Growth Charts for Children With Down Syndrome: 1 Month to 18 Years of Age

Cronk, Christine E.; Crocker, Allen C.; Pueschel, Siegfried M.; Shea, Alice M.; Zackai, Elaine H.; Pickens, Gary; Reed, Robert B.

doi:10.1542/peds.81.1.102

Cited by 348 publications

(54 citation statements)

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“…The shorter length of the cochlea and smaller tympanic cavity (Figure 10; Table 1) are likely related to the smaller body weight in DS mice compared to WT littermates (Figure 2). These observations are consistent with the reduced size and slow growth rates in patients with DS (Cronk et al, 1988;Styles et al, 2002).…”

Section: Hair Cells Cochlear Length and Body Weightsupporting

confidence: 89%

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Hearing impairment in murine model of Down syndrome

Chen¹,

Li²,

McCall

et al. 2022

Front. Genet.

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Hearing impairment is a cardinal feature of Down syndrome (DS), but its clinical manifestations have been attributed to multiple factors. Murine models could provide mechanistic insights on various causes of hearing loss in DS. To investigate mechanisms of hearing loss in DS in the absence of the cadherin 23 mutation, we backcrossed our DS mice, Dp(16)1Yey, onto normal-hearing CBA/J mice and evaluated their auditory function. Body weights of wild type (WT) and DS mice were similar at 3-months of age, but at 9-months, WT weighed 30% more than DS mice. Distortion product otoacoustic emissions (DPOAE), a test of sensory outer hair cell (OHC) function negatively impacted by conductive hearing loss, were reduced in amplitude and sensitivity across all frequencies in DS mice. The middle ear space in DS mice appeared normal with no evidence of infection. MicroCT structural imaging of DS temporal bones revealed a smaller tympanic membrane diameter, oval window, and middle ear space and localized thickening of the bony otic capsule, but no gross abnormalities of the middle ear ossicles. Histological analysis of the cochlear and vestibular sensory epithelium revealed a normal density of cochlear and vestibular hair cells; however, the cochlear basal membrane was approximately 0.6 mm shorter in DS than WT mice so that the total number of hair cells was greater in WT than DS mice. In DS mice, the early and late peaks in the auditory brainstem response (ABR), reflecting neural responses from the cochlear auditory nerve followed by subsequent neural centers in the brainstem, were reduced in amplitude and ABR thresholds were elevated to a similar degree across all frequencies, consistent with a conductive hearing impairment. The latency of the peaks in the ABR waveform were longer in DS than WT mice when compared at the same intensity; however, the latency delays disappeared when the data were compared at the same intensity above thresholds to compensate for the conductive hearing loss. Future studies using wideband tympanometry and absorbance together with detailed histological analysis of the middle ear could illuminate the nature of the conductive hearing impairment in DS mice.

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Section: Hair Cells Cochlear Length and Body Weightsupporting

confidence: 89%

“…Growth retardation and short stature are cardinal features of humans with DS ( Cronk et al, 1988 ; Myrelid et al, 2002 ; Styles et al, 2002 ). Therefore, we recorded the body weights of 30 mice at 3 and 9 months of age (six male DS, nine female DS, nine male WT and six female WT).…”

Section: Resultsmentioning

confidence: 99%

Hearing impairment in murine model of Down syndrome

Chen¹,

Li²,

McCall

et al. 2022

Front. Genet.

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“…11 The other findings that may occur more frequently were omphalocele, tracheoesophageal fistula, pyloric stenosis, ileal and jejunal atresia, imperforated anus. 7,12,13,14…”

Section: Gastrointestinal Abnormalitiesmentioning

confidence: 99%

Study of the clinical features of Downs’s syndrome and relation between dermatoglyphics and congenital heart disease if any

Joseph¹,

Teja

Srinivas

2022

ijhs

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Introduction: Down syndrome is one of the most common cause of congenital malformation with mental retardation seen with an estimated prevalence of 1/600 – 1/800 births. A wide range of abnormalities are seen, some of which are characteristic of Downs syndrome. Objectives and Aim: The present study was aimed at studying the various clinical features including dermatologlyphic patterns in Down syndrome and to find any correlation with congenital heart disease. Materials and Methods: 34 children £18 years with features of down syndromes were assessed for various clinical presentation and dermatoglyphic patterns analyzed. Those with congenial heart disease were also noted to check for any correlation with dermatoglyphic patterns in Downs syndrome.3 ml blood was drawn and thyroid function were recorded. Results: The majority of clinical features observed include slanted palpebral fissure, icrocephaly, epicanthal fold, macroglossia, hypotonia, flat facies, brachycephaly, high arched palate. Dermatoglyphic patterns show increased prevalence of simian crease clinodactly ulnal loops on the finger tips and atd angle >45 in the children. Statistically significant correlation was found when clinodactly of the left hand, left third inter digital space pattern, open field defect and sandal gap of the toes was compared with down syndrome children with CHD.

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“…4,5 Diagnosis-specific growth curves have been developed for conditions, such as Down syndrome and Turner syndrome where genetic abnormality directly influences stature. [6][7][8][9] Reference growth curves for children with CP may be helpful to clinicians and beneficial to children; however, any representative sample is heterogeneous and likely to include many children with differing degrees of acute and chronic malnutrition and, possibly, GH deficiency. 10 The CP charts demonstrate significant deviations from general population reference centiles, with deviations increasing with age and with severity of functional disability (as measured by Gross Motor Function Classification System [GMFCS] levels).…”

Section: Introductionmentioning

confidence: 99%

Cerebral palsy specific growth charts for Indian children: need of the hour

Choudhary

Inamdar

Mauskar

2022

Int J Contemp Pediatr

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Background: Children with cerebral palsy (CP) are generally undernourished and growth retarded. The nutritional assessment and anthropometry have important role in management of these problems. Reference growth curves for Indian children with CP may be helpful to clinicians and beneficial to children for better assessment of nutritional status and to monitor growth. This study was conducted to compare the anthropometric inferences derived from specific growth curves for CP developed by centers for disease control (CDC) with that of using WHO and IAP growth curves.Methods: Cross sectional observational study conducted on 40 children with CP aged 2 to 12 year. Through universal sampling. Anthropometric variables measured and plotted on age and gender specific growth curves for cerebral palsy and on WHO / IAP growth curves. Nutritional assessments on these growth curves are then compared.Results: Number of underweight children were more on WHO/IAP growth curves 52.5% (n=21) compared to CP specific growth curves 20% (n=8) (p=0.002) similarly, number of stunted children were more on WHO/IAP growth curves (40% versus 0%) compared to CP specific growth curves (p=0.0001) both being statistically significant. No significant difference was seen on comparing BMI (p=0.067).Conclusions: Significant difference in nutritional status is observed when anthropometric variables are compared on two curves (CP specific versus WHO/IAP curves). Applying WHO/IAP growth curves, to assess the growth of children with cerebral palsy may not be appropriate and may have fallacies with interpretations of growth and nutritional status. Apart from nutrition, functional status and co morbidities also play a vital role in deciding nutritional status. Disease specific or standardized growth charts for Indian children with CP may play an important role in early diagnosis and management of growth faltering.

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Growth Charts for Children With Down Syndrome: 1 Month to 18 Years of Age

Cited by 348 publications

References 0 publications

Hearing impairment in murine model of Down syndrome

Hearing impairment in murine model of Down syndrome

Study of the clinical features of Downs’s syndrome and relation between dermatoglyphics and congenital heart disease if any

Cerebral palsy specific growth charts for Indian children: need of the hour

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