Assessment of body adiposity in preterm children at the beginning of school age

et al. 2021

Sci Rep

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Microcephaly and macrocephaly can be considered both cranial growth defects and clinical symptoms. There are two assessment criteria: one applied in dysmorphology and another conventionally used in clinical practice. The determination of which definition or under which paradigm the terminology should be applied can vary on a daily basis and from case to case as necessity dictates, as can defining the relationship between microcephaly or macrocephaly and syndromes or diseases associated with neurodysfunction. Thus, there is a need for standardization of the definition of microcephaly and macrocephaly. This study was designed to investigate associations between abnormal cranial development (head size) and diseases or syndromes linked to neurodysfunction based on essential data collected upon admission of patients to the Neurological Rehabilitation Ward for Children and Adolescents in Poland. The retrospective analysis involved 327 children and adolescents with medical conditions associated with neurodysfunction. Two assessment criteria were applied to identify subgroups of patients with microcephaly, normal head size, and macrocephaly: one system commonly used in clinical practice and another applied in dysmorphology. Based on the results, children and adolescents with syndromes or diseases associated with neurodysfunction present abnormal cranial development (head size), and microcephaly rarely co-occurs with neuromuscular disease. Macrocephaly frequently co-occurs with neural tube defects or neuromuscular diseases and rarely with cerebral palsy (p < 0.05); microcephaly frequently co-occurs with epilepsy and hypothyroidism (p < 0.001). Traditional classification facilitates the identification of a greater number of relationships and is therefore recommended for use in daily practice. There is a need to standardize the definition of microcephaly and macrocephaly and to include them in ‘Human Phenotype Ontology’ terms.

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Abnormal cranium development in children and adolescents affected by syndromes or diseases associated with neurodysfunction

et al. 2021

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“…The z-score indicators were used to identify the developmental disorders described below. A similar use of the z-score indicators had been approved previously [ 13 , 23 , 26 ].…”

Section: Methodsmentioning

confidence: 99%

Abnormal Head Size in Children and Adolescents with Congenital Nervous System Disorders or Neurological Syndromes with One or More Neurodysfunction Visible since Infancy

2020

JCM

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The current study was designed to investigate co-occurrence of absolute/relative microcephaly, absolute/relative macrocephaly and congenital nervous system disorders or neurological syndromes with symptoms visible since infancy, based on fundamental data acquired during the admission procedure at a neurological rehabilitation ward for children and adolescents. The study applied a retrospective analysis of data collected during the hospitalization of 327 children and adolescents, aged 4–18 years, affected since infancy by congenital disorders of the nervous system and/or neurological syndromes associated with a minimum of one neurodysfunction. To identify subjects with absolute/relative microcephaly, absolute/relative macrocephaly in the group of children and adolescents, the adopted criteria took into account z-score values for head circumference (z-score hc) and head circumference index (z-score HCI). Dysmorphological (x+/−3s) and traditional (x+/−2s) criteria were adopted to diagnose developmental disorders of head size. Regardless of the adopted criteria, absolute macrocephaly often coexists with state after surgery of lumbar myelomeningocele and hydrocephalus, isolated hydrocephalus, hereditary motor and sensory polyneuropathy, and Becker’s muscular dystrophy (p < 0.001, p = 0.002). Absolute macrocephaly is often associated with neural tube defects and neuromuscular disorders (p = 0.001, p = 0.001). Relative microcephaly often occurs with non-progressive encephalopathy (p = 0.017, p = 0.029). Absolute microcephaly, diagnosed on the basis of traditional criteria, is often associated with epilepsy (p = 0.043). In children and adolescents with congenital nervous system disorders or neurological syndromes with one or more neurodysfunction visible since infancy, there is variation in abnormal head size (statistically significant relationships and clinical implications were established). The definitions used allowed for the differentiation of abnormal head size.

“…Normative values published earlier 13 were applied as a reference. This approach has been used previously 14 . The analyses examined the relationships between the co-occurring growth defects, in particular short stature, and the neurodysfunction diagnostic subgroups.…”

Section: Methodsmentioning

confidence: 99%

Growth disorders in children and adolescents affected by syndromes or diseases associated with neurodysfunction

2019

Sci Rep

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We have observed that one in three patients admitted to the Neurological Rehabilitation Ward for Children and Adolescents due to a syndrome or disease associated with neurodysfunction is short of stature for their age. In order to identify the relationship between growth defects (short stature) and syndromes or diseases associated with neurodysfunction, we analyzed data collected during admission to the Neurological Rehabilitation Ward for Children and Adolescents. The study applied a retrospective analysis of data collected during hospitalization of 327 children and adolescents, aged 4–18 years, affected by congenital disorders of the nervous system and/or neurological syndromes associated with a minimum of one neurodysfunction. Two assessment systems were taken into account – one system traditionally applied, and another one in accordance with indications approved by the Food and Drug Administration, related to diagnosing short stature. The findings show more frequent co-occurrence of, as well as statistically significant correlations between, short stature in certain groups: operated myelomeningocele with hydrocephalus in the subgroup of neural tube defects (p = 0.029), tetraplegia in the subgroup of spastic cerebral palsy (p < 0.001), and hypothyroidism (p = 0.04) in the entire study group.