Bone mineral density and biochemical and hormonal indicators in children with quadriplegic cerebral palsy / Densidad mineral ósea e indicadores bioquímicos y hormonales en niños con parálisis cerebral cuadripléjica

Introduction: children with cerebral palsy (CP) have multiple risk factors for low bone mineral density or osteoporosis. Objective: to explore the association between bone mineral density (BMD) and biochemical and hormonal indicators of bone metabolism in children with quadriplegic cerebral palsy (CP). Methods: a cross-sectional analytical study included 59 participants from six to 18 years of age with quadriplegic CP. Serum concentrations of calcium, phosphorus, 25OHD metabolite, parathyroid hormone (PTH), alkaline phosphatase, and thyroid hormones were determined using standardized methods. The BMD measurement was obtained from the lumbar spine expressed in g/cm2 and Z-score. Unpaired Student's t-test, Chi-square test, odds ratio and Pearson's correlation were performed. Results: participants with CP and malnutrition had lower serum concentrations of calcium, phosphorus and alkaline phosphatase. Those who had low BMD showed lower serum concentrations of calcium, phosphorus and alkaline phosphatase. Most participants with low and normal BMD had vitamin D deficiency (27.1% and 10%) and insufficiency (35.4% and 30%), respectively. There was a significant correlation between BMD and serum concentrations of calcium, phosphorus, alkaline phosphatase, vitamin D and thyroid-stimulating hormone (TSH). There were no differences in the biochemical and hormonal indicators by level of gross motor function, use of anticonvulsants and oral versus enteral feeding method. Conclusion: malnutrition and alteration of vitamin D nutritional status were associated with low BMD and alterations of biochemical indicators of bone metabolism in pediatric patients with quadriplegic CP. The relationship between BMD and biochemical indicators of bone metabolism in children with quadriplegic CP was also demonstrated

Introducción: los niños con parálisis cerebral (PC) presentan múltiples factores de riesgo de densidad mineral ósea baja u osteoporosis. Objetivo: explorar la asociación entre la baja densidad mineral ósea (DMO) e indicadores bioquímicos y hormonales del metabolismo óseo en niños con PC cuadripléjica. Métodos: un estudio transversal analítico incluyó a 59 participantes de entre seis y 18 años de edad con PC cuadripléjica. Las concentraciones séricas de calcio, fósforo, metabolito 25OHD, hormona paratiroidea (PTH), fosfatasa alcalina y hormonas tiroideas se determinaron utilizando métodos estandarizados. La medición de DMO se obtuvo de la columna lumbar expresada en g/cm2 y puntaje Z. Se realizaron pruebas t de Student no pareada, Chi-cuadrado, razón de momios y correlación de Pearson. Resultados: los participantes con PC y desnutrición tenían concentraciones séricas más bajas de calcio, fósforo y fosfatasa alcalina. Los participantes con DMO baja tuvieron menor concentración sérica de calcio, fósforo y fosfatasa alcalina. Los participantes con DMO baja y normal tenían deficiencia de vitamina D (27.1% y 10%) e insuficiencia (35.4% y 30%), respectivamente. Hubo una correlación significativa entre DMO y las concentraciones séricas de calcio, fósforo, fosfatasa alcalina, vitamina D y hormona estimulante de la tiroides. Conclusión: la desnutrición y la alteración del estado nutricio de la vitamina D se asociaron con DMO baja y alteraciones de los indicadores bioquímicos del metabolismo óseo. Se demostró una asociación entre DMO e indicadores bioquímicos y hormonales del metabolismo óseo en niños con PC cuadripléjica

Assessment of anthropometric indicators in children with cerebral palsy according to the type of motor dysfunction and reference standard / Evaluación de indicadores antropométricos en niños con parálisis cerebral de acuerdo con el tipo de disfunción motora y estándar de referencia

Aim: The study aimed to demonstrate that the assessment of the anthropomorphic measurements of children with cerebral palsy (CP) varies according to the type of motor dysfunction and references standard used for comparison. Method: In a cross-sectional design, 108 children 2 to 16 years were classified according to the type of motor dysfunction by gender and age group. Weight, mid-upper-arm-circumference (MUAC), and alternative measures for height were performed. Height/age and weight/age indexes and BMI were evaluated with percentiles and/or Z-scores with reference to a number of previously published references of growth, including those of the World Health Organization (WHO). Results: Fifty-three (49.1%) were females and 55 (50.9%) males. Spastic type was predominant (73.1%) and 26.9% were other types of dysfunction. Most of the children were located on level IV (14.6%) and level V (73.1%) of the Gross Motor Function Classification System (GMFCS). Significant differences were found, suggesting that weight (p = 0.002), height (p = 0.001), and MUAC (p = 0.05) are higher in the spastic group than in other groups. Conclusions: The anthropometric indicators were significantly higher in the spastic group than in other groups. Upper-arm length (UAL) seemed less appropriate than knee height (KH) and lower-leg length (LLL) for measuring height. The WHO reference standard was not useful to evaluate the majority of anthropometric indexes in children with CP, other references as the growth charts of Day and Brooks have been more suitable (AU)

Objetivo: demostrar que la evaluación de las mediciones antropomórficas de los niños con parálisis cerebral (PC) varía según el tipo de disfunción motora y la referencia estándar utilizada. Método: en un diseño transversal se incluyeron 108 niños de 2 a 16 años clasificados de acuerdo con el tipo de disfunción motora por sexo y grupos de edad. Se obtuvieron el peso, circunferencia media de brazo y mediciones alternas para la talla. Los índices talla/edad, peso/edad y el IMC fueron evaluados con los percentiles y/o puntuaciones Z con referencia a estándares de crecimiento previamente publicados, incluyendo los de la Organización Mundial de la Salud (OMS). Resultados: cincuenta y tres (49.1%) eran mujeres y 55 (50.9 %) hombres. Predominó la PC tipo espástico (73.1%) y 26.9% otros tipos de disfunción. La mayoría de los niños se encontraron en el nivel IV (14.6%) y en el nivel V (73.1 %) de la Gross Motor Function Classification System (GMFCS). Se encontraron diferencias significativas, lo que sugiere que el peso (p = 0,002), talla (p = 0,001), y la circunferencia media del brazo CMB (p = 0,05) son mayores en el grupo espástico que en otros grupos. Conclusiones: los indicadores antropométricos fueron mayores en el grupo espástico. La longitud del brazo pareció menos apropiada que la altura de la rodilla y la longitud de la pierna para la medición de la talla. El estándar de crecimiento de la OMS no resultó útil para evaluar los índices antropométricos en niños con parálisis cerebral; otras referencias como las tablas de crecimiento de Day y Brooks fueron más adecuadas (AU)

A girl with spastic tetraparesis associated with biotinidase deficiency.

Biotinidase deficiency is a disorder of biotin metabolism that manifests with cutaneous, ophthalmological and neurologyical symptoms in childhood. Spinal cord involvement has rarely been reported and all of the reported cases are spastic paraparesis. A 3 year-old girl with biotinidase deficiency was admitted to our clinic with hyperventilation, hair loss and spastic tetraparesis. To our knowledge, our case is the first reported tetraparesis associated with biotinidase deficiency. She was treated with oral biotin and benefited significantly from this therapy.

[Celiac crisis with quadriplegia due to potassium depletion as presenting feature of celiac disease]. / Crise coeliaque associée à une tétraplégie par hypokaliémie de déplétion révélatrice d'une maladie coeliaque.

INTRODUCTION: Adult coeliac disease revealed by coeliac crisis and quadriplegia due to potassium depletion is an extremely rare situation. CASE REPORT: A 26-year-old woman presented with a suddenly developed weakness of all four limbs and a severe diarrhea. Authors emphasize coeliac crisis, which is a presenting feature of coeliac disease, characterized by acute diarrhea with life-threatening acid base and electrolyte abnormalities. The patient improved with correction of hypokalemia and gluten-free diet. CONCLUSION: A severe acute diarrhea with metabolic and systemic complications, the so-called coeliac crisis, is a possible presenting clinical feature of a previously undiagnosed adult celiac disease.

Effect of nutritional support in children with spastic quadriplegia.

Malnutrition is a common problem in patients with cerebral palsy. We evaluated the effect of nutritional support on clinical findings in children with spastic quadriplegia. Feeding history, numbers of lower respiratory tract infections, and gastrointestinal and neurologic findings were evaluated via questionnaire. Weight, height, head circumference, midarm circumference, and triceps skinfold thickness were measured. Height for age, weight for age, weight for height, body mass index, and weight and height z-scores were calculated. Clinical findings and anthropometric parameters were re-evaluated after nutritional support for 6 months. Forty-five patients were enrolled. No difference was evident between the first and the last height z-scores of 31 patients who completed the follow-up. Weight, height, weight z-scores, weight for age, weight for height, body mass index, midarm circumference, and triceps skinfold thickness exhibited improvement. Moreover, a significant decrease in number of infections was evident. Frequency of seizures and Gross Motor Function Classification System status did not change. Constipation decreased significantly. Nutritional therapy revealed improvements in some anthropometric findings and a decrease in number of infections. Although there was no difference regarding motor development or seizure frequency, further studies with a longer follow-up are required.

Energy requirements of spasticity.

Direct measurement of energy expended by spasticity in children with severe spastic quadriparesis is difficult. Insertion of an intrathecal baclofen pump in a 13-year-old boy with severe spasticity and profound mental retardation resulted in an estimated 30 to 40% decrease in his spasticity. As he had been on a carefully calculated ketogenic diet and fed by gastrostomy, his precise caloric intake was known. Decrease in spasticity, on the same caloric intake, led to marked weight gain. Reduction of 100 calories intake resulted in new weight stability. It was possible therefore, to estimate indirectly energy used by his spasticity. This 100 calories, representing 34% of calories above his resting energy requirement, corresponded to an independently estimated 30 to 40% of caloric expenditure of his spasticity. It was concluded that when calculation of calories is critical, energy utilization by spasticity must be taken into consideration.

Nutrition in spinal cord injury patients.

The nutrient intake and some indices of nutritional state were studied in 22 quadriplegic and 15 paraplegic patients. When measured over two 5-day periods, the average caloric intake recommended for comparable healthy men (2,700 kcal/day) was not achieved by 75% of the quadriplegic and 47% of the paraplegic patients. No overt signs of malnutrition or vitamin deficiencies were observed, but the average hematocrit and serum albumin levels were at the lower limits of normal. The reduced energy needs of spinal cord patients should be considered in the development of desirable nutritional norms for this patient population.