[Organ transplantation in children]. / Organtransplantasjon hos barn.

Over a period of close to 30 years, children in Norway with organ failure have been offered the option of a transplanted organ. Kidney transplantations have been performed since 1971, heart and liver transplantations since 1983, when ciclosporin was introduced. We present a review of somatic aspects on kidney transplantation in children, with special emphasis on 136 renal transplantations in 107 Norwegian children, eight of whom have died. Organ transplantation has a major psychosocial impact on the child and the child's family. A review of the psychosocial aspects of organ transplantation is given.

[Gastroenteritis in children. Peroral rehydration, intravenous fluid therapy and nutrition]. / Gastroenteritt hos barn. Peroral rehydrering, intravenøs vaeskebehandling og ernaering.

In pediatrics, oral rehydration treatment is increasingly used in the management of gastroenteritis, and based on third world experiences, it is becoming used also in hospitals in western countries. In this paper, the advantages, limitations and practical use of oral rehydration treatment are discussed. Clinical examination of dehydrated children is described, and guidelines are given for intravenous fluid treatment. Furthermore, the article reviews modern research on the nutritional management of gastroenteritis. The incidence of secondary lactose intolerance has been dramatically reduced in recent decades, and this necessitates some changes in our traditional nutritional therapy. Rest of the bowels, and removal of lactose from the diet are seldom justified.

Autonomous hyperparathyroidism in X-linked hypophosphataemia.

Four patients with familial hypophosphataemic rickets developed significant hypercalcaemia which persisted after discontinuation of vitamin D therapy. They had increased PTH levels and were operated for hyperparathyroidism at the ages of 18, 20, 24 and 45 years, respectively. Three of the patients had previously received phosphate treatment and one patient developed hyperparathyroidism 7 years after treatment with calcitriol. Histological evaluation revealed different degrees of parathyroid hyperplasia in all patients, with persistently increased PTH and/or calcium levels after surgery. The possibility of autonomous hyperparathyroidism should be evaluated in the follow-up of patients with X-linked hypophosphataemic rickets.

[Glutaric aciduria type I]. / Glutarsyreuri type I.

Glutaric aciduria type I is a congenital metabolic disease caused by an enzymatic defect in the degradation of the amino acids lysine and tryptophane. This article presents five Norwegian patients with this condition. Early clinical features may be similar to those of encephalitis. The further clinical course is dominated by choreoathetosis, hyperkinesis and spasticity. The diagnosis is made by tracing enhanced glutaric acid in the urine. The treatment is a low protein diet containing only small quantities of lysine and tryptophane. Four of our patients underwent a neuropsychological examination. Despite the fact that such patients are difficult to test, our examination indicates that the condition has a greater effect on motor than on cognitive functions.

The clearance of iohexol as a measure of the glomerular filtration rate in children with chronic renal failure.

The plasma clearances of technetium-99m-labelled DTPA ([99Tcm]-DTPA) and the non-ionic contrast medium iohexol were estimated in 11 children with chronic renal failure for determination of the glomerular filtration rate (GFR). Equal values were obtained with the two substances provided plasma sampling was simultaneous, but when plasma was sampled within 3.5 h after injection of iohexol and [99Tcm]-DTPA the GFR was overestimated by more than 50%. For clearance values below 20 ml min-1 1.73 m-2, valid GFR estimates were obtained both from two plasma samples taken 3 h and 24 h after the injection of iohexol and from a single plasma sample taken 24 h after the injection.

A single plasma sample method for estimation of the glomerular filtration rate in infants and children using iohexol, II: Establishment of the optimal plasma sampling time and a comparison with the 99Tcm-DTPA method.

The glomerular filtration rate (GFR) can be determined from the plasma disappearance rate of the non-ionic contrast medium iohexol. A preceding study established the empirical formulae enabling the development of a single plasma sample method for estimation of GFR in infants and children. In the present study the validity of these empirical formulae was confirmed in examinations in 143 patients. The results of the single plasma sample method were similar to those of a standard 99Tcm-DTPA method, and also with those of a two plasma sample iohexol method. Evaluation of the results obtained with plasma sampling 1 h, 2 h, 3 h and 4 h after the injection of the contrast medium showed that the optimal sampling time was about 3 h after the injection.

Pharmacokinetics of intravenous cefetamet and oral cefetamet pivoxil in children.

The pharmacokinetics of cefetamet were determined after intravenous (i.v.) administration of cefetamet and oral administration of cefetamet pivoxil syrup to patients between the ages of 3 and 12 years. The patients were hospitalized for reconstructive urological surgery; to prevent infection, prophylactic i.v. cefetamet was administered on the day of surgery and oral cefetamet pivoxil was administered 2 days later. After i.v. administration, the mean (+/- standard deviation) half-life of cefetamet was 1.97 +/- 0.60 h (n = 18), which was different from the 2.46 +/- 0.33 h reported for nine adults (22 to 68 years old) in a previous study. The average values for the mean residence times were 2.35 +/- 0.94 and 2.83 +/- 0.34 h and the average values for the fraction of the dose eliminated unchanged in the urine were 79.9% +/- 8.99% and 80% +/- 11% in children and adults, respectively. Plots of mean systemic clearance and steady-state volume of distribution versus body weight for the children and comparative adults were linear on log-log coordinates, and the slopes of the plots were 0.661 and 0.880, respectively. These slope values suggested that mean systemic clearance values per unit of body surface area were similar in children and adults and that maintenance doses for children should be the adult maintenance dose multiplied by the child's surface area divided by 1.73 m2. The mean (+/- standard deviation) oral bioavailabilities of cefetamet pivoxil were 49.3% +/- 15.7% in 3- to 7-year-old children who received a 500-mg dose and 37.9% +/- 10.0% in 8- to 12-year-old children who received a 1,000-mg dose. These values were not different from that observed in the adult group after two 500-mg tablets. Likewise, the peak concentration of cefetamet in plasma and its time of occurrence in children were in line with the values which have been observed for adults.

Glomerular filtration rate estimated by X-ray fluorescence technique in children: comparison between the plasma disappearance of 99Tcm-DTPA and iohexol after urography.

The total plasma disappearance of the non-ionic contrast medium iohexol was determined by X-ray fluorescence technique following intravenous urography in 10 children aged between 2 and 13 years. For comparison the plasma disappearance of 99Tcm-DTPA was estimated both 2 days before and simultaneously with the iohexol study. High correlations between the three sets of data were found and no change in the glomerular filtration rate was detected following injection of contrast medium. It was also found that reliable estimates of the glomerular filtration rate can be obtained from two plasma samples of 1 ml each, taken 3 h and 4 h after the injection of the contrast medium.

Influence of urography on renal function in children. A double blind study with metrizoate and iohexol.

Thirty-two children were given either metrizoate or iohexol for urography in a double blind study. Mild to moderate adverse reactions were observed in all patients receiving metrizoate (15/15) and in 4 receiving iohexol (4/17). Alkaline phosphatase in urine was significantly increased 4 hours after the injection of both media, but had returned to pre-injection levels 16 hours later. The excretion of beta 2-microglobulin and albumin was not altered. In 9 children in the metrizoate group and 11 in the iohexol group the glomerular filtration rate (GFR) was determined before urography by the single injection 99Tcm-DTPA-technique and 3 to 4 hours after urography by measuring the plasma disappearance of the contrast medium with the x-ray fluorescence technique. No reduction of GFR was observed.

Dental features in congenital persistent renal tubular acidosis of proximal type.

The dental features in a hitherto unknown type of renal tubular acidosis (capillary blood pH 7.07-7.15) of proximal type are reported. The patient presented agenesis of three second premolars, delayed development and eruption of permanent teeth, delayed shedding of the primary dentition and severe enamel hypoplasia of the permanent teeth. Apart from exceptionally thin enamel, histologic, fluorescent and polarization microscopic and microradiographic investigation of three primary teeth did not reveal unusual findings. The changes are most probably due to a generalized, acidosis-induced defect in several highly differentiated ectodermal tissues.

Liver enzyme ratios in neonatal liver disease.

The gamma-GT/ASAT (aspartate aminotransferase) and gamma-GT/ALAT (alanine aminotransferase) ratios were examined in 6 children with neonatal hepatitis (NH), 14 children with extrahepatic biliary atresia (EHBA), and 8 children with intrahepatic cholestasis (IHC) (of which 3 with the Aagenaes syndrome). A ratio above 1 is suggestive of EHBA. Both ratios differentiate very well between NH and EHBA, but there is considerable overlap in the enzyme ratios between the EHBA and the IHC groups. Gamma-GT/transaminase ratios may prove to be a useful indicator in the diagnostic work-up of children with liver and biliary tract disease, allowing for early surgery in patients with EHBA, and with a low risk of subjecting NH patients to unnecessary surgery. In our cases the gamma-GT/ALAT ratio separated better between EHBA and IHC than the gamma-GT/ASAT ratio. Furthermore, the case histories made EHBA seem unlikely in two out of three IHC patients with a gamma-GT/ALAT ratio above 1.

Imerslund-Gräsbeck anemia. A long-term follow-up study.

A follow-up study has been performed on 14 patients, now aged 6-46 years, with Imerslund-Gräsbeck anemia (congenital, hereditary selective malasorption of vitamin B12). On intramuscular vitamin B12 therapy, the patients are clinically and hematologically normal. Those who had constant proteinuria in childhood continue to excrete protein in the urine. Our patients excrete an average of 750 mg of protein per 24 hours (range 13-1460 mg). The proteinuria is predominantly of glomerular origin, but some is also of tubular origin. Renal biopsies of the two oldest patients were normal on light microscopy. Electron microscopy revealed moderate signs of chronic glomerulopathy of mesangioproliferative type in both patients. The renal lesions do not seem to be progressive.

Hereditary renal adysplasia.

A family with unilateral renal "agenesis" through 3 generations is presented. In two of the members, a small tissue bud with a ureteric remnant was observed, compatible with the syndrome of hereditary renal adysplasia. Small kidneys and small compensatory renal hypertrophy appear to be characteristic in this family.

Renal biopsies in cystic fibrosis.

Pharmacokinetic studies on antibiotics as well as other studies on renal function indicate renal malfunction in cystic fibrosis. Renal biopsies from two children, and post mortem examination from one child, with this disorder were normal by light microscopy and close to normal by immunofluorescence examination and transmission and scanning electron microscopy, even in cases with advanced disease and marked renal malfunction. Renal biopsy interpretation thus seems to be of little value in the evaluation of renal malfunction in this disorder, and this suggests that the functional abnormalities are caused by metabolic/biochemical defects.

Prostaglandin synthetase inhibitors in the treatment of nephrogenic diabetes insipidus.

Two boys with classical NDI have been treated with prostaglandin synthetase inhibitors. A boy, 7 years old, was treated with low solute-load diet and diuretics from his first year of life. His main complaint was nocturnal enuresis. He responded within one day to indomethacin 25 mg twice daily, and the urine volume was reduced from 4 1/2--6 litre/day to 2 1/2--3 litre/day. There is almost no enuresis. A boy, 7 months old, had a basal daily urine volume of 1.6--1.8 litre. A low solute-load diet and diuretics reduced urine volume to 1 litre, but he still needed gastric tube feeding. With the addition of acetylsalicylic acid, 75 mg three times daily, the urine volume was reduced to 600 ml, and he needed no more tube feeding. Both boys are doing well on the above-mentioned regimens, and no side effects have been observed after 1 year of treatment.