Effect of Developmental Disorders of Maxillary First Molars on Orofacial Morphology / 대한소아치과학회지

This study was aimed to evaluate orofacial morphologies on the cases of developmental disorders of maxillary first molars.Panoramic radiographs, lateral cephalographs, and clinical photos of 2983 children who attended the Pediatric Dental Clinic of Pusan National University Dental Hospital from 2006 to August 2017 were assessed retrospectively. 34 patients were selected whose maxillary first molars were missed or developmentally delayed unilaterally or bilaterally. Demirjian' s method was used for estimating dental age, then which was compared to chronologic age of children. Parameters expressing skeletal and dentoalveolar disharmony were checked and compared with control. Additionally, occlusion relationship was evaluated.Maxillary dental age was significantly delayed compared to chronologic age. Several parameters which show skeletal open-bite tendency and skeletal class III malocclusion with maxillary retrusion were statistically significant. Anterior crossbite and edge-bite were expected in most of these cases, but compensation by occlusion and soft tissue was also verified which might mask skeletal class III tendency.Congenital missed or developmentally delayed maxillary first molars might be related with declined growth of maxilla. If developmental disorders of maxillary first molars were verified during clinical examination, careful monitoring of orofacial growth was necessary during puberty and timed orthopedic and orthodontic intervention were considered.

Triagem urinária para erros inatos do metabolismo em crianças com atraso no desenvolvimento / Urinary screening for inborn errors of metabolism among children with delayed development

Objetivo: verificar a freqüência de positivos na triagem urinária para erros inatos do metabolismo, em crianças com atraso no desenvolvimento e correlacionar aspectos clínicos da população estudada com os resultados da triagem. Método: estudo transversal, descritivo, realizado no Programa de Estimulação Precoce da Unidade de Referência Materno-Infantil e Adolescente do Estado do Pará, em 197 crianças com atraso no desenvolvimento, através da realização de testes qualitativos de urina. Resultados: cerca de 52,8% dos pacientes apresentaram resultados positivos para, pelo menos, um teste. As crianças oriundas de outros municípios do Estado do Pará detiveram maior proporção de positivos, com p=0,003*. Dentre as manifestações clínicas investigadas, as convulsões e os problemas de alimentação destacaram-se pela freqüência. Conclusões: os resultados encontrados mostram a relevância da pesquisa de erros inatos do metabolismo em crianças com atraso no desenvolvimento. A triagem urinária ainda é um recurso laboratorial importante a ser utilizado, na investigação inicial de erros inatos.

Objectives: to investigate the frequency of positive urinary screening for inborn errors of metabolism among children with delayed development and to correlate clinical characteristics of the population studied with the screening results. Methods: this was a cross-sectional descriptive study, performed within the Early Stimulation Program of the Mother-Child and Adolescent Referral Unit of the State of Pará, on 197 children with delays development, by means of undertaking qualitative urine tests. Results: around 52.8% (104/197) of the patients presented positive results for at least one test. Children originating from other municipalities outside of Belém, the State Capital, had a higher proportion of positive tests, with p=0,003*. There was a higher proportion of positive tests when similar cases were reported in the family, or when there were stillbirths or deaths among siblings. Among the clinical manifestations investigated, convulsions and feeding problems had prominent frequencies. Conclusions: the results encountered show the relevance of research regarding inborn errors of metabolism among children with delayed development. Urinary screening is also an important laboratory tool for use in initial investigations.

Serum immunoreactivity to S-100 in children with cerebral palsy and delayed development and in their healthy parents

The passive immunization of pregnant female rats to S-100 protein often leads to ultra-structural abnormalities in the brain glial structures of the offspring of these rats and induces signs of delayed development in the fetal brain. Additionally passive immunization of pregnant animals with certain antigens induces permanent Ag-specific changes in the immune response of their offspring. The purpose of this study was to investigate serum immunoreactiviy (SIR) to S-100 in cerebral-palsied and developmentally-delayed children as well as in their healthy parents and to evaluate its significance related to radiologic findings of brain MRI and single photon emission computed tomography (SPECT). The subjects were children with cerebral palsy and delayed development that had abnormal findings on brain MRI or Brain SPECT. SIR to S-100 protein was measured by ELISA method in the patients, their healthy parents, 20 normal adult controls and 22 normally developed children. The SIR to S-100 protein was significantly higher in the cerebral-palsied and developmentally-delayed children when compared to that of the normal control group children. Increased SIRs were detected in healthy mothers but not in their fathers. There was no difference of SIR between the cerebral-palsied and developmentally-delayed children or any significant difference of SIRs according to the findings of the brain MRI or to developmental quotients. But, the SIRs to S-100 protein were higher in the group of more abnormal findings on brain SPECT.

Prospective evaluation of perinatal risk factors for cerebral palsy and delayed development in high risk infants

Prematurity, intrauterine infection and perinatal brain injury have been reported to be significant risk factors of cerebral palsy (CP). We examined the perinatal predictors of cerebral palsy and delayed development (DD) in 184 high risk infants. Thirty-five infants were diagnosed as cerebral palsy and delayed development at 12 months corrected age. Antenatal, intrapartum, and neonatal factors were prospectively evaluated in 2 groups of high risk infants compared with controls; Group A (n = 79), infants weighing less than 2,000 g; Group B (n = 43), infants weighing 2,000 g or more. In univariate analysis, there were no significant antenatal and intrapartum factors associated with cerebral palsy and delayed development in either group. We found that significant postnatal risk factors of CP in group A included sepsis (p = 0.008), BPD (bronchopulmonary dysplasia) (p = 0.028), IVH (intraventricular hemorrhage) (p = 0.042), ventriculomegaly (VM) (p = 0.001) and a longer duration of mechanical ventilation (p = 0.001); while in group B, sepsis (p = 0.047) and neonatal seizure (p = 0.027) were significant risk factors. In multivariate analysis, sepsis in group B was a moderate risk factor of CP (OR (odds ratio) 1.47; 95% CI (confidence interval) 1.02-2.13). In conclusion, neonatal sepsis may contribute to the development of cerebral palsy and delayed development. We suggest that high risk infants who have sepsis should be carefully followed for cerebral palsy and delayed development. The prevention of cerebral palsy may be feasible by decreasing neonatal risk factors such as sepsis during the neonatal period.

Early Treatment Effect in Children with Cerebral Palsy and Delayed Development

OBJECTIVE: To compare the effects of early and late treatment and to evaluate the contributing factors for the therapeutic outcome in cerebral palsy children. METHOD: Three hundred twenty four children with delayed development including cerebral palsy who were admitted to the Department of Rehabilitation at Yonsei University Medical Center from January 1992 to December 1995 were studied. They were divided into two groups according to the initiation of treatment: early and late treatment groups. Early treatment was defined as treatment started at or before 6 months and the late treatment was after 6 months. They were also divided into groups according to the diagnosis, responsiveness to treatment and initial motor quotient. And then the motor developments during the follow-up period between each groups were compared as the effects of treatment. RESULTS: The development of the motor milestone was faster in the early treatment group than in the late treatment group. The group which initially showed a higher motor quotient had a higher response rate to the treatment and a faster development of the motor milestone than the group with a lower motor quotient. CONCLUSION: These results suggest that the initiation of treatment and the severity of delayed development are important contributing factors for an outcome of treatment.

Gillespie Syndrome with Partial Aniridia, Cerebellar Ataxia, Delayed Development: A case report

In 1965 Gillespie reported a new syndrome of bilateral aniridia, cerebellar ataxia, and oligophrenia (mental retardation). This new syndrome was named Gillespie syndrome. Since then only 17 cases of Gillespie syndrome have been reported in UK, Brazil, Ireland, Belgium, Australia, and US. A case of Gillespie syndrome was not reported in Korea. A 4 year-old girl has triad of Gillespie syndrome, which are partial aniridia, cerebellar ataxia and mental retardation. We confirmed this with ophthalmologic examination, brain MRI, and developmental delay. We report the typical manifestation of Gillespie syndrome in a 4 year-old girl with the brief review of literature.

Comparison of Speech-Language, Mental-Motor Development and Brain Radiologic Findings in Children with Cerebral Palsy and Other Delayed Development

OBJECTIVE: To evaluate the characteristics of speech-language development and to find out the relationship between them and radiological findings, and mental/motor developmental quotient in the children with cerebral palsy and other delayed development. METHOD: Fifty-eight children with cerebral palsy or delayed development were evaluated with Bayley scales of infant development, brain magnetic resonance imaging (MRI), and single photon emission computerized tomography (SPECT). At the same time, the speech-language development using several evaluation batteries was assessed. RESULTS: Most of the children with cerebral palsy or delayed development showed delay in speech-language development. There was no relationship between speech-language development and presence of the lesion on brain MRI or SPECT, and mental/motor developmental quotient. CONCLUSION: Speech-language development was delayed in most of the children with cerebral palsy or other delayed development. Therefore, early interventions for speech-language development and comprehensive speech therapy are required for improving functional outcome in these children.

Effect of Seizure Disorders on Developmental Disability in Patients with Cerebral Palsy or Delayed Development

OBJECTIVE: To investigate the effect of epilepsy and neonatal seizure on development in children with cerebral palsy (CP) or delayed development (DD). METHOD: The subjects were 135 patients with CP or DD. Development was evaluated by Bayley Scale of Infant Development II (BSID II). Epilepsy was diagnosed on the base of clinical features, past history and electroencephalography. RESULTS: The incidence of epilepsy was 18.4% in CP, and 37.8% in DD. Spastic quadriplegia of CP has the highest incidence of epilepsy (38.4%). First seizure attack was occurred before 6 months old of age in 66.7% of CP with epilepsy and in 64.3% of DD with epilepsy. The prevailing type of epilepsy was generalized seizure in DD (57.1%), partial seizure in CP (50.0%). The group with epilepsy had lower psychomotor and mental development quotient on BSID II than the group without epilesy (p<0.05). Polytherapy was more used to control epilepsy than monotherapy. Valproate (50.0%), phenobarbital (37.5%), carbamazepine (31.3%) were commonly used drugs for controlling epilepsy. CONCLUSION: The epilpesy has a negative effect on psychomotor and mental development in the children with CP or DD.

The Development of Evaluation Tool for the Early Diagnosis of Cerebral Palsy

Early diagnosis of cerebral palsy may help to provide early therapeutic intervention. The early treatment of cerebral palsy is critical in the prevention of deformity and promotion of normal movement patterns. However, it is difficult to identify those who are at substantial risk of cerebral palsy before the age of one, particularly in its mild and moderate forms. The purpose of this study was to analyze and establish an efficient early diagnostic tool for cerebral palsy. The subjects in this study were 247 infants selected from outpatients at the Rehabilitation Hospital of Yonsei University from December, 1987 to March, 1996. The infants had problems in motor development or a past history of perinatal risks and all were under one year of corrected age. The assessments included a structured developmental history taken from the child's parents, evaluation of motor development status, and neurological examiniations including assessment of muscle tone, several primitive reflexes and Vojta's postural reactions. These infants were assessed every 2-3 months. According to the findings of developmental milestones, muscle tone, and Vojta's postural reactions, we divided the patients into high and low risk groups. The final diagnosis confirmed that 126 children had cerebral palsy, 43 children had other diagnoses while 78 children turned out to be normal. We found that diagnosis of cerebral palsy during the first six months of postnatal period relied mainly on abnormal muscle tone, Vojta's postural reactions, developmental motor delay, a positive finding of asymmetric tonic neck reflex and a negative finding of optical righting reaction. The sensitivity and specificity of the evaluation methods were 91.1% and 66.7%, respectively in the first six months of postnatal period. In conclusion, we have proved that the delayed motor development, abnormal muscle tone and abnormal Vojta's postural reactions are very valuable tools for the early diagnosis of cerebral palsy.

Psychological characteristics of mothers of children with disabilities

The purpose of this study was to investigate the psychological characteristics of mothers of children with disabilities. This study was performed under the hypotheses that, at the initial diagnosis of the children's disabilities, (1) the mothers suffered from serious psychological distress; (2) the more severe the child's disability was, the more serious the mother's psychological distress was; and that (3) the mother's psychological distress might be resolved within 8 weeks of rehabilitational interventions. The results were as follows: 1) mothers of children with disabilities showed significant (p<0.05) somatization, depression, anxiety, hostility, and phobic anxiety more so than the control group; 2) there was no significant difference in T scores of 9 dimensions of the Symptom Checklist-90-Revision (SCL-90-R) of the mothers at the initial diagnosis of children's disabilities according to severity of child's disability; 3) there was no significant difference in T scores of 9 dimensions of SCL-90-R in mothers of children with disabilities between at initial evaluation and after 8 weeks of rehabilitational interventions. In conclusion, : 1) mothers of children with disabilities suffered from serious psychological distress at the initial diagnosis of their child's disability; 2) the severity of the child's disability had little influence on the degree of the mother's initial psychological distress; 3) this distress did not resolve with only 8 weeks of rehabilitational interventions. Therefore, effective rehabilitational programs should provide sufficient opportunities for repeated follow-up interviews which offer not only adequate information on the children's disabilities but also psychological support for the mothers.

Linear and whorled nevoid hypermelanosis with delayed psychomotor development

We report a case of a 25-month-old girl presented to us for the evaluation of a severe delayed psychomotor development who also has pigmentary abnormalities. Linear and whorled hyperpigmentations following Blaschko's lines were noticed on her entire body except on her face, palms, soles, eyes and mucous membranes, which closely resembled those found in hypomelanosis of Ito, but inversely pigmented. Histologic examination revealed basal layer hyperpigmentation without incontinence of pigment or dermal melanophages. Chromosomal analysis of cultured peripheral leukocytes and fibroblasts from the hyperpigmented and the hypopigmented skin revealed normal female karyotype with no evidence of mosaicism or chimerism. This entity represents a kind of neurocutaneous syndrome-referred to by some authors as linear and whorled nevoid hypermelanosis.

Neonatal Neurosonographic Findings, Cerebral Doppler and Electrophysiological Findings in Infants with Motor Idsturbance

PURPOSE: Neurosonography, cerebral doppler and electrophysiological study are valuable in predicting neurodevelopmental outcome. The purpose of this study was to analyze characteristics of neonatal neurosonographic findigs, cerebral doppler and electrophysiological findings in infants withmotor disturbance. METHODS: The subjects were 33 infants hospitalized at Yonsei Severance Medical Center NICU from January, 1990 to December, 1992. 19 of these patients underwent neonatal cranial ultrasonogram and cerebral doppler and were diagnosed with cerebral palsy(CP) after Vojta treatment. 14 patients alose received Vojta treatment after discharge but showed only delayed development(DD). The cases were studied retrospectively and color doppler sonograpy was performed within 3 days of birth, 5-7 days after birth and weekly thereafter, and the cerebral blood flow velocity of the anterior cerebral artery was measured and comparison between normal pre-term and full-term infants done. Electroencephalogeraphy(EEG) and brainstem auditory evoked potential(BSAEP) were performed before discharge, with follow-up studies done only in cases showing abnormal findings, whereas visual evoked potential(VEP) was performed after discharge interhemispheric fissure was seen in 7 cases of CP and 1 cases of DD, showing a singnificatn frequency in CP, whereas there was no difference in the linear echogenecity of the basal ganglia. 4) Abnormal EEG was seen in 12 cases(63%) of CP group and 4 cses(29%) of DD group. The follow up EEG was normalized in all DD patients but CP patients conhtinues to show significant EEG abnormalities (7 cases out of 9). BSAEP and VEP abnormalities showed no significant difference between the 2 groups. 5) There was no significant difference in the average resistance index(RI) which was calculated from the cerebral blood flow velocity measured by cerebral doppler among normal newborns, CP and DD patents group. But average RI of CP patients was significantly lower than that of normal newborns, indicating an increase in cerebral blood flow in early life with severe neurologic sequelae. CONCLUSIONS: Neurosogography, cerebral doppler and electrophysiological studies performed in the perinatal period of children with motor disturbance show characteristic abnormal finding, with significant differences among childrem diagnosed with CP and DDD.

Neonatal Neurosonographic Findings, Cerebral Doppler and Electrophysiological Findings in Infants with Motor Idsturbance

PURPOSE: Neurosonography, cerebral doppler and electrophysiological study are valuable in predicting neurodevelopmental outcome. The purpose of this study was to analyze characteristics of neonatal neurosonographic findigs, cerebral doppler and electrophysiological findings in infants withmotor disturbance. METHODS: The subjects were 33 infants hospitalized at Yonsei Severance Medical Center NICU from January, 1990 to December, 1992. 19 of these patients underwent neonatal cranial ultrasonogram and cerebral doppler and were diagnosed with cerebral palsy(CP) after Vojta treatment. 14 patients alose received Vojta treatment after discharge but showed only delayed development(DD). The cases were studied retrospectively and color doppler sonograpy was performed within 3 days of birth, 5-7 days after birth and weekly thereafter, and the cerebral blood flow velocity of the anterior cerebral artery was measured and comparison between normal pre-term and full-term infants done. Electroencephalogeraphy(EEG) and brainstem auditory evoked potential(BSAEP) were performed before discharge, with follow-up studies done only in cases showing abnormal findings, whereas visual evoked potential(VEP) was performed after discharge interhemispheric fissure was seen in 7 cases of CP and 1 cases of DD, showing a singnificatn frequency in CP, whereas there was no difference in the linear echogenecity of the basal ganglia. 4) Abnormal EEG was seen in 12 cases(63%) of CP group and 4 cses(29%) of DD group. The follow up EEG was normalized in all DD patients but CP patients conhtinues to show significant EEG abnormalities (7 cases out of 9). BSAEP and VEP abnormalities showed no significant difference between the 2 groups. 5) There was no significant difference in the average resistance index(RI) which was calculated from the cerebral blood flow velocity measured by cerebral doppler among normal newborns, CP and DD patents group. But average RI of CP patients was significantly lower than that of normal newborns, indicating an increase in cerebral blood flow in early life with severe neurologic sequelae. CONCLUSIONS: Neurosogography, cerebral doppler and electrophysiological studies performed in the perinatal period of children with motor disturbance show characteristic abnormal finding, with significant differences among childrem diagnosed with CP and DDD.