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Autoimmune polyglandular syndrome (APS) is an entity, defined by autoimmunity towards two or more endocrine organs. APS is classified in 3 subgroups (type-1, type-2a, type-2b), according to the organs involved. A case is presented of a 13-year old girl referred to the Department of Paediatrics with hypothyroidism, subsequently diagnosed adrenocortical insufficiency and impending Addison crisis, typical for APS-type 2a. In the paper we discuss the need for more attention to APS in clinical work.

Today there are 19 paediatric departments in Denmark. Most of the children's departments are placed in obsolete buildings. Centralisation in children's hospital provides a child-oriented environment with staff, who are knowledgeable about the needs of children, have the competence in both basic and highly specialised medical care, there will be greater opportunities to conduct research in children's diseases and be at the cutting-edge of both national and international excellence in the paediatric area of specialisation, accomplishing this ambition through research and medical improvements.

Introduction: Beta-thalassemia major occurs with increasing frequency among Danish children as a result of immigration. The aim of the study was to estimate the occurrence of beta-thalassemia major in Denmark, analyse the treatment and organ functions, and identify areas for an improved treatment strategy., Material and Methods: During 1998-99 all Danish pediatric departments were contacted for identification of children aged 0-18 years with beta-thalassemia major. Blood transfusions and chelation therapy were registered, and for Eastern Denmark clinical, endocrine, cardiac, and serologic parameters were performed., Results: Twenty-six children had beta-thalassemia major. Out of these, 20 received blood transfusions, and 17 patients were chelated. Eight patients were not chelated owing to previous bone marrow transplantation, treatment with hydroxyurea or ferritin < 2000 micrograms/l and young age. One patient had died. The body height was between 1.5 and -5.4 SDS (median -1.7) and the sitting height was -0.6 to -5.6 SDS (median -2.3). The bone age was delayed 1-5 years (median -2.5) in six out of ten examined patients, and puberty delayed in four out of five. A dilated left ventricle was documented in one out of eight patients examined. All patients were HIV and hepatitis C negative. For 75% of the children, the parents were related., Discussion: Children and adolescents with beta-thalassemia major in Denmark experience major heterogenicity with regard to treatment and late effects. An earlier and more effective iron chelation therapy together with improved patient support may reduce growth disturbances and endocrine and cardiac late effects.

The aim of the study was to evaluate the benefit from participation in parent groups for parents of extremely premature new-born babies. The participation in the parent groups led by the psychologist took place during the hospitalization of the child on the neonatal ward. All parents of extremely premature new-borns admitted to the neonatal department, Hvidovre Hospital, Copenhagen, Denmark in the period from 1 January 1992 to 30 June 1994, were asked to fill in questionnaires. The study population comprised the parents of 58 children. The parents of 14 children did not want to participate. Of the remaining 44 children, 36 were alive and eight had died at the time of the study. Most parents participated in the parent group and the majority stated they had benefited from the participation. It is therefore considered a relevant psychological task to establish parent groups for parents of extremely premature new-borns as a mean of crisis intervention in the neonatal department.

The aim of this study was to evaluate the attitude among parents of extremely premature newborn children towards fixed lower limits for treatment and towards parent involvement in decisions about the treatment of their child. All parents with extremely premature newborns admitted from January 1, 1992 to June 30, 1994 to the Neonatal Department, Hvidovre Hospital, Copenhagen, Denmark, were asked to fill in questionnaires. The study population comprised the parents of 58 children. The parents of 14 children did not want to participate. Of the remaining 44, 36 children were alive and eight had died at the time of the study. Almost all parents stated that neither birth weight nor gestational age were acceptable as criteria for treatment or non-treatment of premature newborns. This attitude contrasted with the recommendation in 1994 from The Danish Council of Ethics. Half of the parents expressed a wish to be involved in the decisions about the treatment of their newborn child. This attitude agreed with the recommendation from the Danish Council of Ethics.

The AIDS-committee of The Danish Society of Paediatrics has done a nation-wide study among infants and children under the age of 15 with AIDS or HIV-antibodies in preparation for planning prevention and treatment. Clinical data have been collected from the Departments of Paediatrics and Infectious Diseases, Haemophilia, Dermatology and Internal Medicine up to 1 March 1993. The study includes 44 infants with a risk of vertical transmission from the mother and 16 children with haemophilia. No cases were found to be infected by blood-transfusion. Twenty of the 44 infants with congenital HIV-antibodies were HIV-infected. Seven of them died from AIDS, 10 currently have AIDS and three are asymptomatic. Seventeen infants are well and HIV-antibody negative after the age of 18 months. Seven infants still have unclarified status, but all are well. Three of the patients with haemophilia are dead. The 13 others do not have AIDS. It is surprising that most of the infected infants' mothers were not known to be infected before the infants got sick. Thus infected infants exist in families who are not suspected to be HIV-infected. The AIDS-committee of The Danish Society of Paediatrics has proposed recommendations for HIV-testing of infants and children. HIV-infected families need comprehensive psychosocial care. The risk-factor from blood-transfusion is now eliminated, but vertical transmission will continue to be a risk-factor. The size of the problem in Denmark will not be known until an epidemiological study of pregnant women has been conducted.

In the period 1985 to 1991, 80 infants with gestational age below 28 completed weeks were born at Hvidovre Hospital, Copenhagen and transferred to the neonatal intensive care unit of the hospital. The incidence of extreme prematurity was 3.6 0/00. Twenty-eight infants died during the neonatal period (35%) and nine infants died later in infancy (11.3%). Forty-three infants (54%) survived. Forty four percent of surviving infants had one or more sequelae related to their prematurity or neonatal complications, mainly blindness or reduced vision, cerebral palsy and mental retardation. Neither gender, mode of delivery or birth asphyxia were important for survival and sequels. Neonatal complications such as patent ductus arteriosus, septicaemia, necrotizing enterocolitis, pneumothorax and cerebral haemorrhages were significantly related to survival and sequelae.

In a retrospective study, all deliveries at Hvidovre Hospital, Copenhagen, with birth weights < or = 1500 g and gestational ages of 23 to 32 completed weeks were examined to find out how they were recorded and the survival of the baby. Sixty-one were registered as abortions, 151 as births. Sixteen infants (10.2%) were registered as stillborn and 9 (6.7%) were live born without major malformations but died in the delivery ward without transfer to the neonatal intensive care unit (NICU) of the hospital. The survival for infants without severe malformations was 72.4% for all liveborn infants, and 77.6% for infants transferred to the NICU. The youngest surviving infant was born after 24 completed weeks of gestation. Because of different criteria for selection, comparison between different studies is difficult. This investigation shows that registration of very premature deliveries can be heterogenous. This is of great importance for survival and outcome rates because of the small number of very premature infants. We suggest a registration of all deliveries taking place after 22 completed weeks of gestation including birth weight, gestational age, pulse, respiratory movements, lethal malformations and time of death to ensure a better knowledge of the prognosis of these tiny infants.

Two cases of congenital tuberculosis are presented and the literature is reviewed. One child survived without sequelae while the other survived but was severely handicapped. The importance of early diagnosis and treatment of this rare but serious manifestation of tuberculosis is emphasized.