[Glucose-galactose malabsorption. The first reported case in Denmark]. / Glukose-galaktose-malabsorption. Det første beskrevne tilfaelde i Danmark.

The first diagnosed case of glucose-galactose malabsorption (GGM) in Denmark is presented. GGM is an autosomal recessive disorder characterized by neonatal début of severe osmotic diarrhoea. Untreated, GGM is potentially fatal. The disease is chronic and caused by a defect in the Na+/glucose co-transporter, SGLT1, located on the jejunal brush border. Diagnosis is based upon oral glucose tolerance test, stool reducing substances, and may be substantiated by genetic analysis. Treatment consists in eliminating alimentary glucose and galactose. Nurtured on this diet the patient will develop normally.

[Neonatal mortality, morbidity and late sequelae in infants with birth weight under 1.501 gr or gestational age under 31 weeks, primarily admitted to a central hospital]. / Den neonatale mortalitet, morbiditet og senfølger hos børn med fødselsvaegt mindre end 1.501 g eller svangerskabslaengde mindre end fulde 31 uger, primaert indlagt på et centralsygehus.

The purpose of the study was during a three year period from 1990 to 1992 to describe the neonatal mortality, morbidity and the late complications of 75 very low birth weight infants primarily treated in the neonatal department of a County Hospital. The study was performed retrospectively. The mean birth weight was 1292 g in (range 755-2046 g) and the mean gestational age 29.9 weeks (range 25-37 weeks). The primary choice of treatment was nasal continuous positive airway pressure (nasal-CPAP) and "minimal handling" regime. When needed surfactant administration and/or mechanical ventilation was used. Twenty neonates (27%) received mechanical ventilation, and 14 (19%) were treated with surfactant (Curosurf). The mortality (8%) (95% confidence limits 2.9%-16.6%) and morbidity is low. Late complications such as cerebral palsy or mental retardation were seen in eight infants (12.1%) (95% confidence limits 5.4-22.5%). The results of our treatment seem acceptable, and confirm an increasing survival among very low birth weight infants.

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)

[Pseudotumor cerebri caused by Lyme borreliosis]. / Pseudotumor cerebri forårsaget af Lyme-borreliose.

Lyme borreliosis may cause a wide range of clinical symptoms. The two previously described paediatric cases of Lyme borreliosis with symptoms of pseudotumor cerebri were associated with characteristic cutaneous symptoms. A case of seven year-old girl with six weeks of headache, vomiting nausea and fatigue as the only symptoms was shown to suffer from pseudotumor cerebri with papilloedema and increased intracranial pressure caused Borrelia burgdorferi. Specific treatment with intravenous penicillin and later intravenous ceftriaxone (due to development of penicillin allergy) for a total of ten days caused the symptoms to gradually disappear over following three to four weeks. Hence, even if pseudotumor cerebri is the only symptom, it is recommended that testing for Lyme borreliosis should be carried out.

Lignocaine gel used for lubrication of intranasal and endotracheal tubes in premature neonates.

In this study, we have measured the plasma concentration of lignocaine and its metabolite, monoethylglycinxylidin, in 19 premature neonates (gestational age < or = 33 weeks) when lignocaine gel was used for lubrication of an intranasal tube (during continuous positive airway pressure treatment) or an endotracheal tube (for intubation). We did not find any correlation between plasma concentration of lignocaine or monoethylglycinxylidin and weight of the infant (range 795-2530 g). None of the neonates had toxic levels of lignocaine. One neonate had an exceptionally high but not toxic plasma level of monoethylglycinxylidin. However, this neonate had been treated for severe seizures with an iv infusion of lignocaine up to 13 h before the study. In conclusion, we found it safe to use moderate amounts of lignocaine (i.e. 0.3 ml/kg of lignocaine gel 20 mg/ml) for lubricating both intranasal and endotracheal tubes.

The role of Mycobacteria Other Than Tuberculosis (MOTT) in patients with cystic fibrosis.

The purpose of this study was to estimate the frequency of and evaluate the clinical impact of pulmonary mycobacterial infections among cystic fibrosis (CF) patients. 185 CF patients aged 2.2-38.5 years were screened by sputum samples and by intracutaneous skin tests against tuberculin and sensitins produced from Mycobacterium chelonae subsp. abscessus, M. avium, M. intracellulare and M. scrofulaceum (the MAIS complex). The skin tests towards the sensitins in BCG-vaccinated patients (n = 60) were significantly influenced by the vaccination. 26 of the remaining 125 non-vaccinated patients had > or = 1 positive skin test (95% confidence limits 15-29%). The majority reacted against the MAIS complex. However, the reactions were similar to those of healthy siblings and an age-matched control group. Moreover, the lung function, growth and HbA1c were similar among skin test positive and negative patients. Three patients had repeated positive sputum cultures, the point prevalence being 1.6% (M. intracellulare, n = 2 and M. chelonae subsp. abscessus, n = 1). During the subsequent 4 years, 4 additional patients with M. chelonae subsp. abscessus were identified. Based on clinical observations, 5 of the infected patients were considered asymptomatic, while 2 might have been symptomatic. In 1 patient, M. chelonae subsp. abscessus disappeared spontaneously. Despite intensive treatment with new antibiotics against Mycobacteria Other Than Tuberculosis (MOTT) in 4 patients, the mycobacteria were not eradicated. In conclusion, MOTT infection was rare and the clinical impact difficult to prove. Treatment should focus on clinical improvement in the individual patient suspected of suffering from significant symptomatic infection. Eradication of the bacteria should not be expected.

[Giardiasis in children with chronic diarrhea. Incidence, growth, clinical symptoms and changes in the small intestine]. / Giardiasis hos børn med kronisk diaré. Incidens, voekst, kliniske symptomer og tyndtarmsforandringer.

During a six-year period, 29 children (aged 0.7-13.5 years, mean 3.3 years) suffering from chronic diarrhoea due to giardiasis were studied. The incidence of this illness was 81 per 1,000,000 per year among children aged 0- < 7 years. According to growth charts, relative height and weight of the patients decreased significantly (both approximately 0.5 SD) from before the onset of diarrhoea to the time of diagnosis and subsequently increased up to the end of catch-up growth. Small intestinal mucosal specimens were studied. Two patients had severe villous atrophy, 8 moderate abnormalities, 6 only slight changes and 13 biopsies were normal. D-xylose or lactose malabsorption was detected in 25% of the patients. The lactose malabsorption was due to hereditary low lactase levels. None of the patients with a Danish ethnic background showed lactose malabsorption. D-xylose absorption and the relative weight loss of the patients correlated with the degree of mucosal damage. Patients with persistent diarrhoea (n = 19) were younger and had a shorter duration of diarrhoeal illness and a more significant weight reduction than those with intermittent diarrhoea (n = 10). However, the age at onset of symptoms was similar in the two groups (medians 1.3 years). Seven patients contracted the disease abroad. They all developed persistent diarrhoea and had a more severe course of the illness than those who acquired the disease in Denmark.

Giardiasis causing chronic diarrhoea in suburban Copenhagen: incidence, physical growth, clinical symptoms and small intestinal abnormality.

During a six-year period, 29 children (aged 0.7-13.5 years, mean 3.3 years) suffering from chronic diarrhoea due to giardiasis were studied. The incidence of this illness was 81 per 1,000,000 children aged 0- < 7 years per year. According to growth charts, relative height and weight of the patients decreased significantly (approximately 0.5 SD) from before the onset of diarrhoea to the time of diagnosis and subsequently increased up to the end of catch-up growth. Small intestinal mucosal specimens were studied. Two patients had severe villous atrophy, 8 moderate abnormalities, 6 only light changes and 13 biopsies were normal. D-xylose or lactose malabsorption was detected in 25% of the patients. The lactose malabsorption was due to hereditary low lactase levels. None of the patients with a Danish ethnic background showed lactose malabsorption. D-xylose absorption and the relative weight loss of the patients correlated with the degree of mucosal damage. Patients with persistent diarrhoea (n = 19) were younger and had a shorter duration of diarrhoeal illness and a more significant weight reduction than those with intermittent diarrhoea (n = 10). However, the age at onset of symptoms was similar in the two groups (medians 1.3 years). Seven patients contracted the disease abroad. They all developed persistent diarrhoea and had a more severe course of the illness than those who acquired the disease in Denmark.

Candida albicans septicemia in a premature infant successfully treated with oral fluconazole.

A premature male infant, birth-weight 1460 g, was treated successfully for a Candida albicans septicemia with orally administered fluconazole for 20 days. Dosage was 5 mg/kg/day. No side effects were seen. Fluconazole may present a major progress in treatment of invasive C. albicans infections in neonatology.

Giardiasis: haematological status and the absorption of vitamin B12 and folic acid.

The haematological status, as well as the fractional absorptions of folic acid (FAFol) and vitamin B12 (FAB12) were studied in 29 children aged 0.7-13.5 years (mean 3.3 years) with chronic diarrhoea due to giardiasis. Small intestinal biopsies revealed mucosal damage in 20 children; the biopsies of the remaining nine children were normal. At the initial investigation the FAFol and FAB12 values were below normal in approximately one-sixth and one-third of patients, respectively. Bacterial overgrowth of the small intestinal tract did not seem to play a role in FAB12 malabsorption. About one-fifth of patients had mild anaemia. None of the patients showed FAB12 insufficiency and only one patient suffered from folate depletion. At follow-up, FAFol, FAB12, haemoglobin and Erc-folate concentrations increased significantly while P-B12 and P-folate remained unchanged. Iron status, as well as dietary intake of iron, appeared insufficient prior to, as well as after treatment. Serum iron, transferrin saturation and haemoglobin concentrations were lower in patients who had acquired the disease abroad or suffered from persistent diarrhoea.

The role of the exocrine pancreas in early-onset vitamin B12 malabsorption in gluten-challenged celiac children.

The fractional absorption of vitamin B12 (FAB12) was measured by a double-isotope technique specially adapted for children. In six celiac children on a strict gluten-free diet and with a normal small intestinal biopsy, the FAB12 performed in the fasting patient averaged 30% (23-40%). After gluten challenge for a mean of 2 months (range 1-4), when mucosal damage was demonstrated by biopsy, the average fasting FAB12 in these patients decreased to 10% (0-17%) (p less than 0.05). However, when the FAB12 test was repeated by means of stimulation by a B12-free meal 1-3 weeks later, while the patients were still on a diet containing gluten, a significant increase was observed (mean 21%, range 14-27%) (p less than 0.05). In four of the six patients the B12 absorption was further evaluated by repeating the FAB12 test by means of intravenous cholecystokinin (CCK) stimulation (n = 3) or by administration of exocrine pancreas enzyme supplementation (EPES) (n = 2) or cobinamide (n = 1). These tests all showed FAB12 values within the range of the meal-stimulated FAB12. Moreover, in eight gluten-free celiac children with normal biopsies, no difference was found between fasting and meal-stimulated FAB12 values. Therefore, it is likely that the early-onset B12 malabsorption observed in the gluten-challenged celiac child with upper-small-intestinal mucosal damage is in part due to an insufficient stimulation of the exocrine pancreas when using the standard fasting B12 absorption test.(ABSTRACT TRUNCATED AT 250 WORDS)

[Nosocomial virus infections in pediatric departments. Rotavirus and respiratory syncytial virus]. / Nosokomielle virusinfektioner i børneafdelinger. Rotavirus og respiratorisk Syncytialvirus.

Rotavirus and Respiratory Syncytial virus are common causes of acute gastroenteritis and upper and lower respiratory diseases among infants and toddlers. Both viruses occur as epidemics during the winter season. The symptoms may be quite severe and often lead to hospitalisation in paediatric wards i.e. about 2,000 children per year. The viruses frequently give rise to nosocomial infections in the infant/toddler wards about one third of the cases being nosocomial. Recent studies have revealed that both viruses are spread via contaminated hands. The density of virus in faeces and upper respiratory secretions is very high less than one microgram being sufficient for spread of the disease. The traditional precautions (i.e. handwashing and isolation) have failed to reduce the numbers of nosocomial infections to an acceptable level. However, the routine use of gloves have been shown to reduce the spread of RS virus effectively. This method is suggested in paediatric wards during the winter since it presumably will also reduce the spread of Rotavirus.

Retrospective clinical study of hypersensitivity reactions to aztreonam and six other beta-lactam antibiotics in cystic fibrosis patients receiving multiple treatment courses.

A total of 2,793 courses of treatment with seven beta-lactam antibiotics were administered to 121 cystic fibrosis patients chronically infected with Pseudomonas aeruginosa, and the patients were evaluated with respect to clinical hypersensitivity reactions. Seventy-five patients (62%) experienced 125 reactions, for an overall frequency (based on the number of courses) of 4.5%. Immediate reactions occurred in 34 patients (28.1%) during 53 courses (1.9%). The highest rate of reactions involved piperacillin (50.9% of patients), and the lowest rate involved imipenem and aztreonam (4.0% and 6.5% of patients, respectively); intermediate reaction rates were noted for carbenicillin (23.6% of patients), azlocillin (20.8%), cefsulodin (17.1%), and ceftazidime (13.0%). Cross-reactivity did not appear to be a major problem. Reactions to aztreonam seemed to be restricted to a small group of patients with a high propensity for beta-lactam hypersensitivity.

Nitrofurantoin versus trimethoprim prophylaxis in recurrent urinary tract infection in children. A randomized, double-blind study.

The efficiency of nitrofurantoin and trimethoprim prophylaxis in preventing recurrent urinary tract infections (UTI) was compared by means of actuarial percentage recurrence-free curves in a randomized, double blind study in 130 children (126 girls, 4 boys) aged 1 to 14 years (mean 7.5). The children received the antibiotics for 6 months. Nitrofurantoin proved to be the most efficient prophylactic drug in patients with abnormal urography and/or reflux (n = 60) as evaluated by actuarial percentage recurrence-free analysis (p = 0.0025). However, no differences was found in patients without urinary tract abnormalities. Nitrofurantoin prophylaxis altered neither the pattern of resistance nor the bacteriological constellation, while patients receiving trimethoprim prophylaxis had 76% trimethoprim resistant bacteria during prophylaxis, compared with 8% before (p less than 0.0001) and 17% after (p less than 0.0001) prophylaxis. The percentage of recurrences due to E. coli (70-80%) was unaffected by trimethoprim prophylaxis, but the proportion due to trimethoprim resistant E. coli was significantly higher during prophylaxis (65%) than before (6%, p less than 0.0001) and after (11%, p less than 0.001). The percentage of Staphylococcus epidermidis UTI was significantly higher during trimethoprim prophylaxis (27%) than before (2%, p less than 0.0003). Following prophylaxis there was no difference in the actuarial percentage recurrence-free curves of the two regimens. Side effects occurred more frequently in the nitrofurantoin group (37%) than in the trimethoprim group (21%) (p = 0.05). The majority of side effects in the nitrofurantoin group derived from gastrointestinal symptoms. In conclusion, nitrofurantoin is recommended as the first choice prophylactic treatment of children with recurrent UTI and urinary tract abnormalities.

Postenteritis enteropathy in infancy. A prospective study of 10 patients with special reference to growth pattern, long-term outcome and incidence.

Ten patients, who developed postenteritis enteropathy with chronic diarrhoea, failure to thrive and small intestinal mucosal damage after an episode of acute gastroenteritis, were studied prospectively. All patients experienced severe growth retardation. Maximum deviation of height and weight from mean values of age-correlated normal children were -1.7 and -3.1 SD (median values), respectively, as compared to -0.1 and 0 SD before onset of the illness. One child died after 14 months of illness. The long-term outcome was satisfactory for the 9 surviving children. For these children, treatment with an elemental diet (in some cases supplemented initially with parenteral nutrition) and later a lactose-free diet lead to resolution of diarrhoea within periods ranging from 0.5 to 10 months. The phase of catch-up growth lasted from 4 to 36 months, resulting in final height and weight deviating -0.6 and -0.8 SD (median values), respectively, from normal mean values. Attempt to predict duration of diarrhoea or length of catch-up growth phase by means of age, weight before illness, characteristics of small intestinal biopsy or maximum deviation of height or weight were unsuccessful. The incidence of postenteritis enteropathy for children of North European ethnic origin was estimated to be 7.6/1,000,000 children below 7 years of age/year, corresponding to 1.2/1,000 children hospitalized for acute gastroenteritis.

Vitamin B12 and folic acid absorption and hematological status in children with postenteritis enteropathy.

In a group of nine children with postenteritis enteropathy (i.e., persisting small-intestinal mucosal damage and failure-to-thrive after an acute episode of gastroenteritis), absorption capacities for vitamin B12 and folic acid were studied and compared with hematological status in peripheral blood. The fractional absorptions of vitamin B12 (FAB12) and folic acid (FAFol) were determined by means of a double-isotope technique employing a single-stool-sample test. The children were examined when growth retardation was maximal, and examinations were repeated during the late recovery period. In spite of considerable small-intestinal mucosal damage, only the absorption of vitamin B12 was markedly affected, while that of folic acid was almost intact. When growth retardation was maximal, FAB12 was below the normal age-correlated range in half of the children. FAB12 was also severely reduced in all longitudinally observed children when compared with the results obtained during the late recovery period (p less than 0.005). FAFol was below the normal range in one fourth of the children, but the reduction was modest and insignificant when compared with the results of repeated examinations during the late recovery period. A moderate iron deficiency was detected in half the children. High levels of plasma vitamin B12, folic acid, and erythrocyte folate were detected at both early and later examinations, indicating that these parameters were not affected by the reduced absorption capacities. However, if malabsorption and chronic diarrhea are combined with low dietary intake of vitamin B12, as is the case for many children in the Third World, depletion of vitamin B12 stores may result.