Essential fatty acids in the nutrition of severely neurologically disabled children.

Essential fatty acids (EFA) are important for the normal development and functioning of the brain, retina and immune competent cells. Severely neurologically handicapped children often have feeding difficulties, and the composition of the diet may be critical with respect to an optimal nutrient content. The aim of the present investigation was to evaluate if the dietary intakes and serum phospholipid concentrations of EFA were adequate in a group of severely neurologically impaired children in an institution. To achieve this, a prospective study was done. The investigation showed low dietary intakes of both n-6 fatty acids (FA) and n-3 FA. The serum concentrations of total n-6 FA, linoleic acid and 22:6n-3 (docosahexaenoic acid) as proportions of the total serum phospholipid FA concentration were initially low. The serum concentrations of 20:3n-9 and 22:5n-6 cholesterol, triacylglycerol, total saturated FA, total monounsaturated FA and apolipoproteins A-I and B were high compared with levels in a reference group of healthy children. Following supplementation with fish oil and soyabean oil, the serum lipid profile approached normal. We conclude that the study children had suboptimal intakes of EFA and that elevated serum concentrations of 20:3n-9 and 22:5n6 were useful serological markers of suboptimal EFA status. Recommended dietary allowances for EFA given as a percentage of energy underestimate EFA requirements in children with a low energy intake. Severely disabled children with feeding difficulties should probably be monitored with serum phospholipid FA measurements or calculation of dietary absolute intakes of EFA.

[Long-term follow-up of patients with congenital heart defects]. / Langsiktig oppfølging av pasienter med medfødte hjertefeil.

There are about 500 new cases of congenital heart disease per year in Norway. Modern diagnostic skills, surgical techniques and follow-up programs have contributed to higher survival rates among patients. Based on international experience, 85-90 per cent of these children will survive into adulthood. Half will suffer from conditions which should be followed up by cardiologists. This article is based upon recommendations on long-term follow-up of patients with congenital heart disease.

Early clinical screening of neonates for congenital heart defects: the cases we miss.

In a population-based study of 35,218 infants born alive during the 15 years from 1982 to 1996, 353 (1%) were diagnosed as having a congenital heart defect, of whom 84 (24%) were diagnosed subsequent to discharge from hospital after birth (2.4/1000). Of these, 40 (48%) had a ventricular septal defect, 14 (17%) an atrial septal defect, 9 (11%) a patent arterial duct, 8 (10%) an aortic stenosis and 13 (15%) other defects. Compared with those in whom diagnosis was made before discharge, the group of patients with defects detected late had an increased prevalence of atrial septal defects, patent arterial duct and aortic stenosis, but less decreased prevalence of ventricular septal defects (p < 0.05). Median age at detection of the defects subsequent to discharge was 6 months (range 2 weeks-11 years). Seven patients (8%) presented with clinical symptoms of cardiac decompensation. The mortality rate was significantly lower in those in whom defects were detected late (1/84; 1%) as compared with those detected immediately after birth (37/269; 14%) (p < 0.05). The total rate for early detection was the same after using one clinical examination (8.2/1000) of newborns as our basic routine instead of two (7.1/1000) (p > 0.05). A substantial proportion of congenital cardiac malformations are detected after discharge from hospital after birth. Some patients with these lesions present with cardiac decompensation and are in need of medication and surgery. One clinical examination of newborns detects congenital malformations of the heart as efficient as two.

Studies on nutrition in severely neurologically disabled children in an institution.

Severe neurological handicaps in children are frequently accompanied by growth retardation. We have studied 13 severely neurologically impaired children in an institution to see if their poor growth was related to a low intake of energy and nutrients, if this was reflected in biochemical nutritional parameters, and to modify their diet according to the results. The investigation showed low dietary intakes of energy and of several of the nutrients, with corresponding low Hb values and serum values of ferritin, selenium and vitamins E and D in some of the children. All the children were initially light for age, with catch-up growth after intervention. We conclude that severely disabled children are at high risk for under- and malnutrition, and that this may partly explain the growth retardation in the study group. To avoid the potential detrimental effects of malnutrition, it is important to aim at providing an optimal diet.

[Treatment of supraventricular tachycardia in children]. / Behandling av supraventrikulaer takykardi hos barn.

Although young infants may have severe symptoms from supraventricular tachycardia the majority responds to treatment. In 60-90% of the infants the arrhythmias disappear within 6-12 months, whereas in older children the supraventricular tachycardia tends to reoccur. Our recommended acute treatment in infants less than six months of age is to emmerse the face in cold water, but adenosine should be used for pharmacological termination of supraventricular tachycardia in all age groups. If this fails, direct current cardioversion should be applied without delay. Intravenous verapamil should not be used, however, in infants for termination of supraventricular tachycardia. Flecainide can be used for acute and prophylactic treatment.

Pulmonary vascular resistance in complete atrioventricular septal defect. A comparison between children with and without Down's syndrome.

Invasive data obtained in children with complete atrioventricular septal defect over the last three years are presented to determine the age at which pulmonary vascular obstructive disease develops. Comparisons were made between children with (n = 21) and without Down's syndrome (n = 12). The investigation was restricted to patients less than one year of age at initial catheterization. Patients with complicated associated heart defects were excluded. Patients with Down's syndrome had a higher ratio of pulmonary to systemic vascular resistance than children without Down's syndrome in the basal situation. This difference almost disappeared after 100% oxygen had been given to patients with elevated pulmonary vascular resistance, indicating that hypoxia and/or hypoventilation is of importance, especially in children with Down's syndrome. Fixed elevated pulmonary vascular resistance was found in 11% of Down patients under one year, with the youngest patient being 5.5 months old. One child without Down's syndrome may have had fixed elevated pulmonary vascular resistance. It is concluded that all children with complete atrioventricular septal defect should be evaluated and operated on at a very young age.

[Primary peritonitis in children]. / Primaer peritonitt hos barn.

Primary peritonitis occurs rarely in childhood. The causative microbe is usually either streptococcus or pneumococcus. However, the incidence of gram negative anaerobic bacteria is increasing. The bacteria are cultured from fluid taken from the abdominal cavity during celiotomy. The abdominal viscera are normal, which excludes secondary peritonitis. We discuss the case of an infant who presented this disease entity.

Cardiac myxoma as the cause of death in an infant.

Cardiac myxoma is a rare disease most frequently encountered in adults, usually localized in the left atrium. It rarely occurs in infants. We report a 3 1/2 month old girl whose sudden death was caused by a large atrial myxoma, completely occluding the tricuspidal orifice. The importance of possible prodromal symptoms suggesting a cardiac disease is stated, and the role of echocardiography as an excellent examination technique is discussed.

[Transportation of newborn infants. A 6-year case load]. / Transport av nyfødte. Et seks års materiale.

We have evaluated neonatal transports to Ullevål hospital over a six-year period. These were either carried out by helicopter, using a trained transportation team, or by ambulance, the transport being improvised from one transport to another. The investigation shows great variation in the handling of the transports, with the most significant problems in the group without a structured transport system. In addition to generally rather casual and often hazardous transport, particular problems arose in connection with maintaining an adequate body temperature. In addition, the transports were very inadequately documented.

Congenital neuronal ceroid lipofuscinosis. A case report.

A case of congenital neuronal ceroid lipofuscinosis, a 9-day-old male child of Pakistani parents, is reported. Only 5 congenital cases have been described previously. The cerebral and cerebellar cortex showed extensive nerve-cell loss. Granular material with histochemical characteristics of ceroid-lipofuscin was deposited in neurons, macrophages and glial cells throughout the brain. Similar material was found in macrophages in the lymphoid system and in certain other organs. Ultrastructurally, the material was identical with that described in the infantile type of ceroid lipofuscinosis. It is pointed out that the metabolic exchange between the blood of the mother and the foetus is not sufficient to prevent the intra-uterine progression of the disease.

Halothane-associated liver damage and renal failure in a young child.

A case of halothane-associated liver damage and renal failure is described in an 18-month-old girl after her third halothane anaesthetic. There had been two uneventful enflurane anaesthetics. The diagnosis was confirmed by detecting antibodies specific for halothane-associated liver failure using a recently developed enzyme-linked immunosorbent assay.