Cesko-Slovenska Pediatrie最新文献

The authors submit their experience with the treatment of idiopathic precocious central puberty in two girls with the GnRH analog (Decapeptyl-Depot, Ferring Co.). The drug was administered for one an a half and two years resp., 80 mg/kg once per month. Apart from the effect on gonadarche and thelarche the pubertal growth rate was reduced to a prepubertal level and the originally accelerated bone maturation was reduced.

More than 100 patients with central precocious puberty are participating in this international multicenter study using monthly i.m. injections of the slow-release GnRH agonist Decapeptyl-Depot. In 15 patients, Decapeptyl-Depot treatment could be discontinued after 2 years of therapy. Gonadal suppression was promptly reversible in all of them, as shown by prepubertal low gonadotrophin and sex steroid levels. Of the remaining 90 patients, 40 have been treated for more than 3 years, including 33 girls and 7 boys. Plasma levels of LH, FSH, estradiol and testosterone dropped to the prepubertal range after one month of Decapeptyl-Depot and remained there for the whole period of therapy. At start of therapy, mean chronologic age of these 40 children was 6.6 +/- 1.4 (SD) years, mean bone age 10.2 +/- 1.9 years. Mean predicted adult height increased in the boys from 173.6 +/- 13.8 (SD) cm at start of therapy to 184.6 +/- 17.0 cm after 3 years. Predicted adult height increased in girls from 158.0 +/- 12.2 to 161.0 +/- 7.5 cm. Undue side effects were not seen, long term tolerance was good. It is concluded that Decapeptyl-Depot injected i.m. every 4 weeks suppresses the pituitary gonadal axis in children with central precocious puberty without clinical or biochemical escapes, and leads to an increase in predicted adult height by more than 3 cm in all boys and in 53% of the girls after three years of treatment.

The authors examined a group of 10 girls with true sexual precocity. The age of the patients was within the range of 11 months and seven and a half years. Height acceleration was on average almost one year, skeletal age acceleration was on average almost one and a half years; when BA was evaluated according to tables of visualization methods of the Motol Clinic and the TW2 and C method, a marked acceleration of BA was observed in the RUS method. To differentiate incomplete puberty-thelarche-in all girls the stimulation test, using Relefact LHRH (Hoechst), was performed, the diagnosis of true puberty was suggested by LH serum levels elevated above 12 IU/l. In incomplete puberty only FSH was elevated. In all patients oestrogenization of the vaginal epithelium, breast development (M2-3) and pubic hair was present, in three patients menarche. For treatment Decapeptyl-Depot (Ferring) was administered, the longest period of treatment was eight months. Therefore the change of HA and BA was not evaluated, but even during this brief period of treatment regression of pubertal sex signs occurred.

The authors investigated C1-inhibitor (C1-INH) plasma levels in blood collected from mothers during delivery: mothers with a normal pregnancy and a group of mothers who displayed during the perinatal period direct or indirect signs of infectious disease. Both groups, as compared with a group of healthy donors, had low C1-INH levels. The neonates were divided into two groups (normal children and children with perinatal risk of infection) with sub-groups of mature full-term neonates (38-41 weeks of gestation) and premature infants (29-36 weeks of gestation). In the group of normal infants the neonates had on average somewhat lower C1-INH levels, as compared with healthy donors, in premature neonates of this group the lowest mean C1-INH level was recorded. In this group the authors observed a correlation between the inhibitor level and the gestation period. The results of C1-INH assessment in the group of neonates with a perinatal risk of infection were different. In premature neonates a higher average C1-INH level was observed than in mature neonates and the relationship between the C1-INH level and the gestation period was a linear negative regression. The postnatal increase of C1-INH levels on the 1st to 5th day was more rapid in premature neonates of both groups.

The authors describe a 14-year-old gypsy girl with the clinical picture of exudative enteropathy where they observed on X-ray examination of the abdominal cavity circular formations which changed position. On laparotomy they found on the serous membrane of the entire enlarged and inflamed ileum numerous pseudo-polypous formations the majority of which were released and floated freely in the chylous ascites. The description of similar globular lesions of the lymphatic system was found in the literature only in accounts on post-mortem findings.

The authors evaluated a group of 256 children operated in 1983-1992 at the Orthopaedic Clinic of the Faculty Hospital on account of congenital pes equinovarus. These children account for 25% of the total number of children treated because of this inborn defect by conservative treatment. The authors draw attention to the lack of uniformity of contemporary treatment of congenital pes equinovarus, and based on their own experience, they recommend an exact therapeutic plan. They emphasize the importance of early treatment by means of corrective plaster bandages, they describe the tactics of treatment by this method, as any deviation from this therapeutic pattern leads to incomplete elimination of the defect and to relapses. The authors present also a general review of possible surgical treatment and emphasize the tactics of postoperative treatment, incl. intensive rehabilitation and correction by footwear. They emphasize in particular the necessity of systematic and uninterrupted conservative treatment which is essential as preoperative preparation, as only by intensive care before and after surgery, incl. a correctly indicated and implemented operation, the desired goal, i.e. an anatomical position of the foot can be achieved.

Transvaginal echocardiography makes it possible to diagnose serious congenital heart disease already during the first trimester of pregnancy. The authors present their experience with this examination method in pregnant women with a high risk of foetal cardiopathies and in women whose foetus is affected by another serious organ anomaly. The authors evaluate the successful visualization of the cardiac anatomy in different projections in relation to foetal age. They present the diagnosis of a serious inborn heart disease detected during the 15th week of gestation.

The authors examined 25 kidneys in 14 healthy neonates in order to evaluate the resistive index (RI) calculated from the maximal systolic and end-diastolic rate of blood flow in the renal artery and some intraparenchymatous arteries. They revealed a significant drop of all absolute rates of blood flow in arteries in the direction to the renal periphery. Consistent with rather few data in the literature, they assessed a normal RI value of intraparenchymatous arteries in healthy neonates (0.76 +/- 0.04) which differs significantly from values assessed in the renal artery (0.82 +/- 0.05) (p < or = 0.001). The authors discuss the normal RI value in the neonatal period. In the conclusion they mention that the RI can be obtained much more easily from the blood flow in the renal artery than the blood flow in intraparenchymatous arteries but the former does not reflect representatively the intraparenchymatous conditions in the kidneys and this restricts its use in evaluations of intraparenchymatous renal processes.

The authors made two series of experiments in rabbits, using liquid ventilation. The first group was ventilated manually using RM 101 solution with an equilibration period of 30 and 60 sec., Tv = 10 ml/kg after previous administration of 30 ml/kg RM 101 at the beginning. The second group was ventilated using a specially developed liquid ventilator--part of the animals was ventilated with an equilibration interval of 30-90 sec., Tv = 10 ml/kg with administration of an initial dose od 30 ml/kg of RM 101 solution, a part of the animals in a continual manner, i.e. without an equilibration period and without administration of the initial dose of the solution. From the results it is apparent that the optimal way as regards blood gases and minimal manner of liquid ventilation. When this method was used during the 180 minutes of liquid ventilation paO2, paCO2 and pH were within the normal range. The other methods of liquid ventilation led rapidly to hypercapnia and a drop of pH as well as to serious changes in the circulation (hypertension, bradyarrhythmia).