Diagnostic value of tissue polypeptide-specific antigen (TPS) in neuroblastoma and Wilms' tumour.

Although tissue polypeptide-specific antigen (TPS) has been described as a potentially useful serum marker of tumour activity in adult epithelial tumours, few data are available for childhood malignancies. Neuroblastomas and Wilms' tumours are the commonest types of solid malignancies found in the retroperitoneum of children. At this time, a widely used marker for Wilms' tumour is not available. Using an enzyme-linked immunosorbent assay (ELISA) kit, serum TPS levels in 23 children with neuroblastomas, nine with Wilms' tumours and 22 with benign tumours were evaluated to test the usefulness of the marker in identifying malignancies. Compared with healthy children (n = 110), the preoperative least-square means (LSM) of serum TPS were considerably elevated in both neuroblastoma (LSM = 209 U l(-1)) and Wilms' tumour (LSM = 235 U l(-1)), whereas values in benign tumours were only slightly elevated. Although the Wilms' tumours were associated with higher preoperative serum TPS levels, there was no statistically significant difference compared with neuroblastomas. Receiver operating characteristic analysis (ROC curves) showed a high sensitivity and specificity for both malignancies. Successful treatment resulted in decrease in TPS serum values. Serum TPS measurements in children presenting with abdominal masses can help in diagnosing the two commonest extracranial solid malignancies of childhood. Furthermore, TPS could acquire a pivotal role in monitoring therapy.

[Value and importance of genital urography in the diagnosis of intersexuality and other malformations of the urogenital tract]. / Aussagekraft und Bedeutung der Genitourographie in der Diagnostik der Intersexualität und anderer Malformationen des Urogenitaltraktes.

The accuracy of genital urography in the diagnosis of intersex and for the demonstration of the internal genitalia has been studied in 16 patients. It was found that the radiological appearances agreed in all cases with the surgical findings. Individual malformations of the genitals have been considered with reference to the classification of Shopfner and Prader and the advantages and disadvantages of various examination techniques are discussed.

[Hypertrophic pyloric stenosis--an indication for sonography]. / Hypertrophe Pylorusstenose--eine Indikation zur Sonographie.

The pyloric region of 58 infants who presented with vomiting was examined by real-time ultrasound. Transverse diameter of the pylorus and single wall thickness were measured. In 18 patients sonographic diagnosis of hypertrophic pyloric stenosis (HPS) was made and subsequently confirmed by surgery. The mean (+/- one standard deviation) of the transverse diameter and of the pyloric wall thickness in patients with HPS was 15 (+/- 1) mm. and 5.5 (+/- 0.7) mm., respectively. Forty infants matched for age and sex distribution without gastrointestinal symptoms served as controls. The measurements for the transverse diameter were 9.6 (+/- 1.8) mm. and for the wall thickness 1.9 (+/- 0.5) mm. Diameter values of patients with HPS were significantly (p less than 0.001) higher than those of the control group and those of the remaining 40 patients without HPS. Real-time sonography proved a reliable tool in the diagnosis of HPS.

Glycated plasma proteins in normal and diabetic mothers and their offsprings.

Glycated plasma proteins (GPP) and glycated hemoglobin (G Hb) has been evaluated in 134 non-diabetics (ND), 299 women with potential abnormality of glucose tolerance (pot.AGT), 75 with impaired glucose tolerance (IGT) and 34 insulin dependent diabetics (IDDM) during pregnancy or postpartum including 94 cord blood determinations. Mean HbA1c levels were significantly elevated in IDDM (6.6 +/- 1.3% M +/- SD) compared to ND (5.1 +/- 0.7%; P less than 0.01), but were similar for the other groups studied. Mean GPP were increased for the IDDM (0.58 +/- 0.29 nmol 5- HMF/mg protein; M +/- SD) and the IGT-group (0.53 +/- 0.22) over ND (0.3 +/- 0.13; P less than 0.01) and the Pot.AGT group (0.37 +/- 0.14; P less than 0.01). 6% of the ND, 15% of the Pot AGT-, 52% of the IGT- and 62% of the IDDM group were found to have GPP values exceeding the 97% confidential limit of the ND. However, the large overlap of individual values from patients with different degrees of glucose intolerance with the normal range of pregnancy precludes the use of GPP as a screening parameter for IGT during pregnancy. A 30-35% reduction of fetal hemoglobin- and plasma protein glycosylation relative to maternal values was observed.

[Associated abnormalities in the VACTERL syndrome--case report with autopsy findings]. / Assoziierte Missbildungen bei einem VACTERL-Syndrom--Fallbericht mit Obduktionsbefunden.

We report on a male newborn with a VACTERL-association. Our patient had the characteristics of the syndrome: vertebral defects, anal atresia, cardiac anomalies, tracheal stenosis and radial dysplasia. In addition to this combination of malformations we have found a left pulmonary artery sling, cholecystaplasia and biliary dysplasia-not previously reported. Etiology, embryology, coincidence and differential diagnosis are discussed.

Neonatal nesidioblastosis--diagnosis and preoperative management.

Two infants were admitted for severe, intractable hypoglycemia. "Fasting" tests lasting 45 and 30 minutes respectively revealed hypoglycemia with inappropriately elevated levels of plasma insulin and plasma C-peptide. Subsequently, somatostatin was infused to test the individual sensitivity of the B-cells. Basal and glucose-induced insulin and C-peptide secretion were significantly reduced. Preoperative treatment with somatostatin was introduced, which controlled the hypoglycemia. In both patients, subtotal pancreatectomy was effective in restoring normal glucose homeostasis.

[Wide gap esophageal atresia without esophagotracheal fistula (author's transl)]. / Langstreckige Oesophagusatresien ohne Oesophagotrachealfistel (Typ II nach Vogt).

Standard treatment of infants with "wide gap" esophageal atresia usually requires a staged procedure consisting of gastrostomy at birth (with or without cervical esophagostomy) and then a bowel interposition between the oesophagus and stomach at 1 year of age. Because this approach procedures significant morbidity and swallowing dysfunction, an alternative method for treating the wide gap atresia is recommended. The upper and lower esophageal pouch can be elongated by preoperative bougienage. This technique allows successful primary esophageal reconstruction in infants with wide gap esophageal atresia previously considered uncorrectable except by use of colonic or gastric tube interpositions.

Reduced susceptibility of glycosylated hemoglobin to proteolytic cleavage.

Four different experimental designs were selected to study, whether glycosylation of hemoglobin alters susceptibility against proteolytic degradation. We compared the resistance of different hemoglobin fractions against degradation by pure proteolytic enzymes, by liver cell culture, by live organ culture and the resistance of in vitro glycosylated vs. "non-glycosylated" hemoglobin solutions. The hemolysates were characterized by high performance liquid chromatography. The degree of glycosylation of hemoglobin was further estimated by the thiobarbituric-acid assay. From the data presented it is concluded, that the glucose moiety linked to the N-terminal valine and lysine residues of the alpha- and beta chain of the hemoglobin exerts a protective effect against proteolytic cleavage. This could explain the extended lag phase in response of glycosylated hemoglobin to an improved metabolic control in diabetic patients and the discrepancy between the rate of synthesis and the rate of catabolism.

[Omphalocele and gastroschisis: clinical differences and surgical considerations. A ten year review (author's transl)]. / Omphalozele und Gastroschisis: Klinische Unterschiede und chirurgische Konsequenzen.

31 infants with anterior abdominal wall defects were treated over the past 10 years. Despite the falling birth rate gastroschisis occurred almost twice as often as omphalocele (19 and 12 cases, respectively). The mortality rate of 71% in omphalocele were related to large defects, rupture of the sac and associated cardiac malformations. The 73% incidence of prematurity, additional malformations and sepsis contributed to the mortality rate of 58% in gastroschisis.