Acta paediatrica Scandinavica最新文献

Pre- and postoperative growth was analyzed in 22 children with craniopharyngioma. In 19 children a growth failure preceded the diagnosis by a mean of 4 years. Six children were obese preoperatively. During the first 3 postoperative months relative weight increased greater than 10% in 14/21 children (there was one surgical death). One year after surgery 13/21 were obese. Neither the size of the tumor nor the mode of surgery was decisive in the development of the obesity. Serum insulin and insulin-like growth factor I (IGF-I) were assessed in four children with growth hormone deficiency (GHD) who, after surgery for craniopharyngioma, were growing normally without GH substitution. One of them was normal in weight and had normal insulin and IGF-I levels; the others were obese and had supranormal insulin and subnormal IGF-I levels. One of the four and two other children with unsubstituted GHD reached final height SDS -0.8, -2.0 and -2.4. One child with normal postoperative GH response reached final height SDS -0.7. Final height SDS greater than or equal to -2.5 was gained with GH substitution by 6/11 children. It was greater than 2.0 SD below the height SDS expected from the heights of the parents in 7/11. An adequate monitoring of children's growth would lead to earlier diagnosis and probably better outcome.

The concentrations of IgG subclass immunoglobulins were determined by radial immunodiffusion in serum from 126 patients with cystic fibrosis (CF). The results were compared to values from age-matched healthy children and adults and correlated to patients age, duration of chronic Pseudomonas aeruginosa infection and lung function parameters. Fifty-two percent of the patients had an elevated concentration of at least one of the IgG subclasses; IgG1 28%, IgG2 16%, IgG3 18% and IgG4 48%. There was significant correlation between elevated serum levels of IgG2, and to a lesser extent IgG3, with decreased lung function (for FEV1; p = 0.0001, and p = 0.001 respectively) and high levels of antipseudomonas precipitins (p = 0.008, and p = 0.002). A similar correlation was not found for IgG1 and IgG4. IgG subclasses vary in their ability to promote phagocytosis and to activate complement and it is possible that individual differences in the IgG subclass pattern could explain the variable course of this disease.

We have treated 14 girls with central precocious puberty for a mean period of 2.3 years (range, 0.5-3.9) with intranasal (D-Ser6) GnRH analogue administered in a mean dose of 28 micrograms/kg/day (range, 15-56). With the onset of treatment there was an initial increase in sitting height compared to subischial leg length, but overall there was no significant change in height standard deviation score for bone age. In this respect our results were indistinguishable from untreated children with central precocious puberty. There was a decrease in physiological GH secretion, associated with decreased sex steroid secretion, which probably accounts for the growth deceleration which has been described during GnRH analogue therapy. The effect of this growth deceleration combined with slowing of the rate of epiphyseal maturation may explain the absence of alteration in height prognosis.

The success of familial compliance with a dietary manipulation programme was studied prospectively in 91 newborn babies from atopic families for up to 12 months. The control group consisted of 72 infants from non-atopic families. The percentages of infants breast-fed at the age of six months were 58% and 38% in the study group and control group, respectively. The introduction of cow's milk based formulas was postponed until the age of three months in 63% of the infants in the study group and 47% of the infants in the control group. Solid food was avoided during the first three months in 76% and 45% of the infants in the study group and control group, respectively. Maternal age, smoking and low social class were associated with poor compliance. The existence of atopic dermatitis in any member of the family was associated with good compliance. The main advantages of our programme were the increased number of children breast-fed until the age of six months, and the reduced number of infants introduced to cow's milk based formulas or solid food before three months of age.

Four patients with portal hypertension, oesophageal varices and severe hypersplenism were treated by partial splenic embolization. All showed improvement of blood and platelet counts early in the postoperative period. Three months after embolization IgA and C3 levels increased significantly. All patients had a decrease in the incidence of variceal bleeding and this procedure provides an acceptable alternative to splenectomy.

The coagulation inhibitors heparin cofactor II (HC II), antithrombin (AT) and protein C (PC) were measured in healthy term and preterm infants in order to establish reference standards. The mean value for HC II in term infants was found to be about half of the adult values. Values below 25% in healthy infants may suggest hereditary deficiency states. One girl with congenital HC II deficiency was detected. Mean AT and PC levels were somewhat higher than HC II. Healthy preterm infants have significantly lower HC II and AT values than healthy term infants. Serial AT measurements have been used in monitoring seriously ill infants and used as a prognostic indicator. In a small number of unhealthy neonates HC II was reduced to an even greater extent than AT, and on recovery normalized more rapidly than AT.