Postoperative assessment of porcine-valved right ventricular-pulmonary artery conduits.

Insertion of extracardiac right ventricular (RV)-pulmonary artery (PA) conduits for repair of severe forms of RV-PA discontinuity has become a widely used technique. During a 10-year period, 76 patients had open heart repair with a porcine-valved RV-PA external conduit. The most common diagnoses were truncus arteriosus (15%) and pulmonary atresia with ventricular septal defect (15%). The patients were 18 days to 37 years old (median 7 years). The postoperative median follow-up period was 4 years. Four patients (8%) have been lost to follow-up. Nineteen patients (25%) died in the early postoperative period and there were 10 late deaths (13%). Calculated probability of survival was 66% at 8 years. Thirty-six patients have undergone late hemodynamic studies. A residual shunt was present in 14% of the patients; moderate to severe PA hypertension was present in 25% and significant RV-PA gradients (greater than 30 mm Hg) in 50%. The mean RV pressure for the group was 78.5 +/- 38 mm Hg. The severity of the gradients appeared to be greater in patients who had been followed longer. Ten patients have undergone reoperation because of conduit obstruction or residual shunting; 6 are alive and well. Of the surviving patients, 95% are acyanotic and without significant exercise intolerance and only 5% have cardiovascular symptoms. Thus, open heart repair using an external RV-PA conduit provides a marked improvement in the quality of life in patients who survive.

Acute renal failure in children. An ultrasonographic-clinical study.

Acute renal failure (ARF) may be due to obstructive uropathy or renal parenchymal disease. Twenty-five children with acute renal failure secondary to renal parenchymal disease underwent ultrasonographic examination of the kidneys. Changes of renal size and cortical echogenicity were correlated with renal function. All patients presented with bilaterally enlarged kidneys with the exception of those in the neonatal age group (12%). Improvement in renal function resulted in normalization of renal size. With regard to cortical echogenicity two groups were formed. Group A comprised 11 patients whose kidneys had the same echogenicity as the liver, while in group B the kidneys were more echogenic (14 patients). Cortical echogenicity was always increased. Determination of creatinine levels showed a statistically significant difference between group A (3.32 mg% +/- 1.40 S.D.) and group B (5.95 mg% +/- 1.96 S.D.), p less than 0.001. Changes in renal function were paralleled by rapid changes in renal size and cortical echogenicity.

[Modern detergents and their application in pediatrics]. / Sind die neuen Waschmittel in der Pädiatrie von Vorteil?

During the past half of this century there has been a tremendous development of chemical products. Their application in every day life is widespread, and problems arose summarized under the term of civilisation damages. An important aspect is the effect of detergents on human skin. Every day more than 100 million people have contact with surface active substances--an influence on the physiologic steady state of the skin or its protective mechanisms was thought to be possible. After reviewing the relevant literature of the past ten years following conclusions can be drawn: As to the problem of tolerance we can say that there is no objection against the use of anionactive detergents. Under extreme conditions, e.g. constitutional anomalies, or intense application on previously damaged skin, injury of the skin can be observed. In the age group between 6 and 12 years there exists no difference in the resistance against alkali or acids in comparison to adults. It has not been demonstrated yet, however, whether the buffering capacity is already active in the skin of the newborn, or when it becomes effective after birth. We can recommend the use of modern detergents in pediatrics, they represent an important part of modern hygiene in childhood, especially in infancy. For optimal efficacy one has to adjust the substance to every constitutional skin type and to preexisting skin damages.

[Emergencies in neonatology--what is the status of diagnostic imaging today?]. / Notfallsituationen in der Neonatologie--Wo steht die Bildgebung heute?

Some diagnoses in neonatology require prompt therapeutic intervention to ensure survival of the newborn child. The purpose of this report is to emphasize the validity of modern diagnostic imaging in the rapid assessment of emergency situations in the neonate.

[Familial juvenile nephronophthisis--a cause of chronic renal failure in childhood]. / Die familiäre juvenile Nephronophthise--eine Ursache des chronischen Nierenversagens im Kindersalter.

Familial juvenile nephronophthisis (FJN) represents an important cause of chronic renal insufficiency in the first two decades of life. Its frequency is reported to vary between 7 and 20% of all cases of terminal renal failure in childhood. Usually the onset is insidious, with polyuria, polydipsia and anaemia being the main clinical features. The diagnosis is based on clinical, laboratory and pathological findings. The purpose of our report is to emphasize the importance of this pathological entity with respect to the clinical symptoms and signs and diagnostic approach on the basis of the case reports of four patients.

[Clinical use of sonography in neonatology]. / Der Einsatzbereich der Sonographie in der Neonatologie.

During the neonatal period, sonography mostly represents the primary imaging modality of choice. Noninvasiveness, no exposure to ionizing radiation and no harmful side effects in biologic tissues contribute to the advantages of this technique. Validity of ultrasonic imaging in the neonatal age group lies primarily in the detection of congenital anomalies. Intraacerebral (2.39 per 1000 live births) as well as intracardiac (6 per 1000 live births) and intraabdominal anomalies can be diagnosed sonographically. Anatomic sites of origin of neonatal abdominal masses are renal (55%), genital (15%), gastrointestinal (15%), non-renal retroperitoneal (10%) and hepato-spleno-biliary (5%). Furthermore, changes of echogenicity reflect structural changes and thus impairment of function in various organs. Finally, Duplex sonography, the combination of real time imaging and Doppler flow measurement, can differentiate between vascular and avascular lesions and normal and abnormal flow patterns. The application of diagnostic ultrasound in the neonatal period is demonstrated.

[The relevance of duplex Doppler sonography in pediatrics]. / Die Relevanz der Duplex-Doppler-Sonographie in der Pädiatrie.

Since the introduction of duplex Doppler sonography the noninvasive assessment of hemodynamic changes in children has become possible. The clinical application comprises the depiction of cerebral hemodynamics in infants, where characteristic flow patterns can be found in patent ductus arteriosus, hypoxic-ischemic encephalopathy, cerebral hemorrhage, hydrocephalus or in brain death. Moreover, Duplex Doppler Sonography play an important role in the evaluation of renal hemodynamics, especially in kidney transplants. Finally, the examination of the portal venous system can demonstrate thrombosis of the portal vein, portosystemic collaterals or a hepatofugal flow in severe portal hypertension. Thus, invasive investigative procedures may become unnecessary in certain cases. The future potential of this technique lies in the development of quantitative measurements of volume flow and in Doppler color flow imaging.

[Malformations of the urinary tract in neonates. Current status of imaging procedures]. / Harntransportstörungen bei Neugeborenen.Wo steht die Bildgebung heute?

UNLABELLED: Malformations of the urinary tract are one of the most common types of anomalies in the neonatal period. Approximately 15-20% of all prenatally detected malformations require acute postnatal imaging. Sonography is the primary imaging modality in the evaluation of nephro-urologic problems in infancy. Hydronephrosis represents the most common pathology, followed by multicystic-dysplastic kidney. DISCUSSION: The result of the sonographic examination directs further imaging. An adequate diagnostic algorithm is decisive for accurate diagnosis, for the appropriate therapeutic regimen and for the prognosis of the patients.

[Effect of vesicoureteral reflux on kidney density in children]. / Der Einfluss eines vesikoureteralen Refluxes (VUR) auf die Nierendichte bei Kindern.

In 59 children with vesicoureteric reflux (VUR) renal echogenicity was analyzed. The density of the kidneys increase with the intensity of the VUR. The children with VUR grade 1 and 2 mainly showed a normal report. In 50% of the cases with VUR grade 3, a normal report was also found, whereas in the other 50% echogenicity was increased to 1 and 2. Highly significant increases in renal density were seen in ultrasound in children with VUR grade 4-5.

[Changes in renal echogenicity in pediatric obstructive uropathies]. / Die Anderungen der renalen Echogenität bei kindlichen obstruktiven Uropathien.

Renal cortical echogenicity of 40 children with obstructive uropathies was analysed in relation to the grade of obstruction and thinning of the renal cortex. In 35 percent of the children an increase in echogenicity was noted. The increase in cortical echogenicity paralleled the grade of dilatation of the intrarenal collecting system and the grade of thinning of the renal cortex. Evaluation of the cortical echogenicity should be integrated into the primary diagnostic setup as well as into the follow-up of children suffering from obstructive uropathies.

[Allergic alveolitis in cow milk allergy]. / Allergische Alveolitis bei Kuhmilchallergie.

The case history of a 12 months old boy with recurrent pneumonia following ingestion of cow's milk is described. After receiving cow's milk containing formula he presented with fever, tachypnea, diffuse rales and crepitations over both lungs. The chest X-ray revealed marked bilateral alveolar shadows. A high titer of antibodies against the four major antigenic components of cow's milk protein was demonstrated by Ouchterlony's precipitation test and the fluorescent immunosorbent test. Withdrawal of cow's milk from the diet resulted in complete resolution of clinical and radiological findings.

[Noonan syndrome in the differential diagnosis of Turner syndrome]. / Noonan-Syndrom in Differetialdiagnose zum Turner-Syndrom.

In children with growth retardation and the typical face, particularly with valvar pulmonary stenosis, the Noonan syndrome is the be suspected. In early infancy, these patients show very severe developmental retardation and muscular hypotony. Mental retardation is frequent but not compulsory. The most important diagnostic tool is the chromosomal analysis. In our patient, the syndrome was diagnosed becaused of the typical appearance and the angiocardiographic findings in connection with a normal chromosomal sequence.

Cerebral blood flow in newborn infants with and without mechanical ventilation.

The influence of mechanical ventilation with low mean airway pressure (MAP) on cerebral blood flow (CBF) veolocity in newborn infants was assessed in fifteen ventilated infants by Duplex Doppler Sonography (Duplex DS). As a control, CBF velocities were examined in 15 age and weight matched non-ventilated infants. For quantitation, maximal systolic velocity, enddiastolic velocity and the semiquantitative Pourcelot index were determined as representative flow variables. There was no significant difference of these flow variables between ventilated and non-ventilated infants. The pH, pO2 and pCO2 did not differ significantly between the two groups and there was no correlation between the flow variables, pH, pO2, pCO2 or MAP. Mechanical ventilation with low MAP is not associated with adverse effects on cerebral hemodynamics in newborn infants when significant alterations of the blood gases are avoided.

[Autoimmunhemolytic anemia in infancy (author's transl)]. / Originalarbeiten Autoimmunhämoplytische Anämie im Säuglingsalter.

Autoimmunhemolytic anemia in childhood can be divided into two main groups according to the length of the illness: an acute, transient form and a chronic, prolonged one. The etiopathology of the illness is still unknown. It is most postable that there is a connection with virus infections, whereby the antibodies, appearing within the framework of an immunological reaction, are to be considered as a secondary response to the infection. It could not, unfortunately, be proved in the case of our patient that there had been a virus infection. The role of immunodefects in the case of the acute form is currently being discussed in the more recent literature and the point als made that genetic defects are possible in the connection.

[Bird breeder's lung with conversion to fatal fibrosing alveolitis]. / Vogelzüchterlunge mit Ubergang in letale fibrosierende Alveolitis.

An 11-year-old girl developed typical symptoms of bird breeder's lung after several years of contact with budgerigars and other birds. The clinical diagnosis was confirmed by immunological and lung-function tests. Despite exclusion of exposure to allergens and treatment with steroids and D-penicillamine no improvement occurred, the exogenous allergic alveolitis progressing to fibrosing alveolitis of which the girl died.

Acute renal transplant rejection in children: assessment by Duplex Doppler sonography.

Over a two year period 74 consecutive Duplex Doppler scans were performed in 23 children with renal allografts and were compared to the Doppler sonographic findings in orthotopic kidneys of 25 age matched healthy controls. The Doppler waveforms of renal arterial flow were analyzed qualitatively assessing systolic and diastolic flow amplitudes, for quantitation the Pourcelot index (PI) was used. There was no variation between the Doppler waveforms in recipients with normal allograft function and healthy controls. In 12 patients with biopsy proven acute rejection a decrease or absence of the diastolic flow amplitude was noted, resulting in increased pulsatility of the Doppler waveform. The mean PI in acute rejection differed significantly from the mean PI in normal allograft function. Duplex Doppler sonography is a useful imaging modality in the differentiation between acute rejection and normal allograft function and should therefore be integrated in the screening of children after renal transplantation.

Pharmacokinetics of intra-arterial indomethacin treatment for patent ductus arteriosus.

We present pharmacokinetic data of prolonged, intra-arterial indomethacin treatment (i.e. induction plus maintenance dose) for symptomatic patent ductus arteriosus (sPDA) in 26 ventilated premature infants. sPDA was assessed by two-dimensional and pulsed Doppler echocardiography. Permanent ductal closure occurred in 20 (76%) infants. Plasma levels of indomethacin were 1.18 +/- 0.74; 1.8 +/- 1.0; 1.51 +/- 0.93 and 1.25 +/- 0.98 micrograms/ml (mean +/- SD) at 12, 24, 48 and 72 h after initial dose administration. All except one patient who responded with ductal closure, showed plasma levels above 0.25 microgram/ml throughout the 3 day treatment period and no case of sPDA reopening was noted. Although target concentrations over time were not defined, the data indicate that the maintenance levels measured were within the therapeutic range. A negative correlation was found for plasma drug levels and postnatal age (r = 0.52; P less than 0.01). Volume of drug distribution was 0.23 +/- 0.18 l/kg, total clearance 0.1 +/- 0.11 ml/min and elimination constant 0.06 +/- 0.05 h-1 (mean +/- SD). The great variation in pharmacokinetic data reflects the heterogeneity of the population studied with respect to extracellular fluid space, cardiovascular status, serum protein and other parameters.

High resolution CT in children with cystic fibrosis.

High resolution CT (HRCT) was performed in 24 children (median age 57.9 months) suffering from cystic fibrosis (CF). In 23 patients (one examination unacceptable because of motion artifacts) the most frequent finding was bronchial wall thickening, shown in 21 patients (91%), followed by bronchiectasis in 15 patients (65%). Less frequent findings were mucus plugging and patchy consolidations, which could be demonstrated in 11 patients each (48%). Findings were classified using a CT scoring system and including only irreversible pulmonary changes; a statistically significant correlation with lung function tests (FEV1/FVC; MEF50) could be established. HRCT to date seems to be the most valuable method to determine extent and severity of lung involvement in children with CF and should therefore be routinely used for the staging of this disease.

Color-coded Doppler evaluation of cholecystic varices in portal hypertension.

A 11-year-old white girl presented with a diagnosis of thrombosis of the portal vein after newborn septicemia. Duplex sonography revealed significant narrowing of the portal vein and its right and left branches. A Doppler signal could only be obtained in certain short segments of the portal vein and indicated hepatopetal flow. Color-coded Doppler sonography showed extensive varicose veins in the gallbladder with a bigger draining vessel running to the porta hepatis. Documentation of varices like those in the gallbladder wall confirms the diagnosis of portal hypertension and may increase the sensitivity of Doppler sonography. Color mapping has the potential to detect unexpected flow and to analyze blood flow to better advantage.