Use of ultrasound shear wave to measure muscle stiffness in children with cerebral palsy.

PURPOSE: Cerebral palsy (CP) is a disorder characterized by an increased muscle stiffness that can be contingent on both neurological and biomechanical factors. The neurological aspects are related to hyper-excitability of the stretch reflex, while the biomechanical factors are related to modifications in muscle structure. We used smart-shear wave elastography (S-SWE) to analyze muscle properties and to compare shear wave speed in soleus muscles of patients affected by CP and typically developing children. METHODS: We enrolled 21 children (15 males and 6 females; age range 3-16) with spastic hemiplegia CP and 21 healthy children (11 males and 10 females; age range 3-14). Measurements of soleus S-SWE were performed using a Samsung RS80A ultrasound scanner with Prestige equipment (Samsung Medison Co. Ltd., Seoul, Korea), with a convex array transducer (CA1-7; Samsung Medison Co. Ltd., Seoul, Korea). For each CP child clinical assessment included Modified Ashworth Scale (MAS) score. RESULTS: Children with CP showed greater S-SWE values than the healthy ones (p < 0.001). Our data suggest a significant correlation between the S-SWE values and the MAS scores (Spearman correlation coefficient 0.74; p < 0.001 at Kruskal-Wallis test) in children with CP. CONCLUSIONS: Measuring muscle properties with SWE, a non-invasive and real-time technique, may integrate the physical exam. SWE may be a reliable clinical tool for diagnosis and longitudinal monitoring of muscle stiffness, as well as particularly suitable for grading and for assessing the response to treatments.

Accelerated bone turnover identifies hemiplegic patients at higher risk of demineralization.

Immobilization osteoporosis represents a severe complication in hemiplegic patients (HPs), causing fragility fractures, which may occur during rehabilitation reducing functional recovery and survival. The aim of the study was to investigate determinants of bone loss, independent from length of immobilization, which may be useful in early identification of HPs at higher risk of demineralization. Forty-eight HPs of both sexes underwent anthropometric measurements, evaluation of scores of spasticity and of lower limb motory capacity. Laboratory tests were performed. On serum: calcium; phosphorus; creatinine; ALP; iPTH; 25(OH) vitamin-D; sex hormones; Δ4-androstenedione; DHEA-S; insulin; IGF-1; FT3; FT4; TSH; c-AMP. On urine: c-AMP and calcium/creatinine ratio. Two bone turnover markers were measured: serum osteocalcin (BGP) and urinary deoxypyridinoline (DPD). Bone mineral density was determined at both femoral necks, defining a percentage difference in bone loss between paretic and non-paretic limb, thus controlling for the complex cofactors involved. Only bone turnover markers significantly and directly correlated with the entity of demineralization, controlling for age, sex and length of immobilization in the multivariate analysis (BGP coefficient estimate=0.008; SE=0.003; p=0.020; DPD coefficient estimate=0.005; SE=0.002; p=0.036). BGP and DPD are not dependent on anthropometric and endocrine-metabolic parameters, disability patterns and duration of immobilization, thus represent independent determinants of the degree of demineralization. A cutoff was defined for BGP and DPD above which subjects show significantly greater risk of demineralization. The immobilization event generates more severe bone loss when it occurs in subjects with higher bone turnover. BGP and DPD measurements may be of primary importance for early identification of HPs at risk, with relevant preventive implications.

Nutritional status in active juvenile chronic arthritis not treated with steroids.

Nutritional status and nutrient intake were assessed in 17 children with active juvenile chronic arthritis (JCA) who never received steroids and in 17 controls matched for age and sex. Five patients had systemic, seven polyarticular and five oligoarticular JCA. Values significantly below those of the controls were found in systemic patients for height (p<0.05), upper arm circumference (p<0.05) and arm muscle area (p<0.01), and in polyarticular subjects for arm muscle area (p<0.01). All patients had unremarkable anthropometric fat measurements. All anthropometric measurements were normal in oligoarticular patients. Twelve JCA patients had reduced serum iron (Fe), 6 reduced serum zinc (SZn), 14 reduced intra-erythrocytic zinc (EZn) and 2 reduced serum copper (SCu). SZn was inversely correlated with erythrocyte sedimentation rate (ESR) (p=0.023). EZn was inversely related to lymphocyte count (p=0.022). SCu was related to ESR (p=0.037) and to lymphocyte count (p=0.016). No significant difference in nutrient intake was found between patients and controls. Active JCA was associated with reduced muscular mass, Fe, SZn, EZn. These alterations did not depend on reduced nutrient intake.

The biomechanics of pelvic osteotomy according to the Chiari method.

The authors analyze the biomechanical effectiveness of pelvic osteotomy according to the Chiari method. This type of operation reduces joint loading and increases the joint support surface. The authors examined 18 patients aged an average of 31 years. This series shows that the effectiveness of pelvic osteotomy according to the Chiari method is particularly observed in cases in which joint morphology is best preserved.

A case of osteoporosis associated with adenosine deaminase deficiency.

The authors report a case of early and marked osteoporotic syndrome in a patient affected with adenosine-deaminase deficiency (ADA) of the heterozygotic type. The negative results obtained in all the tests carried out to ascertain any dysmetabolic, nutritional or iatrogenic disease, and the literature reporting homozygotic patients with osteometabolic and skeletal changes similar to those observed in this particular patient, led the authors to suggest a possible pathogenetic role in ADA deficiency. They conclude by describing the mechanisms by which this enzymatic defect could determine the clinical picture, and by presenting a possible type of treatment.

[Paget's disease: a case history, clinical and therapeutic contribution]. / Il morbo di Paget: contributo casistico, clinico e terapeutico.

The authors, after referring the various pathogenetic hypotheses for Paget's disease (till the latest hypothesis of infection by slow-action virus) report their follow-up of forty-five patients with Paget's disease of bone; then explain their diagnostic method, and record complications stressing the sarcomatous degeneration. Finally they give details on their therapeutic treatment emphasizing the need of hematochemical and radiographic periodical controls in order to optimize the treatment case by case.

[Osteitis condensans ilii: therapy and diagnostic problems. Presentation of a case study]. / L'osteite condensante dell'ileo: Problemi di diagnosi e terapia. Contributo casistico.

The authors describe 4 female patients with Osteitis Condensans Ilii (OCI): 3 of them were pluriparous, ranging 32-36 years of age; while the remaining was nulliparous and 20 years old, with a previous trauma suffered in the iliac bone. Due to the inefficacy of the medical and physical treatment, two patients underwent surgical resection of the osteitic bone; then followed by a 45 days cast immobilization and a 3-4 months with restricted load. The other 2 patients were successfully treated with Indomethacin (a 100 mg/day cycle for a month, followed by a maintainance cycle with 300 mg/day for 3 months). The diagnostic aspects are then discussed, pointing out that absence of any peripheric neurologic damage, normal bone scan, female sex and younger age are the relevant characteristics for the diagnosis of OCI.

[Chondroepiphyseal pathology in childhood hypothyroidism. Problems of diagnosis and prevention]. / La patologia condroepifisaria in corso di ipotiroidismo nell'infanzia: problemi di diagnosi e prevenzione.

Congenital hypothyroidism may present in the differential diagnosis of hypoplastic changes of the femoral heads. Failure to recognize early this entity may result in severe developmental changes. Thus, a team effort among member of different subspecialties (paediatrics, orthopedics and radiology) is necessary for prompt and effective treatment. This paper presents and discusses some cases of the disease as examples of such approach.