RARE-MR-urography (Rapid Acquisition with Relaxation Enhancement) is a fast MR imaging technique (6.4 s/acquisition) that selectively depicts fluid by heavy T2-weighting. From 9/1989 to 11/1990, RARE-MR urograms were prospectively evaluated in the diagnosis of upper urinary tract abnormalities in 55 children. The method is performed in several planes and combined with a coronal, T1-weighted spin-echo sequence. Forty out of 42 kidneys with dilated renal pelvis, and 21 out of 24 dilated ureters were identified, only the mildly dilated ones were missed. Even in non-functioning kidneys the urinary tract was clearly depicted by RARE-MR-urography. However, no differentiation could be made with this technique between vesicoureteral reflux and non-refluxing dilatation of ureter and/or renal pelvis. All 19 pelviureteric obstructions and all eight renal duplications with a dilated segment were identified. RARE-MR-urography is a new tool for diagnosing urinary tract abnormalities in children without having to employ ionizing radiation, contrast media, or general anesthesia. A dilated urinary tract can be shown in one image displaying the entire urinary system, similar to excretory urography. The technique is presently not able to provide the information of voiding cystourethrography or renal scintigraphy, nor is it as easy to perform as ultrasound. However, in certain cases it may replace excretory urography.

Marked hypoxia secondary to intrapulmonary shunting is a characteristic of respiratory failure in human neonates. To investigate the effect of iNO on intrapulmonary right-to-left shunting, two typical pulmonary diseases of the newborn (respiratory distress syndrome and meconium aspiration) were mimicked in 32 mechanically ventilated white New Zealand Rabbits weighing approximately 2 kg each. After tracheotomy, catheters were inserted into a jugular vein, a carotid artery and the right ventricle (to measure right ventricular systolic pressure, RVSP, and mixed venous oxygen content for calculation of shunt by Fick equation). Repeated airway lavage(LAV) with warm normal saline (20 ml/kg) or repeated instillation of a 20% slurry of human meconium (MEC) in normal saline (2 ml/kg) was continued until the a/A-ratio was ≤0.14 and a PIP≥22 mbar was needed to keep the tidal volume constant at 10 ml/kg. Measurements were made after completion of lung damage (baseline) as well as 60 minutes (60 min) after administering iNO (at 80 ppm). Four groups of rabbits (n=8 in each group) were studied: lavage control (LAV-Con) and intervention (LAV-iNO), meconium control (MEC-Con) and intervention (MEC-iNO) (Table, values represent MEAN±SD). Also, a positive influence on dead space (% of tidal volume, calculated as PaCO2-PECO2/PaCO2) could be seen (LAV: 32±10 vs 25±10, p<0.01, MEC: 26±16 vs 18±10, p=0.05 at 60 min). These results demonstrate that iNO decreases intrapulmonary shunting(as well as RVSP and dead space). We speculate that iNO may be beneficial in human newborns with severe respiratory failure even if no extrapulmonary shunting via ductus or foramen ovale has been proven.

We report the case of a 29 weeks gestation female premature infant who suffered from severe postnatal asphyxia following spontaneous vaginal delivery. Prenatally lung hypoplasia due to prematurely ruptured membranes with subsequent oligohydramnios was suspected sonographically. Echocardiography revealed right-to-left shunting via PDA and foramen ovale, in addition to that tricuspid incompetence with a pulmonary arterial pressure gradient of 40 mmHg was demonstrated. At an oxygenation index (OI) of 34, an arterio-alveolar oxygen difference (AaDO2) of 639 mmHg, an FiO2 of 1.0 and a maximal paO2 of 37 mmHg during high frequency ventilation (HFV), we applied inhaled nitric oxide (up to 70 ppm) for a duration of approximately 30 hours. Within two hours the inspiratory oxygen concentration could be weaned to an FiO2 of 0.21, mean airway pressures were reduced markedly. Echocardiographically tricuspid incompetence had disappeared, the PDA was closed and now left-to-right shunting across the foramen ovale was demonstrated. The infant was extubated on day 5 and subsequently had oxygen requirements up to an FiO2 of 0.3 during spontaneous breathing for 20 days.

Zusammenfassung Das hyperammonämische Koma ist eine akut lebensbedrohliche Stoffwechselentgleisung. Die häufigsten kongenitalen Ursachen sind Enzymdefekte im Harnstoffzyklus und im Abbau von organischen Säuren. Die Frühdiagnose und eine sofortige aggressive Therapie sind die entscheidenden Voraussetzungen zur Vermeidung irreversibler Hirnschäden und letaler Verläufe. Das Therapieziel ist eine rasche Senkung des Ammoniaks und anderer neurotoxischer Metaboliten. Die therapeutischen Grundprinzipien beinhalten 1. eine restriktive Proteinzufuhr unter dem Erhalt essentieller Aminosäuren, 2. die Unterbrechung des Proteinkatabolismus mittels hochkalorischer Ernährung, 3. eine medikamentöse Aktivierung alternativer Wege der Stickstoffausscheidung sowie 4. apparative Blutreinigungsverfahren. Das optimale Dialyseverfahren ist umstritten. Wir haben 4 Neugeborene und Säuglinge in einem hyperammonämischen Koma im Rahmen von Stoffwechselerkrankungen mittels Hämodialyse oder Hämofiltration behandelt. Die Kasuistiken bestätigen die Effektivität und Komplikationsarmut beider Verfahren. Diskussion: In der Behandlung von lebensbedrohlichen Hyperammonämien bei Neugeborenen und Säuglingen sind Hämodialysen und Hämofiltrationen die Behandlungsmethoden der Wahl. Die invasive Blutreinigung ist mit einer konsequenten diätetischen und medikamentösen Therapie zu optimieren. Der prognostische Nutzen einer effizienten Blutreinigung bei ausgeprägter Hyperammonämie rechtfertigt einen unverzüglichen Transport in das nächstgelegene pädiatrische Dialysezentrum.

Diphtheria has become a rare disease in Germany. We report on an unimmunized 3.5-year-old German girl with a 7-day history of respiratory distress and fever, presenting a clinical picture mimicking typical bacterial tracheitis without pharyngeal and laryngeal manifestation. Diagnosis of diphtheria was not made until culture of tracheal secretions yielded growth of a toxigenic strain of Corynebacterium diphtheriae. The patient died from toxic cardiac failure despite treatment with diphtheria antitoxin. This is the second reported case of isolated bacterial tracheitis caused by Corynebacterium diphtheriae.Conclusion The observation of a lethal course of diphtheric tracheitis emphasizes the paramount importance of immunization against diseases like diphtheria.

108 adolescents with type 1 diabetes as well as their parents and physicians in charge were studied in a prospective longitudinal design. Most of the juvenile patients were of good metabolic control. The results from different questionnaires of coping are presented. The opinions of the patients, their parents and the physicians in charge differ in some important aspects. Furthermore there are significant differences between the patients of best versus worst metabolic control as far as the extent of self reported psychosocial problems related with the disease is concerned.

ANTIMICROBIAL ACTIVITY OF NITRIC OXIDE (NO) IN CONCENTRATIONS USED DURING INHALATIVE NO THERAPY 1993

108 adolescents with type 1 diabetes as well as their parents and physicians in charge were studied in a prospective longitudinal design. Most of the juvenile patients were of good metabolic control. The results from different questionnaires of coping are presented. The opinions of the patients, their parents and the physicians in charge differ in some important aspects. Furthermore there are significant differences between the patients of best versus worst metabolic control as far as the extent of self reported psychosocial problems related with the disease is concerned.

Introduction: Nitric oxide innalational therapy requires a dosage unit, consisting of flow controlllers for bias and NO flow as well as NO and NO, monitoring devices.Aim Of the study: We examined the accuracy Of each component as well as the accuracy Of the complete system in combination with a high frequency oscillatory ventilator (HFO-V).Materials and Methods: We Compared accuracy Of digital mass flow controllers IMFC) (BrOnkhorst Hi-Tec, Veenendaal, The Netherlands) versus Conventional analog flow controllers (Brooks Europe, Veenendaal, The Netherlands) for NO and biasFloW control.NO and NO, concentrations were measured With Chemiluminescence (CLD 700, EcoPhysics, DUrnten, Switserland) in dry gas containing 21% oxygen.Furthermore accuracy of NO measurement in clinical Conditions was assessed, with NO and bias flow MFC controlled.NO and NO, concentrations were measured using both ChemiluminescenCe (CLD 700) and electrochemical analysers (SensorNOx, Sensor Medics Europe, Bilthoven, The Netherlands).The HFO-V ventilator used was a Sensor Medics 3100-A (Sensor Medics, Yorba Linda, Ca).Results: We found major influences of used flow controllers, humidification, and measurement method used.Data are presented in the table as mean (95%Cl limits) Of the ratio Of pre-calculated to measured NO value.Dosage accuracy (Cnemiluminescence) 2 MFC 0.99 (0.983-0.998) 3100 A biasfiow, MFC (NO) 0.856 (0.835-0.877) 3100 A biasflow, rotameter (NO) 1.175 (0.793-1.74)Measurement accuracy 12 MFC) electrochemical, dry gas 1.017 (1.006-1.029)electrochemical, humid gas 1.131 (1.089-1.175)chemoluminescence, humid gas 1.136 (1.126-1.136) Conclusions:We conclude, that a system consisting of one MFC for NO dosage, rotameter for biasflOw control and electrochemical NO and NO, analyser has adequate accuracy for clinical use during HFO-V.Our electrochemical analyser uses a cell, with limited sensitivity to high oxygen levels, sampled gas was dried via permapure tubing and pressure swings were not allowed to reach the analyser.

A formerly premature, exclusively breast-fed infant with severe zinc deficiency syndrome is presented. He showed the characteristic erosive skin changes, including alopecia, as seen in acrodermatitis enteropathica. In addition, he manifested a failure to thrive and irritability. The diagnosis was confirmed by reduced serum levels of zinc (2.3 μmol/l) and alkaline phosphatase (45 U/l). We consider the reduced zinc supply in the breast milk (5.7 μmol/l) as the most likely cause of the disease. Therapy consisted of oral zinc supplements (50 μmol/kg/day) for a period of 30 weeks. Symptoms and laboratory values normalized completely and did not recur on a normal diet.

A one year prospective surveillance of nosocomial infections (NI) in a neonatal intensive care unit (NICU) was performed. Among 229 neonates the infection rate was 27.1%, the infection proportion 20.1%, and the incidence density 21.9 infections per 1000 patient days. Infants were stratified into four birth weight categories. Degrees of infection ranged from 44.4% in the < or = 1000 g group to 10.1% in the > 2500 g group. Differences between the groups were statistically significant (P < 0.01). The mean birth weight of infants with NI was significantly lower than that of infants without NI (1711 g, SD +/- 841 g vs. 2213 g, SD +/- 896 g; P < 0.01). Mortality of < or = 1000 g babies was 44.4 and 7.6% in > 2500 g neonates. Major sites of infection were pneumonia (32.3%), blood-stream infections (27.4%), infections of the skin, and surgical site infections (11.3% each). The predominant pathogen was Staphylococcus aureus (24.2%) whilst Gram-negative bacteria accounted for 22.7% of the total. Other major infective agents were Staphylococcus epidermidis, Escherichia coli, and Group B streptococci. It is concluded, that low birth weight was a major risk factor for the acquisition of NI in the observed NICU population.

Restrictive dermopathy is a recently described lethal congenital disorder of the skin with an autosomal recessive mode of inheritance. The rigidity of the skin impairs fetal movements in utero and causes arthrogryposis, as well as highly characteristic facial features and pulmonary hypoplasia. We report two cases of restrictive dermopathy in prematurely born infants, describe the typical pathological findings and discuss this disorder in the context of the fetal akinesia/hypokinesia deformation sequence.

Recent studies have shown that in boys a steady-state of blood lactate is maintained at exercise levels above the anaerobic threshold. Therefore, the explanation hitherto provided for the steeper increase in blood lactate beyond the anaerobic threshold, i.e. the onset of anaerobic metabolism, needs modification. Investigations were carried out in ten boys, aged 11–12 years, during treadmill running. Maximal oxygen uptake (\(\dot VO_{2max} \)) and maximal blood lactate were determined during incremental exercise. Subsequently each boy performed four runs at different high constant speeds of 16 min duration, in order to determine maximal steady-state blood lactate. The underlying data also served to estimate roughly the lactate anaerobic threshold. Oxygen uptaken (\(\dot VO_2 \)) was measured at 0.5 min intervals during the initial 7.5 min of each constant-speed run. Maximal steady-state blood lactate was 5.6 mmol/l corresponding to 92% of\(\dot VO_{2max} \). The mean blood lactate at which the anaerobic threshold was reached or just exceeded was 2.7 mmol/l corresponding to 82% of\(\dot VO_{2max} \). Oxygen transport transient kinetics were computed from the mean 0.5 min\(\dot VO_2 \)-values during the constant-speed runs near the maximal steady-state blood lactate and from runs near the anaerobic threshold. Half-times of\(\dot VO_2 \) response were shorter than values previously reported for adults due to a faster increase in\(\dot VO_2 \) at the onset of exercise. Half-times increased with increasing work rates as did the oxygen deficit, due to a slower increase in\(\dot VO_2 \) along with a longer time required to attain a steady-state at higher work rates. A linear relationship between oxygen deficit and blood lactate could be demonstrated beyond the anaerobic threshold in five boys. Our results show that the dysequilibrium between lactate production and its elimination at work rates beyond the anaerobic threshold is only transient. As soon as a steady-state for\(\dot VO_2 \) has been re-established blood lactate remains stable. A definitive dysequilibrium with progressive accumulation of blood lactate will occur only when the work rate exceeds the rate corresponding to the maximal steady-state blood lactate.