Arteritis of the large vessels originating at the aorta (Takayasu arteritis) in childhood (author's transl)]

A 9-month-old female child with extensive inflammatory lesions in the large arterial trunks originating at the aorta (Takayasu arteritis) was reported. Severe cerebral, intestinal and coronary circulatory disturbances as well as insufficient blood supply in both arms, particularly the left arm, developed as a result of the stenoses and obliterations. Clinical course, postmortem findings and the results of EKG, EEG and carotid angiography were discussed. The onset in infancy and the very rapid progression of the disease were unusual.     

Immunohistochemical localization of troponin-C in cultured neurons

Our previous immunofluorescence studies on neurons have demonstrated the presence of myosin in regions of neurons which contained actin. To determine if a system similar to the troponin complex of striated muscle is present in neurons, antibody shown to be specific for the calcium-binding component of troponin (troponin-C) was applied to cultures of embryonic chick and rat dorsal root ganglia. Neurites treated with anti-troponin-C exhibited a bright fluorescence. Accompanying non-neuronal cells were less reactive than the neuronal elements. Immunodiffusion and immunofluorescence showed that the anti-troponin-C did not react with calmodulin, whereas homogenates of the ganglia elicited a positive immunochemical reaction with the anti-troponin-C in Ouchterlony tests. Our results suggest that some intra-axonal movements may be generated by the interaction of actin and myosin and controlled in part by a calcium-troponin-C-dependent mechanism.     

Relative uptake, metabolism, and beta-receptor binding of (1R,2S)-4-(18)F-fluorometaraminol and (123)I-MIBG in normotensive and spontaneously hypertensive rats.

The objective of the study was to compare relative uptake, metabolism, and beta-receptor affinity of the new positron-emitting uptake-1 tracer (1R,2S)-4-(18)F-fluorometaraminol (4-FM) with those of the SPECT pharmaceutical meta-(123)I-iodobenzylguanidine (MIBG) in Wistar Kyoto (WKY) rats and spontaneously hypertensive (SHR) rats. METHODS: No-carrier-added 4-(18)F-FM was applied to SHR and WKY rats in vivo and to retrogradely perfused hearts in vitro. Cardiac and extracardiac distribution was assessed, and metabolite formation was determined by thin-layer chromatography. The in vivo experiments were repeated with no-carrier-added (123)I-MIBG. By means of autoradiography, the beta-receptor affinity of 4-FM was compared with that of MIBG and propranolol (10 micromol/L) through displacement of (125)I-iodocyanopindolol (1.5 pmol/L) in slices of heart and spleen. RESULTS: Cardiomyopathic hearts showed heterogeneous 4-(18)F-FM uptake with gradients up to 3.6 in vivo and in vitro between different regions of the heart. Control hearts showed such gradients in 4-(18)F-FM uptake only in vitro. (123)I-MIBG exhibited a less heterogeneous in vivo distribution in SHR hearts. Extracardiac differences between WKY and SHR were found for uptake of 4-(18)F-FM in the spleen (63.3% plus minus 4% vs. 38.8% plus minus 5.7% of cardiac activity) and for renal uptake of (123)I-MIBG (373% plus minus 27% vs. 81.4% plus minus 17% of cardiac activity). Metabolites of 4-(18)F-FM were found only in the liver and those of (123)I-MIBG were found in the liver and kidney with a nearly equal relative fraction in both types of animals of about 20%, 60%, and 30%, respectively. 4-FM suppressed cardiac-specific beta-receptor binding of (125)I-iodocyanopindolol in heart and spleen of both types of animals significantly, whereas MIBG had almost no effect. CONCLUSION: The more heterogeneous cardiac distribution of 4-(18)F-FM suggests that it reflects alterations in uptake-1 better than (123)I-MIBG in addition to the possibility of quantification and higher spatial resolution by PET compared with SPECT. Altered biotransformation in cardiomyopathic diseases may also impair the evaluation of (123)I-MIBG-SPECT data. The beta-receptor binding of 4-(18)F-FM must be further elucidated.     

Die Peritonealdialyse zur Behandlung reifer und unreifer Neugeborener mit Atemnosyndrom

Some authors have recommended peritoneal dialysis to improve the therapy of the respiratory distress syndrome (RDS) of newborns. In 1968 we published a report estimated on a newborn suffering from RDS. As a pH of 6.73, was 2 hrs after birth, we administered standard therapy and peritoneal dialysis. The baby has survived and his development has been uneventful. This case and the results obtained by other authors in the treatment of RDS with peritoneal dialysis have led us to treat prematures and newborns suffering from severe RDS with peritoneal dialysis in addition to standard therapy (infusions of THAM, glucose, electrolytes, amino acids, administration of antibiotics and oxygen, and, when indicated, intermittent positive-pressure respiration). Peritoneal dialysis was carried out with bland hypertonic solution in continuous flow technics. We have treated 13 prematures and 1 newborn suffering from RDS and a premature suffering from erythroblastosis with hydrops and RDS. Except for the mature newborn, the gestational age was 25 to 33 weeks. Four prematures had birth weights below 1250 g. The Apgar score of 10 newborns was 3 or less 1 min after birth. Thirteen newborn needed resuscitation in the delivery room. Eight newborns were treated with respiration during the first few hours after birth and 4 other prematures at a later stage. Clinical and laboratory data after birth, on admission to the pediatric hospital and during the course of the disease are shown in tables and figures. The indications for the peritoneal dialysis are described in tables. In 2 patients we saw no positive effect, and we saw only a slight effect in 4 patients. Four prematures survived. The physical and psychological development was normal in 3 infants. One premature with a birth weight of 1200 g had recovered before she died of Candida sepsis at the age of 26 days. Results of the treatment and the efficacy and technical problems of peritoneal dialysis in RDS of newborns are discussed. Our opinion is that the peritoneal dialysis is a good additional aid in the treatment of RDS of prematures and newborns.     

Wirkungen von Ketoconazol auf das Immunsystem III. In vitro-Testungen von Ketoconazol und Therapie-Verlaufskontrollen bei chronischen Dermatophytosen/Effects of Ketoconazole on the Immune System III. In Vitro Drug Testing and Follow-up Studies in Chronic Dermatophytoses

Zusammenfassung: Die Resultate der In-vitro Testung von Ketoconazol sowie Therapiever-laufskontrollen unter Ketoconazol von 7 Patienten mit Dermatophytosen werden vorgestellt Es gelang, eine "krankheitstypische" Immunreaktion herauszufinden, die sich im Falle des Ansprechens auf die Therapie Normwerten angleicht Wir konnten beobachten, daß eine Normalisierung der Befunde unter Therapie mit einem gleichartigen In-vitro-Effekt von Ketoconazol vor Therapiebeginn korrelierbar war. Kulturen mononukleärer Zellen der Patienten fielen durch erhöhte spontane Proliferationen, durch spontane IFNγ-Synthese sowie spontane Interleukinsynthese auf.
Die Hemmung der Spontanproliferation durch Ketoconazol in vitro korrelierte mit der späteren Ansprechbarkeit der Patienten auf die Therapie.
Summary: We present the results of in vitro testing of ketoconazole sensitivity in comparison to follow-up studies under ketoconazole therapy of 7 patients suffering from chronic dermatophytosis. We are able to demonstrate an "illness-related" immuno-reactivity of patients' mononuclear cells which come back to normal levels in the case of successful therapy with ketoconazole. The normalisation of immune parameters under therapy was correlated to analogous in vitro effects of ketoconazole prior to therapy. Patients' lymphocytes proliferate spontaneously, secrete IFNγ and interleukins, also spontaneously. The reduction of spontaneous proliferation by ketoconazole in vitro was correlated to successful ketoconazole therapy.     

Steroid hormone-binding proteins in blood plasma

Two types of proteins form dissociable complexes with the circulating steroid hormones in blood serum: albumin, the most abundant plasma protein, and the highly specific glycoproteins that occur in low concentrations: corticosteroid-binding globulin (CBG or transcortin), sex steroid-binding protein (SBP) and progesterone-binding globulin (PBG). Albumin interacts with low affinity, predominantly by hydrophobic bonding; the affinity constants of the steroid complexes with the specific globulins are higher by several orders of magnitude. CBG and SBP have molecular weights similar to that of albumin; PBG appears to be larger. A fairly general characteristic of these steroid-binding glycoproteins is their tendency to polymerize. Rat CBG forms dimers, tetramers and octamers upon removal of steroid from the complex; stoichiometric recombination with corticosterone completely reverses the polymerization. Estrogenic hormones increase the level of CBG and of SBP; androgenic hormones have the opposite effect. A CBG-depressing influence of corticosteroid hormones has been observed in the rat; adrenalectomized rats have increased CBG activity. The thyroid-stimulating hormone of the pituitary is required for the estrogen-induced rise of CBG synthesis in the rat; the effect is mediated by the thyroid gland. In accordance, thyroidectomy reduces the CBG activity whereas administration of thyroxin results in an increase. In contrast to all other species investigated, guinea pig serum contains separate proteins specific for binding of corticosteroid (CBG) and of progesterone (PBG). PBG levels increase about hundred-fold in pregnancy. PBG is a glycoprotein of χ₂- or β-globulin mobility with an approximate molecular weight of 100,000 and an association constant for progesterone-binding higher than any of the other steroid-serum protein complexes. Association with serum proteins suppresses the biological activity of steroid hormones. Bound hormone in blood constitutes an inactive pool which protects the hormone from metabolic and chemical alterations and provides, by reversible dissociation, a buffer against sudden changes in active hormone concentrations.     

Psychometric validation of a German language version of a PROM for urethral stricture surgery and preliminary testing of supplementary ED and UI constructs

Purpose

To validate a German language version of the patient-reported outcome measurement (PROM) following urethral stricture surgery (USS) in a cohort of men undergoing one-stage buccal mucosa graft urethroplasty (BMGU) for urethral stricture. Furthermore, to explore the responsiveness of erectile function (EF) and urinary incontinence (UI) constructs in the context of this intervention.

Methods

The USS-PROM captures voiding symptoms (ICIQ-MLUTS) and health-related quality of life (HRQoL) (EQ-5D). To evaluate EF and UI, the IIEF-5 and ICIQ-UI SF were included. Between March 2012 and April 2013, all patients undergoing BMGU at our institution were prospectively enrolled in this study. Psychometric assessment included internal consistency, test–retest reliability, criterion validity and responsiveness.

Results

Ninety-three men completed the USS-PROM before and 3 months after surgery, with 40 (43 %) also completing the USS-PROM 6 months after surgery to assess reliability. Internal consistency: for the ICIQ-MLUTS, Cronbach’s α was 0.83. The test–retest intraclass correlation coefficient was 0.94. There was a negative correlation between change in ICIQ-MLUTS total score and change in Q _max (r = ?0.40). All values exceeded our predefined thresholds. Significant improvements of voiding symptoms and HRQoL demonstrate responsiveness to change (all p values <0.001). While ICIQ-UI scores did not change (p > 0.05), IIEF-5 scores improved significantly (p = 0.048).

Conclusions

The German language USS-PROM shows similar psychometric properties to the English language version. This instrument can be improved by assessing EF by the use of IIEF-5. Further studies with larger patient cohorts are needed to evaluate the significance of measuring UI in urethroplasty patients.

     

Maternal risks and predictor factors for the termination of pregnancy in fetuses with severe congenital anomaly: experience from a single reference center in Brazil

Objective: To assess the maternal complications in pregnant women with fetuses with several congenital anomaly as well as the predictor variables for the termination of pregnancy.

Methods: We performed a retrospective cohort study with 94 medical records of pregnant women with fetal infeasibility confirmed in the postnatal period by clinical, radiological or anatomopathological exams. To compare the categorical variables regarding the termination and nontermination of pregnancy, we used analysis of variance (ANOVA) and the Mann–Whitney U-test. To assess the variables that were more associated with the judicial request for the termination of pregnancy, we used logistic regression.

Results: The termination of pregnancy was performed in 41 (43.6%) and nontermination of pregnancy in 53 (56.4%) pregnant women. Pregnant women who did not terminate the pregnancy had more complications in the gestational period (p?<?0.0001) and in the postpartum period (p = 0.0088). After multiple logistic regressions, the following variables influenced the decision to terminate the pregnancy: type of congenital anomaly (OR: 18.59; 95%CI: 1.96; 175.87) and living children (OR: 0.45; 95%CI: 0.25; 0.80).

Conclusion: Most of the pregnant women with fetal infeasibility opted for nontermination of pregnancy and these patients had more obstetrical complications. The type of congenital anomaly and living children were the factors most associated with the choice for the termination of pregnancy.