Orthotopic liver transplantation in a malignant hyperthermia susceptible patient

We present a patient with hepatitis C and D and hepatocellular carcinoma who underwent preoperative evaluation for orthotopic liver transplantation. In his past medical history, he reported a life-threatening event during tonsillectomy in 1975. Intubation was impossible due to extreme jaw muscle tension, followed by excessive elevation in body temperature, tachycardia, and coma for a few days. We evaluated him for malignant hyperthermia, according to the European Malignant Hyperthermia Group Protocol, and found him highly positive in both the halothane and caffeine test, respectively. Three months later, we performed an orthotopic liver transplantation. During retransplantation 4 years later, due to ischemic-type biliary lesions, he suffered massive intraoperative bleeding. Blood products, as well as coagulation factors and aprotinin, were well tolerated. Anesthesia was performed in a trigger-free total intravenous technique without dantrolene prophylaxis, but dantrolene was readily available in sufficient quantities in the operating room. The patient did not encounter a malignant hyperthermia crisis in either perioperative period.     

Efficacy of Repeat Isolated Limb Perfusions With Tumor Necrosis Factor α and Melphalan for Multiple In-Transit Metastases in Patients with Prior Isolated Limb Perfusion Failure

Background Isolated limb perfusion (ILP) is an effective treatment modality for multiple in-transit melanoma metastases confined to the limb. Recurrences after ILP, however, occur in approximately 50% of patients and are a challenge for further treatment. The efficacy of repeat ILPs to prolong local control in this patient category is evaluated in this article.Methods We used a prospective database in a tertiary referral center. Out of 100 tumor necrosis factor (TNF)-based ILPs with TNF and melphalan (TM-ILPs) in melanoma patients between March 1991 and July 2003, 25 repeat ILP procedures were performed in 21 patients in whom prior ILP treatment failed. All patients had bulky and/or numerous lesions and were treated with mild hyperthermic TM-ILP by using 2 to 4 mg of TNF and 10 to 13 mg/L of limb volume for the leg and arm, respectively.Results The complete response rate was 76%, a partial response occurred in 20%, and no change was recorded in 4%. There was no difference in the complete response rate or local toxicity between first and repeat perfusions. Local recurrence occurred in 72%; the median time to local progression was 14 months. The 5-year survival rate was 47%, which compares favorably with known survival rates of stage IIIA/AB patients. The median follow-up of the patients was 26 months.Conclusions Patients who experience treatment failure after previous ILP treatment respond very well to repeat perfusion, and prolonged local control can thus be obtained. The subgroup of patients qualifying for repeat ILP represents a relatively favorable biological behavior of the melanoma.     

Isolated Limb Perfusion With Tumor Necrosis Factor and Melphalan Prevents Amputation in Patients With Multiple Sarcomas in Arm or Leg

Background Treatment for extremity soft tissue sarcoma (STS) has shifted in recent years from amputation to local wide excision combined with irradiation. For multiple sarcomas, this limb-sparing approach is often not possible. To avoid amputations, isolated limb perfusion (ILP) with tumor necrosis factor and melphalan is an attractive treatment option for patients with multiple extremity sarcomas.Methods We investigated a prospective database at a tertiary referral institute. From July 1991 to July 2003, out of 217 ILPs, 64 ILPs were performed for either multifocal primary sarcomas or multiple sarcoma recurrences in 53 patients. All ILPs were performed under mild hyperthermic conditions by using 1 to 4 mg of tumor necrosis factor and 10 to 13 mg/L of limb volume for leg and arm perfusions, respectively.Results The overall response was 88%, with 42% complete response, 45% partial response, 11% no change, and 2% progressive disease. This response rate is significantly better than our experience in 153 locally advanced single-STS cases (88% vs. 69%). The toxicity of the procedure was mild to moderate in almost all cases; no treatment-related amputation had to be performed. The time to local recurrence was 29 months and differed significantly between multiple primary and multiple recurrent STS. The 5-year survival rate was 39%. Limb salvage was achieved in 45 (82%) of 55 treated limbs.Conclusions In a group of patients who are uniformly candidates for amputation, ILP can achieve limb salvage in approximately four out of five patients. Because this treatment option provides excellent local control, it should be considered before an amputation is planned.     

Quality of life following living donor nephrectomy comparing classical flank incision and anterior vertical mini-incision

In this study we focused on the quality of life and satisfaction of living kidney donors comparing traditional lumbar (LDN) and mini-incision donor nephrectomy (MIDN). From May 1996 to December 2002, 174 donor nephrectomies including 127 cases of LDN and 47 cases of MIDN were performed. Donors were evaluated using the SF-36 quality-of-life survey as well as a questionnaire dealing with donors‘ attitude towards kidney donation, financial burdens, pain, cosmetic satisfaction and duration of sick leave. Our donors achieved comparable or even higher scores in all the SF-36 categories in comparison to the general US population. Following MIDN, quality of life tended to be superior compared to that of LDN donors; however, statistical significance was reached only in one of the eight categories. Duration of sick leave following surgery was in favor of MIDN compared to LDN donors. Statistically significant differences favoring MIDN were observed regarding postoperative hospital stay and cosmetic satisfaction. The procedure would be again undergone by 94 of LDN and 97% of MIDN donors. Open-donor nephrectomy is a safe and cost-effective procedure. Introduction of the here-described MIDN has led to comparable or even improved results compared to LDN.     

Outcome and quality of life in medicine: a conceptual framework to put quality of life research into practice

It is rather counterproductive to get caught up in never ending discussions on what quality of life (QOL) is and whether it can be assessed. This article is based on the idea that it is much more important to improve the conceptual frameworks that allow the use of the QOL concept in clinical practice. Survival of the QOL concept within the medical community will depend on its contributions to a better understanding of patients and to improving patient care. It is important to accept that QOL should not be viewed in isolation but in synopsis, with other psychologic concepts and clinical data. We propose a profile format that presents QOL data in a way that is easily accessible for clinicians, allowing action to be taken immediately. QOL profiles are never a substitute but a starting point for a patient-doctor interaction. A profile driven interaction has the potential to be structured, efficient, and leading to action.     

The palliative value of tumor necrosis factor alpha-based isolated limb perfusion in patients with metastatic sarcoma and melanoma

BACKGROUND: Both patients with soft tissue sarcoma (STS) and patients with melanoma have limited treatment possibilities once the tumor has metastasized systemically. In patients with extremity STS or bulky melanoma in-transit metastases, the local tumor burden may be so problematic that, even in patients with systemically metastasized disease, an amputation may be inevitable. Isolated limb perfusion (ILP) has proven to be an excellent, local, limb-saving treatment option in patients with locally advanced extremity tumors. In this study, the authors investigated the palliative value of the ILP procedure to avoid amputation in patients who had Stage IV STS and melanoma. METHODS: From 1991 to 2003, of 339 tumor necrosis factor alpha (TNF)-based ILPs, 51 procedures were performed for either Stage IV STS (n = 37 patients) or Stage IV melanoma (n = 14 patients). All patients underwent an ILP with TNF and melphalan of the upper limb (n = 4 patients) or the lower limb (n = 47 patients) with 26-140 mg melphalan and 2-4 mg TNF. RESULTS: The overall response in patients with Stage IV STS was 84%, and their median survival was 12 months after ILP. Limb salvage was achieved in 36 of 37 patients, with 1 patient undergoing amputation due to treatment toxicity. In the patients with Stage IV melanoma, the complete response rate was 43%. All patients with melanoma preserved their limb during a median survival of 7 months. CONCLUSIONS: TNF-based ILP is an excellent procedure that provided tumor control and limb salvage for the short survival of patients with metastasized, very bulky, limb-threatening tumors of the extremity.     

Outcome and prognostic factor analysis of 217 consecutive isolated limb perfusions with tumor necrosis factor-alpha and melphalan for limb-threatening soft tissue sarcoma

BACKGROUND: Extensive and mutilating surgery is often required for locally advanced soft tissue sarcoma (STS) of the limb. As it has become apparent that amputation for STS does not improve survival rates, the interest in limb-preserving approaches has increased. Isolated limb perfusion (ILP) with tumor necrosis factor-alpha (TNF) and melphalan is successful in providing local tumor control and enables limb-preserving surgery in a majority of cases. A mature, large, single-institution experience with 217 consecutive ILPs for STS of the extremity is reported. METHODS: At a prospectively maintained database at a tertiary referral center, 217 ILPs were performed from July 1991 to July 2003 in 197 patients with locally advanced STS of the extremity. ILPs were performed at mild hyperthermic conditions with 1-4 mg of TNF and 10-13 mg/L limb-volume melphalan (M) for leg and arm perfusions, respectively. RESULTS: The overall response rate was 75%. Limb salvage was achieved in 87% of the perfused limbs. Median survival post-ILP was 57 months and prognostic factors for survival were Trojani grade of the tumor and ILP for single versus multiple STS. The procedure could be performed safely, with a perioperative mortality of 0.5% in all patients with no age limit (median age, 54 yrs; range, 12-91). Systemic and locoregional toxicity were modest and easily manageable. CONCLUSION: TNF+M-based ILP can provide limb salvage in a significant percentage of patients with locally advanced STS and has therefore gained a permanent place in the multimodality treatment of STS.     

Zum Problem der sogenannten Simultanagnosie

Zusammenfassung Bei einem Patienten mit einem vorzugsweise lobär verlaufenden hirnatrophischen Prozeß wurden die Störungen der Gesamtauffassung bildlich dargestellter Situationen (Simultanagnosie), die er neben Wortfindungsschwierigkeiten, Paraphasien, Dysgraphie, Dyskalkulie und Merkschwäche bot, einer Analyse unterzogen. Die gestörte Intellektualität der Wahrnehmung zeigte sich als ein Darniederliegen ihrer sinngerichteten Aktivität. Der Patient war nicht in der Lage, Strukturen in eine Wahrnehmungsgegebenheit zu bringen, war unfähig, eine Gestalt aufzubauen. Durch Störungen des Übersehens hatte die Überschaubarkeit des Wahrnehmungsfeldes gelitten. Bei schneller Absättigung des Sinnfindungsbedürfnisses kam es zum sinngemäßen Illusionieren innerhalb kurzschlüssig hergestellter Sinneinheiten. Die durch das Zurücktreten der Gestalt gegenüber der Physiognomie des Gegenstandes gekennzeichneten Zeichnungen des Patienten wurden den raumagnastischen Gestaltungsstörungen zugeordnet. Die Bedeutung der Störungen für das Leben des Patienten erhellen sein Verzicht auf Wahrnehmung und seine Unsicherheit gegenüber einer eindringlicher gewordenen, nicht mehr dahingestellten Welt.     

Polybrominated biphenyls, polychlorinated biphenyls, body weight, and incidence of adult-onset diabetes mellitus

BACKGROUND: Prior studies have reported an increased risk of diabetes related to polychlorinated biphenyl (PCB) exposure. No study has yet investigated whether polybrominated biphenyls (PBBs), which are similar in chemical structure, increase the incidence of diabetes. METHODS: The Michigan PBB cohort was established in 1976 and surveyed again in 1991-1993 and in 2001. PBB and PCB serum levels were measured from blood collected at enrollment. To determine the incidence of adult-onset diabetes, we analyzed cohort members without diabetes at enrollment, ages 20 years and older, with known PBB and PCB levels, who participated in at least 1 follow-up survey (n = 1384). Using Poisson regression, we determined the incidence density ratio (IDR) of diabetes for different serum levels of PBB and PCB, controlling for age, body mass index, smoking, and alcohol consumption at enrollment. RESULTS: Analyzing 25 years of follow-up data, we did not find that higher PBB serum levels were a risk factor for the incidence of diabetes mellitus. However, in women, but not in men, higher PCB serum levels were associated with increased incidence of diabetes (IDR = 2.33; 95% confidence interval = 1.25-4.34 in the highest PCB group compared with the lowest). In both men and women, overweight and obesity increased the diabetes incidence. CONCLUSIONS: We found no association between PBB serum levels and diabetes incidence. In women, there was a positive linear association of diabetes incidence with PCB serum levels at enrollment. This finding is in agreement with 2 prior studies indicating a higher relative risk of diabetes in PCB-exposed women.