Cystic fibrosis in Down's syndrome--diagnostic pitfalls and implications for the clinician.
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Cystic Fibrosis↗
Biomedical subjects
R Sailer
Publications and source records attributed to R Sailer.
Selective examination of plasma membrane-associated photosensitizers using total internal reflection fluorescence spectroscopy: correlation between photobleaching and photodynamic efficacy of protoporphyrin IX.
Fluorescence spectra, fluorescence decay kinetics, photobleaching kinetics and photodynamic efficacy of protoporphyrin IX (PP) were investigated in endothelial cells in vitro after different incubation times. Fluorescence spectra and photobleaching kinetics were determined during total internal reflection (TIR) illumination or epi-illumination. Because penetration depth of the excitation light during TIR illumination was limited to about 100 nm, plasma membrane-associated PP was almost selectively examined. Spectra obtained by TIR fluorescence spectroscopy (FS) showed a very low background, whereas spectra obtained by epi-illumination exhibited considerable background by autofluorescence and scattered light. For photobleaching kinetics during TIR illumination after 1 h or 24 h incubation, a biexponential fluorescence decrease was observed with a rapidly and a slowly bleaching portion. After 1 h incubation, the rapidly bleaching portion was the predominant fraction, whereas after 24 h incubation comparable relative amounts of the rapidly and slowly bleaching portion were determined. The rapidly and slowly bleaching portion were assigned to PP monomers and aggregated species in close vicinity to the plasma membrane. Fluorescence decay measurements after epi-illumination support the decrease of PP monomers within the whole cell with increasing incubation time. In contrast to TIR illumination, photobleaching of PP during epi-illumination was characterized by slow monoexponential fluorescence decrease after 1 h or 24 h incubation. Photodynamic efficacy of PP using epi-illumination was found to depend strongly on incubation time. Considerable cell inactivation was determined for short incubation times (1 h or 3 h), whereas photodynamic efficacy was diminished for longer incubation times. Reduced photodynamic efficacy after long incubation times was assigned to the lower amount of photodynamically active monomers determined close to the plasma membrane as well as within the whole cell. In conclusion, TIRFS measurements are suggested to be an appropriate tool for the examination of the plasma membrane-associated photosensitizer fraction in living cells.
Animals↗
Time-gated fluorescence microscopy in cellular and molecular biology.
An experimental set-up for time-gated fluorescence spectroscopy and microscopy is described, and some recent applications in cellular and molecular biology are summarized. Selective detection of intrinsic fluorophores, in particular nicotinamide adenine dinucleotide (NADH) and flavins was demonstrated in living cells. Non-radiative energy transfer from reduced NADH to the mitochondrial marker rhodamine 123 was evaluated for probing mitochondrial malfunction in living cells. An increase of "energy transfer efficacy" up to a factor 4 was detected after inhibition of enzyme complexes of the respiratory chain. Two different fluorescence lifetimes of calcium orange were evaluated, whose relative intensities depended on calcium concentration. Therefore, fluorescence measured within two different time gates appeared to be suitable for ratio fluorometry of calcium. Time-gated fluorescence spectra of the membrane marker laurdan showed more pronounced changes than steady state spectra when temperature was increased from 24 degrees C to 38 degrees C. This may improve measurements of intracellular temperature. Time-gated detection of small amounts of porphyrins and their discrimination from a large fluorescent background caused by chlorophyll in transgenic tobacco plants again proved the advantages of time-gated fluorescence spectroscopy.
2-Naphthylamine↗
[Interventional occlusion of systemicopulmonary collateral arteries using metal spirals].
Major systemicopulmonary collateral arteries (spca) frequently contribute to collateral lung perfusion in patients with pulmonary atresia and ventricular septal defect or in children with tetralogy of Fallot. Since the surgical access to these vessels is difficult, corrective surgery may become impossible. We report our experience with interventional occlusion of spca in 10 patients. In these patients (age range 13 months-19.5 years) selective injections demonstrated a total of 27 spca. Sixteen of these were coil-occluded by interventional cardiac catheterization. Total occlusion was achieved in 15 cases, in one case we found a small residual shunt. Occlusion was performed using platinum coils (2 cases), Gianturco coils (11 cases) and detachable steel coils (4 cases, including 1 with prior incomplete occlusion by Gianturco coils). 4 patients required 2 interventional cardiac catheterizations. Complications occurred only in procedures that were performed with conventional coils (peripheral pulmonary embolizations of platinum coils in 2 patients, dislocation of the delivery catheter with a partially extruded coil to the descending aorta in 1 patient). Operative ligation had to be performed in 6 spca, since those vessels were not suitable for interventional occlusion. The remaining 5 spca were left unoccluded, since they were of minor hemodynamic relevance due to a subsequent decrease in size. At the end of the follow-up period corrective surgery had been completed in 8 of our 10 patients. In the remaining 2 patients corrective surgery is planned in the near future. According to our experience interventional occlusion of spca is a major improvement in the management of a selected cohort of patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot. The introduction of detachable steel coils facilitates the embolization of those vessels.
Adolescent↗
Photodynamic efficacy of naturally occurring porphyrins in endothelial cells in vitro and microvasculature in vivo.
Photodynamic therapy (PDT) has been described in terms of cellular and vascular effects. The precise mechanisms of cellular and vascular damage are still unknown. In this study, the photodynamic inactivation of endothelial cells in vitro and damage to the microvasculature in vivo by naturally occurring porphyrins (uroporphyrin III (UP), coproporphyrin III (CP) and protoporphyrin IX (PP)) were investigated. The chick chorioallantoic membrane model (CAM model) was used, which is convenient for the study of damage to the microcirculation induced by PDT. The hydrophilic porphyrins UP and CP exhibited low cytotoxicity towards endothelial cells. Only small amounts of UP and CP were taken up, resulting in weak inactivation after irradiation. In contrast, the more lipophilic PP showed a marked cytotoxicity. Considerable amounts of PP were accumulated in the cells, leading to pronounced inactivation after light exposure. For the three porphyrins, damage to the microvasculature was observed. The damage caused by the hydrophilic porphyrins UP and CP was strongly dependent on the drug and light dose. For vascular injury, the efficacy was graded as UP < CP < PP.
Animals↗
Energy transfer spectroscopy for measuring mitochondrial metabolism in living cells.
Microscopic energy transfer spectroscopy was established using mixed solutions of reduced nicotinamide adenine dinucleotide (NADH) and the mitochondrial marker rhodamine 123 (R123). This method was applied to probe mitochondrial malfunction of cultivated endothelial cells from calf aorta incubated with various inhibitors of specific enzyme complexes of the respiratory chain. Autofluorescence of the coenzyme NADH as well as energy transfer efficacy from excited NADH molecules (energy donor) to R123 (energy acceptor) were measured by time-gated fluorescence spectroscopy. Because intermolecular distances in the nanometer range are required for radiationless energy transfer, this method is suitable to probe selectively mitochondrial NADH. Autofluorescence of endothelial cells usually exhibited a weak increase after specific inhibition of enzyme complexes of the respiratory chain. In contrast, pronounced and statistically significant changes of energy transfer efficacy were observed after inhibition of the same enzyme complexes. Detection of NADH and R123 in different nanosecond time gates following the exciting laser pulses enhances the selectivity and improves quantification of fluorescence measurements. Therefore, time-gated energy transfer spectroscopy is suggested to be an appropriate tool for probing mitochondrial malfunction.
Animals↗
[Optical dual canal measuring system for the detection of disorders of mitochondrial metabolism in situ].
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Animals↗
Intracellular fluorescence behaviour of meso-tetra(4-sulphonatophenyl)porphyrin during photodynamic treatment at various growth phases of cultured cells.
Meso-tetra(4-sulphonatophenyl)porphyrin (TPPS4) taken up by cells is mainly localized in lysosomes as previously shown by fluorescence microscopical and fluorescence spectroscopical investigations. In the present study the intracellular fluorescence behaviour and the intracellular amount of this dye at various growth periods of cells were examined. For cells irradiated in the growth phase a relocalization of TPPS4 from the lysosomes into the cytoplasm and finally into the nucleus was observed. In contrast, for cells irradiated in the stationary phase no redistribution could be detected and therefore no evidence for severe damage of the lysosomal membranes and subsequently for the release of lytical enzymes is given. In both cases lethal damage of the cells was achieved as examined using the trypan blue exclusion test. This indicates that damage of the lysosomes is less important in the photodynamic inactivation of cells sensitized by TPPS4.
Animals↗
Time-resolved pH-dependent fluorescence of hydrophilic porphyrins in solution and in cultivated cells.
Fluorescence decay kinetics and time-gated (nanosecond) emission spectra of the hydrophilic photosensitizers meso-tetra(4-sulfonatophenyl)porphyrin (TPPS4) and uroporphyrin III (UP III) are reported. These substances are characterized by low aggregation, preferential accumulation within lysosomes and a pH-dependent composition of unprotonated and protonated species. A comparison of TPPS4 and UP III in buffer solutions and in confluently growing RR 1022 epithelial cells showed that the intracellular pH value of the environment of both photosensitizers was about 4.7. A slight decrease by 0.10-0.15 pH units occurred after light exposure which (in the case of TPPS4) was concomitant with a lethal damage of the cells. A photoproduct at 640 nm with a characteristic fluorescence lifetime of 4.3 +/- 0.8 ns was detected for UP III in buffer solutions at pH values above 5. The absence of this photoproduct in epithelial cells again indicated that UP III was located within lysosomes.
Animals↗
[Comminuted open tibial fracture. An alternative treatment method].
The treatment of comminuted fracture of the tibia is one of the problem cases in operative fracture care. In our department we favour stabilization with the aid of interlocking nails, providing absolute fixation of the interlocking screw is possible. The patient is positioned on the Maquet table, and the fracture of the tibia is temporarily reduced by the Goetze percutaneous cerclage wiring technique. The special feature of our method is that we remove the wires after nailing. In this way the advantages of both techniques, anatomical reduction with cerclage wires and the stability and possibility of early mobilization afforded by interlocking nailing are combined. Poor blood flow in the periosteum is avoided by the immediate removal of the wires.
Fibula↗
[Oxygen conserving nasal catheters. Oxymizer pendant].
The Oxymizer pendant consists of a 40 ml pendulating reservoir bag which is connected to the prongs by two tubes. Both reservoir and tubes serve as oxygen reservoirs. We wanted to examine if it was possible to save oxygen by changing from the usual nasal cannula to Oxymizer pendant. 11 patients with hypoxaemia due to chronic obstructive pulmonary disease were examined. We were able to reduce the oxygen requirement by approximately 65% without significant changes in SaO2. All 11 patients evaluated the Oxymizer pendant to be more comfortable than the usual nasal cannula.
Catheterization↗
Pulmonary artery sling associated with tracheobronchial malformations.
We describe three cases of pulmonary artery slings associated with tracheal stenoses by complete cartilaginous rings and abnormalities in the tracheobronchial branching pattern. This association implicates special problems of management that are different from the simple pulmonary artery sling. Pathologic anatomy, symptoms, diagnostic procedures, and the problems of therapy are described. Considering similar cases in the literature, we conclude that thorough diagnostic evaluation of the tracheobronchial and the cardial system should be carried out in all cases of pulmonary artery sling. Simple correction of the aberrant vessel without correcting the tracheal stenosis is of no value in these cases. In some milder cases, a conservative approach is possible and probably less harmful than an operation.
Abnormalities, Multiple↗
[Dorsal compression fractures of the distal radius metaphysis. Long-term follow-up with conservative therapy].
Conservatively treated compressed fractures of the distal radius dorsal metaphysis healed despite primarily good reduction and consequent treatment with a decrease in dorsal length. The amount of this decline depends on the primary loss of dorsal length. If the fracture showed a loss of dorsal length at the time of the accident of up to 2 mm, the long-term results were almost anatomical. If there was primarily a larger loss of dorsal length, a decline of the distal fragment into the dorsal cave resulted. Most of the dorsal length regained after reduction was lost when the cast was removed. The long-term follow-up examinations were made after about 9 years.
Adolescent↗
[Plate osteosynthesis of the radius. Own implant and technique].
In 1989 and 1990, 46 acute distal radius fractures with die-punch compression cavities underwent open reduction and internal fixation using a new compression plate and surgical technique. In 34 of these patients, functional and radiological results after plate removal were studied. Surgical exposure was palmar or dorsal, or combined palmar with a supplementary dorsal incision, depending on the location of the compression cavity and the extent of fracture dislocation. A cortico-cancellous graft from the iliac crest was fitted into the compression cavity, thus supporting the reduced joint surface against the radius shaft. Superior maintenance of the reduction was achieved using cortico-cancellous instead of simple cancellous graft. A new titanius radius reconstruction plate was used for internal fixation. This two-fold support of the fracture allowed for earlier wrist mobilization.
Adolescent↗
[Snowboarding injuries].
This retrospective study includes 59 people treated at our trauma centre for snowboard accidents that happened in the 1989 winter season. 22 People were severely injured. 2 people died due to a polytrauma. One fracture of the cervical vertebrate column was followed by complete hemiplegia. Reasons for these accidents were a lack of knowledge how to behave outside the marked alpine regions, as well as lack of technical equipment and theoretical education.
Adolescent↗
[Malignant hyperthermia in a child with acute lymphatic leukemia].
A 5-year-old boy with acute lymphatic leukemia in remission developed signs of malignant hyperthermia (MH) during general anesthesia for removal of a central venous access port. The anesthetic procedure for implantation of the port 17 months before had been uneventful despite use of the same triggering agents, halothane and succinylcholine. Meanwhile, the patient had received chemotherapy (COALL-03-85). The first sign of MH was masseter spasm following succinylcholine; then tachycardia, acidosis, myoglobinuria, and CPK elevation (8953 IU) appeared. There was only moderate temperature elevation to 37.8 degree C. Rapid improvement and complete recovery occurred after dantrolene i.v. The patient's father was found to have undiagnosed muscle pain and an elevated CPK level. An in vitro contracture test with halothane and caffeine revealed susceptibility to MH and supported the patient's diagnosis and genetic predisposition. Referring to several other cases in the literature concerning MH in patients with lymphomas and leukemias, a possible correlation between the two diseases is discussed. As the MH crisis in our patient was most probably genetic in origin, a common acquired cause such as a viral infection seems less probable. We do not believe the chemotherapy our patient received between the two anesthetics was the cause since about one-half of the patients in the literature had not had chemotherapeutic pretreatment at the time of the MH crisis. We believe that a common genetic predisposition is the most likely link between the two diseases. In any case, patients with leukemias and lymphomas should be monitored very carefully for symptoms of MH.(ABSTRACT TRUNCATED AT 250 WORDS)
Child, Preschool↗
[Closed tendon injuries of the hand].
Cases of subcutaneous tendon ruptures of the hand were compiled over a period of 10 years; the lesions were all traumatic rather than due to degenerative illness. The 914 injuries fall into two categories: 867 extensor and 47 flexor tendon ruptures. The localisation of the lesions is most often distal. The extensor tendon lesion is accompanied more often by a ruptured bony fragment; the distal flexor tendon is mostly torn-off bony fragment. The extensor tendon ruptures can be subdivided as follows: distal injuries of the extensor aponeurosis in the DIP joint (751 cases), IP joint (31), tractus intermedius (27), ext. carpi radialis longus (5) and brevis (1), ext. carpi ulnaris (6); proximal ruptures of the ext. pollicis longus (42), ext. digitorum communis II and indicis (1 each) and ext. carpi radialis longus and brevis (1 each). The flexor tendon lesions are as follows: distal injuries to flex. digitorum profundus (32 cases), flex. pollicis longus (9), and the opponens pollicis, which is classified under this heading (2). Proximal lesions to flex. pollicis longus (2) and flex. digitorum profundus and superficialis (1 each).
Hand Injuries↗
Revascularization of a partially necrotic talus with a vascularized bone graft from the iliac crest.
A 16-year-old patient had a compound dislocation of the right talus. Following primary treatment, which included a subtaler screw arthrodesis, the talus developed clinical, radiological, and isotope scan signs of necrosis. In spite of a walking caliper to prevent weight bearing on the ankle, the talar articular cartilage of the ankle joint also showed signs of degeneration. The talus was revascularized with a vascularized corticocancellous iliac crest bone graft. Six months postoperatively, there were clinical, radiological, and bone scan signs of significant revascularization. The patient is free of pain and able to walk with full weight bearing on the foot.
Adolescent↗