Evaluation of a standardized protocol for processing adrenal tumor samples: preparation for a European adrenal tumor bank.

Storage and tissue handling of surgical tumor specimen have been recognized as critical steps that can potentially affect reproducibility and comparability of molecular endpoints between laboratories. In the preparation of adrenal tumor tissue banking, three different protocols that simulate warm ischemia upon tumor removal (protocol I), thawing and refreezing cycles (protocol II), as well as storage of vital tumor samples (protocol III) were applied. For the first two protocols, samples were subdivided and either snap frozen or treated with a RNA preserving agent (RPA) while in protocol III different storage media were compared. Following these procedures, recovery and integrity of DNA, RNA, and protein by means of pulsed field electrophoresis, long-range PCR, real-time PCR, immunoblot, and immunohistochemistry (protocol I and II) as well as cell viability and steroidogenic capacity (protocol III) were investigated. While DNA integrity was not influenced by different treatment modalities, expression levels of adrenal marker genes were more affected in samples after snap freezing in comparison to RPA pretreatment. Moreover, storage at room temperature before and after freezing could be demonstrated to decrease the relative amount of protein phosphorylation (ERK) and enzymatic activity (succinate cytochrome c reductase) while overall protein levels were not significantly affected. Similarly, morphological or immunohistochemical evaluation was comparable between groups. For primary cell cultures generated after storage of tumor samples similar rates of viability were observable while steroid output varied between the groups. Overall, on the basis of the presented endpoints standardized operational procedures can be defined for a proposed European adrenal tumor biobank.

[Disseminated nocardiosis : Diagnostic challenge]. / Disseminierte Nocardiose : Diagnostische Herausforderung.

Nocardiosis is a rarely found bacterial infection in Europe which can particularly affect immunocompromized patients. Localized infections of the dermis and lungs, as well as disseminated infections can be observed. Suspicion of nocardiosis should be reported to the microbiological laboratory so that goal-directed molecular genetic techniques and extended cultivation can be implemented for identification of the causative agent. A multitude of antibiotics can be used for successful therapy but the duration of therapy must be extended over 6-12 months. The mortality of disseminated infections ranges between 15-85% depending on the underlying immune status of the patient. The polymorphic appearance of nocardiosis is described based on the case of an intensive care patient.

Thoracic outlet syndrome: do we have clinical tests as predictors for the outcome after surgery?

OBJECTIVE: Thoracic outlet syndrome (TOS) is a clinical phenomenon resulting from compression of the neurovascular structures at the superior aperture of the thorax which presents with varying symptoms. Regarding to the varying symptoms, the diagnosis of TOS seems to be a challenge and predictors for the outcome are rare. The purpose of this study was therefore to analyze the different clinical examinations and tests relative to their prediction of the clinical outcome subsequent to surgery. METHODS: During a period of five years, 56 patients were diagnosed with TOS. Medical history, clinical tests, operative procedure and complications were recorded and analysed. Mean follow-up of the patients was 55.6 +/- 45.5 months, median age of the patients was 36.4 +/- 12.5 years. RESULTS: Different clinical tests for TOS showed an acceptable sensitivity overall, but a poor specificity. A positive test was not associated with a poor outcome. Analyses of the systolic blood pressure before and after exercise showed, that a distinct decrease in blood pressure of the affected side after exercises was associated with a poor outcome (p = 0.0027). CONCLUSIONS: Clinical tests for TOS show a good sensitivity, but a poor specificity and cannot be used as predictors for the outcome. A distinct decrease in blood pressure of the affected side after exercises was associated with poor outcome and might be useful to predict the patients' outcome.

Diagnosis and pre-operative management of multiple injured patients with explorative laparotomy because of blunt abdominal trauma.

INTRODUCTION: In trauma patients, injury of solid abdominal organs secondary to blunt trauma is a major source of morbidity and mortality. Different diagnostic options such as FAST sonography or CT scan have been described. METHODS: Our trauma registry was used to identify multiple injured patients with blunt abdominal trauma during 2001 to 2006. Patient demographics, diagnostic and operative findings, treatment, complications, length of stay and mortality were reviewed. RESULTS: Of 438 multiple injured patients, 58 patients were diagnosed with blunt abdominal trauma. During examination, free fluid or organ injury could be seen in 72.4% during sonography and in 84.3% of the patients who received CT scan, giving a sensitivity of 92% for initial FAST Sonography. Nevertheless, CT scan showed a higher sensitivity in detecting bowel (84%) or mesenteric (75%) injuries, if compared to FAST. 30 (51.7%) of the 58 patients had to undergo laparotomy because of blunt abdominal trauma, giving a laparotomy rate of 6.8% because of blunt abdominal trauma in multiple injured patients. CONCLUSION: Sonography is the method of choice for initial screening and CT scan in detecting bowel or mesenteric injuries. A large intraperitoneal fluid accumulation during initial sonography in combination with unstable vital signs should lead to an immediate exploratory laparotomy.

Recurrent ischemic colitis in a patient with leiden factor V mutation and systemic lupus erythematous with antiphospholipid syndrome.

Ischemic colitis results from insufficient blood supply to the large intestine and is often associated with hypercoagulable states. The condition comprises a wide range presenting with mild to fulminant forms. Diagnosis remains difficult because these patients may present with non-specific abdominal symptoms. We report a 51- year-old female patient with known Leiden factor V mutation as well as systemic lupus erythematous along with antiphospholipid syndrome suffering from recurrent ischemic colitis. At admission, the patient complained about abdominal pain, diarrhea and rectal bleeding lasting for 24 hours. Laboratory tests showed an increased C-reactive protein (29.5 mg/dl), while the performed abdominal CT-scan revealed only a dilatation of the descending colon along with a thickening of the bowel wall. Laparotomy was performed showing an ischemic colon and massive peritonitis. Histological examination proved the suspected ischemic colitis. Consecutively, an anti-coagulation therapy with coumarin and aspirin 100 was initiated. Up to the time point of a follow up examination no further ischemic events had occurred. This case illustrates well the non-specific clinical presentation of ischemic colitis. A high index of suspicion, recognition of risk factors and a history of non-specific abdominal symptoms should alert the clinicians to the possibility of ischemic disease. Early diagnosis and initiation of anticoagulation therapy or surgical intervention in case of peritonitis are the major goals of therapy.

A rare case of primary actinomycosis of the anterior abdominal wall: diagnosis and treatment.

We present the case of a 60-year-old man who presented with a left hypochondrial swelling first noticed 4 weeks prior to admission to our clinic. Based on the findings of the ultrasound and magnetic resonance imaging investigation, a tumour of uncertain origin of the abdominal wall was suspected, also involving the small bowel. The swelling, including the affected lateral and transverse oblique muscles as well as the subcutaneous tissue and the adjacent omentum majus, was completely excised. The resulting myoaponeurotic defect of the left lateral abdominal wall was closed with interrupted Vicryl sutures and stabilised with a PTFE prosthesis (20 x 10 cm) that was placed intraabdominally and secured by spiral tackers and interrupted transfascial monofilament Prolene sutures. Microscopic examination of the excised specimen revealed an actinomycosis of the anterior abdominal wall, which is extremely rare. The surgical treatment was followed by antibiotic therapy for 6 months. This treatment resulted in full recovery with no further complications.

Gluteal compartment syndrome after prostatectomy caused by incorrect positioning.

Gluteal compartment syndrome is an uncommon and rare disease. Most reasonable causes for the development of this disease are trauma, drug induced coma, Ehlers-Danlos syndrome, sickle cell associated muscle infarction, incorrect positioning during surgical procedures and prolonged pressure in patients with altered consciousness levels. The diagnosis requires a high index of suspicion, especially in postoperative patient where sedation or peridural anaesthesia can confound the neurological examination. Early signs include gluteal tenderness, decrease in vibratory sensation during clinical examination and increasing CK in laboratory findings. We present a case of a 52 year-old patient, who developed gluteal compartment syndrome after radical prostatectomy in lithotomic position. After operation, diuresis decreased [<50 ml/h] and CK [93927 U/l], LDH [1528 U/l], creatinin [1.5 mg/dl] and urea [20 mg/dl] increased in laboratory findings. Despite peridural anaesthesia, the patient complained about increasing pain in the gluteal region and both thighs. His thighs and the gluteal region were swollen. Passive stretch of the thighs caused enormous pain. The compartment pressure was 92 mmHg. Therefore, emergency fasciotomy was performed successfully. The gluteal compartment syndrome was most likely caused by elevated pressure on the gluteal muscle during operation. We suggest heightened awareness of positioning the patient on the operating table is important especially in obese patients with lengthy operating procedures.

Incidence of dysarthria in children with cerebellar tumors: a prospective study.

The present study investigated dysarthric symptoms in children with cerebellar tumors. Ten children with cerebellar tumors and 10 orthopedic control children were tested prior and one week after surgery. Clinical dysarthric symptoms were quantified in spontaneous speech. Syllable durations were analyzed in syllable repetition and sentence production tasks. Localization of the cerebellar lesions were defined after manual transfer from individual 2D-MR images onto 3D images of a spatially normalized healthy brain. Cerebellar children showed few and mild clinical signs of dysarthria. No difference was present in the sentence production task compared to controls. In five cerebellar children, syllables were prolonged in the syllable repetition task after surgery. Syllable duration normalized in an additional four-week session in all but one case. The MR-analysis showed that superior paravermal cerebellar areas likely involved in dysarthria in adults (paravermal lobules HVI, Crus I) were not significantly affected. In children, speech impairments appear to be rare after cerebellar surgery because tumors most commonly affect posterior-inferior and medial parts of the cerebellum while critical cerebellar regions are likely spared. The results suggest a similar localization of speech functions in the cerebellum in children and adults.

Cerebellar mutism--report of four cases.

The aim of the present study was to investigate the manifestations of mutism after surgery in children with cerebellar tumors. Speech impairment following cerebellar mutism in children was investigated based on standardized acoustic speech parameters and perceptual criteria. Mutistic and non-mutistic children after cerebellar surgery as well as orthopedic controls were tested pre-and postoperatively. Speech impairment was compared with the localization of cerebellar lesions (i. e. affected lobules and nuclei). Whereas both control groups showed no abnormalities in speech and behavior, the mutistic group could be divided into children with dysarthria in post mutistic phase and children with mainly behavioral disturbances. In the mutistic children involvement of dentate and fastigial nuclei tended to be more frequent and extended than in the nonmutistic cerebellar children. Cerebellar mutism is a complex phenomenon of at least two types. Dysarthric symptoms during resolution of mutism support the anarthria hypothesis, while mainly behavioral changes suggest an explanation independent from speech motor control.

Malignant melanoma of the anal region.

Malignant melanoma (MM) of the anal region is an uncommon disease. In many cases, the disease is undetected or mistaken for a benign polyp or haemorrhoids until it reaches an advanced state. Owing to delayed diagnosis and early metastases, the prognosis is often poor. In contrast to melanomas of the skin, a history of sun exposure does not seem to have an impact in development of MM in this region. Anorectal melanomas (AM) are most common in the rectum, followed by the anal canal and anal verge. Ras mutations, especially in codon 61 of the N-ras oncogene, are common in CM and rare in melanomas of the vulva and anorectum. The diagnosis of an AM is usually made using a biopsy. Histopathological examinations show spindle-shaped and pleomorphic cells. Adjuvant immunohistological markers are the calcium-binding protein S-100, the melanoma antigen HMB-45, the melanoma-expressed protein Melan A, and microphthalmia-associated transcription factor (MiTF). To date, there are few published guidelines for the correct management of AM, and surgery remains the mainstay of treatment. We report on a 39-year old man who presented with a 5-week history of recurrent prolapse of an anal tumour. The tumour was histologically confirmed to be malignant melanoma.

Do children with focal cerebellar lesions show deficits in shifting attention?

More recent findings suggest a possible role of the cerebellum in nonmotor functions. Disability of individuals with cerebellar damage in rapidly shifting attention is one frequently used example to support cerebellar involvement in mental skills. The original proposal was based on findings in five children with chronic surgical lesions of the cerebellum and a young adult with a degenerative disorder. The aim of the present study was to repeat Akshoomoff and Courchesne's initial findings in a larger group of children with focal cerebellar lesions. Ten children with cerebellar lesions and 10 age- and sex-matched controls were tested. Neocerebellar areas were affected in all children with cerebellar damage except one based on detailed analysis of MRI scans. Subjects had to perform a focus and a shift attention task. Two visual and two auditory stimuli were presented in a pseudorandom order. An ellipse and a high-pitched tone were presented less frequently than a circle and a low-pitched tone. Rare stimuli were presented at five different time intervals. In the focus tasks, subjects had to react to the same rare stimulus of one of the two modalities. In the shift task, subjects had to switch between the two rare stimuli. Motor deficits based on reaction times were small in cerebellar children compared with controls. The ability of target detection did not significantly differ in the children with cerebellar lesions compared with the control children in both the focus and the shift attention task. In particular, children with cerebellar damage showed no significant impairment in rapid (<2 s) shifts of attention. The present findings indicate that the cerebellum may be less critical in attention related processes than suggested previously.