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Bone mineral density and biochemical variables of the IGF system in small for gestational age children
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ABSTRACTS / Bone 38 (2006) S1 – S17
significantly one month after the first supplementation(April): 35.3 T 4.4 ng/ml ( P < 0.001), and decreased significantly three months after the second supplementation: 22.4 T 4.6 ng/ml (September ( P < 0.001). No child had neither deficiency (<10 ng/ml) nor insufficiency (10 – 15 ng/ml) of vitamin D. On April, a month after the first supplementation, no children had levels of vitamin D intoxication (>50 ng/ml). Calcium intake were below to FDA recommendations: 770 T 420 mg/day. These results disclosed that double supplementation of 100,000 IU of vitamin D, on March (beginning of winter) and June (winter), is adequate and safe in the prevention of vitamin D deficiency for children at zones of risk at the south of our country. doi:10.1016/j.bone.2006.02.047
42 Familiar primary hyperparathyroidism associated to tumors in the maxilla M.C. Fernandez, M.S. Parisi, A. Bagur, B. Oliveri Seccio´n Osteopatı´as Me´dicas, Hospital de Clı´nicas, School of Medicin, UBA Familiar primary hyperparathyroidism associated to tumors in the jaw syndrome(HPT-JT) is an autosomal dominant predisposition to hyperparathyroidism(HPT), ossifying fibromas of the mandible and maxilla and renal cyst or Wilms tumor. A mutation of a suppression gene, the HPRT2, is responsible for this syndrome. Because cases of HPT TM present aggressive behaviour, parathyroid carcinoma must be ruled out. Diagnosis is performed isolating the mutation. Ossifying fibromas do not remit after parathyroidectomy (PTX). Objective: To present a case of HPT with clinical features of HPT TM. Case Report: A 39-year-old woman presented for consultation due to severe bone pain and 4 tumors in the maxilla with a 7 month course. Laboratory determinations revealed severe hypercalcemia, hypophosphatemia and increased intact PTH. Bone scintigram showed alterations in the calvaria and maxilla. Radiographs exhibited multiple lytic images. The patient presented renal lithiasis. Severe osteopenia was observed in total skeleton. Craniofascial CT scan revealed osteolysis in the left hemimaxilla extending to the midline and floor of the mouth. Upper right PTX was performed; histologic diagnosis was principal cell adenoma. PTH was normal postPTX. Based on the acute and aggressive presentation with bone and metabolism significantly compromised, the absence of remission of tumors, and reports of a sister with similar tumor, presumptive diagnosis was HPT TM. Genetic diagnosis remains pending. Conclusion: Cases of severe HPT associated to tumors in the maxilla must be evaluated rigorously ruling out parathyroid carcinoma, detecting associated tumors, and investigating alterations in the patient’s family. Patient follow-up postPTX is recommended due to possible recurrences. doi:10.1016/j.bone.2006.02.048
43 Bone mineral density and biochemical variables of the IGF system in small for gestational age children L. Silvano, A. Pe´rez, M. Miras, L. Mun˜oz, E. Pretel, G. Picotto, S. Martin, V. Elı´as, G. Sobrero, S. De Grandis, P. Armelini, N. Tolosa de Talamoni Hospital de Nin˜os, Bioquı´mica y Biologı´a Molecular, Ciencias Me´dicas, UNC. Univ. Cato´lica Co´rdoba, Argentina Body mass is associated with bone mineral density (BMD), which is determined by genetic and environmental factors. The present work was designed to relate BMD and calcium and phosphorus metabolism biochemical variables in small for gestational age (SGA) children, with vitamin D (VDR) and estrogen receptor (ER) genotypes. Children (n = 72) included controls, SGA with postnatal catch-up and without catch-up. Serum calcium and phosphorous, PTH, osteocalcin and beta cross-laps (EQLIA), IGF-I and IGFBP3 (IRMA) and acid labil subunit (ALS) by EIA, were measured. DNA was isolated from blood. VDR genotypes were determined using BsmI and FokI restriction enzymes, while for ER genotypes, PvuII y XbaI were employed. BMD were analyzed in lumbar spine and femoral neck using DEXA (Norland). SGA children without catch-up have lower femoral neck and lumbar spine BMD than those from SGA children with catch-up or control ones ( P < 0.01); they also showed lower levels of serum IGF-I, IGFBP3 and ALS ( P < 0.01). VDR and ER polymorphism distribution did not show statistically significant differences between groups. Nevertheless, a better femoral BMD in the control group is correlated with the Ff genotype. In conclusion, SGA children without catch-up have biochemical variables related to the IGF system completely different of those from other groups and lower BMD values at both skeletal sites. A BMD – FokI association is observed in control children while the same tendency has been shown in SGA children. doi:10.1016/j.bone.2006.02.049
44 In vivo effect of calcium (Ca) on brush border intestinal alkaline phosphatase (BBIAP) in the rat M.L. Brance, R.M. Brun, L. De Candia, A. Rigalli, R.C. Puche Lab Biol O´sea, Facultad Medicina, Rosario, Argentina In vitro, purified BBIAP binds Ca, is inhibited in a concentration dependent process, and displays molecular aggregation. The objective of this work was to investigate in vivo the effect of Ca on the activity and expression of the enzyme in the rat. Ten cm of the small intestine of adult rats were isolated by two ligatures: in the pylorous and 10 cm in caudal direction. In 4 treated rats, the isolated portion was instilled with 2 mL of a buffer containing CaCl2 50 mM. Four controls received the solution without Ca. Luminal samples
ABSTRACTS / Bone 38 (2006) S1 – S17
significantly one month after the first supplementation(April): 35.3 T 4.4 ng/ml ( P < 0.001), and decreased significantly three months after the second supplementation: 22.4 T 4.6 ng/ml (September ( P < 0.001). No child had neither deficiency (<10 ng/ml) nor insufficiency (10 – 15 ng/ml) of vitamin D. On April, a month after the first supplementation, no children had levels of vitamin D intoxication (>50 ng/ml). Calcium intake were below to FDA recommendations: 770 T 420 mg/day. These results disclosed that double supplementation of 100,000 IU of vitamin D, on March (beginning of winter) and June (winter), is adequate and safe in the prevention of vitamin D deficiency for children at zones of risk at the south of our country. doi:10.1016/j.bone.2006.02.047
42 Familiar primary hyperparathyroidism associated to tumors in the maxilla M.C. Fernandez, M.S. Parisi, A. Bagur, B. Oliveri Seccio´n Osteopatı´as Me´dicas, Hospital de Clı´nicas, School of Medicin, UBA Familiar primary hyperparathyroidism associated to tumors in the jaw syndrome(HPT-JT) is an autosomal dominant predisposition to hyperparathyroidism(HPT), ossifying fibromas of the mandible and maxilla and renal cyst or Wilms tumor. A mutation of a suppression gene, the HPRT2, is responsible for this syndrome. Because cases of HPT TM present aggressive behaviour, parathyroid carcinoma must be ruled out. Diagnosis is performed isolating the mutation. Ossifying fibromas do not remit after parathyroidectomy (PTX). Objective: To present a case of HPT with clinical features of HPT TM. Case Report: A 39-year-old woman presented for consultation due to severe bone pain and 4 tumors in the maxilla with a 7 month course. Laboratory determinations revealed severe hypercalcemia, hypophosphatemia and increased intact PTH. Bone scintigram showed alterations in the calvaria and maxilla. Radiographs exhibited multiple lytic images. The patient presented renal lithiasis. Severe osteopenia was observed in total skeleton. Craniofascial CT scan revealed osteolysis in the left hemimaxilla extending to the midline and floor of the mouth. Upper right PTX was performed; histologic diagnosis was principal cell adenoma. PTH was normal postPTX. Based on the acute and aggressive presentation with bone and metabolism significantly compromised, the absence of remission of tumors, and reports of a sister with similar tumor, presumptive diagnosis was HPT TM. Genetic diagnosis remains pending. Conclusion: Cases of severe HPT associated to tumors in the maxilla must be evaluated rigorously ruling out parathyroid carcinoma, detecting associated tumors, and investigating alterations in the patient’s family. Patient follow-up postPTX is recommended due to possible recurrences. doi:10.1016/j.bone.2006.02.048
43 Bone mineral density and biochemical variables of the IGF system in small for gestational age children L. Silvano, A. Pe´rez, M. Miras, L. Mun˜oz, E. Pretel, G. Picotto, S. Martin, V. Elı´as, G. Sobrero, S. De Grandis, P. Armelini, N. Tolosa de Talamoni Hospital de Nin˜os, Bioquı´mica y Biologı´a Molecular, Ciencias Me´dicas, UNC. Univ. Cato´lica Co´rdoba, Argentina Body mass is associated with bone mineral density (BMD), which is determined by genetic and environmental factors. The present work was designed to relate BMD and calcium and phosphorus metabolism biochemical variables in small for gestational age (SGA) children, with vitamin D (VDR) and estrogen receptor (ER) genotypes. Children (n = 72) included controls, SGA with postnatal catch-up and without catch-up. Serum calcium and phosphorous, PTH, osteocalcin and beta cross-laps (EQLIA), IGF-I and IGFBP3 (IRMA) and acid labil subunit (ALS) by EIA, were measured. DNA was isolated from blood. VDR genotypes were determined using BsmI and FokI restriction enzymes, while for ER genotypes, PvuII y XbaI were employed. BMD were analyzed in lumbar spine and femoral neck using DEXA (Norland). SGA children without catch-up have lower femoral neck and lumbar spine BMD than those from SGA children with catch-up or control ones ( P < 0.01); they also showed lower levels of serum IGF-I, IGFBP3 and ALS ( P < 0.01). VDR and ER polymorphism distribution did not show statistically significant differences between groups. Nevertheless, a better femoral BMD in the control group is correlated with the Ff genotype. In conclusion, SGA children without catch-up have biochemical variables related to the IGF system completely different of those from other groups and lower BMD values at both skeletal sites. A BMD – FokI association is observed in control children while the same tendency has been shown in SGA children. doi:10.1016/j.bone.2006.02.049
44 In vivo effect of calcium (Ca) on brush border intestinal alkaline phosphatase (BBIAP) in the rat M.L. Brance, R.M. Brun, L. De Candia, A. Rigalli, R.C. Puche Lab Biol O´sea, Facultad Medicina, Rosario, Argentina In vitro, purified BBIAP binds Ca, is inhibited in a concentration dependent process, and displays molecular aggregation. The objective of this work was to investigate in vivo the effect of Ca on the activity and expression of the enzyme in the rat. Ten cm of the small intestine of adult rats were isolated by two ligatures: in the pylorous and 10 cm in caudal direction. In 4 treated rats, the isolated portion was instilled with 2 mL of a buffer containing CaCl2 50 mM. Four controls received the solution without Ca. Luminal samples