M. Vignolo

Growth and development in simple obesity.

It is well known that fat children tend to be taller than their peers and to present a slight acceleration of skeletal and pubertal maturation. To verify this tendency and to examine some of the points that are still controversial, auxological data were studied concerning 303 subjects (141 males and 162 females, aged 6–16 years) affected by simple obesity. Subjects were seen to be taller than average by about 1 SD from 6 to 9 years of age, becoming close to or shorter than average at later ages. Height below the 10th percentile was common in 17% of males and 8% of females, due to hereditary shortness, growth delay or late puberty. Girls had early puberty and menarche; the rate of sexual maturation was variable in boys.

Modified Greulich-Pyle, Tanner-Whitehouse, and Roche-Wainer-Thissen (knee) methods for skeletal age assessment in a group of Italian children and adolescents

Modified Greulich-Pyle (GP), Tanner et al. 2, radius, ulna and short bones (TW2-RUS), TW2-20-bone and Roche-Wainer-Thissen RWT (knee) skeletal age assessments were made in an Italian population sample of 128 males and 93 females aged 4.1–16.9 years. All the scales appear to be well-suited to the Italian population despite minor differences. A very high correlation was found between the assessment of knee skeletal ages by the RWT method and that of the hand-wrist by the GP and TW2 systems in the same subject without sex and age-associated variations.

Assessment of skeletal maturity of the hand-wrist and knee: A comparison among methods.

This study evaluated the influence of height growth and nutritional status on skeletal maturation of the knee and hand‐wrist. Radiographs of 589 subjects (250 girls and 339 boys) from 2 to 15 years were rated according to Greulich‐Pyle, TW‐20 bone and TW‐RUS, RWT knee, and FELS hand‐wrist methods and a method combining FELS and RWT indicators. The subjects were referred to the Genoa University Paediatric Department from 1980 to 1987 for short stature, simple obesity, or acute diseases. Bone age was closer to chronological age using the RWT knee method rather than the hand‐wrist methods, while bone age assessed at the hand‐wrist was closely related to height and BMI. When skeletal maturation was delayed, Greulich‐Pyle, TW‐20‐bone, TW‐RUS, and FELS bone ages tended to be lower than RWT knee estimates. Conversely, if maturation was advanced the hand‐wrist estimates tended to be higher than RWT knee bone ages. The combined estimates are close to FELS bone age values. These findings show true intraindividual variability of skeletal maturity at the hand‐wrist and knee. A certain “laziness” in knee maturation seems to be confirmed. Am. J. Hum. Biol. 12:610–615, 2000.

Ectodermal abnormalities in Kabuki syndrome.

We describe a girl with Niikawa-Kuroki (Kabuki) syndrome (NKS) with conical incisors, hypodontia, hypoplastic nails, and brittle hair. Abnormal teeth are common in NKS and support a hypothesis of autosomal dominant inheritance of the syndrome [Halal et al., 1989; Silengo et al., 1996]. Hair abnormalities have never been investigated in NKS. The ectodermal involvement in NKS could represent an important clue for the understanding of the pathogenesis of this syndrome.

Relationship between body mass index and asthma characteristics in a group of Italian children and adolescents

The prevalence of asthma and obesity, two often associated conditions, is influenced not only by age and gender but also by lifestyle factors. This study aimed to determine whether, in a Mediterranean northern Italian region, Liguria, an increased prevalence of obesity could be detected in asthmatic children and adolescents and to evaluate the possible relationship between body mass index (BMI) and the characteristics and/or severity of asthma. BMI was determined in 554 asthmatic subjects (2.2-16.1 years) and 625 age-matched controls; BMI was expressed as a continuous variable in standard deviation score (SDS) units, determined as difference between the individual observed value and the reference mean for age and sex, divided by the corresponding standard deviation (BMI-SDS). Overweight/obesity was set at BMI-SDS of 2 or more. BMI-SDS was significantly higher in controls than in asthmatics (p = 0.04); however, the proportion of overweight/obesity subjects (BMI-SDS > or = 2) was similar in controls and in asthmatic patients (p = 0.08). Evaluation of the asthmatic group revealed that BMI-SDS was independent of gender (p = 0.57), atopic sensitization (p = 0.69), and comorbidity with other allergic symptoms (p = 0.60). By contrast, BMI-SDS was lower in preschool-age children than in school-age children and adolescents (p < 0.0001), in subjects with a high rate of acute respiratory tract infections (p = 0.04), and in those not treated with inhaled corticosteroids (IGCs) (p = 0.02). Although an increase in the prevalence of overweight/obesity was not detected in asthmatic children and adolescents, the results reported here suggest a preventive surveillance of calorie intake and a promotion of physical activity in children requiring long-term treatment with inhaled glucocorticosteroids.

Evaluation of insulin resistance in a cohort of HIV-infected youth

OBJECTIVE Metabolic abnormalities, including impairment of glucose homeostasis, have been well characterized in HIV-infected patients. In contrast to adults, insulin resistance and diabetes mellitus appear to be relatively uncommon finding in youth. DESIGN We assessed insulin resistance, and associated risk factors, in a population of vertically HIV-infected children and young adults, when compared with a control population of healthy children. METHODS At the time of enrolment, weeks of pregnancy, birth weight, sex, age, weight, height, body mass index (BMI), pubertal stages, CDC classification, blood pressure, clinical lipodystrophy, hepatitis B or C co-infection, antiretroviral therapy, CD4 T lymphocyte counts, and HIV-RNA levels were recorded. Fasting plasma glucose and insulin levels and homeostatic model assessment-insulin resistance (HOMA-IR) were determined. These parameters were compared between HIV patients and healthy controls with multivariate analyses. RESULTS Fasting insulin levels (OR=1.21, P<0.001) and glycemia (OR=0.89, P<0.001) were significantly different between HIV-infected patients and controls. Antiretroviral therapy duration (r=0.281, P<0.05), triglyceride levels (r=0.286, P<0.05), age (r=0.299, P<0.05), and BMI SDS (r=0.485, P<0.001) were significant predictor variables of insulin resistance, expressed as HOMA-IR. Moreover, clinical lipodystrophy seems to be strongly correlated to glycemia (P<0.05), triglyceride levels (P<0.05), serum insulin levels (P<0.001), HOMA-IR (P<0.05), and also with therapy duration (P<0.05). CONCLUSIONS Both HIV infection and antiretroviral therapy demonstrate differential effects on glucose metabolism in HIV-infected children. Targeted prevention of insulin resistance and diabetes mellitus in HIV-infected children and young adults is needed in order to avoid the associated long-term complications that would otherwise occur, given the improvement in life expectancy of HIV-infected individuals.

Linear growth and skeletal maturation in subjects with treated celiac disease

BACKGROUND The aim of this study was to report on the long-term growth and development in a group of treated patients with celiac disease. METHODS The study includes 26 patients (11 boys and 15 girls) with typical celiac disease who were younger than 2.5 at diagnosis and were followed by means of a growth longitudinal monitoring from the introduction of a gluten-free diet (mean age, 1.7 +/- 0.5 years) until adulthood, over a median period of 15.3 years. Growth indicators used were: height, skeletal age, weight and BMI. RESULTS At the time of admission, the patients had a general tendency to short stature, underweight and retarded skeletal maturation. They did not catch up completely in height and skeletal age after a dietary treatment period of 3 years. Most of them were seen to be slightly below average height for age during childhood and adolescence with skeletal maturity retardation, even if a fairly large interindividual variation of height profiles was evident. CONCLUSIONS Notwithstanding the early treatment, the careful follow-up, and the good adhesion to the dietary rules of the patients under study, slight negative effects of the disease on growth were not avoided.

Comparison of growth retarding effects induced by two different glucocorticoids in prepubertal sick children: An interim long-term analysis

SummaryThe low interference with growth expected in child for a cortisol analogue, deflazacort (DFZ), prompted us to verify if DFZ could affect growth less than prednisone (PDN). An interim analysis relative to 27 girls and 38 boys (out of 100 expected) age 3–12 yrs, after a median period of 14 mo.s is reported. Children with connective tissues (CTD) and glomerular disorders (KD) were randomly allocated to DFZ or PDN. Anthropometric measurements and maturity ratings were performed. Mean daily doses of PDN (or DFZ equivalent), from 0.57 to 0.64 mg/kg (DFZ 0.92 to 0.94 mg/kg) to induce control and from 0.19 to 0.93 mg/kg (DFZ 0.34 to 0.36 mg/kg) to maintain disease under control were given in CTD and KD, respectively. The increase in bone age delay over time was significantly>for PDN(-4.0 mo/yr) than DFZ (-1.8 mo/yr) in the overall group. The increases in statural age delay and loss over time were significantly> for PDN (-5.9 and-5.9 mo/yr) than DFZ (-2.4 and-2.4 mo/yr), only in children with “taller” midparents. Although doses of DFZ 1.1–1.8 times those of PDN were given, growth retardation in PDN-treated children was nevertheless 2.3–2.5 times that in DFZ-ones.

FELS, Greulich‐Pyle, and Tanner‐Whitehouse bone age assessments in a group of Italian children and adolescents

Assessments of skeletal maturity are usually made from handwrist radiographs, using either the method of Greulich‐Pyle (GP) or Tanner‐Whitehouse (TW). Recently the FELS method has been developed, and it represents a potentially valuable approach to skeletal age assessment. The present study evaluates the accuracy and precision of FELS skeletal age assessments compared with ratings by the GP and TW methods in a group of Italian children and adolescents. The hand‐wrist radiographs of subjects (171 males and 156 females 1 to 17 years) referred to the “Istituto di Puericultura e Medicina Neonatale” of the University of Genoa in Italy between 1985 and 1990 were assessed according to each method. Two independent observers rated the radiographs and one observer reassessed them after 6 months or more. GP estimates rather closely match chronological age; TW ratings tend to overestimate chronological age by 7–9 months around puberty, mainly in boys; and the FELS method tends to overestimate chronological age by amounts that increase with chronological age. The degree of precision of skeletal age assessments is within the usually accepted limits. Higher levels of repeatability and reproducibility are apparent for the FELS estimates than for GP and TW assessments. Thus, skeletal maturity is likely to be adequately interpreted by the FELS method as well as by the more commonly used GP and TW systems in Italian children and adolescents.

Bone quality assessed by phalangeal quantitative ultrasonography in children and adolescents with isolated idiopathic growth hormone deficiency

Objective: Some observations indicate that GH deficiency (GHD) may have little impact on bone mineralization in contrast to its effects on bone growth and maturation. The aim of the present study was to evaluate the effects of isolated GHD and GH-replacement therapy on bone quality assessed by a quantitative ultrasound (QUS) technique at the proximal phalanges of the hand. Design: Growth and QUS data of 68 subjects (50 males and 18 females) aged 5–18 yr with isolated GHD were retrospectively examined. A cross-sectional series of 120 observations was collected and compared with data obtained from a control population (1227 healthy children, 641 males and 586 females, aged 3–16 yr). Methods: QUS variables amplitude-dependent speed of sound (AD-SoS) and bone transmission time (BTT) were assessed by the sonographic device DBM Sonic BP IGEA. Height and weight measurements were performed according to standard techniques. In patients, skeletal age (SA) was determined by Tanner-Whitehouse method (3rd version). Results: Before treatment height, SA, AD-SoS and BTT were reduced in patients. Height SD score (SDS), SA SDS, AD-SoS SDS, and BTT SDS improved during treatment. Significant associations of both AD-SoS and BTT with age, SA, height, and therapy duration were observed. Using multivariate regression models the disease state, SA, and height proved to be significant variables in predicting BTT and AD-SoS. Conclusions: QUS measurements adjusted for body size and skeletal maturity in GHD patients seem to be only slightly reduced. A body size and skeletal maturity adjustment should be incorporated in studies on bone mass in GHD children and adolescents. A non-invasive technique such as QUS technology opens new perspectives.