Evaluation of plasma 3,4-dihydroxyphenylacetic acid (DOPAC) and plasma 3,4-dihydroxyphenylalanine (DOPA) as tumor markers in children with neuroblastoma.

Catecholamines and their metabolites are important in the diagnosis of neuroblastoma (NB). Plasma (p-) levels of 3,4-dihydroxyphenylalanine (DOPA) are increased in most NB, probably reflecting decreased DOPA decarboxylase activity. Urine (u-) homovanillic acid (HVA), a DOPA and dopamine (DA) metabolite. is also increased in most NB. DOPAC (3,4-dihydroxyphenylacetic acid) is an important metabolite of DA in tissues with monoamine oxidase (MAO) activity. Because MAO is expressed in NB tumor cells, we studied the importance of measuring p-DOPAC and p-DOPA as compared to u-HVA and u-vanillylmandelic acid (VMA) in the diagnosis and follow-up of NB. DOPAC, DOPA, dopamine, noradrenaline, adrenaline, VMA and HVA were measured by reverse-phase HPLC with electrochemical detection in 106 children (28 with NB (13 newly diagnosed), 25 with other solid tumors, 28 hospitalized for nonneoplastic diseases, and 25 healthy children). P-DOPAC or p-DOPA concentrations were above the upper normal range in 92% of untreated NB patients, as were u-HVA or u-VMA levels. None of these tumor markers was correlated to tumor stage or survival. P-DOPA but not p-DOPAC was correlated to age in NB children. Increased values of p-DOPAC and p-DOPA were found in one patient surviving NB for 10 years. Plasma DOPAC concentrations were decreased in children hospitalized for non-NB diseases, probably reflecting reduced food intake. Plasma analyses of DOPA and DOPAC seem to be useful alternatives in the diagnosis and follow-up of NB if urine sampling is to be avoided. Plasma DOPAC may be an index of nutritional status in various diseases.

Methaemoglobinaemia among neonates in a neonatal intensive care unit.

After detection of a few clinical cases of methaemoglobinaemia (methb) in our NICU, a prospective clinical study was undertaken to determine the extent of the problem and to identify the causes. Consequently, during the following 8 months all haemoglobin tests included simultaneous measurements of methb on an OSM 3 hemoximeter (Radiometer): 8% (n = 33) of 415 neonates were found to be methb positive (defined as > or = 6% methb). Mean methb was 19% (range 6.5-45.5%). Maximum methb concentrations were found on day 4-31 postpartum (mean 12 days) and the number of days with a positive methb sample ranged from 1 to 18 days (mean 6 days). About 40% of the neonates born at 25-30 weeks of gestation and 60% with a birth weight < 1000 g were methb positive. Also, there was a negative correlation between the size of the methb positive concentration and gestational age (r = -0.38, p = 0.02). Measurements of C-reactive protein and leucocytes, NADH reductase, pH, Cl, nitrate and nitrite were carried out in methb positive patients. The tests were repeated 1 week after cessation of methb. The only significant difference was an increase in NADH reductase at the second measurement. Likewise, a wide range of clinical parameters were registered and they occurred with a higher frequency among the methb positive patients when compared with a methb negative control group matched with regard to gestational age and the closest possible birth weight. The mean birth weight of methb positive patients was 1170 g and that of negative controls 1380 g (p < 0.006).(ABSTRACT TRUNCATED AT 250 WORDS)

[Adrenogenital syndrome--diagnosis, treatment and therapeutic control]. / Adrenogenitalt syndrom--diagnostik, behandling og behandlingskontrol.

Congenital adrenal hyperplasia is a rare, genetic disorder. In Denmark about 90 cases have been diagnosed over the last 20 years. It is potentially life-threatening in early childhood, and must be intensively controlled in order to achieve a normal growth, pubertal maturation and fertility. This article reviews the pathogenesis and genetic and clinical characteristics underlying congenital adrenal hyperplasia, and outlines pre- and postnatal approaches to the diagnosis and monitoring, with special emphasis on 21-hydroxylase defect.

[Acute lymphoblastic leukemia in children. A retrospective study: 1970-1991]. / Akut lymfoblastaer leukaemi hos børn. En retrospektiv opgørelse: 1970-1991.

From January 1970 through December 1991, 94 girls and 130 boys with a median age of 4.8 years were diagnosed with non-B cell acute lymphoblastic leukaemia (ALL) at the University Hospital, Rigshospitalet. Intensive risk-group adapted therapy based on age and white-cell count (WBC) at diagnosis, the presence of a mediastinal mass, central nervous system (CNS) or testicular leukaemia, T-cell disease, and certain cytogenetic translocations have been used since July 1981. Ninety-seven percent of all patients achieved complete remission (all patients diagnosed since July 1986). Ninety patients relapsed, all within five years from diagnosis: 59 in bone-marrow (BM), 20 in CNS (no BM-involvement), nine in testes (all isolated), one in the eye, and one in a lymph node. The five-year event-free survival increased from 0.20 to 0.72 from the first to the last five-year period (p < 0.0001). Age and WBC at diagnosis were of prognostic significance during the period 1970-1981, but not during the last 10-year period, when risk-adapted therapy was applied. Following a relapse, patients with an isolated testicular focus had the best five-year survival as compared to patients with CNS- and/or BM-involvement (1.00 vs 0.19; p = 0.003). Patients relapsing following cessation of therapy had a better prognosis than did patients relapsing on therapy (five-year survival 0.58 vs 0.17; p = 0.002). Identification of new risk factors, more individualized therapy and monitoring of minimal residual disease is expected to have increasing influence on the management of children with ALL.

Management of prelabor rupture of membranes at term. A randomized study.

OBJECTIVE: To compare the rate of obstetric interventions, length of labor, and maternal morbidity in pregnancies with prelabor rupture of membranes at term after either early or late induction of labor in both primiparous and pluriparous women. DESIGN: Prospective, randomized study. SUBJECTS: 362 women with singleton pregnancies, cephalic presentations, gestational age of 36 completed weeks or more were allocated at random to induction with oxytocin either 6 hours after PROM (n = 62) (early) or 24 hours (n = 62) (late). Those eligible, but not participating in the study, totalled 238 women. MAIN OBSTETRIC MEASURES: Time of spontaneous labor in the late induction group, length of labor, obstetric intervention rate, maternal morbidity, and the degree of histologic chorioamnionitis. RESULTS: The length of labor was longer in the late induction group than in the early induction group in both primiparous and pluriparous (p < 0.05). There were no overall differences in the rate of obstetric interventions or maternal morbidity, but there were marked differences between primiparous and pluriparous women. Increasing time span between the period from rupture of membranes to delivery increased the degree of histologic chorioamnionitis. CONCLUSION: If a woman wants a short labor, she will benefit from early induction. We did not find statistical differences in the rate of obstetric intervention or in the maternal morbidity, but there was a tendency towards adverse effects of late induction.

[Diphtheria in Denmark 1956-1989. Occurrence of Corynebacterium diphtheriae and other diphtheria toxigenic bacteria]. / Difteri i Danmark 1956-1989. Forekomsten af Corynebacterium diphtheriae og andre difteritoksigene bakterier.

The public immunization program against diphtheria, established in 1941, has almost eradicated the disease in Denmark, and 1956 became the first year without any notified cases. Since then, toxigenic strains have only been isolated five times--three cases of clinical diphtheria due to Corynebacterium diphtheriae biovar. mitis and two cases of tonsillitis/pharyngitis due to Corynebacterium ulcerans. The source of the infection was not identified in any of the cases. The first case of diphtheria in 1968 was imported from abroad. The following two cases in 1983 and 1985 were due to strains of the same phage type and peptide profile as the strains isolated during the epidemic in Sweden in 1984-1986. This indicates that the Danish cases and the Swedish epidemic derived from the same source. The diphtheria immunity of the Danish population is decreasing, and the level of protection is approaching the Swedish level. The impact is that a situation like that in Sweden may be anticipated with diphtheria epidemic in the lowest socio-economical groups--the skid row dwellers, alcoholics and drug abusers--if the immunization program against diphtheria is not intensified.

Captopril treatment in Bartter's syndrome.

A 13-year-old girl presented with lassitude, polyuria and hypokalemia. Plasma renin concentration and urinary prostaglandin excretion were elevated, whereas plasma aldosterone concentration, urinary aldosterone excretion and blood pressure were normal. A diagnosis of Bartter's syndrome was made. The result of treatment with oral potassium was unsatisfactory. Treatment with acetylsalicylic acid had some effect, but an allergic reaction rendered withdrawal necessary. Treatment with the angiotensin converting enzyme inhibitor captopril and oral potassium led to clinical and biochemical improvement.

Evaluation of renal function and morphology in children by 99mTc-DTPA gamma camera renography.

The purpose of the present investigation was to evaluate the use of 99mTc-DTPA (diethylenetriaminepenta-acetate) gamma camera renography for the study of kidney function as well as morphology. Sixty-nine children with urinary tract infection or congenital hydronephrosis aged 6 days to 13.6 years were studied. A method for determination of the glomerular filtration rate (GFR) from the recorded curves without use of blood or urine samples was tested in 65 consecutive children. From each renogram an uptake index was determined. After a weight/height correction the sum of right and left kidney uptake index correlated with total GFR determined from plasma clearance of 51Cr-EDTA (ethylenediaminetetra-acetate) measured on the same day (r = 0.96). The relative standard error of estimating GFR from the renograms was 10.7% at GFR = 100 ml/min and 14.6% at GFR = 50 ml/min. In a subgroup including 20 children no difference was found in kidney morphology and length studied by renography and intravenous urography, the latter providing more details concerning the urinary tract. Relative kidney length did not correlate with relative renal function determined by renography, emphasizing the difficulty in determination of relative renal function by urography. In conclusion, not only relative but also absolute renal function (GFR) can be determined in children by 99mTc-DTPA renography; at the same time kidney and urinary tract morphology are evaluated. In our hands, 99mTc-DTPA renography has become a useful diagnostic tool, lowering the need for the more cumbersome intravenous urography and 51Cr-EDTA plasma clearance. However, intravenous urography is mandatory for the detailed study of the urinary tract, and use of the 51Cr-EDTA plasma clearance technique is necessary when a very reliable determination of GFR is wanted.