Skeletal disproportion in children with chronic renal disease

OBJECTIVES: To assess stature and skeletal disproportion in children with chronic renal disease. METHODS: Cross-sectional study of height (HT), sitting height (SH), subischial leg length (SILL), sitting height/height ratio (SH:HT) and disproportion score (SH SDS minus SILL SDS) in 56 children (M:35) with median age 11.4 years (range 4.5,18.7) with chronic renal disease. RESULTS: There were 19 children with chronic renal insufficiency, 6 receiving peritoneal dialysis and 31 after renal transplant. The median HTSDS for the whole group was -1.21 (-2.8, 0.35). The median SH:HT ratio in non-transplanted children and renal transplant were 0.51 (0.49, 0.53) and 0.50 (0.48, 0.53), respectively (p = 0.02). The median disproportion score of the whole group was -3.2 (-4.8, -1.8). There was a significant correlation between disproportion score and SH:HT (r = 0.5, p = 0.005). SH:HT ratio was negatively related to duration of illness (r = 0.4, p = 0.005). CONCLUSION: Children with chronic renal disease have significant body disproportion and this may be due to a disproportionately greater effect of disease and treatment on spinal growth.     

Skeletal development inAcropora cervicornis

Scanning electron microscopy, field studies using dyes which become incorporated into the skeleton of living corals as time markers, and petrographic and mineralogic techniques were used to describe the diel pattern of calcium carbonate accretion in the extending axial corallite ofAcropora cervicornis. The axial corallite extends by the formation of randomly oriented fusiform crystals at the distal tip of the branch. Morphological and mineralogical characteristics suggest that these might be calcite crystals. They form a framework upon which needle-like aragonite crystals (initially small tufts) begin to grow. As the needles elongate, groups of them form well defined bundles, fasciculi, which compose the primary skeletal elements. There is a diel pattern in the deposition of the skeleton. At night (1800–0600 hours) the distal spines are pointed and composed primarily of fusiform crystals. During the day (0600–1800 hours) mineral accretion occurs on all surfaces of the skeleton, apparently by epitaxial growth on the aragonite needles of the fasciculi.     

Skeletal development in Acropora cervicornis

Monthly linear extension and calcium carbonate accretion were measured over a year in the Caribbean staghorn coral, Acropora cervicornis. X-radiographs were made of cross sections of branches to analyze radial growth. Correlations were made between parameters of skeletal growth and four environmental parameters monitored over the same sampling periods: temperature, daylight hours, sun hours, plankton abundance. The results indicate that linear extension does not change during the year with the possible exception of April. It is suggested that temperatures outside an optimal range (ca. 26°–29°C for staghorn Acroporas) might cause a decrease in linear extension, however. Specific accretion (mg. mm^-1) does show significant variations through the year. Calcium carbonate accretion (mean specific accretion times mean linear extension, mg. tip^-1) is most strongly correlated with number of sun hours. A comparison is made between diel patterns of extension and accretion and longer term measurements. It is suggested that the accretion process is probably most influenced by some activity influenced by light. There are no annual growth bands in X-radiographs of cross-sections of the branches of A. cervicornis. This may result from secondary infilling in the skeleton.     

Intelligence and physical features of children of women with epilepsy

The teratogenicity of maternal epilepsy has been attributed to several factors, including the antiepileptic drugs taken to prevent seizures during pregnancy, the occurrence of seizures during pregnancy, and the factors in the mother that caused her to have epilepsy. We have addressed the hypothesis that the children of women who have a history of epilepsy (seizure history), but who took no antiepileptic drugs (AED) and had no tonic-clonic seizures in pregnancy, have an increased risk of malformations and diminished intelligence. The frequency of cognitive dysfunction was determined in 57 seizure history and 57 matched control children aged 6-l6 years. The masked evaluation of the children included a physical and neurologic examination and testing with the Wechsler Intelligence Scale for Children-Revised (WISC-R) and a systematic physical examination for the features of the fetal AED syndrome. The evaluation of both parents of each child included a test of reasoning (Ravens Progressive Matrix) and a physical examination. There were no differences between the two groups of children in either IQ scores or physical features; none of the seizure history children was judged to have the "anticonvulsant face" or digit hypoplasia. This study had 80% power to rule out a difference of seven or more IQ points between the two groups, based on a two-sided test at a 5% level of significance. Our confidence in concluding that there was no difference between seizure history and control infants was strengthened by the fact that no statistically significant differences were observed with respect to multiple outcomes, including eight related measures of intelligence. Thirty (53%) of the seizure history mothers resumed taking AED after the birth of the child we evaluated. Additional studies are needed to address the teratogenicity of the antiepileptic drugs as monotherapy.     

Skeletal maturation of children in Shiraz, Iran

Hand-wrist radiographs were made of 4,551 Shiraz school children and well babies of known age who were also weighed and measured. Medical examinations were not made and selection was biased in favor of the advantaged child because the urban standard of living is higher than the rural in Iran and because the poorest children may not attend elementary school and never attend high school. Nevertheless, height, weight and bone age fell rapidly below the tenth Iowa percentile by the age of two years and did not begin recovery until the age of six years. The distribution of the bone ages in each group was symetrical, not bi-modal. Bone age offered no advantage over height age as an index of development but analysis of the radiographs provided evidence that the retardation was pathologic rather than genetic. Ossification of individual epiphyses was recorded in 3,333 hands and a marked discrepancy between carpal and phalangeal bones, with the carpal bones most retarded, was documented. A high incidence of pseudoepiphyses was noted.     

Skeletal maturity of Japanese children in Western Kyushu

This paper describes the skeletal maturity status of Japanese children in Western Kyushu and its variation within Japanese populations. Hand-wrist skeletal maturity was assessed by the Tanner-Whitehouse (1975) (TW2) method from radiographs of 500 boys and 485 girls aged from 4 to 15 years. Western Kyushu children showed retarded skeletal maturity scores (RUS, carpals, and 20-bone) under the age of 12 years for boys and 10 years for girls, and thereafter they were advanced in relation to the British standard. Within Japanese populations the present sample showed delayed maturity compared to Tokyo children, but was close to that of Sapporo children throughout the age range studied. However, the expected effect of secular trend suggested skeletal maturity more advanced for Tokyo children and somewhat advanced one for Sapporo children compared to that of Western Kyushu children.     

Skeletal development in monozygotic and dizygotic twins]

The maturation and development of 27 monozygotic and 23 dizygotic twins were studied over a 10 year period with data collected at one year intervals from age 9 to adulthood. In this manner the ossification process was recorded based on X-ray films of the carpal bones. Further information was acquired through anthropometric and somatoscopic data reproduced with standard photography. Sex and phase specific genetic factors influencing the maturation process are presented and analysed.     

Intelligence and genetic markers in Chilean children.

Genetic markers and total intelligence quotient (IQ) assessed by WISC (Wechsler Intelligence Scale for Children) were studied in children of both sexes from Santiago, Chile. Heterozygous boys for phosphoglucomutase 1 (PGM) and heterozygous girls for haptoglobin (Hp) had lower IQ than homozygotes. For ABO system, B girls had lower and B boys had higher IQ than children with other ABO phenotypes. These differences were highly significant with the two tailed t'-test (Student's t-test with the Welch-Satterthwaite correction for degrees of freedom), and most of them remained significant after the correction for multiple comparisons. Girls had greater variance of IQ than boys. Relationships between homozygotes and heterozygotes were found in two independent studies. Thus, the genetic relationship found here seems likely to be a true biotic effect.     

Early development of children with Williams syndrome.

Developmental observations in ten young children with Williams syndrome (1-6 years old) are presented from developmental tests, symbolic play sessions and play sessions with a special educator following the non-directive Montessori approach. There is a considerable individual variability in performance. Overall, the children are engaged in goal-directed activities for more than 35% of the time during play sessions. Overactivity and distractability seem to be more age-dependent and situation-specific than thought before. Developmental interventions may include play sessions following the Montessori approach.     

Skeletal growth in school children: maturation and bone mass

Skeletal growth and development was evaluated in 322 white children (age 6 to 14) using three different methods: (1) ¹²⁵I photon absorptiometry, (2) compact bone measures on radiographs, and (3) Greulich-Pyle skeletal age from hand-wrist radiographs. Bone mineral content, measured by photon absorptiometry, increased at an incremental rate of about 8.5% each year. Skeletal age was a poor predictor of skeletal status, i.e., bone mineral content (14% error), and did not decrease the predictive error substantially more than did chronological age. Gross morphology (height and weight) was in fact a better predictor of bone mineral content than were skeletal age, chronological age, and radiographic morphometry. Skeletal age deviations were correlated with deviations in body size. A bone mineral index was devised which was independent of body size and this index was also independent of skeletal age. Skeletal age is imprecise (3 to 6 months error) and the range of variation in normal children (13 months) overlaps the maturational delay of the malnourished and diseased. The difficulties in using skeletal maturation are discussed and it is suggested that particular maturational indices be used which better indicate skeletal growth than does a composite skeletal age.     

Skeletal development in Xenopus laevis (Anura: Pipidae).

Postembryonic skeletal development of the pipid frog Xenopus laevis is described from cleared-and-stained whole-mount specimens and sectioned material representing Nieuwkoop and Faber developmental Stages 46-65, plus postmetamorphic individuals up to 6 months old. An assessment of variation of skeletogenesis within a single population of larvae and comparison with earlier studies revealed that the timing, but not the sequence, of skeletal development in X. laevis is more variable than previously reported and poorly correlated with the development of external morphology. Examination of chondrocranial development indicates that the rostral cartilages of X. laevis are homologous with the suprarostral cartilages of non-pipoid anurans, and suggests that the peculiar chondrocranium of this taxon is derived from a more generalized pattern typical of non-pipoid frogs. Derived features of skeletal development not previously reported for X. laevis include 1) bipartite formation of the palatoquadrate; 2) precocious formation of the adult mandible; 3) origin of the angulosplenial from two centers of ossification; 4) complete erosion of the orbital cartilage during the later stages of metamorphosis; 5) development of the sphenethmoid as a membrane, rather than an endochondral bone; and 6) a pattern of timing of ossification that more closely coincides with that of the pelobatid frog Spea than that recorded for neobatrachian species.     

Platelet selenium in children with normal and low selenium intake

The concentration of selenium was determined by instrumental neutron activation analysis in erythrocytes, platelets, and plasma of eight dietetically treated children with phenylketonuria (n=6) or maple-syrup-urine disease (n=2) with low selenium intake and for ten children with normal selenium intake. The normal selenium concentration in platelets was about 600 ng/g and about five times higher than in erythrocytes of the same children. A decreased selenium concentration in platelets was seen only when the corresponding concentrations in erythrocytes and plasma were very low. This suggests a special role of selenium in platelets.     

Aggression in low functioning children and adolescents with autistic disorder

Background

Parents, caregivers and mental health professionals have often reported violence and aggression in children or adolescents with autistic disorder. However, most of these observations derived from anecdotal reports, and studies on frequency and characterization of aggression in autism remain limited. Our objective was to better characterize and understand the different types of aggressive behaviors displayed by a large group of individuals with autism in different observational situations.

Methodology/Findings

The study was conducted on 74 children and adolescents with autism and 115 typically developing control individuals matched for sex, age and pubertal stage. Other-Injurious Behaviors (OIB) were assessed in three observational situations (parents at home, two caregivers at day-care, a nurse and a child psychiatrist during blood drawing) using validated scales. The frequency of OIB was significantly higher in individuals with autism compared to typically developing control individuals during the blood drawing (23% vs. 0%, P<0 .01). The parents observed significantly less OIB in their children than caregivers (34% vs. 58%, P<0.05). In addition, the most frequent concurrent behaviors occurring just before the appearance of OIB in individuals with autism were anxiety-related behaviors and excitation according to the parental as well as the caregiver observation.

Conclusions/Significance

The results suggest that in a stressful situation, such as the blood drawing, individuals with autism release their stress through behaviors such as OIB, whereas typically developing individuals regulate and express their stress through cognitive skills such as mental coping strategies, symbolization skills with representation and anticipation of the stressful situation, social interaction and verbal or non-verbal communication. The findings underline also the key role of the environment in assessing OIB and developing therapeutic perspectives, with an individual who modulates his/her behavior according to the environment, and an environment that perceives this behavior and reacts to it with different tolerance thresholds according to the observers.     

Mental development of unselected children with sex chromosome abnormalities

Summary Twenty-five children with sex chromosome aberrations found among 11000 consecutively newborn children from 1969 to 1974 have been followed with psychologicalpsychiatric examinations at four different times from the age of 1 year till the ages of 7 to 11. The results of the follow-up studies are presented, and it is concluded that diagnosis of sex chromosome aberrations at birth or during early childhood with full information and guidance to parents and educators are of great importance for the development of the children, especially concerning learning and behavior.     

Skeletal stem cells in bone development,homeostasis, and disease

Tissue-resident stem cells are essential for development and repair, and in the skeleton, this function is fulfilled by recently identified skeletal stem cells (SSCs). However, recent work has identified that SSCs are not monolithic, with long bones, craniofacial sites, and the spine being formed by distinct stem cells. Recent studies have utilized techniques such as fluorescence-activated cell sorting, lineage tracing, and single-cell sequencing to investigate the involvement of SSCs in bone development, homeostasis, and disease. These investigations have allowed researchers to map the lineage commitment trajectory of SSCs in different parts of the body and at different time points. Furthermore, recent studies have shed light on the characteristics of SSCs in both physiological and pathological conditions. This review focuses on discussing the spatiotemporal distribution of SSCs and enhancing our understanding of the diversity and plasticity of SSCs by summarizing recent discoveries.     

Skeletal development in the fossorial gymnophthalmids Calyptommatus sinebrachiatus and Nothobachia ablephara

The development of the cartilaginous and bony elements that form the skull and axial and appendicular skeleton is described in detail for the post-ovipositional embryonic development of the fossorial gymnophthalmid species Calyptommatus sinebrachiatus and Nothobachia ablephara. Both species have a snake-like morphology, showing an elongated body and reduced or absent limbs, as well as modifications in skull bones for burrowing, such as complex articulation surfaces and development of bony extensions that enclose and protect the brain. Similar morphological changes have originated independently in several squamate groups, including the one that led to the snake radiation. This study characterizes the patterns of chondrogenesis and osteogenesis, with special emphasis on the features associated with the burrowing habit, and may be used for future comparative analyses of the developmental patterns involved in the origin of the convergent serpentiform morphologies.