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.
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.
213 www.journals.viamedica.pl/neurologia_neurochirurgia_polska Agata Czarnowska et al., SARS-CoV-2 infection in MS patients treated with disease-modifying therapies
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.
Aim: The main aim of this study was to determine some malnutrition risk factors among children with cerebral palsy (CP). Children with CP often require the assistance of physical therapy centers. Experience suggests that, apart from physical disabilities, this group often suffers from malnutrition. Methods: Data were gathered in the hospital among 128 children aged 3-18 years who were suffering from CP. The children were admitted from 2011 to 2013 to the Center for Neurological Physical Therapy for children in the Regional Hospital No. 2. St. Queen Jadwiga in Rzeszow (RORE). Statistical analyses were conducted for data on gender, age, type of CP, motor function level according to Gross Motor Function Classification Scale (GMFCS), body mass index (BMI) and hemoglobin levels in blood. Results: The risk of anemia differs based on gender - the risk is 6 times greater among boys than among girls (p = 0.0398). Risk of malnutrition is 3.5 times higher in children with tetraplegia than in children with diplegia or hemiplegia (p = 0.0043). Higher GMFCS scores are connected to greater proportions of malnourished children (for BMI z-score <-1.64, p = 0.0010). Conclusions: Among children with CP, malnourishment risk factors are male gender for anemia and tetraplegia and high GMFCS values.
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