[Selected biomarkers in the primary tumors of the central nervous system: short review]. / Vybrané biomarkery primárnych nádorov centrálneho nervového systému: krátky prehlad.

Classification, grading and treatment of central nervous system tumors is currently based on morphology. Advances in molecular biology help to clarify pathogenesis, refine prognosis and detect potential targets for targeted therapy in a wide spectrum of CNS tumors. In this short review we present our view on selected diagnostic, prognostic and predictive biomarkers of primary CNS tumors, with an emphasis on application in daily praxis.

[Carney complex]. / Carneyho komplex.

Carney complex is a clinically and genetically heterogeneous disease, with at least two genetic loci including the PRKAR1A gene located on chromosome 17 and the CNC2 locus mapped to chromosome 2. Clinically this syndrome is characterized by multiple myxomas occurring in different anatomic sites, mucocutaneous pigmentary lesions, and a variety of non-endocrine and endocrine tumors, often causing endocrine abnormalities, involving various organs. Knowledge of morphological findings in CNC patients with their typical locations is necessary to raise suspicion of this syndrome by pathologists. Confirmation of the diagnosis allows regular clinical check-ups and early treatment of these patients.

Fatal venous thrombembolism complicating imatinib therapy in a patient with metastatic gastrointestinal stromal tumor.

Imatinib, a tyrosine kinase inhibitor, is currently the therapy of choice for gastrointestinal stromal tumor (GIST). The toxic effects of imatinib treatment are usually mild, and serious adverse events are rare. We report here the case of a patient with peritoneal metastases of GIST involving the pelvis treated by imatinib. Abdominal pain deteriorated early in the course of the therapy along with the enlargement of the tumors. The patient died suddenly, and the autopsy revealed pulmonary embolism originating from the deep vein thrombosis caused by the compression of common iliac vein by the tumor. The possibility of deep vein thrombosis caused by the compression of the veins by necrotic tumor should be considered in patients with abdominal or pelvic metastases of GIST, including patients treated with imatinib.

[Tumorous diseases in patients with the testicular feminization syndrome ("androgen insensitivity" syndrome)--description of two cases]. / Náidorová onemocnení pacientu se syndromem testikulární feminizace ("androgen insensitivity" syndrom)--popis dvou prípadú.

OBJECTIVE: To describe tumors occurring in two cases of testicular feminization syndrome. SUBJECT: Case report. SETTING: Dpt. of Special Diagnostics SPAU, University Hospital Plzen. SUBJECT AND METHOD: Two cases of testicular feminization syndrome were selected from four cases in our registry. Patients were 45 and 84-year-old Caucasian "females". Resected material was fixed in formaldehyde, routinely processed and stained with hematoxiline-eosin, inhibin, cytokeratines 20, placentar alkaline phosphatase, CD 99, Melan A, hCG. Sertoli cell adenoma was diagnosed in both patients. Older patient had in addition unclassified sex cord tumor of Leydig cell type. The number of sex chromosomes was examined using FISH analysis in both patients. CONCLUSION: Patients with testicular feminization syndrome are frequently affected by benign or malignant tumors in the cryptorchid testes. We documented two benign Sertoli cell adenomas and one sex cord tumor of uncertain biological behavior in our patients. The testes should be removed after puberty with subsequent estrogen therapy in patients with testicular feminization syndrome.

The efficacy of laparoscopic examination of the internal inguinal ring in children.

The ability of physicians to identify a patent processus vaginalis by laparoscopic examination of the internal ring is now well established, but the efficacy on patient outcome is not. The authors reviewed their experience to determine the effect of diagnostic laparoscopy of the internal ring on the management of children with inguinal hernias. The records of 150 children who underwent inguinal surgery were reviewed--75 before (group 1) and 75 after (group 2) pediatric laparoscopy was introduced into the authors' practice. The children in group 1 were selected for unilateral or bilateral surgery based on history, age, sex, side of presentation, and parental preference. For group 2, laparoscopy was an additional option offered to appropriate patients. Laparoscopy was performed in 43 group 2 patients, using an infraumbilical site. The minimum follow-up period was 2 years for group 1 and 1 year for group 2. The mean ages for groups 1 and 2 were 41.2 and 39.7 months, respectively. There were 61 boys and 14 girls in each group. The percentages of right (R), left (L), and bilateral (B) findings, based on clinical observation, were 56.0 (R), 29.3 (L), and 14.7 (B) for group 1, and 58.7 (R), 26.6 (L), and 14.7 (B) for group 2. The incidence of bilateral surgical exploration was similar for the two groups (group 1, 58.6%; group 2, 61.3%). The addition of laparoscopy significantly lowered the incidence of negative explorations (group 1, 16.0%; group 2, 2.6%; P < .01).(ABSTRACT TRUNCATED AT 250 WORDS)

[Traumatic odontoid synchodrotic slip in a 2-year old child]. / Traumatisk densløsning hos et to år gammelt barn.

We report a case of a two-and-a-half year-old child involved in a head-on motor vehicle accident, which is a typical mechanism of traumatic injury of the odontoid process in small children. The child was neurologically intact and the first X-ray was without signs of fractures. Two weeks later, X-ray revealed a 1 cm anterior luxation of the odontoid process. After closed reduction the child was treated conservatively with a Minerva jacket, which led to consolidation.

[Traumatic tricuspid valve insufficiency]. / Traumatisk trikuspidalinsufficiens.

We report a case of tricuspid insufficiency in connection with blunt chest trauma. The patient was involved in a car accident. The central venous catheter showed a right ventricular pressure curve, suggestive of a tricuspid valve insufficiency. A transoesophageal echocardiographic examination supported this by revealing a papillary muscle rupture. This kind of injury has been seen more frequently during the last 35 years, partly because of better diagnostic procedures and a better understanding of the pathology. The decelerating force in the right ventricular chamber produces a regurgitation thereby initiating rupture of the papillary muscle and/or the chordae tendinae. In a literature study twelve out of thirteen patients were involved in car accidents. Some of them had a dominant V-wave in the venous pulse curve, but no clinical observation is directly diagnostic. Therefore, cardiac lesions should be kept in mind whenever there is a history of blunt chest trauma. The best diagnostic approach is echocardiography.

Time-resolved near-edge x-ray absorption fine structure spectroscopy on photo-induced phase transitions using a tabletop soft-x-ray spectrometer.

We present a table-top soft-x-ray spectrometer for the wavelength range λ = 1-5 nm based on a stable laser-driven x-ray source, making use of a gas-puff target. With this setup, optical light-pump/soft-x-ray probe near-edge x-ray absorption fine structure (NEXAFS) experiments with a temporal resolution of about 230 ps are feasible. Pump-probe NEXAFS measurements were carried out in the "water-window" region (2.28 nm-4.36 nm) on the manganite Pr(0.7)Ca(0.3)MnO(3), investigating diminutive changes of the oxygen K edge that derive from an optically induced phase transition. The results show the practicability of the table-top soft-x-ray spectrometer on demanding investigations so far exclusively conducted at synchrotron radiation sources.

Extraordinary response to erlotinib therapy in a patient with lung adenocarcinoma exhibiting KRAS mutation and EGFR amplification.

Case reports on the co-incidence of Kirsten rat sarcoma (KRAS) mutation and epidermal growth factor receptor (EGFR) amplification in patients with NSCLC are very rare. This combination is usually considered a negative prognostic factor, despite EGFR amplification alone having positive predictive value. The whole course of treatment of a patient with both EGFR amplification and KRAS mutation present is decribed. The patient in question was a smoker for whom both first- and second-line chemotherapy had been unsuccessful. In stage IV disease biological therapy was administered and proved highly beneficial. Today, 38 months since commencing the treatment, the patient still has no signs of progression and the therapy is still in progress.

[Hemodialysis in children with kidney failure in the GDR]. / Hämodialyse niereninsuffizienter Kinder in der DDR.

From our experiences with more than 9.000 haemodialyses in children we render the following three general conclusions: 1. The chronic haemodialysis and the transplantation of a kidney stood the test in the therapy of chronic renal insufficiency in childhood. 2. The most favourable conditions for the performance of the treatment with dialysis in children consist within a special paediatric institution which is achieved by the formation of dialysis centres for children in the GDR. 3. The success of the treatment with dialysis essentially depends on the preparation of the child and on the good cooperation of all persons participating in the treatment. The care system in the GDR which was built up together with the working team children nephrology in 1970 essentially contributes to this fact.

[Dislocation following total hip endoprosthesis. Association with surgical approach and other factors]. / Luxationen nach Hüft-TEP-Implantationen: Abhängigkeit vom operativen Zugang und anderen Faktoren.

During a period of eight years (1984 to 1992) we found 45 dislocations in 1734 patients treated by total hip arthroplasty. We compared this retrospectively evaluated group with a randomized control group of 61 patients. As a cause for an increasing number of dislocations in 1989 we found: age over 70 years, high CCD-stem-angle (142 degrees), dorsal approach, abduction of the acetabular component under certain circumstances (left side). Revision operations always are a high risk procedure. Acetabular anteversion, anaesthesia, way of component fixation did not induce an increased rate of dislocation. We now left the dorsal approach and use only stems with an CCD-angle of 135 degrees or less by now. Careful positioning of the acetabular components and a better patient-instruction helped to decrease the number of dislocations. Because of these measures we reduced the THA-dislocations rate from 5.6 to 0.17 per cent (first operations) and from 12.1 to 6.5 per cent (revision operations).

[The effect of pH, temperature and albumin content on the vitality of fetal rat liver cells during in vitro tests]. / Einfluss von pH, Temperatur und Albumingehalt auf die Vitalität von Leberzellen fetaler Ratten bei in vitro-Versuchen.

The influence of pH, temperature, and albumin content in the incubation medium on the vitality incubated hepatocytes was tested in vitro as well as the effects in the trypan-blue exclusion test. pH changes between 6.8 and 7.4 in the incubation medium have not any influence on the vitality of the hepatocytes or the trypan-blue exclusion test. Reduced vitality results occurred in an initial pH 7.8 in the incubation medium. The vital dye exclusion test is not useful in case of incubation temperature of 4 degrees C, since the results regarding the vitality of the hepatocytes are only apparently better. An addition of human serum albumin is necessary for a good survival of the hepatocytes during the incubation.

[Metabolic changes in bones and modification of growth in children with chronic renal failure, dialyzed and with kidney transplant]. / Stoffwechselstörungen der Knochen und Wachstumsbeeinflussung chronisch niereninsuffizienter, dialysierter und transplantierter Kinder.

Forty to fifty percent of the children with chronic renal insufficiency show a growth retardation. The cause of this disorder is supposed on the cellular level of the target organ bone. Objective prospective studies on the growth characteristics of children with chronic renal insufficiency after dialysis and transplantation, respectively, are necessary in consideration of the internal milieu and external influences.

[Future occupational plans of patients after orthopedic rehabilitation: how are they expressed? What influences them?]. / Berufliche Zukunftsvorstellungen von Patienten nach orthopädischer Rehabilitation: Wie drücken sie sich aus? Welche Faktoren beeinflussen sie?

Perceptions about one's occupational future are regarded as the initial phase in an ongoing process of occupational resettlement towards achieving gainful activity. Actual gainful activity, however, tends to be increasingly dependent on a number of labour market variables. At the end of an orthopaedic rehabilitation measure, 341 patients with pain syndromes of the locomotor system were interviewed concerning their vocational perspectives and notions, and the data obtained were studied in association with anamnestic and psychosocial factors. It is assumed that both plain expectations (such as returning, or not returning, to work) and multi-layered statements alike (such as the dual message of returning and of not returning to work, at first glance incompatible) will frequently be masked in the interview. Distinct, differing patterns were nevertheless found for the various patient groups regarding pain description, pain course, subjective disablement by pain, subjective job burden, subjective well-being, rehab expectations, and judgement of therapy outcome. Similar differences were stated for the social situation, indicated inter alia by (in)capacity for work, or application for benefit award (severely disabled person status, invalidity pension). Pain drawings, pain severity scaling, differentiated description of various pain qualities, the Pain Disability Index, all emerged as useful and practical additional tools in elucidating these patterns.

[Initial results of tm-PAH studies in children with kidney transplants]. / Erste Ergebnisse der Tm-PAH-Untersuchungen bei nierentransplantierten Kindern.

In 15 children 2 months to 5 years after a transplantation of a cadaver kidney the renal function was determined by means of a clearance- and Tm-PAH-examination. Only in 4 children the Tm-PAH reached the normal value of a kidney. The calculation of the glomerulo-tubular balance results in increased values. The result for the relative tubular blood supply is normal. Compared with the values of children with an overcome haemolytic-uraemic syndrome or with a chronic glomerulonephritis or pyelonephritis shows that the renal function in children who underwent a transplantation similarly behaves as in a chronic pyelonephritis.

[Growth of transplanted kidneys]. / Das Wachstum transplantierter Nieren.

Two or more years after a kidney transplantation were determined the GFR as 51Cr-EDTA-clearance and the effective renal plasma flow as 125J-hippuran clearance. In 14 children the results of the clearance with the HLA-identity (HLA-I), the quantity of the transfusion units (TU) received before the transplantation and the duration of the cold ischaemia time (CIT) were correlated. While with HLA-I and TU no connections could be proved, the results of the clearance seemed to be reversedly proportional to the CIT (r = -0.53 and -0.64 for GFR and ERPF) for a longer time after transplantation. Different age between donor and recipient shows worse results of clearance in the group of the adults who received a kidney of a child, in contrast to the other three possible combinations of the kidney transplantation.

[Sonographic diagnosis of the juvenile tarsus. Clinical application possibilities demonstrated by examples]. / Sonographische Diagnostik am kindlichen Tarsus. Klinische Einsatzmöglichkeiten anhand von Beispielen.

Radiologic assessment of foot deformities in young children is limited especially by the ossification status. Ultrasonography proved to be a useful diagnostic supplement e.g. in idiopathic talipes equinovarus, vertical talus, and tarsal coalitions. Effects of treatment and three-dimensional movements of the tarsal joint complex can be delineated. Sonographic assessment of tarsal alignment is applicable already in the first months of life. This study deals with first experiences in clinical application of this new diagnostic technique.

[Glomerulonephritis (GN) in childhood. I. Epidemiology, clinical aspects and kidney function in 125 children]. / Zur Glomerulonephritis (GN) im Kindesalter. I. Mitteilung: Epidemiologie, Klinik und Nierenfunktion von 125 Kindern.

The blood pressure, urinary symptoms (proteinuria, hematuria, casts), the albumin- and cholesterol concentration in the serum and the renal function (GFR, RPF) of 125 children with chronic glomerulonephritis (GN) were analysed. 76 children had only single symptoms (93% proteinuria, 33% hypertension), which indicated a GN. The serum albumin and cholesterol concentration were pathological in 43% of the patients and serum creatinine level was pathological in 20% of the children. After 6 years the individual courses of renal function demonstrated a deterioration of GFR and RPF for most of the children. It can be concluded, that the summary consideration of epidemiology, symptomatology and renal function of different glomerular lesions has only a limited application to the clinical practice.