Cesko-Slovenska Pediatrie最新文献

This study was designed to asses the clinical course of five children with a short bowel syndrome after massive intestinal resection during neonatal period. On the basis of their experience the authors analyze some properties that may be advantageous in this syndrome and discuss the most important factors, including enteral feeding at an early postresection stage, which influence a full development of the small bowel adaptive mechanisms. They point out that so called "short gut colitis", sometimes occurring during advancement of enteral nutrition, can be successfully treated by sulphasalazine. The authors come to the conclusion that the prognosis for newborn infants undergoing an excessive bowel resection is far well thanks to enhancement of the intestinal adaptation by a combination of a long-term parenteral nutrition with enteral feeding.

The authors elaborated data, using the retrospective method, of a group of 146 patients with affective respiratory and reflex paroxysms. They focused attention on clinical manifestations of the disease, anamnestic data suggesting possible damage or immaturity of stem structures, the influence of heredity and the family environment. They found that in 63.7% the disease was manifested before the age of 1 year, most frequently at the age of 9-12 months. The cyanotic type of paroxysms was found in 67.5% of the patients, the pallid type in 21% and 3.5% of the patients suffered from both types of paroxysms. In 27.4% perinatal risks were recorded. The influence of a family-history was statistically significant in relation to the patient's age during the first attack: in patients with a positive family-history the mean age being by 1.8 months lower. 82.9% of the patients had a normal neurological finding, the EEG was evaluated as normal in 89.6% of 125 thus examined children. Psychological examinations made in 12 children revealed in all instances anomalies of personality with a predominance of lack of compliance and adaptability to the environment. Deterioration of the health status as a result of affective respiratory and reflex paroxysms was not recorded in any of the patients.

In the submitted case-history the authors publish the case of successful treatment of a 9-year-old girl whose life was at risk after intoxication with a large dose of amitriptyline. Concurrently they deal with the social background of emotional disorders and some peculiar features in this girl with suicidal behaviour; attention is also drawn to specific features of the psychopathological picture of psychiatric child morbidity.

The authors describe in a neonate with thanatophoric nanism histopathological changes of the bones. According to X-ray findings type I was involved, the histopathological changes corresponded to type II according to Langer's classification. The consanguinity of the parents suggested the autosomal recessive type of heredity and the presence of pseudo-diastrophic dysplasia in two grade 1 cousins is considered by the authors as an incidental coincidence. The described case confirms the assumed clinical and genetic heterogeneity of thanatophoric nanism.

At the beginning of the eighties the Czech Republic had a chance to cure completely less than 30% of children treated on account of acute lymphoblastic leukaemia (ALL). Introduction of a very intensive protocol ALL-BFM 83 in all leukaemia centres in the Czech Republic in the mid-eighties led to radical improvement of the prognosis of the sick children. In 1985-1990 339 children were treated by therapy differentiated according to the degree of risk. Remission was achieved in 90.5%, by Dec. 31 1991 61% of the children lived in their first complete remission. Based on the above results the 5-year probability of survival was calculated for the whole group as 62%. Children with a standard risk of an adverse course and children where the diagnosis was made at the age of 1-10 years had the most favourable prognosis. The poorest therapeutic results were recorded in children with a high risk of an adverse course and in children under one year at the time of the diagnosis. During the investigation period a relapse of the disease occurred in 25% patients, most frequent was an isolated medullary relapse in 13%; leukaemia of the CNS was diagnosed in 7.5% and 5.5% of the boys suffered from leukaemic infiltration of the testes. The newly introduced nation-wide protocol ALL-BFM 90 gives further hope of an improved prognosis of children with ALL.

The authors demonstrate two cases of non-immunological foetal hydrops. In the first case the initial cause of foetal hydrops was hypoalbuminaemia (hypoproteinaemia), in the second case intrauterine cardiac failure resulting from supraventricular tachycardia of unknown aetiology. The authors explain the pathogenesis of the condition, its early diagnosis and therapy. They draw attention to possible intoxication of the neonate by digoxin administered to the mother during pregnancy.

The author compared in 88 13-year-old children evaluation of the size of the thyroid gland by palpation and sonography. Evaluation by the two methods was in agreement in 64 cases (73%) and controversial in 24 cases (27%). Palpation led in 24 instances to overestimation of the size and in 7 children (8%) to underestimation, as compared with sonography. Sonography is an essential method for evaluation of the thyroid gland in children.