Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.1.3.1 (alkaline phosphatase)
47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 6-year-old girl with cerebral palsy developed conscious disturbance and generalized convulsion after one-hour hot herb drug bath. Physical examination on admission revealed rectal temperature 41 degrees C, hot skin, respiration 46/min, regular heart beat 98/min, BP 130/60 mmHg, Glascow coma scale 4 (E2M1V1), soft and flat abdomen, no hepatosplenomegaly, no skin rash, no focal neurological sign, increased generalized muscle ton. Laboratory data showed CBC: WBC 20400 cumm (Neutrophils 31%, Lymphocytes 69%), Hb 11.6gm%, ESR 11 mm/hr, arterial blood gas: PH 7.077, PO2 43mmHg, PCO2 57.1mmHg, HCO3- 16 mEq/L, BE-11.5mEq/L, serum sodium 143 mEq./L, potassium 5.2 mEq/L, chloride 101 mEq/L, free calcium ion 3.8mg%, GOT 63IU/L, GPT 263 IU/L, amylase 193 IU/L, alkaline phosphatase 388 IU/L, LDH 1245 IU/L, CPK 677 IU/L, total bilirubin 0.8 mg/dl, direct type 0.1 mg/dl, BUN 18 mg/dl, Glucose 35 mg/dl. Urinalysis revealed proteinuria( ) trace hematuria and pyuria, but no cast. Lumbar puncture is within normal limits. Bacteriology including blood and CSF are normal. Multiple organ failure was noted at that time. Intensive cooling methods were performed including central and peripheral cooling. We used luminal and valium to control the seizure. Condition didn't improve. Afterwards cardiopulmonary arrest developed. Patient expired 8 hours after admission despite of resuscitation. Heat stroke in infancy and childhood is different from that in adulthood. The predisposing factors are high ambient temperature, dehydration, very young baby, sweat gland dysfunction, or ectodermal dysplasia. Definition of heat stroke includes 1) rectal temperature above 41 degrees C, 2) behavioral change, 3) warm skin, wet or dry.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Status epilepticus induced by prolonged immersion in hot herb bath: report of one case]. 263 19

Bone-mineral density was studied in a heterogeneous group of 139 children (mean age, nine years; range, three to fifteen years) who had spastic cerebral palsy. The evaluation included serum analyses and a nutritional assessment based on a dietary history and anthropometric measurements. The bone-mineral density of the proximal parts of the femora and the lumbar spine was measured with dual-energy x-ray absorptiometry and was normalized for age against a series of ninety-five normal children and adolescents who served as controls. Bone-mineral density varied greatly but averaged nearly one standard deviation below the age-matched normal means for both the proximal parts of the femora (-0.92 standard deviation) and the lumbar spine (-0.80 standard deviation). Ambulatory status was the factor that best correlated with bone-mineral density. Nutritional status, assessed on the basis of caloric intake, skinfolds, and body-mass index, was the second most significant variable. The pattern of involvement, durations of immobilization in a cast, and a calcium intake of less than 500 milligrams per day were additional factors of less significance. The age when the child first walked, previous fractures, use of anticonvulsants, and serum vitamin-D levels did not correlate with bone-mineral density after adjustment for covariance with the ambulatory status and the nutritional status. Serum levels of calcium, phosphate, alkaline phosphatase, and osteocalcin were not reliable indicators of low bone-mineral density.
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PMID:Bone-mineral density in children and adolescents who have spastic cerebral palsy. 759 76

Calcitriol (1,25-dihydroxy vitamin D) is an important hormone in calcium and phosphate metabolism. Levels of calcitriol and its precursor, 25-hydroxy vitamin D (calcidiol), were measured in a heterogeneous group of 125 noninstitutionalized children and adolescents with spastic cerebral palsy. Levels of each were correlated with: (1) clinical factors including mobility, prior fracture, and use of anticonvulsants; (2) nutrition and growth parameters including skinfolds, body mass index, and use of vitamin supplements; and (3) other serum analyses including osteocalcin as a marker of bone formation, calcium, and alkaline phosphatase. Levels of calcidiol and calcitriol did not correlate with any of the various clinical, nutritional, or growth parameters examined. The prevalence of low (< 10 ng/mL) levels of calcidiol was significant (19%), and dependent on the season of the year in which the level was measured. In contrast, less than 2% of the patients were found to have a low (< 20 pg/mL) level of calcitriol and the mean was comparable to normal pediatric subjects. Levels of calcitriol are maintained in noninstitutionalized children with cerebral palsy despite anticonvulsants, poor nutrition, and calcidiol levels that vary greatly with the seasons.
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PMID:Vitamin D levels in noninstitutionalized children with cerebral palsy. 937 1

A high incidence of long-bone fractures has been observed in children and young adults with quadriplegic cerebral palsy in residential care. This study aimed to determine factors that contribute to these fractures and to institute preventive treatment. Twenty individuals (12 males, eight females) of a cohort of 88 residents with spastic quadriplegia in residential care in Gauteng, South Africa who had sustained fractures were compared with a random sample of age-matched control participants (10 males, 10 females) from the same facility. Participants ranged in age from 6 to 29 years (median 17.5 years). The majority of fractures were in the upper extremities. There was radiological and biochemical evidence of rickets and osteomalacia in both groups. However, the severity of the disease was more pronounced in the group with fractures. There was a significant relation (p=0.002) between the number of fractures and the use of anticonvulsant therapy (ACT). Three months of vitamin D administration (calciferol 5000 iu/day) resulted in a marked clinical improvement. There were no fractures during this period in either group. In addition, the mean serum calcium (Ca) and phosphate (Pi) levels increased (Ca from 2.17 to 2.35 mmol/L and Pi from 1.13 to 1.66 mmol/L) and mean total alkaline phosphatase level decreased (from 1123 to 423 U/L). We concluded that vitamin D deficiency was the major factor contributing to the occurrence of fractures in this population. Unless sunlight exposure can be guaranteed, vitamin D supplementation should be considered for children and adults in residential care, especially if they are on ACT, even in areas with year-round sunshine.
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PMID:Pathological long-bone fractures in residents with cerebral palsy in a long-term care facility in South Africa. 1184 8

In the present study, bone mineral density of 40 children with cerebral palsy (study group) and the effects of various risk factors on bone mineralization in these children were investigated by comparing with 40 age-matched healthy children (control group). Weight, height, skinfold thickness, body-mass index measurements, and serum levels of calcium, phosphorus, alkaline phosphatase and 25 OH vitamin D were not significantly different between the study and control groups (p>0.05). The mean bone mineral density value of the study group measured by dual-energy X-ray absorptiometry method at L2-L4 levels of lumbar vertebrae was significantly lower than that of the control group (p<0.05). When the patients in the study group were assessed with respect to ambulation status, pattern of involvement, calcium and energy intakes, and whether or not they had taken and/or were taking a regular physical therapy program, there was a significant difference only between the hemiplegic and tetraplegic patients (p<0.05), while there were no significant differences among the patients who were ambulant versus non-ambulant, who had sufficient versus insufficient calcium and energy intakes, and who did and did not take a regular physical therapy (p>0.05). Although the ambulatory status, quantity of calcium and energy intakes, and the presence or absence of a physical therapy program had no effects on bone mineral density values of the children with cerebral palsy in this study, the exact factors and mechanisms responsible for the reduced bone mineral density in children with cerebral palsy should be investigated in further large-scale studies considering the increased risk of pathological fractures in these patients.
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PMID:Evaluation of bone mineral density in children with cerebral palsy. 1271 64

Several studies have examined bone mineral density (BMD) and related factors in children with cerebral palsy, but there are no such studies of adults with cerebral palsy. We evaluated BMD in 123 institutionalized adults (51 men aged 21-41 years and 72 premenopausal women aged 24-46 years) with cerebral palsy, and examined the associations of BMD with mobility level, use of anticonvulsant drugs, and abnormal calcium metabolism status. Hand radiographs were used to measure BMD of the second metacarpal bone (mBMD). Body weight (kg), height (m), and body mass index (BMI) were recorded. Serum calcium, phosphate, and alkaline phosphatase were measured. Abnormal calcium metabolism, defined as calcium <8.5 mg/dl, phosphate <2.6 mg/dl, or alkaline phosphatase >260 U/l, was identified in 28% of the men and 31% of the women. Multiple regression analysis showed that the use of anticonvulsant drugs was significantly associated with lower mBMD in both sexes. Higher alkaline phosphatase level was significantly associated with lower mBMD in men. Mobility level (ambulation) was significantly associated with higher mBMD in women. Neither age nor BMI correlated with mBMD. Our findings indicated poor bone health status in adults with cerebral palsy and the existence of several factors that could affect bone metabolism in these patients.
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PMID:Factors influencing metacarpal bone mineral density in adults with cerebral palsy. 1458 98

Osteopenia is common in children, adolescents, and young adults with severe cerebral palsy (CP; spastic quadriplegia) living in residential care, and frequently results in atraumatic fractures. On clinical grounds 67 patients (34 males, 33 females) with severe CP (gross motor function classification system [GMFCS] levels IV or V) aged 5 to 25 years (median 20 y) were divided into three groups with increasing likelihood of severe impairment of bone quality: (1) patients without fractures and without anticonvulsant medication (n=13); (2) patients without fractures and with anticonvulsant medication (n=45); (3) patients with fractures and with anticonvulsant medication (n=9). Evaluation included measurements of quantitative ultrasound (QUS) of the calcaneus, multiple serum analyses, and determination of urinary bone-resorption markers. Values of the quantitative ultrasound index (QUI) were significantly different (p=0.001): group 1 (median 56.9; interquartile range 43.8-75.3); group 2 (49.9; 40.0-60.0); group 3 (35.6; 30.5-38.5). when comparing values of laboratory serum and urine in the three groups, we found significant differences in values of serum bone alkaline phosphatase (p=0.001), serum parathyroid hormone (PTH; p=0.002), serum albumin (p=0.020), and urinary deoxypyridinoline/creatinine ratio (p=0.004). In multiple regression analysis, no laboratory variable was found to be an independent predictor of QUI. QUS of the calcaneus may be a useful method to assess bone quality and fracture risk in children and young adults with severe cp living in residential care, independent of information from laboratory data.
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PMID:Quantitative ultrasound of the calcaneus in children and young adults with severe cerebral palsy. 1617 14

The aim of this cross-sectional study was to investigate the frequency of decreased areal bone mineral density (aBMD) among patients with cerebral palsy (CP), as estimated by using various aBMD Z-score adjustment methods. In addition, this study examined factors related to decreased aBMD scores. One hundred and two children between the ages of 3.2 and 17.8 years were examined. In patients with severe CP, the incidences of decreased aBMD according to various adjusting methods based on decimal age, bone age, height age, and height-for-age Z-score (HAZ) were 79.5%, 69.5%, 51.9%, and 38.3%, respectively. Abnormal levels of calcium, phosphorus, alkaline phosphatase, parathyroid hormone, or anticonvulsant were not predictive for a decreased aBMD. Mean aBMD Z-scores were significantly lower in all aBMD Z-score adjustment methods in patients with severe CP compared to patients with mild-to-moderate CP, except for the adjustment method based on HAZ.
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PMID:Assessment of bone density in children with cerebral palsy by areal bone mineral density measurement. 2238 86