entire exon 5. Deletion of exon 5 has previously been reported only in two patients with HNPCC, and not in patients with MTS.

Conclusions

For the genetic analysis of MTS, the possibility of rare copy number variations of MLH1, as well as MSH2 variations, should be considered. RNA-based screening using puromycin is recommended in order to identify such variations. It remains unclear why only the proband among the pedigree had skin malignancies, however, the skin carcinogenesis might have been related to occupational radiation exposure.

     

"Anatomic" right hepatic trisectionectomy (extended right hepatectomy) with caudate lobectomy for hilar cholangiocarcinoma

BACKGROUND: The techniques of right hepatic trisectionectomy are now standardized in patients with hepatocellular or metastatic carcinoma, but not in those with hilar cholangiocarcinoma. METHODS: Under preoperative diagnosis of hilar cholangiocarcinoma, 8 patients underwent "anatomic" right hepatic trisectionectomy with en bloc resection of the caudate lobe and the extrahepatic bile duct, in which the bile ducts of the left lateral section were divided at the left side of the umbilical fissure following complete dissection of the umbilical plate. RESULTS: Liver resection was successfully performed, and all patients were discharged from the hospital in good condition, giving a mortality of 0%. All patients were histologically diagnosed as having cholangiocarcinoma. The proximal resection margins were cancer-negative in 7 patients and cancer-positive in 1 patient. Four patients with multiple lymph node metastases died of cancer recurrence within 3 years after hepatectomy. One patient died of liver failure without recurrence 42 months after hepatectomy. The remaining 3 patients without lymph node metastasis are now alive after more than 5 years. CONCLUSIONS: Anatomic right hepatic trisectionectomy with caudate lobectomy can produce a longer proximal resection margin and can offer a better chance of long-term survival in some selected patients with advanced hilar cholangiocarcinoma.     

Hilar cholangiocarcinoma—surgical anatomy and curative resection

We have studied the surgical anatomy of the intrahepatic bile duct, hepatic hilus, and caudate lobe based on intraoperative findings and selective cholangiography of surgical patients and resected specimens, and have established the cholangiographic anatomy of the intrahepatic subsegmental bile duct. Thorough knowledge of the three-dimensional anatomy of the subsegmental bile duct, hepatic hilus, and caudate lobe is indispensable for curative surgery of hilar cholangiocarcinoma. We designed and actually performed 15 kinds of hepatic segmentectomies with caudate lobectomy and extrahepatic bile buct resection in 100 consecutive patients, with curative resection being possible in 82 patients. Postoperative survival after curative resection of hilar cholangiocarcinoma was better than expected, and the 5-year survival rates for all 82 patients with curative resection and for 55 patients with curative surgery without portal vein resection were 31% and 43%, respectively. Hepatic segmentectomy with caudate lobectomy and extrahepatic bile duct resection should be designed not only in accordance with the preoperative diagnosis of tumor extension into the intrahepatic bile ducts but also so that curative surgery for advanced hilar cholangiocarcinoma can be performed.     

High Protein Diet Feeding Aggravates Hyperaminoacidemia in Mice Deficient in Proglucagon-Derived Peptides

(1) Background: Protein stimulates the secretion of glucagon (GCG), which can affect glucose metabolism. This study aimed to analyze the metabolic effect of a high-protein diet (HPD) in the presence or absence of proglucagon-derived peptides, including GCG and GLP-1. (2) Methods: The response to HPD feeding for 7 days was analyzed in mice deficient in proglucagon-derived peptides (GCGKO). (3) Results: In both control and GCGKO mice, food intake and body weight decreased with HPD and intestinal expression of Pepck increased. HPD also decreased plasma FGF21 levels, regardless of the presence of proglucagon-derived peptides. In control mice, HPD increased the hepatic expression of enzymes involved in amino acid metabolism without the elevation of plasma amino acid levels, except branched-chain amino acids. On the other hand, HPD-induced changes in the hepatic gene expression were attenuated in GCGKO mice, resulting in marked hyperaminoacidemia with lower blood glucose levels; the plasma concentration of glutamine exceeded that of glucose in HPD-fed GCGKO mice. (4) Conclusions: Increased plasma amino acid levels are a common feature in animal models with blocked GCG activity, and our results underscore that GCG plays essential roles in the homeostasis of amino acid metabolism in response to altered protein intake.     

Superior efficacy of MMCP regimen compared with VMCP and MMPP regimens in the treatment of multiple myeloma

OBJECTIVE: A newly designed combination chemotherapy for multiple myeloma, MMCP [ranimustine (MCNU), melphalan (MPH), cyclophosphamide (CPM) and prednisolone (PSL)], was analyzed and compared with the results of our previous randomized trial of VMCP [vincristine, MPH, CPM and PSL] and MMPP [MCNU, MPH, procarbazine and PSL]. METHODS: MCNU (33.3 mg/m2, div) on day 1 and MPH (4 mg/m2, po), CPM (66.7 mg/m2, po) and PSL (30 mg/m2, po) from day 1 to 4, were administered. Each cycle was repeated every 3 weeks. PATIENTS OR MATERIALS: From January 1991 until August 1995, 104 patients with multiple myeloma diagnosed at 10 hospitals of Nagoya Cooperative Study Group were enrolled. RESULTS: Of the 87 evaluable patients, partial response rate for MMCP was 65.5% and was significantly higher than that of VMCP (13/47=27.7%, p<0.0001) and that of MMPP (21/47=44.7%, p=0.0196). A plateau attainment was observed in 49.4%. The percentage of the patients who attained plateau was significantly increased in the MMCP arm than in the VMCP arm (19.1 %, p=0.0017) but was not in comparison with that of MMPP arm (42.6%, p=0.6790). Patients treated with MMCP survived significantly longer than those treated with VMCP or MMPP (p=0.0009 by generalized Wilcoxon test, p=0.0023 by log-rank test) with median survival for MMCP being 31.6 months, for VMCP 22.5 months, and for MMPP 22.9 months. No significant differences were observed with respect to adverse effects among the three regimens. CONCLUSION: The newly designed MMCP is a candidate as an induction chemotherapy for multiple myeloma.     

Dermoscopic features distinctive for extraocular sebaceous carcinoma

We examined dermoscopic features of three cases of extraocular sebaceous carcinoma and reviewed the literatures. The yellowish structures, polymorphous vessels and ulceration were common findings in our cases and all cases of the previous reports. The appearance of whitish‐pink areas has not been described previously. Our results suggested that the combination of four dermoscopic features, whitish‐pink areas, yellowish structures, polymorphous vessels and ulceration might be distinctive in extraocular sebaceous carcinoma.