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Stone burden is a predictor of stone free rate (SFR) after ureterorenoscopy (URS). 2D measurements (X-Ray/CT) may unreliably predict actual stone burden. This study was performed to evaluate 3D measurements as a predictor of SFR after URS compared to 2D measurements. A retrospective study (2008–2019) was performed on patients with a single stone and preoperative CT-scans. Stone size was assessed by three conventional 2D methods and an automated 3D measurement. Primary outcome was SFR after four weeks. Secondary outcomes involved the need for subsequent procedure and complications. 227 patients were included, SFR was 74.9%. 3D measurement correlated significantly with SFR (OR: 0.980). Unfortunately, no predictive value was observed. 3D measurement demonstrated a significant correlation to complications (OR: 0.966). Low interobserver variability (0.891 vs 0.782 respectively) was found in the 2D and 3D measurements. It was concluded that, although 3D voluminometry correlates significantly to SFR and complications, these approach harbors no predictive value.

A 34-year-old para V woman was referred to our centre at 35+1 weeks of gestation for an assumed fetal malformation with prenatal renal impairment and anhydramnios. Prenatal ultrasound demonstrated unilateral renal agenesis; the bladder was not detectable. The baby was born by caesarian section at 36+2 weeks of gestation because of placental insufficiency. Postnatal adaptation was uneventful, but the newborn presented external stigmas of trisomy 21 and progressive renal impairment with anuria. Nevertheless, the postnatal ultrasound showed two enlarged kidneys in loco typico with impaired perfusion but without signs of malformations. In the lower abdomen, a rosette-shaped structure of unknown origin was noted. Its origin could not be cleared by imaging including voiding cystourethrography and colon contrast radiography. Explorative laparotomy identified the structure as a persistent urachal cyst with secondary obstruction of the upper urinary tract. After removal of the urachus with reconstruction of the bladder dome, renal function recovered completely while urine was drained continuously via suprapubic catheter. A voiding cystourethrogram 3 weeks later showed a posterior urethral valve as an additional unexpected diagnosis. The valve was slit at the age of 6 months without complications, the renal function remained stable in the further course. In retrospect, the main cause for the renal failure remains unclear. It appears to be the obstruction due to the space-consuming character of the urachal cyst, especially because the megacystis typically associated with urethral valve was not viewable. Alternatively, the additional proximal stenosis may have only masked the typical findings of PUV.Eine 34-jährige 5. Para wurde unserem Zentrum in SSW 36 bei schwerer fetaler Fehlbildung mit Anhydramnion zugewiesen. Die kardiopulmonale Adaptation nach Sectio in SSW 36+1 bei Placentainsuffizienz gestaltete sich weitgehend komplikationslos, jedoch präsentierte das Neugeborene neben äußeren Stigmata einer Trisomie 21 ein akutes Nierenversagen mit Anurie. Postnatal waren jedoch beide Nieren sonografisch ohne Hinweise auf Anlagestörung nachweisbar, sie zeigten sich aber etwas vergrößert und perfusionsgestört. Zudem konnte eine intraabdominell gelegene, kokardenförmige Struktur dargestellt, aber keinem Organsystem zugeordnet werden. Auch weiterführende diagnostische Maßnahmen wie Miktionscysturethrophie und Kolonkontrastdarstellung waren nicht wegweisend. Eine explorative Laparatomie erbrachte die Diagnose einer Urachuszyste mit mechanischer Verlegung der Ureteren und konsekutivem Nierenversagen. Nach erfolgter operativer Entfernung der Zyste mit Rekonstruktion des Blasendaches wurde in der Miktionscysturethrografie als Überraschungsbefund das Vorliegen einer posterioren Urethralkappe diagnostiziert. Diese wurde im Alter von 6 Monaten komplikationslos entfernt. Auch retrospektiv läßt sich in diesem Fall nicht eindeutig klären, welche der vorliegenden Fehlbildungen vorrangig für das akute Nierenversagen verantwortlich gemacht werden kann. Möglicherweise war in diesem Fall die raumfordende Wirkung der Urachuszyste führend ursächlich, zumal die bei Urethralklappe typischerweise assoziierte Megazystis nicht darstellbar war. Alternativ könnten die klassischen Befunde einer Urethralklappe durch die proximale zusätzliche Stenose auch lediglich maskiert gewesen sein.

IgA-Ablagerungen sowohl bei primarer IgA-Nephropathie als auch bei Purpura Schonlein-Henoch-Nephritis lassen trotz unterschiedlicher Klinik auf eine gemeinsame Pathologie und Pathogenese beider Erkrankungen schliesen. Die primare IgA-Nephropathie tritt vor allem bei Jugendlichen und Erwachsenen auf und ist nicht mit extrarenalen Symptomen assoziiert. Entweder besteht eine rezidivierende Makrohamaturie oder eine Mikrohamaturie mit Proteinurie. Die Purpura Schonlein-Henoch-Nephritis ist eine Erkrankung des (fruhen) Kindesalters mit vaskulitischer Symptomatik der Haut, der Niere aber auch des Gastrointestinaltrakts und der Gelenke. Oft besteht nur eine milde renale Symptomatik. Jedoch sind konsequente Nachuntersuchungen notig, um eine signifikante Glomerulopathie zu entdecken. Bei beiden Erkrankungen wird IgA1 vermehrt produziert und nicht korrekt glykosyliert, die Proteinurie ist jeweils wichtigster Risikofaktor. Eine immunsuppressive Therapie ist nur bei schwerem, progredientem Verlauf gerechtfertigt. Der Einsatz von Angiotensin-converting-Enzym-Inhibitoren hat an Bedeutung gewonnen, da dadurch die prognostisch ungunstige Proteinurie reduziert wird und durch einen anti-fibrotischen Effekt eine Nephroprotektion erreicht werden kann.

Steroid sensitive (minimal change) nephrotic syndrome (MCNS) has been regarded as immunological disorder because of clinical and experimental evidence as well as the response to immunosuppressive treatment. Recent work increased dramatically the understanding of podocyte biology which may be the key structure involved in MCNS, Interestingly many treatment options which were thought to work via an immunosuppressive pathway are now known to have a direct -non immunological- impact on the glomerular filtration barrier, i.e. the podocyte. Aim of this review is the presentation of recent research regarding the podocyte biology but also concerning the treatment of this disorder.

Infection with sorbitol-fermenting Shiga toxin-producing Escherichia coli O157:H- (sf STEC O157:H-) is rare, but emerging in Europe. The pathogen is typically isolated from paediatric patients with life-threatening haemolytic uraemic syndrome (HUS). It is unclear whether this observation primarily reflects the pathogen's virulence or its complex laboratory diagnosis, not routinely conducted in diarrhoeal patients. In summer 2009, four boys living in the same suburb in Germany developed diarrhoea-associated HUS: three were infected by sf STEC O157:H- and one died. We conducted two analytical epidemiological studies, an extensive search for diarrhoeal cases in potentially exposed groups, and an environmental investigation. Outbreak cases were residents of the suburb diagnosed with HUS, sf STEC O157:H- infection, or both between 24 July 2009 and 25 August 2009. Overall, we ascertained eight cases with a median age of 4 years (range: from 8 months to 9 years). Stool screening of 220 persons led to the identification of only four additional cases: two asymptomatic carriers and two diarrhoeal cases. HUS was strongly associated with visiting a local playground in July, particularly on 16th July (odds ratio = 42.7, P = 0.002). No other commonality, including food, was identified, and all environmental samples (n = 24) were negative. In this localized non-foodborne outbreak, the place of likely infection was a local playground. Sf STEC O157:H- infection apparently limits itself rarely to diarrhoeal illness and progresses frequently to HUS. Therefore, detection of and response to this hypervirulent pathogen primarily relies on HUS surveillance.

Urinary tract anomalies (UTA) including polycystic kidney disease nowadays can be detected antenatally by ultrasound. The concomitant presence of oligohydramnios has been regarded as a severe risk factor for renal dysfunction and pulmonary hypoplasia, although clinical data after birth are scarce. We report the postnatal course and long-term follow-up of 10 infants with oligohydramnios due to congenital UTA from two pediatric nephrology centers. The underlying final diagnoses were autosomal-recessive polycystic kidney disease (ARPKD, n = 2), familial tubular dysgenesis (n = 2) and bilateral renal hypoplasia (n = 6) including 3 children with posterior urethral valves. Two children died in the neonatal period while 8 children are currently alive at a median age of 2.5 (range 1.1-10) years. In the postnatal period, respiratory failure necessitating mechanical ventilation occurred in 7 infants (including the 2 non-survivors). All surviving children had chronic renal failure, which could be managed conservatively in 6 children (median GFR 45 (range 15-53) ml/min/1.73 m2) while 2 reached end-stage renal disease; one undergoing preemptive kidney transplantation and one peritoneal dialysis. Seven of 8 children reached normal developmental milestones. In conclusion, the presence of antenatal oligohydramnios in infants with UTA does not always carry a poor prognosis. The high incidence of perinatal complications, the complexity of underlying causes and the prevalence of postnatal chronic renal dysfunction calls for a multidisciplinary approach in the management of these children.

Urinary transferrin loss is a typical feature in relapse of the idiopathic nephrotic syndrome, however, the impact on serum iron homeostasis and hematological parameters has not been studied systematically so far.Therefore, we investigated serum iron (Fe), erythropoietin (EPO), ferritin (FN), transferrin (TF), total iron-binding capacity (TEBK), transferrin saturation and the soluble transferrin receptor (sTFR) combined with hematological parameters (hemoglobin, MCV, MCH) in 42 children with relapsing, steroid-sensitive nephrotic syndrome (NS) in remission (RM, n = 26) and relapse (RL, n = 16), including 13 patients who were studied in both states. Thirty-three age-matched healthy children served as controls.Fe, TEBK and TF were significantly reduced in RL compared to RM in cross-sectional as well as in paired studies while ferritin, hematological parameters and EPO levels remained unchanged. A significant increase, however, of the soluble transferrin-receptor could be demonstrated in cross-sectional analysis comparing RL to RM and healthy controls (3568+/-713 mg/ml vs 2625+/-576 vs 2646+/-697; p0.001 respectively) as well as in paired analysis of 13 patients in RL and RM (p0.001).We conclude that transient transferrin and iron deficiency occurs in RL of INS but this seems to be counterbalanced by upregulation of the sTFR, a mechanism that might be important in preventing the development of iron deficiency anemia during the active nephrotic state.

Preemptive isolated liver transplantation (PLTX) can cure the metabolic defect in primary hyperoxaluria type 1 (PH1) but there are no uniformally accepted recommendations concerning the timing of this transplantation procedure. We have performed PLTX successfully in 4 children (age 3-9 years) with PH1 with no mortality or morbidity due to the transplantation procedure. Plasma and urinary oxalate levels normalised rapidly and renal function remained stable including one patient with advanced chronic renal failure who showed a stable course for more than 24 months. Although treatment must be individualised in this severe metabolic disorder and PLTX has to be viewed as invasive procedure, we feel PLTX should be offered and discussed not too late in the treatment of PH1 to prevent or at least delay the progression to end stage renal disease and systemic oxalosis.

To improve our understanding of both diagnosis and treatment of diseases of oxalate metabolism, we first set out to establish a new ion-chromatographic method to determine normal plasma levels of oxalate, citrate and sulfate from single plasma samples. In 50 infants and children (23 girls, 27 boys, aged 0.2 to 17 years) with normal renal function, blood was drawn in Li-heparin tubes, placed on ice and preserved immediately with 40 microliters M HCl/ml plasma in two ultracentrifugation steps. For measurement, plasma was injected onto an ion chromatography system with NaOH as the mobile phase, and then run as a linear gradient from 5 mM to 52.5 mM over 21 minutes. Analysis yielded measurable and reproducible oxalate (6.43 +/- 1.06 microM/liter), citrate (79.3 +/- 27.4 microM/liter) and sulfate (235.0 +/- 85.3 microM/liter) levels, without any age and gender specific differences. The least detectable plasma oxalate level was0.3 microM with a high reliability and reproducibility (coefficient of variance 1.95 to 4.75%). In conclusion, we established a reproducible, precise method to determine the relevant plasma anions involved in mineral metabolism, which heretofore have not been easily measurable. Studies of diseases of oxalate and citrate metabolism are ongoing on the basis of the normal plasma values achieved in this study.

The treatment of frequently relapsing steroid-sensitive nephrotic syndrome in children with established immunosuppressive drugs (steroids, cyclophosphamide, cyclosporin A) sometimes presents problems because of the expected incidence of side effects. Stimulation of the immune system with the anthelminthic drug levamisole in this disease has been documented. Aim of this study was to assess in a prospective but uncontrolled series of observations its value and side effects.25 patients (15 boys, ten girls; median age 10 [3.5-22] years) were given levamisole, 2 mg/kg/48 h. Before this treatment was started eight of the children/adolescents (32%) had frequent relapses and 17 (68%) had become steroid-dependent. Treatment was started during steroid-induced remission and continued for 3-24 (median 6) month, while steroids were discontinued after four weeks.Relapse frequency per patient month was reduced from a mean of 0.5 (0.33-0.83) before to 0.31 (0-0.67) during levamisole administration (P0.001). In 12 patients (48%) no or considerably fewer relapses were observed. Patients with exclusively frequent relapses responded to levamisole better than those with steroid dependence (7/8 [87.5%] vs. 5/18 [27.7%], P = 0.01). Side effects were reversible leukopenia in two patients and nonspecific skin rash as well as epigastric pain in one patient.Levamisole is an efficacious addition or alternative, with a low incidence of side effects, in the treatment of frequently relapsing nephrotic syndrome, particularly so in yet steroid-dependent patients.

Haematuria and proteinuria are important symptoms of primary and secondary nephropathies. We report three african children presenting to our center in whom infection with S. haematobium resulted in haematuria and proteinuria. The third patient concomitantly suffered from steroid-sensitive relapsing nephrotic syndrome with the histological features of focal and segmental glomerulo-sclerosis. The diagnosis was in all cases established by light microscopy and urinary symptoms improved after treatment with praziquantel. In the third patients long term remission of the nephrotic syndrome could be maintained after 4 doses of praziquantel for recurrent bladder symptoms. We conclude that bilharziosis must be considered in the differential diagnosis of children with haeamturia and proteinuria even in Europe. The diagnosis can be established easily by light microscopy and an effective and low-risk treatment (with Praziquantel) can be offered.

Primary hyperoxaluria type 2 (PH2) is a rare disease with only 24 patients reported in the literature so far. It should be considered in any patient presenting with urolithiasis or nephrocalcinosis due to hyperoxaluria. The metabolic defect is deficiency of d-glycerate dehydrogenase/glyoxylate reductase leading to characteristic hyperoxaluria and excretion of l-glycerate, the cornerstone of diagnosis of PH 2. Although development of terminal renal failure seems to be less prevalent than in PH 1, recent reports indicate that chronic as well as terminal renal insufficiency may occur. Therefore specific therapeutic measures should aim at reduction of urinary calcium oxalate saturation by potassium citrate or pyrophosphate to reduce the incidence of nephrolithiasis and nephrocalcinosis and thus improve renal survival. Secondary complications (obstruction, urinary tract infections and pyelonephritis) must be avoided. In patients with terminal renal failure isolated renal transplantation seems to carry a high risk of disease recurrence. Conclusion PH 2 is a rare but important cause of urolithiasis and nephrocalcinosis; long-term follow up is necessary, since the renal prognosis may be worse than previously anticipated.

Disseminated islands of gastric mucosa are very rare in the small intestine. The secretion of hydrochloric acid can lead to ulceration which results in gastrointestinal bleeding. It is often difficult to localize the focus in case of gastrointestinal blood loss especially in the small bowel. Technetium-99m-pertechnetate scintigraphy may be a helpful tool in detecting ectopic gastric mucosa. We report a case of a 21-mo-old boy with recurrent gastrointestinal bleeding. By using pertechnetate scintigraphy, extensive tracer accumulation in the jejunum and proximal ileum was detected. Histologically, multiple islands of ectopic gastric mucosa were found in about 50 excited mucosal and transmural biopsies. The unusual finding of disseminated accumulation of 99mTc-pertechnetate in the small intestine was the diagnostic clue for such a rare disease.

Hyperkalemia is a life threatening emergency and warrants immediate treatment because of its deleterious cardiac consequences. Initial measures in mild cases include restriction and binding of dietary potassium, correction of metabolic acidosis and increasing urinary excretion by furosemide. In moderate and severe hyperkalemia infusion of glucose with insulin has been regarded as the standard medical treatment so far. However, recently also the beta 2 stimulatory drug salbutamol has been shown to be an effective agent to treat hyperkalemia by inducing a shift of potassium into the intracellular compartment. We treated 15 pediatric patients of different age groups (mean age 5.16, range 0.1-16 years) suffering from acute hyperkalemia (mean level 6.6, range 5.9-7.7 mmol/l) by means of a single infusion of salbutamol (5 micrograms/kg over 15 minutes). Serum potassium concentrations decreased significantly to 5.74 +/- 0.53 after 30 minutes and furthermore to 5.19 +/- 0.48 and to 4.92 +/- 0.53 mmol/l after 60 and 120 minutes, respectively (p0.001 at all stages compared to pre-treatment). Since no side effects occurred besides a slight increase of heart rate in 3 patients, we conclude that short-term intravenous salbutamol infusion is an effective, rapid, safe and predictable way to treat children of any age suffering from acute hyperkalemia and therefore has become the first line treatment in our center.

The recent war has provided a great deal of experience in the operation of aircraft and ground equipment under a variety of conditions. This paper is an attempt to condense and correlate some of the points noted into a logical sequence. It is not based on statistics and is more in the nature of a selection of problems and impressions about them. Possibly this is an advantage, as it seems doubtful if many logical conclusions could be drawn from a set of completely comprehensive observations, even if these were available.

In order to establish the necessary data to enable high proof strength austenitic stainless steels to be used for the fabrication of pressure vessels, plates were produced with an increased proof stress obtained by the warm working method. The laboratory investigation then showed that it was practicable to raise the proof strength of this material to twice the normal value for fully softened plate with an improvement in the ultimate tensile strength. Following this, two similar experimental vessels were manufactured in normal and high proof strength 18% Cr-8% Ni-Ti stabilized steel and these were subjected to hydraulic pressure testing. The vessel tests showed how stainless steel vessels behave under static pressure application at room temperature, and the behaviour of the high proof strength vessel indicated that it would be quite safe at nearly double the design pressure for the fully softened vessel. The strain hardening of stainless steel, fabricating techniques and weld quality have also been investigated and these are reported upon in the paper. Proposals for design stress criteria and stress values are made in relation to an examination of existing pressure vessel codes and the experimental data.

In this paper two aspects of gas-turbine design and operation are dealt with. First, an account is given of the development of special types of ‘kinematic’ construction suitable for accommodating the very large temperature changes encountered when a gas turbine is started and put on load rapidly. Examples are given of the application of this type of construction to representative components of a 750-1,000 kW. gas turbine. Secondly, a description is given of the development of combustion chambers to burn fuels ranging from natural gas and gas oil to residual fuels, coal tar fuel and peat. A range of chambers to burn these fuels has been designed to fit interchangeably on the basic components of the standard turbine. A detailed account is given of the development of a combustion chamber of elbow type which takes advantage of the fact that a number of changes of direction through 90 deg. or more are necessary in the ducting of nearly all industrial gas turbines. Development of this chamber is described from early atmospheric test models to the final pressurized combustion chambers which have been fitted to production gas turbines. With only slight adjustments, this type of chamber has shown itself capable of burning hydrocarbon fuels, ranging from natural gas to residual fuel oils, and a modified form of chamber has also successfully burnt low calorific value gases.