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From Jan 1, 1965 to Nov 1, 1980, 155 infants with Hirschsprung's disease have been treated. All had confirmed aganglionosis at multiple rectal biopsies. From 1965 to 1969, 54 patients underwent a Swenson's procedure at an age ranging from 12 to 18 months after a preliminary colostomy. 13 patients died between colostomy and definitive operation. From 1969 to 1977, the delay in performing corrective operation after colostomy was shortened and the definitive procedure was done between 6 and 11 months of age in 69 patients. No fatality was observed but in 9 cases a perianal dermatitis lowered the quality of functional results. From 1977 to Nov 1, 1980, progress in immediate nursing and total parenteral nutrition made colostomy unnecessary in 19 neonates and infants with severe Hirschsprung's disease. The definitive surgical procedure (mainly Swenson's) was performed before 3 months of age. The postoperative results were excellent. After more than 4 years of follow-up for the first cases, the good secondary results, the quality o defecation, and the shortening of total hospital time, allow us to advocate this method in selected cases.

The authors report the experience of the Children's Hospital of Marseille about ten renal transplantations in nine children. Indications, immunosuppressive treatment, surgical techniques employed are detailed. The results study show two mains complications : rejection and arterial hypertension. A better recipient selection, as in adults, would increase good results.

Cystic lesions in childhood are very rare. Since the introduction of sonography the diagnostic approach has become much easier, but surgical treatment is also much simpler nowadays. During the past 10 years we had the opportunity to treat 7 patients. Our results are compared with those published in recent literature.

The authors present a multisource study of 29 kidneys tumors (Wilms excepted) in children. The most common types (18 cases) are tumors from metanephrogen blastema (congenital mesoblastic nephroma, nephroblastomatosis and nodular renal blastema, multilocular cystic nephromas. Mature kidney tumors are much more rare in childhood (8 cases): they are epithelial tumors (adenoma or adenocarcinoma) or mesenchymal neoplasms. In one case, histological study don't permit to list a malignant tumor to one of previous categories. Finely, among secondary tumors, two localisations of hemopathy are reported. After a limited exposure of cases and review of the literatures the authors emphasize the anatomo-clinical aspects, the value of paraclinical exams and the treatment of each type of tumor.

Cardiac and skeletal myosin subfragments 1 cleaved into three fragments were modified by 1-cyclohexyl-3-(2-morpholinoethyl)carbodiimide metho-p-toluene-sulfonate in the presence of the nucleophile nitrotyrosine ethyl ester. The effects observed (first-order kinetics of ATPase inactivation, incorporation of 1 mol of nitrotyrosine/mol of subfragment 1) were similar to those previously observed for the nondigested subfragments 1 [Lacombe, G., Van Thiem, N., & Swynghedauw, B. (1981) Biochemistry 20, 3648-3653; Körner, M., Van Thiem, N., Lacombe, G., & Swynghedauw, B. (1982) Biochem. Biophys. Res. Commun. 105, 1198-1207]. For both native and digested subfragments 1, which were inactivated to the extent of about 70%, the location of the label nitrotyrosine was performed by immunological blotting with 125I-labeled anti-nitrotyrosine immunoglobulins. It was found that the modified residue was essentially located on the heavy chain for the native subfragments 1 and on the 50K peptide for the digested subfragments 1.

The phosphate burst of cardiac myosins from different animal species was studied by a rapid quench-flow technique. The rate constant of the phosphate burst varied from one species to another. Moreover it was lower for cardiac myosins than for skeletal myosin. The phosphate burst rate correlated with the ATPase in the steady state and with the muscle-shortening speed. By contrast, the amplitude of the phosphate burst did not vary significantly. Its value was 0.8--0.9 per myosin site for each of the myosins studied. Deviations from this stoichiometry were related to the mode of preparation of the myosin and to denaturation by a high pH.

23 cases of primary testicular tumors (TT) were observed in 6 different French divisions of Pediatric Surgery during a 10 year period. This series combined with a survey of the literature serve to stress the rarity of such tumors in boys under the age of 15 years. Yolk sac tumors are the most frequent ones (40%), followed by teratomas (32.5%) which, in children and in this localization, are almost always benign tumors of nongerminal origin, in particular rhabdomyosarcomas and tumors of sexual cords, represent 22% of an TT. They are different from what is observed in adults, in whom the most frequent tumors are seminomas, embryonal carcinomas and teratocarcinomas. Most testicular tumors reveal themselves as unilateral testicular masses. High serum alpha-foeto-protein is specific to yolk sac tumors and its post-operative surveillance is mandatory. Scrotal ultrasonography constitutes the main investigation in view of diagnosis and also serves to detect less evident tumors in case of precocious puberty, gynecomastia. Since lymphatic invasions are unusual in children, surgical exploration of the retro-peritoneal space is no longer considered necessary for the stagging and is replaced nowadays by ultrasonography and CT scan. In case of teratomas and others benign tumors, high inguinal orchiectomy is sufficient; in certain cases (well-delineated and superficial tumor in single-testis), tumorectomy alone is possible. As concerns Yolk sac tumors, the addition of chemotherapy from the age of 2, cures more than 90% of stage I tumors; radiotherapy is necessary when it comes to stage II or III. As for testicular rhabdomyosarcomas, in which prognosis is better than in others areas, the combination of surgery, chemotherapy and perhaps radiotherapy is able to cure from 80 to 90% of them.

Caudal anaesthesia in pediatric surgery about 116 cases (children from three weeks to 18 years with 64 below 5 years) is reported. The caudal anaesthesia, at 6 mg/kg, is very usefull for surgery below T 10 because: --it can be used in day surgery, --it avoids intubation and its complications, --it allows feeding as soon as required, --it gives a post operative analgesia. In our practice, this technique is safe and is real progress compare to the general anaesthesia.

Since the first observation by Spann et al., it has become clear that in cardiac hypertrophy induced by a mechanical overloading, the velocity of shortening of the cardiac muscle (Vmax) is reduced (see ref. 2 for review). Most authors agree that this mechanical alteration is accompanied by a decrease in the Ca2+-dependent ATPase activity of myosin (see ref. 3 for review). The molecular basis of such changes was unknown because the structural modifications of the myosin molecule were ill-defined. Nevertheless, it has recently been shown that, like skeletal muscle myosin, cardiac myosin is composed of several polymorphic forms, comparable to isoenzymes. In the skeletal muscle, new functional requirements can induce changes in both contractile activity and type of myosin isoenzyme synthesised. We now report that an increase in cardiac work produced by mechanical overloading in rats induces the preferential synthesis of a cardiac myosin isoenzyme characterised by specific immunological and electrophoretic properties and exhibiting a lower ATPase activity. This adaptive change could account for the reduced shortening speed of this hypertrophied cardiac muscle.

Changes in serum levels of corticotrophin (ACTH), immunoreactive beta-endorphin, antidiuretic hormone and cortisol were compared in children undergoing minor surgery under either general anaesthesia with halothane or epidural anaesthesia by the caudal route. A rapid and major increase in hormone levels was observed under general anaesthesia but not under epidural anaesthesia.

Solen ensisensis muscle arginine kinase (ATP : L-arginine phosphotransferase, EC 2.7.3.3) was isolated in an homogeneous state. Its molecular weight was found to be about 80 000. The properties of this enzyme were compared with those of arginine kinase from Sipunculus nudus, an enzyme which also has a molecular weight of about 80 000. Both enzymes have several reactive thiol groups (8 thiol groups in the Solen kinase and 12 in the Sipunculus enzyme were titrateable with 5,5'-dithio-bis-(2-nitrobenzoic) acid and histidine residues (both enzymes have 6 reactive histidine residues). These kinases were, therefore, highly susceptible to oxidation. Both enzymes show the same pH optimum and absolute specificity towards the guanidine substrate, L-arginine. The reaction kinetics of both enzymes are of the sequential type. In the presence of alpha-aminoacids of Mg2+-ADP, similar spectral effects were obtained. The enzymes differ in their enzymic activities and in their rate of recovery following urea denaturation. The most important difference that appeared to be a special feature of the Sipunculus enzyme is that the spectrum of the Mg2+-ADP-enzyme complex is strongly intensified by L-arginine.