Assessment of risk factors for osteoporosis and fractures in children with meningomyelocele.

PURPOSE: Our objective was to assess bone and muscular mass in children with meningomyelocele (MMC), and to analyze risk factors for osteoporosis and fractures based on densitometric examination. MATERIAL AND METHODS: The study group included 30 patients (15 girls and 15 boys) with MMC, aged 6-17 years, treated in the Department of Pediatric Rehabilitation, University Hospital. Physiotherapeutic assessment and laboratory tests (serum parathormone, alkaline phosphatase levels, calcium, and phosphate levels, and urine calcium levels) were performed. Densitometry was measured by dual energy X-ray absorptiometry using a Lunar DPX-L apparatus. Lean mass (fat-free tissue content) and fat mass (% fat content) was evaluated. RESULTS: Femur fractures were the most common 12/30 (40%); 5/30 (17%) of the children with MMC had multiple fractures. The incidence of fractures correlated significantly with BMI and body fat content (p = 0.03) Children with MMC and fractures had a tendency toward higher BMI, despite the same absolute value of body mass, compared to those without fractures. Body fat levels were higher in MMC patients with fractures than in those without fractures (BMI R = 0.393, p = 0.03). Children with MMC and fractures had significantly higher 24 h calcuria values, despite normal renal function indices (p = 0.03). CONCLUSIONS: Low-energetic fractures in MMC children may result from metabolic disturbances that are a consequence of excessive renal calcium loss or excessive fatty tissue content.

Fractures during growth: potential role of a milk-free diet.

UNLABELLED: Dietary calcium deficiency may increase fracture risk. In girls, 29.4% of fracture cases and 11.8% of controls without fracture had a history of milk-free diet. The odds ratio (OR) for fracture with a milk-free diet in girls was 4.6, p < 0.01. In boys, 23% of cases and 19% of controls had a history of a milk-free diet; OR = 1.3, NS). A milk-free diet due to cow's milk allergy is associated with increased fracture risk in girls. INTRODUCTION: An intake of calcium below the reference daily intake (RDI) of 800-1200 mg/day during growth is thought to increase fracture risk even though convincing evidence for this view is scarce. The paucity of evidence may be partly due to many trial participants being calcium replete. Children and adolescents with cow's milk allergy (CMA) avoid milk and have a calcium intake below the RDI. The aim of this study was to examine the association between consumption of a milk-free diet and fracture risk. METHODS: In this case-control study conducted in Poland, 57 boys and 34 girls aged 2.5-20 years with fractures (cases) were randomly matched by age and sex with 171 boys and 102 girls without fractures (controls). Weight and height were examined using standard methods. Bone mineral density (BMD) and body composition were measured using dual-energy X-ray absorptiometry. Conditional logistic regression and Bayesian analyses were used to determine the proportion of the fracture risk attributable to a milk-free diet. RESULTS: In girls, 29.4% of cases and 11.8% of controls had a history of milk-free diet producing an odds ratio (OR) for fracture associated with a milk-free diet of 4.6 (95% confidence interval [CI]: 1.4-15.5, p < 0.01). In boys, 23% of cases and 19% of controls had a history of a milk-free diet; OR = 1.3 (95% CI: 0.6-2.7, NS). If the prevalence of CMA in the population is 5%, only 6.7% of the fractures occurring are attributable to CMA and the associated nutritional deficit. CONCLUSIONS: Cow's milk allergy is associated with increased fracture risk in girls. Whether this association is due to the illness, calcium deficit or a deficit in other milk nutrients is uncertain. These data suggest that the contribution of milk-free diet to fracture liability among children and adolescents is modest.

Plasma fibrinogen concentration in pediatric patients treated with an elimination diet based on soy proteins and casein hydrolyzate.

PURPOSE: Fibrinogen is one of the most discussed new risk factors of atherosclerosis. The aim of the study was to assess the relationship between fibrinogen concentration and classic risk markers of atherosclerosis in a group of children aged from 2 to 6 with or without a family history of circulatory system diseases (FHCAD) (American Academy of Pediatrics--AAP criteria). The study also considered the impact of allergies/food intolerance treatment with elimination diets on the concentration of atherosclerosis markers specially fibrinogen. INCLUSION CRITERIA: a) family history of early occurrence of circulatory system diseases (FHCAD+) according to AAP standards; b) the type and duration of elimination diet continued in infancy and early childhood. 134 of 388 children were included in the investigation. RESULTS: The analysis of data relating to the so-called classic biochemical risk factors of atherosclerosis (total cholesterol--TC, HDL, LDL, triglycerides, glucose) did not reveal any differences between the tested groups. It was found that in the FHCAD+ group the concentration of fibrinogen was statistically higher than in the group with a negative family history. It was discovered that the type of elimination diet had no effect on fibrinogen level in the FHCAD+ group. In the group of children with negative family history the concentration of fibrinogen was statistically lower in the group on casein hydrolysate than in children treated with soy formula. CONCLUSIONS: The initial interview in pediatrics should include information on the patient's family history of atherosclerosis. In case of a positive family history, fibrinogen, as one of atherosclerosis risk factors, should be monitored.

The duration of breastfeeding and attention deficit hyperactivity disorder.

PURPOSE: To examine whether duration of the breastfeeding is associated with the symptoms of attention deficit hyperactivity disorder in children. MATERIAL AND METHODS: A total of 100 children aged 4-11 years were divided into two groups: 60 children with ADHD symptoms (based on ICD-10) and 40 subjects of normal control grup. The structured interview and the retrospective questionnaire (including the items: number of pregnancy, pregnancy course, gestational age, status of newborn, birth weight, duration of breastfeeding: <3 months; 3-6 months; 6-12 months; >12 months) were used during the study of the both examined groups to indicate the risk factors of development. RESULTS: No significant differences in the percentages of duration of pregnancy, pregnancy complications, delivery complications, condition of the newborn, and birth weight were found between the two groups. The mean of the duration of breastfeeding for group with ADHD was 0.45 year: 5 months and 9 days (median 0.25 year: 3 months). The mean of the duration of breastfeeding of control group was 0.55 year: 6 months and 18 days (median 0.46 year: 5 months) and was significantly greater than that of group with ADHD (p<0.04). The 36 (60%) children with ADHD were breast fed less than 3 months. For comparison 13 (32.5%) controls were breast fed less than 3 months. Significant differences were found among the two children groups (p < 0.05). CONCLUSIONS: The short duration of breastfeeding as environmental factor may be considered a risk factor of ADHD symptoms. However, the further studies are nedeed.

ATM gene founder haplotypes and associated mutations in Polish families with ataxia-telangiectasia.

Ataxia-telangiectasia (A-T) is an early onset autosomal recessive ataxia associated with characteristic chromosomal aberrations, cell cycle checkpoint defects, cancer susceptibility, and sensitivity to ionizing radiation. We utilized the protein truncation test (PTT), and single strand conformation polymorphism (SSCP) on cDNA, as well as denaturing high performance liquid chromatography (dHPLC) on genomic DNA (gDNA) to screen for mutations in 24 Polish A-T families. Twenty-six distinct Short Tandem Repeat (STR) haplotypes were identified. Three founder mutations accounted for 58% of the alleles. Three-quarters of the families had at least one recurring (shared) mutation, which was somewhat surprising given the low frequency of consanguinity in Poland. STR haplotyping greatly improved the efficiency of mutation detection. We identified 44 of the expected 48 mutations (92%): sixty-nine percent were nonsense mutations, 23% caused aberrant splicing, and 5% were missense mutations. Four mutations have not been previously described. Two of the Polish mutations have been observed previously in Amish and Mennonite A-T patients; this is compatible with historical records. Shared mutations shared the same Single Nucleotide Polymorphism (SNP) and STR haplotypes, indicating common ancestries. The Mennonite mutation, 5932 G>T, is common in Russian A-T families, and the STR haplovariants are the same in both Poland and Russia. Attempts to correlate phenotypes with genotypes were inconclusive due to the limited numbers of patients with identical mutations.

The incidence of some civilization diseases in families of children with food allergy/intolerance.

PURPOSE: The aim of the study was to evaluate the incidence of chosen civilization diseases in families of children with food allergy/intolerance. We also wanted to indicate the need for developing and implementing activities preventing these diseases among children. MATERIAL AND METHODS: On the basis of information from questionnaires, two groups of children were distinguished: a group of 80 children suffering from food allergy/intolerance on elimination diet (GR1) and a group of 67 healthy children (GR2) on regular diet. In GR1, the elimination diet with soya bean preparations or casein hydrolysates was introduced before the age of 6 months and continued for at least 12 months. A high risk of hypercholesterolemia according to extended American Academy of Pediatrics criteria including hypertension, diabetes and obesity was determined for children in both groups. RESULTS: The research showed that 31.25% of children examined according to AAP criteria and 46.25% according to extended criteria had a positive family history of premature diseases of the circulatory system. The study proved that hypertension was the most frequent cause of morbidity in families of children from a high risk group and it was found in 67.7% of families with children on elimination diet and with a positive family history and in 78.7% of families with children from GR2 with a positive family history. Obesity, coronary heart disease, hypercholesterolemia, atherosclerosis and diabetes were listed consecutively. CONCLUSIONS: Once a positive family history of cardiovascular diseases is discovered, systematic education promoting health in a family and complex evaluation of physical and psychomotor development of the children should follow. Arterial blood pressure and lipid profile in serum ought to be monitored to eliminate risk factors of these diseases for children.

[The assessment of nutritional status in children with chronic hepatitis B treated with interferon alpha]. / Ocena stanu odzywienia dzieci z przewleklym zapaleniem watroby typu B podczas terapii interferonem alpha.

The aim of the study was an evaluation of nutrition status in children with chronic hepatitis B during IFN-alpha therapy. Observation was carried out in a group of 40 children in the age range of 1-14 years with clinically, diagnosed chronic hepatitis B. Respectful of age 2 subgroups were distinguished. I: 27 children up to 4 years old, II: 13 children in the age range of 4-14 years. The nutritional status was evaluated by anthropometric measurement (body mass, body length/height). Moreover Cole index was estimated at the beginning and completion of the treatment and in the time of the highest weight loss. None of the examined children has body mass deficiency before IFN-alpha treatment: Cole index was in normal range as well. Lack of appetite, connected with IFN-alpha administration, was observed in 42.5 children during therapy. Weight loss was observed in 65% of the examined children especially in the first 2 months of therapy. Return to the initial body mass value was observed within 3 months after IFN-alpha completion in 77% children; but in 2 children only 12 months after IFN-alpha therapy termination. 12 months after IFN-alpha completion Cole index lower than initial was observed in 42.5% of the examined children in spite of return of body mass to initial values. There was no observed unfavourable influence of IFN treatment on body height. 20-weeks IFN-alpha therapy in children with chronic hepatitis B often causes lack of appetite, which may lead to reversible disturbances in nutritional status. Correct dietetic proceeding is necessary to decrease the frequency and intensity of disturbances in nutritional status in this group of children.

Application of derivative spectrophotometry for determination of coenzyme Q10 in pharmaceuticals and plasma.

The use of derivative spectrophotometry is proposed in this work for determination of coenzyme Q10 in formulations and in human plasma. The spectrophotometric procedure is simpler and less expensive than chromatographic techniques commonly used for the analysis of coenzyme. The active compound can be determined in the range 0.25-10 ppm for standard solutions and pharmaceuticals and 0.05-1.5 ppm in plasma. The proposed method was applied for coenzyme determination in real samples. The results agree well with declared value and with these obtained by HPLC.

[Densitometric evaluation of mineral deposits in children and young people with food sensitivity who were treated by diet therapy]. / Densytometryczna ocena zasobów mineralnych u dzieci i mlodziezy z nadwrazliwoscia pokarmowa leczonych dietetycznie.

Bone mineral content (BMC) and bone mineral density (BMD) of the whole skeleton were measured with dual energy x-ray absorptiometry (DEXA) using DPX-L bone densitometer (Lunar) in 240 caucasian subjects with food hypersensitivity, aged 2-18 years, fed milk-free diet (mean duration: 3.6 yrs). DEXA results were compared to the age and sex-matched reference population (n = 473) presenting normal consumption of milk and dairy products, Breast-feeding duration, social conditions and general physical activity did not differ in these groups. All subjects ranged between 3rd and 97th percentile for body weight and height and their nutritional status was similar in both groups, as assessed by means of anthropometric methods (BMI, skinfolds, midarm circumference). Mean values of total BMC and total BMD did not show significant differences between two groups of children, regarding to diet application. Although dietary calcium intake has a significant positive influence on the bone mineral content, our cross-sectional study suggests that the properly applied and controlled elimination diet should not disturb bone mass accumulation in children and adolescents with food allergy.

Assessment of bone density in children with Scheuermann's disease.

Twenty four children with Scheuermann's disease (11 girls and 13 boys) aged 9-18 years measured for bone mineral density. The total skeleton (TB BMD) and lumbar spine (L2-L4 BMD) mineral density were investigated by dual energy X-ray absorptiometry (DEXA). In nine patients with Scheuermann's disease and backache we found lower levels of TB BMD and L2-L4 BMD in comparison with reference population of Lunar database. Osteopenia in these children may be caused by decreased physical activity due to vertebral pain.

Auxological evaluation of children with cow's milk protein intolerance treated an elimination diet.

A somatic development assesment, including the nutritive condition at the moment of diagnosis and after at least a year's dietary treatment was carried out in a group of 55 children with primary cow's milk protein intolerance. The influence of the applied elimination diet and/or anti-allergic supporting treatment on the clinical picture was evaluated as well as calcium--phosphorus--magnesium balance parameters.