Histomorphologic examination of skeletal muscle preparations does not differentiate between malignant hyperthermia-susceptible and -normal patients

BACKGROUND: It has been suggested that malignant hyperthermia (MH) can be diagnosed by specific myopathologic alterations. The purpose of this study was to investigate whether there are characteristic myopathologic changes in skeletal muscles of MH-susceptible (MHS) compared with MH-normal (MHN) patients. METHODS: Four hundred forty patients with clinical suspicion of MH were classified as MHN, MH equivocal (MHE), or MHS by the in vitro contracture test with halothane and caffeine. In addition, a small muscle sample excised from each patient was analyzed by histologic, histochemical, immunohistochemical, and computer-aided morphometric methods. RESULTS: MHN was diagnosed in 243 patients, MHE was diagnosed in 65, and MHS was diagnosed in 132. No myopathologic abnormalities were found in 53.5% of the MHN, 53.9% of the MHE, and 56.1% of the MHS patients. Thirty-five percent of all patients showed one, 9.8% showed two, and only 0.9% showed three different pathologic findings within skeletal muscle preparations. The frequency of pathologic findings did not differ between the MHN and the MHS patients; only fiber type I predominance was observed more often in MHN. MHE patients could not be assigned to a diagnostic group by detection of myopathologic alterations. In six clinically unaffected patients, a former unrecognized myopathy, such as central core disease, was diagnosed. This disease is characterized by a specific alteration (cores). CONCLUSIONS: Histologic differences between MHS and MHN statuses could not be demonstrated in this study. Histopathologic examinations can neither improve the diagnosis of MH nor contribute to a better definition of the MH status. However, histopathologic examinations might be useful to detect formerly unrecognized specific myopathies.     

Korean hand acupressure for motion sickness in prehospital trauma care: a prospective, randomized, double-blinded trial in a geriatric population

Patients with trauma or medical illnesses transported to the hospital by ambulance have a frequent incidence of motion sickness. Because the administration of drugs in the ambulance is prohibited by law in Austria, the noninvasive Korean hand acupressure point at K-K9 may be an alternative against nausea and vomiting. We enrolled 100 geriatric patients with minor trauma, randomizing them into a K-K9 group and a sham acupressure group. We recorded visual analog scores (VAS) for nausea and for the patient's overall satisfaction with the treatment, hemodynamic variables, and peripheral vasoconstriction. In the K-K9 group, a significant (P < 0.01) increase in nausea was recorded in all cases: from VAS of 0 mm to 25 +/- 6 mm. A similarly significant (P < 0.01) increase was registered in the sham group: from VAS of 0 mm to 83 +/- 8 mm. However, at the time of arrival in the hospital, nausea scores were significantly different between the K-K9 group and the sham group (P < 0.01). Although all patients had been vasoconstricted at the emergency site before treatment, there was a significant difference (P < 0.01) between groups with regard to the number of vasoconstricted patients at the hospital (4 and 46 constricted and dilated, respectively, in the K-K9 group versus 48 and 2 constricted and dilated, respectively, in the sham group). On arrival in the hospital, a significant difference (P < 0.01) in heart rate was noted between the K-K9 group and the sham group (65 +/- 6 bpm versus 98 +/- 8 bpm). The patients' overall satisfaction with the provided care was significantly higher (P < 0.01) in the K-K9 group (19 +/- 9 mm VAS) than in the sham group (48 +/- 12 mm VAS). Neither group experienced a significant change in blood pressure. K-K9 stimulation was an effective and simple treatment for nausea during emergency care and significantly improved patient satisfaction. IMPLICATIONS: Korean hand acupressure at the K-K9 point was effective in reducing nausea and subjective symptoms of motion sickness in emergency trauma transport of patients at high risk of motion sickness.     

Differential regulation of lipoprotein kinetics by atorvastatin and fenofibrate in subjects with the metabolic syndrome

The metabolic syndrome is characterized by insulin resistance and abnormal apolipoprotein AI (apoAI) and apolipoprotein B-100 (apoB) metabolism that may collectively accelerate atherosclerosis. The effects of atorvastatin (40 mg/day) and micronised fenofibrate (200 mg/day) on the kinetics of apoAI and apoB were investigated in a controlled cross-over trial of 11 dyslipidemic men with the metabolic syndrome. ApoAI and apoB kinetics were studied following intravenous d(3)-leucine administration using gas-chromatography mass spectrometry with data analyzed by compartmental modeling. Compared with placebo, atorvastatin significantly decreased (P < 0.001) plasma concentrations of cholesterol, triglyceride, LDL cholesterol, VLDL apoB, intermediate-density lipoprotein (IDL) apoB, and LDL apoB. Fenofibrate significantly decreased (P < 0.001) plasma triglyceride and VLDL apoB and elevated HDL(2) cholesterol (P < 0.001), HDL(3) cholesterol (P < 0.01), apoAI (P = 0.01), and apoAII (P < 0.001) concentrations, but it did not significantly alter LDL cholesterol. Atorvastatin significantly increased (P < 0.002) the fractional catabolic rate (FCR) of VLDL apoB, IDL apoB, and LDL apoB but did not affect the production of apoB in any lipoprotein fraction or in the turnover of apoAI. Fenofibrate significantly increased (P < 0.01) the FCR of VLDL, IDL, and LDL apoB but did not affect the production of VLDL apoB. Relative to placebo and atorvastatin, fenofibrate significantly increased the production (P < 0.001) and FCR (P = 0.016) of apoAI. Both agents significantly lowered plasma triglycerides and apoCIII concentrations, but only atorvastatin significantly lowered (P < 0.001) plasma cholesteryl ester transfer protein activity. Neither treatment altered insulin resistance. In conclusion, these differential effects of atorvastatin and fenofibrate on apoAI and apoB kinetics support the use of combination therapy for optimally regulating dyslipoproteinemia in the metabolic syndrome.     

Lipofibromatous hamartoma of the median nerve in patients with macrodactyly: diagnosis and treatment of a rare disease causing carpal tunnel syndrome

Lipofibromatous hamartoma is a very rare benign peripheral nerve tumour. It is mostly encountered in the proximal extremities of young adults, involving the median nerve in the majority of cases. We present two patients with macrodactyly and carpal tunnel syndrome caused by lipofibromatous hamartoma of the median nerve and discuss diagnosis and treatment of the disease. A 10-year-old girl with a congenital progressive macrodactyly of her right index finger presented with a slowly growing mass in her right palm and pain and numbness, along with motor and sensory deficits in the median nerve distribution. Treatment consisted of carpal tunnel release, epineurolysis and partial excision of the fibrofatty tissue. The second patient, a 25-year-old man presented with a swelling in his left palm and findings compatible with carpal tunnel syndrome. Intraoperatively, the lesion presented as sausage-shaped enlargement of the median nerve by fibrofatty tissue. After carpal tunnel release, a partial excision of the mass with epineurolysis was performed. In both patients, histology showed nerve bundles separated by abundant fibrofatty tissue. In the girl, a proliferation of dysplastic perineurial cells could be observed. The suspected diagnosis for patients with macrodactyly and clinical signs of carpal tunnel syndrome should be lipofibromatous hamartoma. A carefully taken history, physical examination, X-ray, and MRI are important for its correct diagnosis. The surgical management remains controversial. Treatment should include decompression of the median nerve at points of compression, partial excision of the fibrofatty tissue, and debulking of soft tissue. In some cases, an epineurolysis can be additionally performed.     

Comparison of clonidine and midazolam as anxiolytic premedication before wisdom tooth surgery: a randomized, double-blind, crossover pilot study

Purpose

Anxiolytic and possible side effects of clonidine 150?μg compared to midazolam 7.5?mg for premedication in surgical wisdom tooth extraction were evaluated.

Methods

In a prospective, randomized, double-blind crossover trial, ten patients undergoing bilateral wisdom tooth surgery received clonidine or midazolam orally 1?h before the treatment. Patients receiving midazolam for the first surgery received clonidine at the second surgery and vice versa. The anxiolytic efficacy was evaluated with a visual analogue scale (VAS) upon admission and 30, 50 and 60?min after administration of the medication. Patient satisfaction was recorded on a VAS after surgery and 7?days postoperatively.

Results

As soon as 30?min after administration of midazolam (p?<?0.03) and clonidine (p?<?0.02), an anxiolytic effect was recorded. Both medications did not differ in patient satisfaction.

Conclusion

Oral administration of clonidine 150?μg and midazolam 7.5?mg were rated as medications with equal anxiolytic effects before wisdom tooth surgery under local anesthesia.     

Selected disorders of connective tissue: pseudoxanthoma elasticum, cutis laxa, and lipoid proteinosis

There has been progress made in the understanding of 3 Mendelian disorders: pseudoxanthoma elasticum, cutis laxa, and lipoid proteinosis cutis and mucosae. While they are primary connective tissue diseases, their names imply a connection to the skin, and in fact, it is often the dermatologist who makes the diagnosis. It seems rational that defects in various extracellular matrix proteins cause lipoid proteinosis or subtypes of cutis laxa, yet the discovery of a liver- and kidney-based transmembrane transporter as the culprit of pseudoxanthoma elasticum was rather surprising and may shed new light on elastic tissue homeostasis.     

Cosmetic results of histographically controlled excision of non‐melanoma skin cancer in the head and neck region

Background: Beside the primary goal of complete eradication, the cosmetic result is an important aspect of the treatment of non‐melanoma skin tumors especially in the head and neck region. Patients and Methods: From 1990 to 2000, we treated a total of 5,227 large basal cell carcinomas (BBC) and 1,189 squamous cell carcinomas (SCC) in the head and neck region by surgical excision in 4,239 inpatients at the Department of Dermatology, University of Tübingen. The procedure used in all patients was a conservative excision controlled by complete three dimensional histology of all margins (3D‐histology) and specifically targeted follow‐up surgery where required (histographic surgery). As part of the prospective tumor follow‐up, we asked the treating outdoor physician one and four years later to evaluate the results of our surgical procedures. Results: Of the 5,565 follow‐up questionnaires sent back, 4,868 contained answers regarding the cosmetic result. The data from both answers were pooled. In 1,972 (40,5 %) patients the cosmetic result was evaluated as “excellent”, in 1,992 (40,9 %) as “good”, in 662 (13,6 %) as “satisfactory”, in 191 (3,9 %) as “mediocre” and in 51 (< 1,0 %) as “poor”. In 697 of the responses, the physician did not comment the cosmetic results or the patient was lost for follow up. Conclusion: With respect to both long term safety and cosmetic outcome, tumor surgery with 3D‐histology of excisional margins has set very high quality standards in the treatment of non‐melanoma skin cancer of the head and neck area.     

Baseline serum levels of mast cell tryptase are raised in hemodialysis patients and associated with severity of pruritus

Background: The pathogenesis of pruritus in renal disease is not yet understood. Evidence suggests that mast cells play a role; for example, the number of dermal mast cells is increased in patients on hemodialysis. Patients and methods: To investigate a possible role of mast cell tryptase in pruritus of patients undergoing chronic hemodialysis, serum mast cell tryptase concentrations were measured in blood samples taken from 93 such patients, 53 of whom also recorded the severity of their pruritus on a visual analogue scale. Results: Serum mast cell tryptase levels were above 11.4 µg/l (95^th percentile) in 84 of 93 hemodialysis patients (90.3 %). The intensity of pruritus correlated significantly (p = 0.014) with the tryptase levels, an associated not yet shown for other mast cell‐related parameters. Conclusions: Mast cells or even tryptase itself may be involved in the pathogenesis of pruritus of hemodialysis patients.