NECROSIS OF THE FEMORAL HEAD AND HEALTH-RELATED QUALITY OF LIFE OF CHILDREN AND ADOLESCENTS.

OBJECTIVE: To verify the impact of avascular necrosis of the femoral head on the quality of life of children and adolescents with Perthes disease and sickle cell disease. METHODS: A comparative study including 24 children between eight and 18 years old with avascular necrosis of the femoral head secondary to Perthes disease and sickle cell disease (Group with Necrosis) and 24 children considered asymptomatic (Group without Necrosis). Clinical and sociodemographic data were collected and the PedsQL 4.0 and the Charnley score for hip dysfunction were applied. RESULTS: There was no difference in the overall score and in any domain when comparing the Perthes group (global = 73.1) and the sickle cell disease group (global = 65.9). When comparing the groups with necrosis and without necrosis, the Perthes group had a lower overall score only for the Physical Functioning domain (87.5 versus 68.5); sickle cell disease group had a lower overall score (64.9 versus 79.4) and in the Physical Functioning (68.5 versus 87.5) and School Functioning (62.9 versus 73.7) domains. CONCLUSION: Avascular necrosis of the femoral head produces lower quality of life scores both in the global evaluation and in the domains Physical Functioning and School Functioning. Necrosis, bilateral lesion, and hip function were found to be associated with the loss of quality of life. Level of Evidence III, Sectional comparative study.

OBJETIVO: Verificar o impacto da necrose avascular da cabeça do fémur sobre a qualidade de vida de crianças e adolescentes com doença de Perthes e anemia falciforme. MÉTODOS: Estudo comparativo com 24 crianças entre oito e 18 anos com necrose avascular da cabeça do fémur secundária à doença de Perthes e à anemia falciforme (grupo com necrose) e 24 crianças consideradas assintomáticas (grupo sem necrose). Foram coletados dados clínicos e sociodemográficos e aplicado o instrumento PedsQL 4.0 e o escore de Charnley para disfunção do quadril. RESULTADOS: Não houve diferença no escore global e em nenhum domínio comparando os grupos Perthes (global = 73,1) e anemia falciforme (global = 65,9). Quando comparados os grupos sem necrose e com necrose, nota-se que o grupo Perthes tem escore inferior apenas para o domínio Capacidade Física (87,5 versus 68,5); já o grupo anemia falciforme tem escore global inferior (64,9 versus 79,4) e também nos domínios Capacidade Física (68,5 versus 87,5) e Aspecto Escolar (62,9 versus 73,7). CONCLUSÃO: A necrose avascular da cabeça do fémur produz escores de qualidade de vida inferiores tanto na avaliação global, como nos domínios Capacidade Física e Aspecto Escolar. Foram identificadas presença de necrose, lesão bilateral e função do quadril como fatores associados à perda de qualidade de vida. Nível de evidência III, Estudo seccional comparativo.

Necrosis of the femoral head and health-related quality of life of children and adolescents / Necrose da cabeça do fêmur e qualidade de vida relacionada à saúde de crianças e adolescentes

ABSTRACT Objective: To verify the impact of avascular necrosis of the femoral head on the quality of life of children and adolescents with Perthes disease and sickle cell disease. Methods: A comparative study including 24 children between eight and 18 years old with avascular necrosis of the femoral head secondary to Perthes disease and sickle cell disease (Group with Necrosis) and 24 children considered asymptomatic (Group without Necrosis). Clinical and sociodemographic data were collected and the PedsQL 4.0 and the Charnley score for hip dysfunction were applied. Results: There was no difference in the overall score and in any domain when comparing the Perthes group (global = 73.1) and the sickle cell disease group (global = 65.9). When comparing the groups with necrosis and without necrosis, the Perthes group had a lower overall score only for the Physical Functioning domain (87.5 versus 68.5); sickle cell disease group had a lower overall score (64.9 versus 79.4) and in the Physical Functioning (68.5 versus 87.5) and School Functioning (62.9 versus 73.7) domains. Conclusion: Avascular necrosis of the femoral head produces lower quality of life scores both in the global evaluation and in the domains Physical Functioning and School Functioning. Necrosis, bilateral lesion, and hip function were found to be associated with the loss of quality of life. Level of Evidence III, Sectional comparative study.

RESUMO Objetivo: Verificar o impacto da necrose avascular da cabeça do fémur sobre a qualidade de vida de crianças e adolescentes com doença de Perthes e anemia falciforme. Métodos: Estudo comparativo com 24 crianças entre oito e 18 anos com necrose avascular da cabeça do fémur secundária à doença de Perthes e à anemia falciforme (grupo com necrose) e 24 crianças consideradas assintomáticas (grupo sem necrose). Foram coletados dados clínicos e sociodemográficos e aplicado o instrumento PedsQL 4.0 e o escore de Charnley para disfunção do quadril. Resultados: Não houve diferença no escore global e em nenhum domínio comparando os grupos Perthes (global = 73,1) e anemia falciforme (global = 65,9). Quando comparados os grupos sem necrose e com necrose, nota-se que o grupo Perthes tem escore inferior apenas para o domínio Capacidade Física (87,5 versus 68,5); já o grupo anemia falciforme tem escore global inferior (64,9 versus 79,4) e também nos domínios Capacidade Física (68,5 versus 87,5) e Aspecto Escolar (62,9 versus 73,7). Conclusão: A necrose avascular da cabeça do fémur produz escores de qualidade de vida inferiores tanto na avaliação global, como nos domínios Capacidade Física e Aspecto Escolar. Foram identificadas presença de necrose, lesão bilateral e função do quadril como fatores associados à perda de qualidade de vida. Nível de evidência III, Estudo seccional comparativo.

Acute cerebellar dysfunction with neuromuscular manifestations after scorpionism presumably caused by Tityus obscurus in Santarém, Pará / Brazil.

Scorpionism is a public health problem in many tropical countries, especially in North Africa, South India, Latin America and the Middle East. In Brazil, patients with severe scorpion envenoming have mainly cardiovascular events, including acute heart failure, acute respiratory distress syndrome and shock, death is rare. We described 58 accidents presumably caused by Tityus obscurus in Brazilian Amazonia. Patients reported a sensation of "electric shocks" which could last hours. The vast majority of patients presented a clinical picture compatible with acute cerebellar dysfunction, beginning minutes and lasting up to 2 days after the accident. They presented cerebellar ataxia, dysdiadochokinesia, dysmetry, dysarthria, dyslalia, nausea and vomiting. Besides, some patients presented myoclonus and fasciculation which can also be attributed to cerebellar dysfunction or maybe the result of direct action on skeletal muscle. Two patients had evidence of intense rhabdomyolysis and acute kidney injury. The clinical picture in this scorpion envenoming is mainly characterized by an acute dysfunction of cerebellar activities and abnormal neuromuscular manifestations and in some cases muscle injury which are not described in any other region of the world. This work presents clinical, epidemiologic, laboratory and treatment aspects of this unmatched scorpion envenoming in the state of Pará, northern Brazil.

Mesial temporal lobe epilepsy and hippocampal sclerosis: cognitive function assessment in Hispanic patients.

PURPOSE: The goal of this study was to assess the cognitive function status in patients with mesial temporal lobe epilepsy and hippocampal sclerosis (TLE+HS) to determine their cognitive function profile and to correlate material-specific memory deficits with HS laterality diagnosed by MRI. METHODS: Seventy-one patients were assessed with a neuropsychological protocol that includes IQ, attention, handedness, verbal memory, visual memory, language, and the executive function. chi(2) and correlation tests were used. RESULTS: Memory impairment was found in 46 patients (66%): patients without any memory deficit (n=25), patients with verbal memory deficit (n=21), patients with visual memory deficit (n=17), patients with deficit for both types of memory (n=8). Correlation between MRI lesion and memory was 66%. Language was impaired in 33 patients (46%). Eighteen patients (25%) had a deficit of the executive function. CONCLUSIONS: Patients with TLE+HS presented with a deficit in material-specific episodic memory correlating in large proportion with HS lateralization. We also found language and executive function impairments.