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We have studied serum lipid levels and dietetic intake in 38 children whose parents had ischemic heart disease (HPCI) before 55 years of age and in a control group (n = 114). In the HPCI group, 25% had serum levels of total cholesterol higher than 200 mg/dl; only 2 children presented elevated levels of LDL and apo B. Dietetic intakes in both groups were similar, with a high protein (16-17% of calories) and fat (39-42% of calories) intake and a low carbohydrate intake (40.46% of calories). In both groups the percentage of monounsaturated fat was higher than other types of fat. The most frequent phenotype in the HPCI group was IIa (8 children). Only 1 child showed a IIb phenotype. This finding may be due to the variability of this phenotype in the same individual throughout life. In the families (n = 8), we have detected 2 families with polygenic hypercholesterolemia (HP), 2 others with familial combined hyperlipidemia (HFC) and 4 without family history of hyperlipidemia. Taking into account the lipid profile in children of the HPCI group, we have detected the presence of familial dysliproproteinemias. It appears that dietetic intake is not an atherogenic risk factor in these patients.

A series of 439 children (245 boys and 194 girls) ranged between 2.0 and 18.0 years of age have been studied January 1987 to April 1990. They belonged to four groups: I) 306 children (163 boys and 143 girls), "control group"; II) 31 children (22 boys and 9 girls) whose parents had some type of dyslipoproteinemia (HPDLP); III) 38 children (24 boys and 14 girls) whose fathers were survivors of myocardial infarction occurred before 55 years of age (HPCI); and IV) 43 children (23 boys and 20 girls) who had, at least in two occasions, more than three months of time separated between then, over 200 mg/dL of total serum cholesterol levels detected by opportunist search (HDC). For children's identification of risk factors to develop atherosclerotic disease during adult life, two different types of strategy has been utilised. One, "selective search", taking into account children of groups II (HPDLP) and III (HPCI). Other, "opportunist search", taking into consideration children of group IV (HDC). The most frequent primary dyslipoproteinemia between the families of children with high serum levels has been Polygenic Hypercholesterolemia (HP). In the second place were both Familial Hypercholesterolemia (HF) an Familial Combined Hyperlipidemia (HFC). A family with Mixed Hyperlipidemia (HM) was also identified. Familial aggregation, with relation to serum lipid levels, were detected in children of the three groups: HPDLP, HPCI and HDC, as it is reported by another authors. Our results suggest the genetic alterations may contribute to the presence of different types of dyslipoproteinemia in children.

Apolipoprotein (apo)-beta is an atherosclerotic risk factor in adults and children. In families with Familial Combined Hyperlipidemia (FCH) it has been described as a lipoprotein phenotype called Hyperapobetalipoproteinemia (Hyperapo-beta) and characterized by increased numbers of small, dense and apo-beta enriched low density lipoproteins. In our Lipids Clinic, we have studied 267 children, but for the purpose of this paper we have only taken into account the 19 of these children who showed increased plasma total apo-beta levels (Hyperapo-beta-emina). To investigate the type of dyslipoproteinemia of these children, we divided them into two groups: 1) Group 1: 10 children with apo-beta levels greater than mean + 2 standard deviations (SD); 2) Group 2: 9 children with apo-beta levels greater than mean + 3 SD. We have also studied the fathers, mothers, brothers and sisters of all the children. Only one child (Group 1) had type IIb hyperlipoproteinemia. The other children had type IIa hyperlipoproteinemia. In each group, 4 families had FCH and the others had either Familial Hypercholesterolemia or Polygenic Hypercholesterolemia. Families with FCH could also have hyperapo-beta. It is possible that in the future some children of FCH families and those with type IIa hyperlipoproteinemia will have increased plasma triglyceride levels. This could be prevented by a proper diet.

The polyps of the digestive tract, with the exception of the juvenile ones, are not often found in children. Out of 762 endoscopies paediatric patients carried out, 31 were seen to be affected by digestive polyps, of which 16 were juvenile. The rest belonged to other types, among which 5 adenomas are included. In this paper, clinical aspects are analysed, pointing out also the value of the endoscopy as choice method not only for diagnosis but also for treatment of polyps.

The authors have studied the body composition in a group of 36 boys from 7 to 10 years old, through densitometry and anthropometry. They have found significant correlations between the percentage of fat, got by densitometry and some anthropometric indices: Quetelet index (r = 0.79), sum of 4 skinfolds (r = 0.78) and logarithm of the sum of 4 skinfolds (r = 0.80). After a multiple regression analysis, they got equations which permit you to calculate the percentage of fat, using several anthropometric parameters with a 10% of error. They bring two nomograms which facilitate the estimation of body fat. They compare the results obtained with the ones described by others authors and suggest the need to extend this study to boys with other ages as well as to girls.

A case of "iliac horns" syndrome appears in a there month old baby girl, with alterations in the nails of the thumb and index fingers, which gave reason to suspect the existence of an associated osseous dysplasia. Diagnosis is confirmed with radiology, showing pathognomonic iliac horn of the syndrome. An extensive review is made of literature on this affection, extremely infrequent in infancy, establishing that this is the case involving the youngest age described to date. Interest in radiological study of minimal alterations in phenotype of the pediatric patient is emphasized.

The authors present a 12 year old patient affected by a juvenile polyposis syndrome. They describe the clinic and the complementary tests carried out, especially the findings of the digestive fibroendoscopy which allowed multiple polyps in the duodenum, colon and rectum to be shown, confirmed by an anatomopathological study. Two years afterwards, a total colectomy and ileoproctostomy was carried out. In the operative piece included numerous hamartomatous polyps with adenomatous isolated foci. A subsequent endoscopic exploration showed numerous polyps in anus and rectal stump. The adenomatous transformation and recidivation of her polyposis require close vigilance and a follow-up of this female patient in view of the possibility of the precocious development of a colorectal carcinoma.

Congenital adrenal hyperplasia caused by a deficiency of 21-hydroxylase is an inherited disorder of adrenal steroidogenesis. In this study, the deficiency incidence is presented according to different author's and countries' results. Nowadays knowledge concerning its genetics are reviewed, especially HLA linkage. Allelic variants of deficiency and the "linkage disequilibrium" with HLA complete antigens are described. Finally, the main applications of that knowledge are presented: heterozygote detection and prenatal diagnosis.

The persistence of serological positivity was studied in 21 children with surgically proven hydatidosis. The children had been previously operated on between 10 months and 7 4/12 years before serum samples were extracted. Forty-eight people (19 adult and 39 children) were included as a control group. E.L.I.S.A. specific IgE and indirect hemagglutination techniques were used for the diagnosis of hydatid disease. Studies of the immunoglobulins and complement components IgG, IgA, IgM, IgE, C3, C4, C5 and factor B were done on the sera from the 21 children with histories of equinococciasis. The results are expressed according to the location and physical condition of the cyst, the time passed after surgery, the number of cysts and the number of surgical operations. We found persistence of positivity of up to 5 4/12 years for indirect hemagglutination and up to 6 7/12 years for specific IgE, in cases without evidence of clinical parasitation so we think that the postoperative control must be based on several serological determinations. The concordance between the two specific serological tests (specific IgE and indirect hemagglutination) was 80.95%; specific IgE having appeared more sensitive especially in cases of hepatic location. The results concerning the total IgE determination were highlighted in the immunoglobulin studies. We consider total IgE as being only orientative, because of the frequency with which it is found elevated in childhood diseases.

It have been studied 90 children (obese 73, non obese or control 17), trying to analyze the following parameters: mean and standard deviation values, its circadian rhythms and relation between cortisol and insulin, correlation between both hormones and anthropometric parameters of obesity and sexual influence with all the studied parameters. The plasmatic cortisol values were higher in male, obese (180.4 +/- 11.2 ng/ml) and control (377.5 +/- 49.2 ng/ml) children. The plasmatic insulin values higher in obese children (55.3 +/- 9.5 microU/ml) verifying the hyperinsulinemia observed in obesity. There was not correlation between cortisol and insulin values with body fat. All the children showed cortisol and insulin circadian rythm. In the groups of obese children the cortisol circadian rythm was similar to the control one. However, the insulin circadian rythm was disturb. So, the acrophase was delayed two hours when male and female were analyzed all together and only one hour in the obese female group. The acrophase of cortisol and insulin rhythms in obese group is delayed in relation with the degree of obesity. The circadian rhythms of cortisol and insulin, in both obese and control groups, are not dependent. The duration of obesity do not have any relation with the mentioned alterations.

Atherosclerosis and its consequences are a significant cause of death in industrialized countries. In recent years, due to modern techniques which permit DNA analysis, a series of alleles associated with dyslipoproteinemia and heart disease have been identified. In this study these genetic markers, in particular those found at the level of the genes of apolipoproteins, are reviewed. The knowledge of these facts is important for the paediatrician, since these disturbances can be identified in the first days of an individual's life, thus a positive influence can be made over the lifestyle habits.

Six cases of children diagnosed as having osteoid osteoma with different localization are reviewed. They constitute 0.04% of all those admitted to the paediatrics department. The average age is 8 1/12 years and three exists a male predominance over the female in the proportion of 2/1. The clinic has shown it self through characteristic pain, with nocturnal paroxysms, which disappears with aspirin, in addition to functional impotence and muscular atrophy. In the case of vertebral localization, scoliosis was detected. Both, the radiography and the computerized axial tomography showed to be efficient for the diagnosis, which was confirmed anatomo-pathologically in the case operated on. The evolution was satisfactory in all of them.

A study of plasmatic fatty acids was carried out on a group of paediatric patients suffering from cystic fibrosis. These data have been compared with those obtained by others authors. High levels of saturated fatty acids and a reduction of polyunsaturates have been found. The ratio of eicosatrienoic acid to arachidonic acid is high in this group of patients, which indicates a certain lack of essential fatty acids.

The authors present a system for the appraisal of the nutritional and inflammatory condition in patients suffering from cystic fibrosis (CF) in the phase of apparent inactivity of pulmonary infection, using a system of indices based on the quantification of some plasmatic proteins. The plasmatic appraisals of 4 visceral proteins (albumin, thyroxine-binding prealbumin, retinol-binding protein and transferrin) and, as well, of 5 proteins of the acute phase (alpha-1-acid glycoprotein, alpha-1-antitrypsin, alpha-2-macroglobulin, haptoglobin and ceruloplasmin) were obtained in a control group of 16 healthy children and in another of 14 children affected by CF. With the proteic plasmatic appraisals of the control group, the knowledge of their biological value and after a statistical-mathematical analysis, the most sensitive, specific and independent proteins were determined for evaluating the nutritional and inflammatory condition, obtaining two simple formulas which were denominated Nutritional-Inflammatory Prognostic Indices (NIPI) A and B (NIPI A = alpha-1-acid glycoprotein + haptoglobin/albumin + prealbumin; NIPI B = haptoglobin/albumin). From the analysis of the results, it can be deduced that the children with CF are affected by an inflammatory process, very probably infectious.