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1.
In this study, the authors attempted to predict the ultimate leg length in normal children with normally growing legs, using Green-Anderson and Moseley techniques with chronologic and skeletal age. The predictions were based on serial growth studies of children between 5 and 10 years of age. Using skeletal age for prediction, the absolute mean error in predicting the ultimate leg length was 2.4 cm using the Green-Anderson method and 2.58 cm using the Moseley method. Using the Moseley method for length prediction in boys, the mean error was 3.8 cm using skeletal age and 1.5 cm using chronologic age. For girls, using the Moseley method of leg length prediction, the mean error was 1.55 cm with skeletal age and 2.46 cm with chronologic age. The significance of this study is that skeletal age, as determined by the Gruelich and Pyle Atlas, does not improve the accuracy of prediction of ultimate leg length in children younger than 10 years of age, except in girls with advanced bone age.  相似文献   

2.
Administered the Maudsley Personality Inventory to 5 groups of female Ss: (a) 13 Turner Syndrome Ss (mean age 19 yrs 2 mo) with karyotype 45X, (b) 18 Turner Syndrome (TS) Ss (mean age 26 yrs 11 mo) with other types of sex chromosomal abnormalities, (c) 16 sisters of Ss (mean age 24 yrs 2 mo), (d) 9 Ss with growth retardation and primary amenorrhea (mean age 22 yrs 4 mo), and (e) 19 nurses (mean age 22 yrs 8 mo). The results are compared to English and American normative values. The total group of TS Ss obtained a low Neuroticism (N) score compared to the control groups. When the total group was divided on the basis of their karyotypes, the low N score could be referred solely to Ss with karyotype 45X, who scored significantly lower than any other group tested so far with the MPI. TS Ss with chromosomal abnormalities other than 45X scored within normal limits. No variables other than karyotype 45X were related to an extremely low N score. It is concluded that exceptional emotional stability, as reflected in a very low N score on the MPI, is concomitant with the total absence of one sex chromosome, which is probably due to a developmental abnormality of the CNS. (7 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   

3.
We report an unusual case of a 55 year old Japanese woman with a seminoma but relatively normal menses. The patient was a phenotypic female with late onset menarche (18 years of age), who was amenorrhoeic for the first year, followed by menses of one to three days' slight flow with dysmenorrhoea, but an otherwise normal menstrual history. A typical seminoma was removed from the left adnexal region and an immature testis was identified separately as an associated right adnexal mass. Repeated karyotypic studies on peripheral blood lymphocyte cultures showed only 46,X,-Y,t(Y;15)(q12;p13). Cytogenetic examination of the patient's younger brother, who had fathered three healthy children, showed an identical karyotype. Mosaicism of 46,X,-Y,t(Y;15)(q12;p13)/45,X cell lines was found in skin samples from the patient's elbow and genital regions, although there were no clinical stigmata of Turner syndrome. An androgen receptor binding assay of cultured genital skin fibroblasts was negative. Molecular analysis using Southern blot hybridisation, PCR, and direct DNA sequencing showed that neither the patient nor her brother had a detectable deletion or other abnormalities of Y chromosome sequences, including the SRY (sex determining region of the Y chromosome) gene sequence. These findings suggest that Turner mosaicism of the 45,X cell line may have contributed to this atypical presentation in an XY female, although we cannot exclude abnormalities of other genes related to sex differentiation.  相似文献   

4.
Several studies have investigated the quality of life (QOL) of GH-deficient (GHD) adults who, as children, had been treated with GH. Variable findings are probably related to sample heterogeneity and disparate research methodologies and designs, particularly the choice of control or comparison groups. In addition to comparing a relatively large sample to questionnaire norms, the present study is the first to compare the QOL adjustment of GHD patients to that of same sex siblings. A total of 140 former patients (76% of those eligible; mean age, 26 yr; n = 95 isolated GHD, n = 45 multiple pituitary hormone deficiencies; 117 males and 23 females) and 53 same sex siblings (84% participation), 18 yr and older, participated in the telephone questionnaire survey. The majority of interviews with GHD patients (78%) and siblings (87%) were conducted blind to the subject's clinical status. Comparisons between GHD patients and norms for standardized questionnaires indicated both better and worse functioning in several domains. In contrast, very limited differences were detected between GHD cases and same sex siblings. Isolated GHD patients were functioning better than those with multiple pituitary hormone deficiencies, but the effect sizes of these differences in most areas were relatively small. Adult height and degree of growth over the course of GH therapy were generally unrelated to QOL outcomes. Findings from the present study underscore the importance of selecting unbiased control/comparison groups in evaluating psychological outcomes among GHD adults.  相似文献   

5.
Bone age maturation in 116 untreated patients with Turner's syndrome was evaluated in a cross-sectional and longitudinal analysis. A total of 265 radiographs were rated using the TW2-RUS method on the computer-assisted skeletal age score (CASAS) system. Bone age was found to be retarded from the chronological age of 3 to 6y. Between the ages of 7 and 12 y bone age almost equalled chronological age and progressed normally at a rate of 1 y y(-1). Bone maturation slowed down thereafter and epiphyseal closure was not reached before the age of 17 y. Reference data are presented on bone age and a bone age maturation curve for untreated patients with Turner's syndrome to be used in clinical practice. In the assessment of bone age and bone age velocity in Turner's syndrome the CASAS system produced reliable and valid results. The absolute difference between repeated bone age ratings was 0.26 "y" (median) with a range of 0.00-0.56 "y". Future studies evaluating the effect of growth-promoting treatment in Turner's syndrome should use a computerized method for the determination of bone age.  相似文献   

6.
Bone age maturation and growth velocity were analyzed longitudinally by the TW2 RUS method standardized for Japanese children in 45 GH-treated boys with idiopathic GH deficiency (GHD). The patients were divided into three groups: Group I consisted of four isolated GHD patients who underwent spontaneous puberty without gonadotropin suppression treatment (GST) and had a mean final height of 151.9 cm; Group II consisted of 24 GHD patients with associated gonadotropin deficiency who received sex hormone replacement treatment (GRT) and had a mean final height of 165.3 cm; Group III consisted of 17 isolated GHD patients who underwent spontaneous puberty and had a mean final height of 158.3 cm after being treated with combined GH and GST. Bone age matured along with chronological age in Group I, whereas bone age in Group II decelerated significantly after a bone age of 12 years and did not reach a bone age of 14 years. Bone age maturation in Group III showed an intermediate pattern between Groups I and II; bone age decelerated significantly after a bone age of 12 years but mean bone age advanced beyond a bone age of 14 years. Height velocity in Group I during GH treatment decelerated rapidly after the pubertal growth spurt, as usually seen in normal puberty. A definite pubertal growth spurt was not observed in the height velocity of Group II during GH treatment before receiving GRT; the mean height velocity gradually declined, remaining at 3.5-4.5 cm/year even after 18 years. Mean height velocity in Group III during GH treatment and GST showed a similar tendency as Group II, but it declined more rapidly. Since a growth velocity of around 3 cm/year was preserved with GH treatment despite the decline in growth velocity, the slower the advance of bone age, the longer the treatment period and, therefore, the taller the final height achieved by GST compared to Group I. It is recommended to start GST at a bone age between 11.5 years and 13 years. The timing, namely when to start GRT in GHD with gonadotropin deficiency or when to stop GST in isolated GHD, can be estimated according to the patient's desired final height and bone age-growth potential.  相似文献   

7.
PURPOSE: We attempted to determine the incidence of vesicoureteral reflux in asymptomatic siblings of children with reflux at different ages and assess the incidence of renal damage in asymptomatic siblings with reflux. MATERIALS AND METHODS: We reviewed radionuclide cystograms of 482 consecutively referred siblings of children with vesicoureteral reflux, including 295 girls and 187 boys 2 weeks to 12.8 years old (mean age 2.8 years). Ultrasonograms and renal cortical scintigrams of children with reflux were evaluated. All siblings were considered asymptomatic by the referring physicians. RESULTS: The overall incidence of vesicoureteral reflux was 36.5%, and the incidence in girls and boys was 39.3 and 32.1%, respectively. Children 24 months old or younger had the highest incidence (45.7%) and the highest risk of bilateral reflux. From ages 25 to 72 months the incidence of reflux was 33.1% and in siblings older than 72 months it was 7%. Reflux of urine to the level of the renal pelvis was detected in 28.6% of all referred siblings. Renal damage was observed on sonography or scintigraphy in 4.7% of the siblings with reflux. CONCLUSIONS: The high incidence of vesicoureteral reflux through age 72 months indicates that it is important to screen siblings of children with reflux at an early age to prevent renal damage, which can occur in the absence of symptomatic urinary tract infection.  相似文献   

8.
Prenatal exposure to anticonvulsant medication has been shown to cause craniofacial dysmorphology, prenatal growth retardation, hypoplastic nails and phalanges, and visceral abnormalities. In this study we examined maxillary and mandibular stone dental casts (45) and panoramic radiographs (39) from 45 individuals with ages 4.5 to 22.0 years for changes in mesiodistal crown size of deciduous and permanent teeth, and the presence of dental anomalies. These individuals had been exposed prenatally to antiepileptic drugs (AEDs). Mesiodistal crown diameters were measured from the dental casts and converted into standard scores (Z), using published normative data from the University of Michigan Longitudinal Craniofacial Growth Series. A significant increase in mesiodistal crown dimensions of the posterior maxillary teeth was observed, specifically in primary molars and their permanent premolar successors, as well as permanent molars. Changes in tooth size were more common in females than in males. Dental maturity, assessed using the panoramic radiographs, was equal to chronologic age. An increased frequency of hypodontia was the only notable dental anomaly.  相似文献   

9.
A total of 183 hematologic malignancies with t(4;11)(q21;q23), including five variant translocations, were collected by the Workshop. Clinical, morphologic and immunophenotypic features were compiled, and karyotypes with variant t(4;11) or secondary chromosomal aberrations were reviewed. All cases were acute leukemias (AL): 173 acute lymphoblastic leukemias (ALL), six acute myeloid leukemias (AML), three unclassifiable AL, and one biphenotypic AL. Ten patients had treatment-associated AL. Females were overrepresented (104 vs 79) and the age distribution was clearly nonrandom; 34% of the cases occurred in infants below the age of 12 months. The remaining AL were evenly distributed among the other age groups, with the oldest patient being 79 years old. An increased white blood cell count (WBC) was reported in more than 90% of the cases, with hyperleukocytosis (> or =100 x 10(9)/l) in 64%. Additional chromosomal changes were detected in 55 (30%) cases, most often gain of the X chromosome, i(7)(q10), and trisomy 8, with frequent breakpoints in 1p36, 1q21, 7q10, 11p15, 12p13, 17p11, and 17p10. All recurrent secondary changes resulted in genomic imbalances, in particular gains of 1q, 7q, 8, and X and losses of 7p and 17p. Event-free and overall survival (EFS and OS) could be ascertained in 170 and 171 patients, respectively. Kaplan-Meier estimates of EFS and OS showed no differences with regard to gender, WBC, or presence of secondary chromosomal abnormalities, and there was no increase of EFS or OS among the 55 cases that had undergone bone marrow transplantation. However, age had an important prognostic impact, with significantly (P < 0.0001) longer EFS and OS in children 2-9 years old than among infants and younger children, patients aged between 10 and 39 years and older adults.  相似文献   

10.
BACKGROUND: Sodium valproate administration during pregnancy may be teratogenic; it is associated with an increased risk of neural tube defect, tetralogy of Fallot, oral clefting and other facial abnormalities. Knowledge of these harmful effects is still poor. CASE REPORTS: Four children, including three siblings, presented with characteristic facial abnormalities (four cases), oral clefting (one case), mental retardation (2/2 cases), and bone anomalies of forearm and hands (one case). The diagnosis of fetal valproate syndrome was only made at the age of 3 1/2 years in the eldest of the three siblings all born from an epileptic mother receiving valproate since the age of 13 years. Prevention advice for further pregnancies was not followed. CONCLUSION: All epileptic mothers should be aware of the risk of antiepileptic drugs during pregnancy, specially those given sodium valproate, a potentially teratogenic drug.  相似文献   

11.
This first known case of concurrent congenital dyserythropoietic anaemia (CDA) and autoimmune haemolytic anaemia (AIHA) which occurred in a hispanic male and spanned 6 years from the age of 2. Light and electron microscopy of bone marrow erythroblasts and immunophenotyping confirmed CDA; serum/eluate warm autoantibodies and positive direct antiglobulin tests (DAT) associated with severe, episodic anaemias established AIHA. Cytogenetic analysis of bone marrow cells and peripheral blood lymphocytes ascertained sex chromosome aneuploidy (48 XY,+ Y,+ Y). Recurrent, life-threatening episodes of transfusion-dependent anaemia refractory to steroids and intravenous immune globulin, were put into stable remission at age 8 years when splenectomy successfully managed both disorders.  相似文献   

12.
In this longitudinal study, skeletal ages assessed with the Fels method and the Tanner-Whitehouse II method (TW II) were compared for boys (n = 30) and girls (n = 30) with a mean chronological age between 12 and 16 years. The subjects, participating in the Amsterdam Growth and Health Study, were measured annually between 1977 and 1980, which resulted in four radiographs of the left hand and wrist of every individual. For boys, the mean TW II skeletal age was 0.32 years older than the mean Fels skeletal age (sd 0.50). Tested at the subsequent chronological ages, the mean TW II skeletal ages were 0.05-0.47 years older, the differences being statistically significant at the mean ages of 13, 14 and 15 years. For girls, the mean TW II skeletal age was 0.20 years younger than the mean Fels skeletal age (sd 0.69). At the subsequent chronological ages, the mean TW II skeletal ages were 0.03 to 0.35 year younger, the differences being statistically significant at the mean chronological ages of 14 and 15 years. As a consequence of the differences between the methods, application of the Fels method resulted in classifying a smaller percentage of boys (10%) as rapid maturers, and a higher percentage (6.7%) of boys as normal maturers in comparison to the TW II method. For girls, a higher percentage of female adolescents were classified as rapid (16.7%) and slow maturers (13.3%), but a smaller percentage was classified as normal mature (30%). Differences in the skeletal ages can be ascribed to differences in maturation of the reference population, but also to fundamental differences in the statistical methods of the scoring system and the scales of maturity. CONCLUSION: There is no agreement in skeletal ages assessed according to the TW II method and the Fels method in adolescence.  相似文献   

13.
STUDY DESIGN: This retrospective study evaluated the progression of deformity after posterior fusion by reviewing 63 consecutive patients with idiopathic scoliosis who were all in Risser sign 0 at the time of surgery. All patients were observed beyond the time of skeletal maturity. Average follow-up time was 9 years and 8 months (range, 5-16 years). OBJECTIVES: To investigate the risk factors for the crankshaft phenomenon after posterior fusion and to build a model for predicting the probability of curve progression until maturation of growth. SUMMARY OF BACKGROUND DATA: There remains considerable controversy concerning the incidence, risk factors, and necessity of combined anterior fusion to prevent the crankshaft phenomenon in patients who are skeletally immature. METHODS: Serial radiographs were measured for Cobb angle, apical rotation according to Perdriolle, and apical rib-vertebra angle of Mehta. Multivariate and univariate logistic regression analysis was performed using seven potential predictors as independent variables and Cobb angle progression and rotational progression as dependent variables. RESULTS: Average progression of deformity was 3 degrees Cobb angle (range, -8-16 degrees) and 3 degrees Perdriolle rotation (range, -9-17 degrees). Progression of deformity more than 5 degrees of either Cobb angle or rotation was observed in 22 (35%) of 63 curves with 7 (11%) of 63 curves greater than 10 degrees. Chronologic age and skeletal age were found to be significantly associated with progression of deformity in univariate analysis. In multivariate analysis, only skeletal age seemed to be independently prognostic. The authors tried to build the logistic model using the three factors of chronologic age, skeletal age, and apical rib-vertebra angle. This model correctly classified 81% of all patients as progressive or nonprogressive. The positive predictive value was 90%. CONCLUSIONS: The results showed that patients with chronologic age of 11 years of younger, especially those with a skeletal age of 10 years or younger, had a high estimated probability of progression of deformity. The progression was fairly moderate, however, with an average Cobb angle of 9 degrees and average rotation of 7 degrees, which neither the patients nor the surgeon believed was of such magnitude as to warrant routine combined anterior fusion.  相似文献   

14.
An incidence survey of Hodgkin's disease in Greater Boston during 1959-1973 detected five sibling pairs under the age of 45. The expected number is 0.7; thus, siblings of young adults with Hodgkin's disease have about a sevenfold excess risk of the disease (P = 0.0008). Eight sibling pairs, not in the incidence series, were also identified. Among all 13 pairs, 12 were sex concordant; the number expected is 6.8 (P = 0.01). The literature includes 46 sibling pairs under 45 of which 30 are sex concordant. The expected number is 23.9 (P = 0.05). Combining the present and the literature series suggests that siblings of the same sex as an affected person have a risk of Hodgkin's disease double that of siblings of the opposite sex. The sex concordance suggests that the excess Hodgkin's disease among siblings of affected persons is due either to inter-personal transmission of an etiologic agent by prolonged or intimate contact or to common-source exposures.  相似文献   

15.
Immunization to eliminate measles is recommended at 15 months of age with the option of giving vaccine at 6 to 9 months of age during measles outbreaks in Korea. Because of the recent resurgence of measles and concern about the possibility of reduced vaccine efficacy caused by genomic differences between vaccine virus and contemporary wild measles viruses, we conducted a measles vaccine efficacy study involving children with household exposure ages 1 to 5 years during measles outbreak that had occurred 1993 in Seoul and Seong-nam city, with the demographic analysis of patients brought to the hospitals. A total of 380 patients (M:F = 216:164) were included in this study. Two hundred nine cases (55.0%) occurred in children less than 5 years of age, and 167 (43.9%) were younger than 16 months of age. The recorded age-specific incidence rates showed bimodal patterns, i.e. highest peak in those below 16 months of age and second peak in those ages 6 to 9 years of age. Only 9.6% (16 of 167) of the measles cases less than 16 months, 59.5% (25 of 42) of those 16 months to 4 years and 91.8% (157 of 171) of the cases in school age children have been vaccinated. Attack rates among the 122 vaccinated siblings and 12 unvaccinated siblings ages 1 to 5 years who contacted measles were 5.7 and 75%, respectively, and the clinical vaccine efficacy was 92.4% (95% confidence interval, 83.6, 96.4). The high vaccine efficacy in household exposures suggests that measles outbreaks in Korea are not caused by reduced vaccine efficacy.  相似文献   

16.
The purpose of this study was to compare the interactions of oldest and youngest children with parents and siblings. Twenty first-born and 17 last-born children between the ages of 4 and 8 from two- or three-child families were selected from a larger sample for this study. Their interactions at home with other family members were observed and coded. Oldest children emitted more of their total behaviors to parents than to siblings, whereas last-born children did not differ significantly in the total number of behaviors emitted to the two family member groups. When the incidence of individual behavior categories was examined, no significant main effect due to ordinal position was found; however, a significant interaction of ordinal position with family member group for some behaviors was found. Both parents and siblings emitted similar levels of prosocial responses toward oldest and last-born children. However, oldest children were more likely to be the recipient of negative responses from parents and siblings than were last-born children of the same age. Implications of these findings for understanding the influence of ordinal position on social behavior are discussed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   

17.
Examined how similarly mothers behave toward infant siblings in 50 families in which mothers were videotaped while interacting with each of 2 siblings when each child was 12 mo old. The average age difference between siblings was 35 mo. Maternal behavior was assessed from the videotapes during feeding and free play. Factor analysis yielded 3 factors: Affection, Verbal Attention, and Control. Results indicate that the mothers behaved very similarly toward their 2 siblings at the same age in infancy: The consistency of maternal behavior toward the siblings approached the reliability of the measures. Although results might differ for older children, and for siblings of different ages, the data suggest that differential maternal treatment of children of the same age in infancy is unlikely to be a major source of the marked individual differences that have been observed within pairs of siblings. (28 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   

18.
INTRODUCTION: Moebius's syndrome is an entity present at birth, characterized by oculofacial paralysis and external ophthalmoplegia. Other cranial nerves can also be affected and associated to skeletal abnormalities and neurologic symptoms. It appears sporadically, sometimes of familiar nature, presenting special facies with total absence of facial expression and severe strabismus. The pathogenesis of the syndrome still remains unknown, being the transitory situation of fetal hypoxia/ischemic the most accepted theory. In some cases chromosomal abnormalities have been detected. CLINICAL CASE: We reported three children with different symptoms, two of them are siblings whose father is affected, but he was not diagnosed until adult age. CONCLUSIONS: We conclude pointing out the different presentation of the disease, the appearance in several members of a family and its chronically evolution.  相似文献   

19.
OBJECTIVE: To define mechanisms accounting for transient deafness in three children (two siblings, ages 3 and 6, and an unrelated child, age 15) when they become febrile. DESIGN: Audiometric tests (pure-tone audiometry, speech and sentence comprehension), tympanometry, middle ear muscle reflex thresholds, otoacoustic emissions (OAEs), and electrophysiological methods (auditory brain stem responses [ABRs], sensory evoked potentials, peripheral nerve conduction velocities) were used to test the children when they were afebrile and febrile. RESULTS: ABRs, when afebrile, were abnormal with a profound delay of the IV-V and absence of waves I-III. The ABR in one of the children, tested when febrile, showed no ABR components. Measures of cochlear receptor function using OAEs were normal in both febrile and afebrile states. Cochlear microphonic potentials were present in the three children, and a summating potential was likely present in two. When afebrile, there was a mild threshold elevation for all frequencies in the 15-yr-old and a mild elevation of thresholds for just low frequencies in the two siblings. Speech comprehension in quiet was normal but impaired in noise. One of the siblings tested when febrile had a profound elevation (>80 dB) of pure-tone thresholds and speech comprehension was absent. Acoustic reflexes subserving middle ear muscles and olivocochlear bundle were absent when febrile and when afebrile. No other peripheral or cranial nerve abnormalities were found in any of the children. Sensory nerve action potentials from median nerve in one of the children showed no abnormalities on warming of the hand to 39 degrees C. CONCLUSION: These children have an auditory neuropathy manifested by a disorder of auditory nerve function in the presence of normal cochlear outer hair cell functions. They develop a conduction block of the auditory nerves when their core body temperature rises due, most likely, to a demyelinating disorder of the auditory nerve. The auditory neuropathy in the two affected siblings is likely to be inherited as a recessive disorder.  相似文献   

20.
The effects of sex and age on patterns of circulating somatotropin (ST) concentration and plasma IGF-I, IGF-II, insulin, and IGF binding protein-3 (IGFBP-3) were studied in ram, wether, and ewe lambs (n = 7 or 8) sampled at mean ages of 81 (I1) and 158 d (I2). Between 81 and 158 d of age, rams grew more rapidly than wethers (P < .01), and wethers grew more rapidly than ewes (P < .01). The sex differences in growth were reflected in empty body weight at slaughter: rams > wethers > ewes (P < .05). Mean plasma ST concentrations, ST pulse amplitude, and integrated plasma ST concentrations were greater (P < .05) in rams than in ewes at I1 and I2. Characteristics of the ST plasma profile in wethers were generally intermediate between those of rams and ewes. The interpulse interval was greater in ewes than in wethers at I2. The IGF-I and IGFBP-3 concentrations were greater in rams than in ewes at both sampling times. Plasma IGF-II was greater in ewes than in rams at I2. Mean plasma ST was approximately two thirds less at I2 than at I1 regardless of sex. Mean plasma ST and IGF-I at both ages were positively correlated with growth. Mean plasma ST at I2 was negatively correlated with fatness at slaughter. Sex and age significantly affected patterns of circulating ST and concentrations of IGF-I and IGFBP-3 in prepubertal growing lambs, under conditions for which growth rates and composition were also sexually dimorphic.  相似文献   

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