Photochromic peptidic NPY Y4 receptor ligands.

The neuropeptide Y (NPY) Y4 receptor is a G protein coupled receptor, which is targeted by pancreatic polypeptide, a homologue of NPY. Selective Y4R agonists were suggested as potential therapeutics for the treatment of obesity. Highly potent dimeric peptidic Y4R agonists, constituting two pentapeptide moieties connected through an aliphatic linker, represent an interesting class of Y4R ligands. Based on this compound class, photoresponsive Y4R ligands, containing an azobenzene, azopyrazole, diethienylethene or a fulgimide chromophore were prepared to explore structural requirements of such Y4R agonists on Y4R binding. The synthesized Y4R ligands, containing a non-aliphatic rigid photochromic linker, switch reversibly in aqueous buffer and exhibited high Y4R affinity throughout. This demonstrated that the replacement of the highly flexible aliphatic linker by a considerably less flexible photochromic linker was well tolerated with respect to Y4R binding. Differences in Y4R affinity and activity between the individual photoisomers (varying in spatial orientation and flexibility) were marginal suggesting that the linking element in the dimeric ligands is less critical for the adaptation of high-affinity binding modes at the receptor.

[Warfarin embryopathy in maternal coumarin therapy for protein S deficiency]. / Warfarin-Embryopathie bei mütterlicher Coumarin-Therapie wegen Protein-S-Mangels.

A case of Warfarin embryopathia is shown. The newborn, whose mother had been treated with Marcoumar (Phenprocoumon 3 mg/day) during the whole pregnancy because of a hereditary protein-S-deficiency showed the typical symptom of nasal hypoplasia. Coumarol derivates pass the placental membrane and are known as teratogenic. Recent retrospective and prospective studies show that the risk of fetal or embryonal teratogenic injury is about 25-30% if Coumarol derivates are given through the 6th to the 9th week of pregnancy. It is possible to nearly avoid those injuries when Heparin is used for anticoagulant therapy in this period and Coumarol itself is used in the lowest possible therapeutic dose.

[Ultrasound image of an unusual intrahepatic site of a bile duct cyst]. / Das sonographische Bild einer ungewöhnlichen intrahepatisch gelegenen Gallenwegszyste.

An intrahepatic cyst was found ventral and cranial to the gallbladder without choliangectasy in an infant of 8 months of age. Final diagnosis of a bile duct cyst was arrived at intraoperatively. The article discusses the possible aetiology, non-invasive diagnosis and sonographic differential diagnosis. Sonography is discussed as the method of choice in assessing intrahepatic and extrahepatic cysts and the bile ducts.

[Nonsurgical removal of iatrogenic intracardiac foreign bodies. A study in a 5-month-old infant]. / Nicht-operative Entfernung iatrogener intrakardialer Fremdkörper. Eine Beobachtung bei einem fünf Monate alten Säugling.

The development of new techniques and special catheter instruments permit iatrogenically embolized polyethylene catheter fragments to be retrieved nonsurgically. A case is reported in which a fragment of a central venous catheter was removed from the right atrium and ventricle by a DORMIA catheter in a 5-month-old child. The literature concerning nonsurgical retrieval of catheters is reviewed and indications for removal and complications discussed.

Pedigree of the major histocompatibility complex in a family with a neonatal alloimmune thrombocytopenia.

Immunization of a mother negative for the platelet-specific antigen P1A1 against the P1A1 antigen of her child induced neonatal alloimmune thrombocytopenia. This prompt immune response occurred in her first pregnancy and we were therefore interested to study whether there is an association with genes located within the major histocompatibility complex. We deduced haplotypes of HLA-A, B, C and DR as well as of complement C2, C4 and Bf in the maternal family members.

[E. coli dyspepsia]. / Die Coli-Dyspepsie.

The incidence of EPEC infections has decreased dramatically in industrialized countries since the 1960s, 1970s, but EPEC remains an important of acute gastroenteritis in developing countries.

[Effect of a synthetic detergent (Syndet) on the pH of the skin of infants]. / Der Einfluss eines synthetischen Detergens (Syndet) auf das pH der Haut von Säuglingen.

The long- and short-term effects on the skin of infants of a synthetic detergent (syndet) with an acid pH were investigated and compared to ordinary soap. The short-term effect was determined by measuring the skin pH on different parts of the body before and 20 min after washing with syndet. The long-term effect was tested in a second group, in which the infants were washed either with ordinary soap or with syndet for 3 days. The skin pH was measured 4-5 h after washing. The results were evaluated statistically. The results show that for a short time syndet displaces the skin pH towards acid pH in younger infants; however, the skin pH is not influenced in older infants. Syndet keeps the skin pH in the physiological range for a longer time after washing than ordinary soap.

[The septic state in infancy]. / Das septische Zustandsbild im Säuglingsalter.

In a retrospective study it was investigated in which a course the septicemia appears during the first year of life and which laborchemical and immunological findings are typical for the specific manifestation and can be used for the diagnosis. 27 sucklings with septicemia were admitted at our hospital during 1976-1982. As the clinical course we found: the acute septicemia with pronounced shocksymptoms (Septic-Toxic-Course, STC), the septicemia with a tardy course and hematogenous dispersion of bacteria in one organsystem, namely in the brain (Meningoencephalitis), in the bone (Osteomyelitis) and in the soft tissue (Phlegmon), the septicemia with a tardy course and forms a septicopyemia with secundary dispersion of bacteria in multiple organsystems. Only STC and septicopyemia show the symptoms which are lead back with the dispersion of bacteria. By the septicemia with a tardy course and hematogenous dispersion of bacteria in one organsystem the clinical symptoms are determined only in the infected organ. As the only course of septicemia the STZ shows laborchemically in the blood a damage to the livercells with a constant elevated levels of plasmaencyms GOT, GPT and LDH; this findings can be used for a diagnostic criterion. By septicopyemia, meningitis and osteomyelitis the findings of sepsis exist in the blood but are rare by phlegmon. By septicemia with a tardy course (2, 3) a humoral and/or cellular immundeficiency exist. This is a point of application for therapy to give biological antibody with a large spectrum.

[DiGeorge syndrome--significance of early diagnosis in cellular immunodeficiency]. / DiGeorge-Syndrom--Die Bedeutung der Frühdiagnose be zellulären Immundefizienzen.

Di George syndrome is caused by anomalous development of the organs arising from the third and fourth pharyngeal pouches and results in congenital aplasia of the thymus, aplasia or hypoplasia of the parathyroid glands and cardiovascular malformations. Clinically, affected children show hypoparathyroidism and, because of depressed cell-mediated immunity, serious bacterial, viral and fungal infections. We present an infant, aged 6 weeks, with convulsions due to hypocalcemia, in which cell-mediated immunodeficiency was detected. Additionally diagnostic and therapeutic possibilities in DiGeorge syndrome are shown.

[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.

[The significance of respiratory syncytial virus as pathogen in respiratory tract infections in infants (author's transl)]. / Die Bedeutung von RS-Viren als Erreger von Infektionen des Respirationstraktes bei Säuglingen.

Ten out of twenty babies aged up to twelve months and admitted during the months February and March 1978 were shown to be infected with Respiratory Syncytial Virus. During the first 6 months of life the disease usually presents as bronchiolitis. Serologically only neutralising antibodies were demonstrable in this age group. IgA was usually raised in babies with bronchopneumonia. An allergic reaction between virus antigen and maternal antibodies is considered to occur, assuming an activation of the infant's own secretory immunoglobulin.

[A contribution to hereditary sensory neuropathy. Electron microscopical studies (author's transl)]. / Beitrag zur hereditären sensorischen Neuropathie. Elektronenmikroskopische Untersuchungen.

A female infant nine months of age, hospitalized several times because of feeding and thriving problems as well as statomotoric retardation, at the occasion of a combustion showed no sign of perceiving pain. Based on clinical symptoms, histological and electron microscopical examinations the disease could be classified as hereditary sensory neuropathy type III.

[Haemoglobin S/beta thalassaemia in a Greek family: a case study (author's transl)]. / Hämoglobinopathie vom Typ HbS-beta-Thalassämie: Ergebnisse einer Familienuntersuchung.

A case report is presented of the rare haemoglobinopathy HbS-thalassaemia, type betaS-betathal-O in a Greek family. The special diagnostic features are discussed, with particular reference to the differential diagnosis.

[Long-time follow-up of rare liver tumours in infancy (author's transl)]. / Langzeitbeobachtung seltener Lebertumoren im Säuglingsalter.

Two rarely-observed tumours of the liver are discussed: firstly, primary liver carcinoma in a 5-month-old infant, and secondly, myxoma in a newborn infant. Both tumours were removed surgically. The child with primary liver cell carcinoma was treated for 2 1/2 years with cyclophosphamide. She has been followed-up for 8 years and can be assumed to be cured. Both children developed normally. The pathogenesis of liver tumours at this early age is discussed.