Plasma carotenoids in German children and adolescents

Carotenoids are important antioxidants and precursors of vitamin A, but only few studies have been carried out on plasma carotenoid levels in paediatric age groups. Using high-performance liquid chromatography we analysed concentrations of four important carotenoids (α-carotene, β-carotene, cryptoxanthin, lycopene) in 129 healthy German children and adolescents (81 boys, 48 girls; age 1–18 years). For all carotenoids analysed, no significant differences between girls and boys were found. α-Carotene values ranged from 0 μmol/l (below detection limits) to 0.73 μmol/l (0–395 μg/l), β-carotene from 0.09 μmol/l to 2.68 μmol/l (48–1443 μg/l), lycopene from 0 μmol/l to 1.51 μmol/l (0–815 μg/l), and cryptoxanthin from 0 μmol/l to 0.30 μmol/l (0–164 μg/l), respectively. Data analyses according to age groups showed a tendency towards higher levels of α- and β-carotene, and lower levels of lycopene in young children. These differences were significant when children younger than 4 years were compared with those above 8 and 12 years, respectively. The data presented may serve as reference values for studies on children with nutritional disorders and diseases associated with a risk of vitamin deficiencies. Conclusion Carotenoids are important antioxidants and singlet oxygen scavengers. Plasma levels of α-carotene, β-carotene, lycopene and cryptoxanthin were determined in 129 healthy German children and adolescents. Highest values were found for β-carotene and lycopene.     

Thyroid carcinoma in children and adolescents

A clinical and pathological study was undertaken to define the prevalence, clinical presentation and outcome of thyroid carcinoma in children and adolescents. Clinical records from 48 patients under 20 years of age at diagnosis, out of 372 patients with thyroid cancer examined between 1980 and 1994, were retrospectively reviewed. Female/male ratio was 3.8/1. None had a previous positive history of head and neck irradiation. Patients underwent near-total (44 patients) or partial (4 patients) thyroidectomy followed by ¹³¹I ablation of residual thyroid tissue. The mean follow up period was 58.4 months, ranging between 2 and 190 months. Clinically a thyroid mass was present in 41 patients, 28 of whom also showed neck lymph node involvement. Node metastases were present in 50% of the patients and lung metastases in 4.2%. Histological type was papillary in 41, follicular in 6, and medullary in 1 case. Surgical complications were observed in 19 patients (40%). In 3 patients papillary thyroid cancer was associated with chronic lymphocytic thyroiditis. All patients were treated with l-thyroxine suppressive therapy. Recurrence of cancer after surgical and radio-iodine treatment was observed only in one patient 8 months after surgery. Conclusion Our experience demonstrates that thyroid carcinoma in childhood cannot be considered a rare occurrence, since it represents about 13% of all thyroid cancers, and is frequently associated with lymph node but rarely with distant metastases. Nevertheless, the prognosis of thyroid carcinoma in childhood is fairly good. Received: 16 January 1996 / Accepted: 1 July 1996     

The effect of obesity, age, puberty and gender on resting metabolic rate in children and adolescents

During puberty fat-free mass (FFM) and fat mass (FM) change quickly and these changes are influenced by sex and obesity. Since it is not completely known how these changes affect resting metabolic rate (RMR), the aim of the present study was to investigate the effect of body composition, age, sex and pubertal development of postabsorptive RMR in 9.5- to 16.5-year-old obese and non-obese children. Postabsorptive RMR was measured in a sample of 371 pre- and postpubertal children comprising 193 males (116 non-obese and 77 obese) and 178 females (119 non-obese and 59 obese). RMR was assessed by indirect calorimetry using a ventilated hood system for 45 min after an overnight fast. Body composition (FFM and FM) was estimated from skinfold measurements. The mean (± SD) RMR was significantly (P < 0.001) lower in non-obese (males: 5600 ± 972 kJ/24h; females: 5112 ± 632 kJ/24h) than in obese (males: 7223 ± 1220 kJ/24h; females: 6665 ± 1106 kJ/24h) children. This difference became non-significant when RMR was adjusted for body composition (FFM + FM). However, the difference between the genders still remained significant (control male: 6118 ± 507, control female: 5652 ± 507, P < 0.001; obese male: 6256 ± 507, obese female: 5818 ± 507 kJ/24h, P < 0.001). The main determinant of RMR was FFM. In the whole cohort, FFM explained 79.8% of the variation in RMR, followed by age, gender and FM adding further 3.8%, 1.1% and 0.8% to the predictability of RMR, respectively. No significant contribution for study group (obese, non-obese), pubertal stage, or fat distribution was found in the regression for RMR. The adjusted value of RMR (for FFM and FM) slightly, but significantly (P < 0.01) decreased between the age of 10–16 years, demonstrating the important effect of age on RMR. Conclusions The resting metabolic rate of obese and control children is not different when adjusted for body composition. The main determinant of RMR is the fat-free mass, however, age, gender and fat mass are also significant factors. Pubertal development and fat distribution do not influence RMR independently from the changes in body composition. Received: 4 March 1996 / Accepted: 21 August 1996     

Plasma and serum micronutrient concentrations in preschool children

Abstract The plasma concentrations of vitamin A, vitamin E, β-carotene and serum concentrations of zinc, retinol-binding protein and prealbumin were examined for a random cluster sample, stratified by socioeconomic status, of 467 healthy preschool children. Children were aged 9–62 months; 44% were females. The mean plasma values were: vitamin A, 1.29 µmol 1^-1; vitamin E, 18.9 µmol 1^-1; and β-carotene, 0.30 µmol 1^-1. The mean serum values were: zinc, 13.9 µmol 1^-1; retinol-binding protein, 25.5mg 1^-1; and prealbumin, 186.2 mg 1^-1. The mean molar ratio of vitamin A to retinol-binding protein for the study group was 1.10. There were no differences in the mean values of any of the measured micronutrients between the genders. The results of this survey do not indicate that the prevalence of micronutrient deficiency in this preschool population is of public health significance.     

Iron overload and urinary lysosomal enzyme levels in β-thalassaemia major

The urinary levels of the lysosomal enzymes n-acetyl-β-d-glucosaminidase (NAG) (EC 3.2.1.52) and α-mannosidase (EC 3.2.1.24) were evaluated in patients with β-thalassaemia major and normal control subjects. Two groups of patients with different degrees of iron overload, as judged by their serum ferritin levels, were investigated. Renal disease was not present in any of the patients. A statistically significant increase in the levels of NAG was observed in the high ferritin (>3,000 mg/dl) group compared to the low ferritin (<3,000 mg/dl) and the control groups. No difference was observed in the urinary α-mannosidase levels between the groups examined. The finding of increased NAG levels in the patients with the increased iron load suggests that kidney lysosomes are a target of iron toxicity. The different behaviour of the two lysosomal enzymes may reflect the intra- and inter-lysosomal heterogeneity in kidney. Conclusion Iron overload resulted in increased urinary levels of the lysosomal enzyme NAG, which has been proposed as an early marker of kidney damage. Reduction of iron load, achieved by regular desferrioxamine infusion, resulted in normalisation of the urinary enzyme levels. Thus kidney lysosomes appear to be a target and possibly a mediator of iron toxicity in this tissue. Received: 25 September 1996 / Accepted: 25 February 1997     

Helicobacter pylori and hypergastrinemia in children with recurrent abdominal pain

Recurrent abdominal pain (RAP) is a significant problem in the pediatric population, and there has been much recent interest in the role that Helicobacter pylori (Hp) might play in this disorder. In this case control study, the authors aimed to determine whether Hp is an agent responsible for RAP, and to assess fasting gastrin concentrations in children with and without RAP in the Hp-positive and -negative groups. The study was conducted in 42 patients with RAP and 50 healthy children attending routine day-case surgery as a control group, aged 3 to 15 years, over a 12-month period. Of the 42 children with RAP, 30 were seropositive (71.4%) for Hp IgG, and of 50 children in the control group, 32 were seropositive (64%) for Hp IgG (P > 0.05). We found that Hp infection was as high in healthy children as in children with RAP. The mean fasting gastrin levels in 62 Hp-seropositive children (60.4 ng/l) were not different from those in 30 Hp-seronegative children (57.3 ng/l) and those in 42 children with RAP (58.2 ng/l) were also not significantly different from those in 50 healthy children (62.9 ng/l). Thus, no association between childhood Hp infection, hypergastrinemia, and RAP was found in our Turkish population. Accepted: 26 May 1998     

Spontaneous growth in German children and adolescents with genetically confirmed Prader-Willi syndrome

Height and weight in children with Prader-Willi syndrome, diagnosed by standard clinical criteria, follow a specific developmental pattern resulting in early childhood obesity, absent pubertal growth spurt and adolescent short stature. New molecular techniques (methylation analysis, fluorescence in situ hybridization) now allow the unequivocal diagnosis of Prader-Willi syndrome (PWS). We investigated the possibility of a bias in syndrome-specific growth standards based on clinically diagnosed patients by comparing these standards with new standards derived from 100 German patients with molecularly confirmed PWS, none of whom had received a growth-promoting therapy. Height centile curves of the German patients fall in the tall range of standards derived from American patients. This is mainly due to an elevation of the lower centile ranges in both sexes. When the height standards derived from German patients are compared to those of a large multinational cohort of patients, 78% of whom were not confirmed by genetic testing, only minor differences in the height centiles become apparent. The population background therefore does not appear to play a major role for the observed differences. In a marked proportion of patients a decreased sitting height/height ratio is found. This was usually associated with scoliosis. Weight standards from our study group show that after 14 y of age German girls with PWS are heavier than their American counterparts. Standards for the body mass index of German patients of both sexes are increased over normal reference standards (p < 0.0001) and do increase with age (boys: p = 0.0038; girls: p = 0.0004). PWS genotypes or sex had no apparent influence on SDS for height, weight and body mass index. Conclusions: Because of the observed differences to other growth standards, use of the newly constructed centile curves is advocated in German patients with molecularly confirmed PWS to avoid delay in the diagnosis of additional growth-compromising conditions.     

Plasma lipid and apolipoprotein concentrations in full term infants fed formula supplemented with long-chain polyunsaturated fatty acids and cholesterol

Recent data indicate that supplementation of infant formula with ω-3 and ω-6 long-chain polyunsaturated fatty acids might offer developmental benefits for full term infants. We investigated biochemical consequences of feeding formula supplemented with egg lipids to provide long-chain polyunsaturated fatty acids and compared triglyceride, cholesterol, lipoprotein cholesterol (HDL₂-cholesterol, HDL₃-cholesterol, non-HDL-cholesterol) and apolipoprotein A-I, A-II and B concentrations in full term infants fed either conventional formula (n = 10) or a formula supplemented with ω-3 and ω-6 long-chain polyunsaturated fatty acids and cholesterol in amounts similar to those found in mature human milk (n = 12). At the age of 5 days, cholesterol, non-HDL-cholesterol and triglyceride concentrations were significantly higher in infants fed supplemented than in those receiving conventional formula. At the age of 30 days, triglyceride concentrations were significantly higher with supplemented than with conventional formula. Thereafter throughout the study, no significant differences were seen between the two groups. Conclusion Full term infants fed formula supplemented with ω-3 and ω-6 long-chain polyunsaturated fatty acids and cholesterol showed significantly higher plasma cholesterol and triglyceride concentrations than infants receiving conventional formula on day 5 and on days 5 and 30, respectively. Thereafter no appreciable effect of diet on plasma phospholipid, triglyceride, cholesterol, lipoprotein cholesterol and apolipoprotein concentrations were seen. Received: 13 March 1996 / Accepted: 21 October 1996     

Effects of gender,age, and heart rate on QT intervals in children

The objective of this study was to determine if gender, age, and heart rate affect corrected QT intervals in children. Electrocardiograms were obtained from 781 healthy children 10–18 years of age. Corrected QT intervals were significantly (p < 0.0005) greater for girls than for boys in the entire population and for each age group over 14 years. The corrected QT interval varied inversely with age and directly with heart rate. Hence gender, age, and heart rate should be considered when diagnosing long QT syndrome.     

Hemisplenectomy for giant splenic cysts in children

To analyse the clinical features and outcome following surgery of giant splenic cysts in␣children, all patients presenting to a children's hospital over the years 1987–1996 were reviewed. Variables analysed included presenting symptoms, method of diagnosis, operative procedure, and complications. Six patients (aged 8–16 years) presented with abdominal pain or a large abdominal mass. Ultrasound examination demonstrated large (>5 cm) unilocular splenic cysts. Five of the six children underwent hemisplenectomy without complication or blood transfusion. The remaining patient had an infected cyst, for which total splenectomy was necessary because of major intraoperative haemorrhage. Hemisplenectomy is based on accurate dissection of the splenic hilum with clear knowledge of the intracapsular vascular anatomy of the spleen. Preoperative vaccination is recommended in the event that splenectomy is required. This retrospective review concludes that hemisplenectomy for splenic cysts is a safe procedure in children. Accepted: 3 November 1997     

Nephrotic syndrome and Hodgkin disease in children: a report of five cases

 This report documents the occurrence of a nephrotic syndrome in five children with Hodgkin disease. In two cases the nephrotic syndrome predated the diagnosis of lymphoma by 6 months and 12 months respectively , while in the other three, the two disorders occurred simultaneously. The nephrotic syndrome resolved in four cases during effective treatment for active Hodgkin disease, while proteinuria remained unchanged in the fifth case with partial control of the lymphoma. The occurrence of a nephrotic syndrome as a manifestation of active Hodgkin disease suggests that some immunological abnormalities play a role in the pathogenesis of the association. Conclusion The possibility of glomerular dysfunction although rare must be considered and actively looked for in all cases of Hodgkin disease. Similarly, any unusual sign or symptom noted in patients with nephrotic syndrome, particularly receiving or having received immunosuppressants, requires thorough investigation to determine the presence or absence of lymphoma. Received: 5 December 1995 / Accepted: 4 July 1996     

Plasma homocysteine levels in children and adolescents with type 1 diabetes

OBJECTIVE: Hyperhomocysteinemia has been established as a risk factor for cardiovascular disease. The objective was to investigate total plasma homocysteine concentrations in children and adolescents with type 1 diabetes and a control group. METHOD: Twenty-seven children with type 1 diabetes and 27 subjects of an age- and sex-matched control group were recruited. Fasting samples were collected for plasma total homocysteine, serum vitamin B12, folate, and creatinine. RESULTS: Fasting total homocysteine concentrations showed no difference between patients and controls (5.6 +/- 2.9 micromol/L vs 5.7 +/- 2.2 micromol/L; p greater than 0.05). The diabetic patients had significantly higher serum folate than the healthy controls (11.4 +/- 3.3 ng/mL vs 9.4 +/- 4.1 ng/mL; P = 0.02 and higher serum B12 than the control group (282.8 +/- 119 pg/mL vs 228.5 +/- 50.9 pg/mL; P = 0.03). Total plasma homocysteine concentration correlated with age (r = 0.44, P = 0.02), weight (r = 0.56, P = 0.002), body mass index (r = 0.57, P = 0.002), folate (r = -0.48, P = 0.01), and creatinine (r = 0.41, P = 0.03) in diabetic patients. In stepwise multivariate regression model for diabetics, the independent correlates for total plasma homocysteine concentration was folate (P = 0.002). CONCLUSION: We concluded that fasting plasma total homocysteine concentrations were within normal limits in children and adolescents with type 1 diabetes who were without any clinical evidence of microvascular and macrovascular complications.     

Antioxidants may mitigate the deterioration of coronary arteritis in patients with Kawasaki disease unresponsive to high-dose intravenous gamma-globulin

During the early stages of Kawasaki disease, a marked increase in oxygen-free-radicals (OFRs), which are produced by activated polymorphonuclear cells, may induce coronary arteritis. Early use of high-dose intravenous gamma-globulin (IVIG) and aspirin effectively blocked this deteriorating course of coronary arteritis; however, late use of IVIG, even using a high-dose schedule, did not achieve the same efficacy. The causes and reactions to the scenario of IVIG refractoriness have rarely been mentioned in the literature. We present an 11-month-old male infant with Kawasaki disease and deteriorating coronary arteritis owing to late use of IVIG who showed dramatic responsiveness to the addition of α-tocopherol and ascorbic acid. We also discuss the possible mechanism.     

Oxygen consumption in infants and children during heart catheterization

Oxygen consumption was measured in infants, children, and adolescents during diagnostic heart catheterizations. A total of 825 measurements of oxygen consumption (VO₂) was performed in 504 subjects using a semiopen hood system and a paramagnetic oxygen analyzer. In 256 subjects under 3 years of age, body dimensions and heart rate were found to be significant factors for oxygen consumption. The regression equation for both sexes was: VO₂/BSA (ml/min · m²) = 3.42 · height (cm) − 7.83 · weight (kg) + 0.38 · HR − 54.1 (r ²= 0.39, SD = 38.7), where BSA is body surface area and HR is heart rate. VO₂/BSA was significantly lower in infants less than 3 months of age (133 ± 33 ml/min · m²) compared with infants of 3–12 months (171 ± 37 ml/min · m²; p < 0.01). In 272 children aged 3 years and older and adolescents, gender was a significant factor in oxygen consumption together with BSA and HR. The regression line equation for males was VO₂/BSA (ml/min · m²) = 0.79 · HR − 7.4 · BSA(m²) + 108.1 (r ²= 0.45, SD = 34.2). The regression line equation for females is VO₂/BSA (ml/min · m²) = 0.77 · HR − 5.2 · BSA(m²) + 106.8 (r ²= 0.43, SD = 34.4). Hematocrit, systemic oxygen saturation, and blood pressure were not significant factors. The predictive value of nomograms for oxygen consumption is limited because of the large interindividual variations not explained by differences in gender, body size, or simple hemodynamic variables. Preferably, oxygen consumption is measured; but if nomograms for oxygen consumption are used for hemodynamic assessment, the wide confidence intervals should be considered.     

Correlation of Plasma Adrenomedullin to Myocardial Preservation During Open-Heart Surgery

Adrenomedullin (ADM) is a vasoactive peptide with potent dilatory effects. We studied whether perioperative myocardial injury could be altered by the presence of ADM. Blood samples from 19 children with congenital heart disease undergoing surgical repair were collected at six time points: preoperative, on cardiopulmonary bypass (CPB), and 0, 3, 6, and 12 hours after CPB. Blood levels of ADM (pg/ml) and troponin-I (Tn-I; ng/ml), a specific marker of myocardial injury, were measured. Patients were divided into three groups based on their 12-hour Tn-I levels (I, < 10, n= 6; II, 10–25, n= 6; III, >25, n= 7). Preoperative Tn-I levels were within the normal range for all patients. Preoperative ADM levels in group I (with little or no evidence of myocardial injury) were significantly greater than those of either group II or III (242.7 ± 15.4 vs 83.8 ± 18 and 85.2 ± 5.5, respectively; p≤ 0.0001 for each). The 12-hour ADM levels in group I remained significantly lower than preoperative levels (242.7 ± 15.4 vs 197.4 ± 11.6, p≤ 0.03) but higher than in the other groups. In group III, ADM increased at the 12-hour time point (159.2 ± 6.5, p≤ 0.0001 vs baseline). Higher preoperative ADM levels are associated with lower levels of myocardial injury (as assessed by troponin-I release) during congenital heart surgery.     

Adrenocortical function in asthmatic children: low levels of adrenocortical hormones in children with persistent attacks

The existence of adrenal dysfunction in asthmatic patients remains controversial. Assessing adrenal function in these patients is difficult because of varying severity and duration of attacks and large fluctuations in the secretion of adrenocortical hormones. Total and free (unbound) levels of serum cortisol and cortisone were measured in asthmatic children and healthy controls using reversed-phase high performance liquid chromatography. The results were correlated with the severity and duration of asthma attacks. In asthmatic patients in remission, total and free concentrations of cortisol and cortisone did not differ significantly from those in healthy controls. During attacks, levels increased in proportion to the severity of the attack. These values decreased to control levels after 24 h. However, in children with attacks lasting for more than 5 days, values remained significantly decreased with no relation to the severity of the attack. Conclusion During an asthma attack, adrenocortical hormone concentrations increase in proportion to the severity of the attack but subsequently decrease with time. It is speculated that the inability to sustain an elevated cortisol level may lead to the persistent asthma attacks.