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In adults, luteinizing hormone (LH) and follicle stimulating hormone (FSH) are secreted in a pulsatile manner. Prior to puberty gonadotropin (GN) levels are low and show only small fluctuations. The following investigation was performed to elucidate the type of GN secretion in infants. LH and FSH were determined every 30 min over a period of 8 h in three different groups: Group 1:2 male and 2 female adults; Groups 2:2 male and 2 female prepubertal children; Group 3:3 male and 3 female infants, aged 6-12 weeks. Group 1 showed a clear pulsatile secretion of LH (4.5-23.5 mIU/ml [range]) and FSH (6.9-16.0 mIU/ml). Group 2 demonstrated a rather constant secretion of LH (less than 1.5-2.3 mIU/ml) and FSH (1.6-4.9 mIU/ml). Group 3: In male infants pulsatile secretion of LH (3.6-34.7 mIU/ml)--and to a lesser degree of FSH (1.8-4.6 mIU/ml)--were found. In female infants the pulsatile secretion of FSH (6.5-22.7 mIU/ml) was more pronounced than that of LH (less than 1.5-4.7 mIU/ml). The secretory pattern in early infancy is of a pulsatile type.

Die artifizielle Ernährung im Kindesalter unterscheidet sich in der Indikationsstellung, aber auch in der Nährstoffzusammensetzung von der des Erwachsenen. Daneben ergeben sich auch technische Unterschiede und damit unterschiedliche Gefahren bei Langzeitanwendung. Ganz besonders soil daher auf die anderen Möglichkeiten in Form enteraler Zufuhr chemisch definierter Diät und in Form der Sondenernährung hingewiesen werden. Immer muβ zuerst der Weg mit den geringsten Gefahren versucht werden.

Im Routinebetrieb der Universitats-Kinderklinik Wien wurde an 49 Neugeborenen die Lage eines in die Nabelvene eingefuhrten Kunststoffkatheters rontgenologisch kontrolliert. Nur bei 7 Patienten (rund 14%) erreichte der Katheter, wie es der allgemeinen Vorstellung entspricht, die untere Hohlvene bzw. den rechten Vorhof. Der hohe Prozentsatz fehlerhafter Positionen wurde bisher kaum beachtet und kann als Ursache spaterer Komplikationen angesehen werden.

Bericht uber neuropathologische Befunde eines Falles von kongenitalem nichthamolytischen Ikterus (Crigler-Najjar-Syndrom) mit anhaltender excessiver Hyperbilirubinamie. Das Gehirn des im Alter von 8 Monaten in einem Decerebrationszustand verstorbenen Knaben zeigte schwere Lasionen verschiedenen Alters: symmetrische Verodung und gliose Vernarbung in makroskopisch blas erscheinenden Teilen der Basalkerne—Pallidum, Corpus Luysii, lateraler Thalamus und reticulare Nigrazone—sowie frischere Veranderungen nach Art spongioser partieller Gewebsnekrosen mit intensiver glios-mesenchymaler Reaktion in den makroskopisch gelb verfarbten Regionen der Groshirnrinde, des Ammonshorns und Striatums. Daneben bestand subtotale diffuse Kleinhirnrindenatrophie mit symmetrischer Zahnkernverodung und Olivenatrophie. Die erhobenen Befunde weisen darauf hin, das es bei anhaltender Erhohung des indirekten Serum-Bilirubins nicht nur in der unmittelbaren Neugeborenenperiode, sondern auch im spateren Alter zur Hirnschadigung kommen kann. Die Pathogenese der ZNS-Veranderungen wird in Hinblick auf einige experimentelle Befunde diskutiert.

Two boys with presumably congenital cystic changes in the lungs are described. In one child multiple cystic deformations led to acute symptoms of severe respiratory distress necessitating urgent resection of the right middle and upper lobes. The other boy had a large thick-walled cyst in the left upper lobe causing no distress whatsoever. This patient was observed for more than 6 years before operation. Multiple vascular anomalies and anastomoses were revealed during surgery. Both cases are presented in detail and the completely different clinical course is outlined. Only when careful supervision of intrapulmonary cystic changes is guaranteed can operation be postponed for longer than 6 months. Pulmonary cysts may lead to emergency procedures. They are always a potential source of serious complications such as rupture or infection. When no tendency for regression can be discovered surgical removal is indicated. Conservative treatment will not influence the course. Differential diagnosis is discussed briefly.

One particular form of an anorectal malformation--the anterior perineal anus--is frequently overlooked in the neonate period because defecation is easy at this age. However, with the change of diet, this malformation as a rule leads to chronic constipation. The lateral view at the beginning of an irrigoscopy usually shows a characteristic picture of a horizontal caudal limitation of the rectum, a dorsal pouch, a beakshaped fistula and an elevation of the anorectal transition. These findings contribute essentially to the clinical diagnosis and permit early operation.

Besides classical achondroplasia various severe osteochondrodysplastic disorders have been differentiated in recent years. Their most important features include obligatory perinatal mortality, severe dystrophy of the thorax and shortening of tubular bones. Additionally some of these syndromes present visceral malformations, cleft-lip, and malformations of the genitals. A detailed case report of a patient is given. Various features: dystrophy of the thorax, micromelia, postaxial polydactyly, metaphyseal dysplasia, malformations of the great vessels, and polcystic kidney suggest the classification as short rib-polydactyly syndrome, type Saldino-Noonan.

Among the various types of hepatic ductular atresias, there is a group of patients with a definable syndrome of malformations: typical physiognomy, malformation of pulmonary arteries, mental retardation and disturbed growth of body and genitals. This syndrome has been defined only in the last two years by Watson et al. (1973) and Allagille et al. (1975). A detailed description of a boy with this combination of malformations is given. Additionally he has aplasia of the right kidney. A second patient out of 4, which we found in our cardiologic department, has hypoblasia of one kidney, too. The prognosis of the liver disease in these patients seems to be better than in other children with biliary atresia.

Krebs kommt beim Kind wesentlich seltener vor als beim Erwachsenen. Trotzdem nehmen die Tumoren in den zivilisierten Landern seit Beherrschung der Infektionskrankheiten hinter den Unfallen die zweite Stelle in den Todesursachenstatistiken ein (Schmid u. v. Rauchhaupt, 1972). Grundsatzlich konnen fast alle beim Erwachsenen vorkommenden Tumoren auch im Kindesalter beobachtet werden. Den ganz uberwiegenden Teil machen jedoch die Hamoblastosen und die vom Erwachsenen kaum bekannten embryonalen Tumoren aus. Auf Grund einer Zusammenstellung von 2.000 kindlichen Tumoren (Burchenal et al., 1962) ergibt sich der Haufigkeit nach folgende Reihe: Leukamie 34,1%, Neuroblastome 11,1%, Knochentumoren 10,8%, ZNS-Tumoren und Retinoblastome 10,6%, Weichteilsarkome 9,1%, Wilmstumoren 6,8%, Lymphome 6,o%, andere 11,5%.

Up until now 39 cases of combined Klippel-Trénaunay syndrome and Sturge-Weber syndrome have been described. Here follows the report of a girl, now 4 years of age, displaying a full combination of these syndromes. Only a small part of the body surface is not covered with naevi teleangiectatici laterales. The patient has clear hypertrophy of the left cheek and of the left lower extremity, less noticeable on the left upper extremity. For therapeutic reasons the left side of the head and the left lower extremity were thoroughly angiographically examined--this revealed typical abnormalities. The vessel-alteration of the lower extremity are not extremely far developed and arteriovenous fistulas on a large scale are also absent. This allows us to dismiss the F.P. Weber syndrome on the one hand, while it explains the absence of complications of the Klippel-Trénaunay syndrome, as described in literature, on the other. The significance of the alterations of lymph nodes in this disease, which we are the first to describe, is at present not fully clear. The cerebral attacks have until now showed only a temporary response to medication.

During the past 2 years 7 children received kidneys from cadaveric donors. 2 transplants had to be removed because of irreversible chronic rejection and 1 recipient died on the 4th day after surgery. The remaining 4 recipients are doing well. In the evaluation of the results special emphasis is placed on the normalization of growth and puberty. The difficulties of psychosocial rehabilitation are stressed.

This report describes the results of a systematic development screening of high risk infants in a provincial district over a period of three and a half years. Criteria for neurological research during the first year of life and organizing problems are presented. Low frequency of medical care during pregnancy seems to be a main factor predetermining cerebral palsy and multiple disabilities. An optimal transport system seems to be a supporting factor for the prevention of such motoric and mental derangements, especially in children, who initially show no pulmonary or cardiac problems.

A ten year old boy with parents of Rumanian and Turkish origin suffered from metaphyseal chondrodysplasia (McKusick type) with typical radiological changes of the metaphyseal endplates. Microscopic examination of his hair revealed only slight diminution of its thickness, and signs of increased brittleness. Because of Hirschsprung's disease resection of colon was performed. No immunologic changes were found.

40 children with clinical diagnosis of bronchial asthma aged 8--14 years, 39 with allergic disposition, were investigated. Acute therapeutic and protective effect on frequency of asthmatic attacks, pulmonary auscultation and lung function (FEV1, PF, FVC) by application of Fenoterol and Ipratropium as combination aerosol were registered. Over a period of 4 weeks we could reduce the frequency of attacks nearly to the half. 10 and 20 minutes after aerosol application we showed a good improvement of pulmonary ventilation. Only on children with severe asthma bronchiale an exclusive treatment with Berodual seems to be of modest protective effect.

Seven cases of the most severe form of tuberculous meningitis, in which a midbrain syndrome developed, are reported. Three different types of progress were observed. Exudative inflammation and cerebral edema dominated in the first group, causing the rapid development of the acute midbrain syndrome, which may turn into a bulbar syndrome. In the second group the development of the midbrain syndrome was delayed and an apallic syndrome followed. The morphological examination disclosed local diencephalic and midbrain lesions caused by herniation and specific vasculitis and vascular compression. The third group showed disintegration of cortical function as a result of parenchymal lesions, apart from local midbrain symptoms which never fully intensified into the midbrain syndrome. Observation of the progress of the disease proved that late diagnosis and delayed therapy were decisive in cases of the most severe form of tuberculous meningitis.

The rare X-ray finding of urinary bladder wall calcifications in a newborn is reported. The newborn suffered from micturition disturbance caused by urethral valves, bilateral hydroureter and bilateral hydronephrosis. No possible definite cause for the calcification of the bladder wall could be found. The calcifications could never be detected by X-ray after the age of 5 months.

Potter-syndrome with agenesia of the kidneys and typical face is only one variation of the big group of renofacial dysplasia. Among our patients which are under treatment because of malformations of the urinary tract very often we see children with dysplastic faces or malformed ears. Therefore vice versa patients with alterations of ears and face should be exactly investigated to exclude urinary tract malformations.

Four examples of short rib-polydactyly syndromes are described. Case 1 represents the Majewski type, and the other three are examples of the Saldino-Noonan type. Both conditions are true multiple congenital anomaly syndromes and neonatally lethal forms of dwarfism with a narrow thorax, polydactyly and associated visceral malformations. They are probably caused by the homozygous state of different autosomal recessive genes. They must be differentiated from similar conditions such as chondroectodermal dysplasia, asphyxiating thoracic dysplasia, the Meckel syndrome and the trisomy 13 syndrome.

Obwohl in den letzten Jahren vor der medizinischen Offentlichkeit sehr viel von der operativen Behandlung des kindlichen Hydrocephalus gesprochen und geschrieben wurde, erleben wir es recht haufig, das der Wert dieser Methoden verkannt und vor allem das der richtige Zeitpunkt der Operation versaumt wird. Es sei daher neuerdings kurz auf die Indikationen zu dieser Operation hingewiesen.