Reduced bone mineral density in adult patients with Langerhans cell histiocytosis

This retrospective study evaluated bone mineral density (BMD) and bone turnover in adults with LCH. Twenty-five adult patients and 25 matched controls were evaluated with BMD measurement and indices of bone metabolism. A BMD value below the expected range for age (Z-score ≤ - 2.0) was found in 20% of patients; in particular, all postmenopausal women and men over 50-years had either osteoporosis or osteopenia. Patients with active disease had significantly lower Z-scores compared to patients with inactive disease and controls. Reduced bone turnover was found in all 14 patients treated with chemotherapy. No fractures due to osteoporosis were identified during 305.15 patient-years of follow-up.     

Low bone mineral density in children with Crohn's disease]

Recent studies have reported low bone mineral density in children with Crohn's disease. The aims of this retrospective study were to quantify its frequency and to search for risk factors. POPULATION AND METHODS: Bone mineral density of 29 children with Crohn's disease was measured by dual-energy X-ray absorptiometry. All the children were taking calcium and vitamin D, during all the follow-up. RESULTS: Osteoporosis (Z-score < or = -2.5 S.D.) was found in 38% of the children, and osteopenia in 38% (Z-score between -1 and -2.5 S.D.). Low bone mineral density was correlated with age, suggesting it begins with puberty. Daily corticosteroid exposure was significantly higher for patients with osteoporosis. Disease severity measured with Harvey-Bradshaw index and exposure to immunosuppressive drugs were almost statistically significant. Sex, height, duration and site of disease, nutritional assistance exposure were not associated with low bone mineral density. CONCLUSION: This study confirms the high frequency of low bone mineral density in children with Crohn's disease, mainly during puberty. Corticosteroid exposure is a risk factor, and the disease severity, a probable one (non significant). New treatment strategy has to be defined to prevent and to treat this complication.     

Late effects of treatment for germ cell tumors during childhood and adolescence

PURPOSE: To evaluate the long-term sequelae of treatment for malignant germ cell tumors (GCT) during childhood and adolescence. PATIENTS AND METHODS: Of 128 patients treated for GCT at St. Jude Children's Research Hospital between 1962 and 1988, 73 are long-term survivors (continuously disease-free for > or =5 years after diagnosis), with a median follow-up of 11.3 years). Survivors' ages at diagnosis ranged from birth to 18.3 years (median, 9.2 years); 64% (47 patients) were female. Initial surgical resection was followed by observation for stage I germinomas (n = 2), testicular tumors (n = 13), and selected cases of ovarian or sacrococcygeal tumors (n = 2), and by radiation therapy (RT) for patients with stage II to III germinoma (n = 8). The remaining 48 patients received postoperative chemotherapy (vincristine, dactinomycin, and cyclophosphamide [VAC] +/- doxorubicin, 1962 to 1978; VAC and/or cisplatin, vinblastine, and bleomycin [PVB], 1979 to 1988). RT was added to the chemotherapy for 21 patients. Late complications involving various organ systems and their relationship to treatment were evaluated. RESULTS: More than two-thirds of long-term survivors (n = 50) had at least 1 complication, and half (n = 38) had > 1 organ system affected. The systems most often involved included the musculoskeletal (41% of survivors), endocrine (42%), cardiovascular (16% excluding those who had only abnormal chest radiograph), gastrointestinal (25%), genitourinary tract (23%), pulmonary (19%), and neurologic (16%) systems. High-frequency hearing loss occurred in 58% (11 of 19) of patients treated with cisplatin. Musculoskeletal, gastrointestinal, and urinary tract abnormalities were most frequent in patients whose treatment included RT. CONCLUSIONS: A high frequency of late effects after treatment for pediatric GCT, particularly in patients who received RT, was demonstrated. Treatment sequelae could be anticipated from the intensity and type of therapeutic modalities. Treatment-directed screening evaluations may improve quality of life in long-term survivors of pediatric GCT through timely identification of sequelae that can be prevented or ameliorated.     

Bone mineral density in cystic fibrosis patients under the age of 18 years

AIM: The increase in life expectancy of cystic fibrosis (CF) patients has brought about a rise in new clinical problems in these patients, such as a decrease in bone mineral density (BMD). The cause of diminished BMD in CF is multi-factorial. METHODS: The aim of this cross-sectional study, conducted on 39 CF patients under the age of 18 years, was to evaluate the degree of bone mineralization and the prevalence of low BMD in these patients during a follow-up at the Cystic Fibrosis Regional Center of Tuscany, using a dual energy X-ray absorptiometry (DXA) scan, and to then study the factors correlated with low BMD. RESULTS: Areas BMD values (g/cm2) and Z-score values were determined. Eighteen patients (46%) out of the our sample had decreased BMD, while 21 patients (54%) had normal values. A statistically significant association was found between BMD Z-score values and pancreatic insufficiency, BMI<5th percentile and DeltaF508 homozygosis. Subjects treated with oral steroid therapy had a 3.9 times greater risk of developing osteoporosis compared to non-treated subjects (95% C.I.: 1.07-22.6; R.R. 4.9). An association was found between BMD Z-score values and FEV1 values (r=0.29; P=0.06), physical activity total score values (r=0.22; P=0.19) and the Chrispin-Norman chest radiographic score (r=-0.31; P=0.06). CONCLUSION: Early identification of reduced bone mass values would permit early intervention to prevent the development of osteoporosis. Maintaining pulmonary function, guaranteeing optimal nutritional status, following an adequate program of physical activity and controlling steroid intake could maintain BMD over time.     

Bone mineral density deficits in survivors of childhood cancer: long-term follow-up guidelines and review of the literature

The development of curative therapy for most pediatric malignancies has produced a growing population of childhood cancer survivors who are at increased risk for a variety of health problems resulting from their cancer or its treatment. Because of the fact that many treatment-related sequelae may not become clinically apparent until the survivor attains maturity or begins to age, the ability of primary care providers to anticipate late effects of treatment is essential for providing timely interventions that prevent or correct these sequelae and their adverse effects on quality of life. Altered bone metabolism during treatment for childhood cancer may interfere with attainment of peak bone mass, potentially predisposing to premature onset of and more severe complications related to osteopenia and osteoporosis. Bone mineral deficits have been reported after treatment for a variety of pediatric malignancies and represent morbidity that can be reduced or prevented through lifestyle changes and attention to other common cancer-related sequelae such as hypogonadism. The Children's Oncology Group long-term follow-up guidelines for survivors of childhood, adolescent, and young adult cancers provide risk-based surveillance recommendations that are based on expert opinion and review of the scientific literature for potential late effects of pediatric cancer therapy including osteopenia. This review summarizes the existing literature that has defined characteristics of cancer survivors at risk for bone mineral deficits and contributed to the surveillance and counseling recommendations outlined in the Children's Oncology group long-term follow-up guidelines.     

骨卡波西型血管内皮细胞瘤1例并文献复习

目的 探讨儿童骨卡波西型血管内皮细胞瘤(Kaposiform hemangioendothelioma,KHE)的临床特征 、病理特点 、诊疗方法 及预后情况,以提高对该病的诊治水平.方法 以2014年11月9日南京医科大学附属儿童医院骨科收治的1例儿童骨KHE患者为研究对象,并回顾分析其临床资料;检索维普、万方、CN...     

Osteopenia, physical activity and health-related quality of life in survivors of brain tumors treated in childhood

BACKGROUND: Osteopenia has been reported in children surviving acute lymphoblastic leukemia and brain tumors, apparently as a consequence of therapy. It has been suggested that cranial irradiation may play a role in the development of this complication. In order to explore that possibility, we examined survivors of brain tumors treated with and without radiation in childhood to investigate associations between radiation, osteopenia, physical activity, health status and overall health-related quality of life (HRQL). PROCEDURE: Subjects were survivors of posterior fossa tumors (astrocytoma or medulloblastoma) or optic glioma, < 18 years of age at diagnosis and > 1 year off treatment. Measurements of growth velocity, body composition, bone densitometry, physical activity and HRQL were undertaken. RESULTS: Twenty-five (62.5%) of the 40 eligible patients participated in the study. Of the 25 patients, 12 (48%) received radiation therapy (R group) while 13 received no radiation (NR group). Growth hormone (GH) deficiency had been detected in three subjects, one had completed GH therapy while two were still on hormone replacement. The prevalence of osteopenia was 44% in the entire group, and 67% versus 27% in the R and NR groups. Florid osteoporosis was present in 20% of the entire group, more than 40% of the R group but none of the NR group. A significant correlation (P < 0.01) was observed between overall HRQL and Z scores of bone mineral density (BMD) of the lumbar spine. Pain and ambulation/mobility utility scores correlated significantly (P < 0.05) with BMD, while levels of physical activity correlated (P < 0.05) with overall HRQL utility scores. CONCLUSIONS: This pilot study demonstrates that in survivors of brain tumors treated in childhood, radiation therapy is associated with significant loss of bone mineral. Among these survivors, HRQL is less, pain is more severe and ambulation is more restricted in those with low BMD scores. The reduction in HRQL is reflected in diminished physical activity. A larger multi-center study is needed to confirm these results.     

Late effects of therapy in adult survivors of osteosarcoma and Ewing's sarcoma

To study the late consequences of primary bone cancer, we interviewed 82 osteosarcoma and 29 Ewing's sarcoma survivors regarding their health, fertility and offspring, employment, annual income, and activities of daily living. All subjects had been diagnosed before age 20 (mean age, 14.6 years), had survived at least 5 years from diagnosis, and were at least 21 years of age. On average, they were 32.5 years of age at interview. As controls, 151 siblings were interviewed. During the follow-up period, eight survivors had died, and eight survivors had been diagnosed with a second malignancy (7.2%; P = .002). No other health condition distinguished survivors from controls. Although the survivors were more likely than controls to have some difficulty climbing stairs and to have had employment disability, employment status and annual income at follow-up were similar. Deficits in marriage and fertility were not significant. Adult survivors of primary bone tumors diagnosed during childhood or adolescence are at high risk for second malignancies and premature death, making continued medical follow-up of utmost importance. Despite the physical impairment following limb amputation for many, the majority of outcomes we measured did not differ from controls, suggesting few adverse psychosocial outcomes in this group of cancer survivors.     

Late effects of therapy in adult survivors of osteosarcoma and Ewing's sarcoma.

To study the late consequences of primary bone cancer, we interviewed 82 osteosarcoma and 29 Ewing's sarcoma survivors regarding their health, fertility and offspring, employment, annual income, and activities of daily living. All subjects had been diagnosed before age 20 (mean age, 14.6 years), had survived at least 5 years from diagnosis, and were at least 21 years of age. On average, they were 32.5 years of age at interview. As controls, 151 siblings were interviewed. During the follow-up period, eight survivors had died, and eight survivors had been diagnosed with a second malignancy (7.2%; P = .002). No other health condition distinguished survivors from controls. Although the survivors were more likely than controls to have some difficulty climbing stairs and to have had employment disability, employment status and annual income at follow-up were similar. Deficits in marriage and fertility were not significant. Adult survivors of primary bone tumors diagnosed during childhood or adolescence are at high risk for second malignancies and premature death, making continued medical follow-up of utmost importance. Despite the physical impairment following limb amputation for many, the majority of outcomes we measured did not differ from controls, suggesting few adverse psychosocial outcomes in this group of cancer survivors.     

Osteoporosis in children with severe congenital neutropenia: bone mineral density and treatment with bisphosphonates

A high incidence of decreased bone mineral density (BMD) has been described in patients with severe congenital neutropenia (SCN). The objectives of the study are to describe changes in BMD in children with SCN treated with granulocyte colony-stimulating factor and evaluate the response to treatment with bisphosphonates in those who had osteoporosis. A prospective open-label study was performed evaluating BMD and metabolism in 9 Chilean patients with SCN, administrating bisphosphonates in those with osteoporosis. Follow-up ranged between 7 months and 3.5 years. Six out of 9 patients had reduced BMD on initial assessment: 3 had osteoporosis (z score <-2) and 3 had osteopenia (z score <-1). Four children presented vertebral fractures. Two presented osteopenia on follow-up without clinical symptoms. Five patients were treated with bisphosphonates, increasing their BMD z score (mean increase 1.2, range 0.27 to 2.62). z Score of hydroxyproline/creatinine ratios, which was elevated in 4 patients with osteoporosis, decreased during treatment (mean decrease 2.18, range 1.56 to 2.53). Four patients remodeled and reexpanded fractured vertebrae during treatment. No side effects of bisphosphonates were seen on follow-up. Osteoporosis is an important comorbidity in SCN patients probably due to increased bone resorption. Bisphosphonates seem to be an effective treatment for osteoporosis in these patients.     

IS THE TREATMENT FOR CHILDHOOD SOLID TUMORS ASSOCIATED WITH LOWER BONE MASS THAN THAT FOR LEUKEMIA AND HODGKIN DISEASE?

Background: Cancer disease and its therapy (e.g., chemotherapeutic agents such as glucocorticoids, methotrexate, antymetabolities, cranial and local irradiation) may severely disturb normal growth, bone mineral acquisition, and skeletal development because most individuals go through the stages of rapid growth when childhood cancer is diagnosed. Procedures: To identify factors associated with reduced bone mineral density (BMD) in survivors of childhood cancer the authors examined 114 patients (70 males) who had been treated for acute lymphoblastic leukemia (ALL; n = 43), Hodgkin disease (HD; n = 35), and solid tumors (ST; n = 36) twice. Median age at diagnosis was 8.4 years; at the consecutive examinations it was 12.8 and 16.3 years, respectively. To assess bone density we used dual-energy x-ray absorptiometry (DXA). Results: In the first examination, patients with a history of steroid therapy had higher total and spine BMD and higher BMI (body mass index) than those who were not given steroids. At the end of the follow-up, no differences were found in BMD between subgroups, although BMI was still higher in both ALL and HD patients than in those with ST. Conclusions: Patients treated for solid tumors have reduced bone mass. Low BMI and local irradiation seem to be the factors responsible for reduced BMD in children treated for ST. The use of steroids does not disturb bone mass accumulation in patients treated for childhood malignancies. However, a long-term effect of cancer treatment on osteoporosis risk remains to be determined.     

No difference between prednisolone and dexamethasone treatment in bone mineral density and growth in long term survivors of childhood acute lymphoblastic leukemia

BACKGROUND: Dexamethasone is known to have both more potent leukemic activity and is associated with a higher incidence of side effects than prednisolone. In this study, we compared the long-term effects of dexamethasone and prednisolone on bone mineral density (BMD), body composition and growth in long-term survivors of ALL in first complete remission. PROCEDURE: Ninety patients (51 male, 49 female; 8.6-38.5 year), treated with either a prednisolone containing protocol (n = 47; n = 19 also with CNS-irradiation) or a dexamethasone containing protocol (n = 43; no cranial irradiation) participated in this cross-sectional single center study. Mean follow-up was 12.7 years (2.0-29.7 years). BMD of lumbar spine and total body, and body composition were expressed as standard deviation scores (SDS) using dual energy X-ray absorptiometry. Bone mineral apparent density of the lumbar spine (BMAD) was calculated to correct for bone size. RESULTS: There was no difference in height, height corrected for target height, BMD, or lean body mass between prednisolone and dexamethasone treated patients. Prednisolone treated patients had an increased percentage body fat (SDS +0.46; P < 0.05) and increased body mass index (SDS 0.88; P < 0.01) compared to normal. Dexamethasone treated patients had only an increased body mass index (SDS 0.52; P < 0.05). Height, total body BMD, and lean body mass were lower in patients treated with cranial irradiation as compared to non-irradiated patients, but differences in the latter two disappeared when corrected for height. BMAD was normal after CNS-irradiation. CONCLUSIONS: Long term survivors of ALL treated with prednisolone or dexamethasone containing regimens do not differ in height, BMD, or body composition.     

Hypothalamic dysfunction after chemotherapy

Cranial irradiation with or without chemotherapy can cause hypothalamic-pituitary dysfunction. Chemotherapy without cranial irradiation has not been thought to cause such deficiency. In order to determine whether chemotherapy without cranial irradiation can lead to hormonal deficiency, we reviewed the medical records of 362 childhood cancer patients who underwent full hypothalamic-pituitary evaluation because of altered growth and development after oncological therapy (1987-2002). Of these, 31 received chemotherapy but no cranial or total body irradiation and had no CNS tumor: 18 had hematological malignancy and 13 had a solid tumor of the torso or extremity. Duration of follow-up was 13.0 +/- 4.1 years (mean +/- SD). Growth hormone deficiency (GHD) was identified in 15 (48%), central hypothyroidism (TSH-D) in 16 (52%), and pubertal abnormalities in 10 (32%). Pubertal abnormalities included precocious puberty in two (6%), gonadal failure in five of 27 who were old enough to assess puberty (19%), and gonadotropin deficiency in three of 27 (11%). GHD and TSH-D were co-existent in eight patients (26%). Overall, 81% (n = 25) had GHD, TSH-D, precocious puberty, and/or gonadotropin deficiency. None had ACTH or ADH deficiency or primary hypothyroidism. Of note, this was not a study of prevalence, but rather an evaluation of clinically referred patients. In conclusion, hypothalamic dysfunction may occur in survivors of non-CNS tumors who receive chemotherapy but do not receive cranial irradiation. We recommend at least annual observation of growth rate and pubertal development of all children treated for pediatric malignancies, with evaluation for GHD, TSH-D, pubertal abnormalities, and other hypothalamic dysfunction in all poorly-growing cancer survivors, even those not treated with cranial irradiation.     

Bone density and 25-hydroxyvitamin D level in extrahepatic biliary atresia

Biliary atresia (BA) represents a common cholestatic affliction of the gastrointestinal tract affecting infants and children. The objective of the present study was to evaluate 42 patients (20 with and 22 without jaundice) diagnosed with extrahepatic BA for bone mineral content and serum 25-hydroxyvitamin D (HVD) levels. Physical examination and anthropometric nutritional assessment were performed. The investigation included liver function tests and serum calcium (Ca), phosphate (P), magnesium (Mg), and 25-HVD levels. Dual-energy X-ray absorptiometry was used to measure the bone mineral density (BMD) of the lumbar spine (L₁–L₄). Our results showed that 16 jaundiced␣patients (80%) and only 3 nonjaundiced patients (13.6%) showed osteoporosis (P< 0.05). All patients had normal serum Ca and P levels. Only 1 nonjaundiced patient had a low serum Mg level. Serum 25-HVD levels (mean ± SD) were 20.71 ± 8.24, 16.12 ± 4.3, and 9.18 ± 5.84 ng/ml, respectively, in subjects with normal bone density (n=7), osteopenia (n=3), and osteoporosis (n=11). Bone disease represents a well-known complication among long-term survivors of BA. To date, the pathogenesis has remained unexplained. Since, as demonstrated in the present study, jaundiced patients develop osteoporosis more frequently than nonjaundiced patients, hyperbilirubinemia may have an influence. Bone-mineral deficiency can be detected earlier by means of BMD measurement (non-invasive method) than by measuring serum Ca, P, and Mg levels in these patients. Accepted: 27 November 2000     

Ten years of extracorporeal membrane oxygenation: neurodevelopmental outcome.

Cf the 87 survivors of extracorporeal membrane oxygenation over a 10-year period, 67 participated in a follow-up study which included neurologic examination (n = 67), cognitive testing (n = 67), and audiologic assessment (n = 33). Matched control subjects for those older than 5 years were also evaluated. Outcome was defined as normal for cognitive scores greater than or equal to 85 and normal neurologic examination results, suspect for cognitive scores 70 through 84 or nonfocal neurologic findings such as hypertonia/hypotonia, and abnormal for cognitive scores less than 70 or abnormal neurologic examination results. Of the 10 school-aged children studied, 9 were normal and there were no differences in mean cognitive scores between subjects and controls (IQ subjects = 109 +/- 12 [SD], IQ controls = 107 +/- 13). For preschoolers aged 2.7 through 4.11 years, the mean cognitive score was 91 +/- 11 and 7 (70%) were normal. For infants 6 through 30 months, the mean cognitive score was 101 +/- 22 and 27 (57%) were normal. A total of 7 children (21% of those studied) had abnormal audiologic assessments. Three children demonstrated mild high-frequency and 4 moderately severe high-frequency sensorineural hearing loss which was bilateral in 3 and of undetermined laterality in 1. Abnormal neurodevelopmental outcome was significantly associated with cerebral infarction and chronic lung disease. Outcome was not related to demographic or perinatal variables, illness severity prior to extracorporeal membrane oxygenation, or underlying diagnosis. Neurodevelopmental outcome among survivors of extracorporeal membrane oxygenation in this series is consistent with previous reports of morbidity among neonates with severe respiratory failure treated conventionally.     

Body composition and bone mass in survivors of childhood cancer

BACKGROUND: The number of survivors of childhood cancer has increased. Several studies in children and adults have shown relationships between lean mass (LM), fat mass (FM), and bone mineral content (BMC). The objective of the study was to examine the association between body composition and bone mass in young survivors of childhood cancer. METHODS: Sixty-eight postpubertal participants (31 females and 37 males) aged between 15.5 and 27 years who were at least 5 years after completion of treatment for leukemia (n = 30), lymphoma (n = 28), or solid tumors (n = 10) were studied. Anthropometry was performed and dual energy X-ray absorptiometry (DXA) was used to assess BMC in the total body (T) and lumbar spine (S), FM, and LM. RESULTS: There were no observed differences in age or time for cessation of treatment. Body mass index (BMI) was a strong determinant of bone mass in both sexes. TBMC correlated positively with LM (males r = 0.9 and females r = 0.76; P < 0.0001, respectively) and with FM (r = 0.54; P < 0.01 in males and r = 0.8; P < 0.00001 in females). SBMC correlated with LM in both sexes (in males r = 0.77 and in females r = 0.64; P < 0.0001, respectively) but only in females, SBMC also correlated positively with FM (r = 44 P = 0.03). There were no differences between patients who received radiation and those who did not. CONCLUSIONS: The associations between bone mass and body composition differ by sex and skeletal site, however, they are similar in survivors of childhood cancer and compared to healthy individuals during growth. Further prospective research is needed in cancer survivors to determine the long-term effect of anti-cancer therapy on body composition and bone mass.     

High-grade astrocytoma in very young children

BACKGROUND: High-grade astrocytomas are rare in young children, but have been reported to have a better prognosis than similar tumors in older patients. PROCEDURE: We retrospectively reviewed the clinical characteristics, survival, and long-term sequelae for patients younger than 3 years old with high-grade astrocytoma, treated at a single institution between 1984 and 2005. RESULTS: Sixteen patients were included. Histology included anaplastic astrocytoma (n = 9), glioblastoma multiforme (n = 5), and malignant glioma (n = 2). All patients underwent biopsy or resection, followed by chemotherapy. Six patients received scheduled irradiation and six were irradiated at the time of disease progression. Ten patients are alive at a median follow-up of 11.6 years (range, 1.7-21.6 years). 5-year overall survival (OS) was 66.3% (SE 12.2%), and 5-year event-free survival (EFS) was 28.6% (SE 12.1%). Age at diagnosis was a significant predictor of the hazard of death in a Cox model (HR 2.871, 95%CI 1.015-8.123). Gender and histology did not predict OS or EFS. Trends toward improved OS were detected for patients with hemispheric tumors and those undergoing complete resection. All evaluable survivors (n = 9) had some neurocognitive impairment, with estimated overall cognitive ability ranging from significantly delayed to average; all survivors attending school (n = 5) performed below grade level on achievement testing. Seven of nine evaluable survivors had endocrine dysfunction. CONCLUSIONS: Young children with high-grade astrocytoma have better long-term overall survival than older patients, but recurrence is common, and most children require irradiation. Long-term complications are frequent and often severe.     

Bone densitometry in pediatric populations: discrepancies in the diagnosis of osteoporosis by DXA and CT

OBJECTIVES: To test the hypothesis that because of errors associated with growth and development, osteoporosis is frequently overdiagnosed in children when using dual-energy x-ray absorptiometry (DXA). This study compared bone density values obtained by DXA with those from computed tomography (CT), which is not influenced by body or skeletal size. STUDY DESIGN: Vertebral bone density was measured by using both DXA and CT in 400 children (100 each, healthy and sick boys and girls). Regression analysis was used to compare DXA and CT Z scores, and the agreement between DXA and CT classifications of Z scores below -2.0 was examined. RESULTS: DXA and CT Z scores were moderately related (r2 = 0.55 after accounting for age and anthropometric measures). DXA Z scores predicted CT Z scores below -2.0 with reasonable sensitivity (72%), specificity (85%), and negative predictive value (98%), but positive predictive value was low (24%). Many more subjects were classified as having bone density lower by DXA (76/400) than by CT (25/400), particularly subjects below the 5 th percentile of height and/or weight for age. CONCLUSIONS: The inability of DXA to account for the large variability in skeletal size and body composition in growing children greatly diminishes the accuracy of this projection technique for assessing bone acquisition and diagnosing osteoporosis in pediatric populations.     

Assessment of bone quality by quantitative ultrasound of proximal phalanges of the hand and fracture rate in children and adolescents with bone and mineral disorders

Bone quality by quantitative ultrasound and fracture rate were assessed in 135 (64 males) children and adolescents aged 3-21 y with bone and mineral disorders such as chronic anticonvulsants or glucocorticoids treatment, juvenile rheumatoid arthritis, celiac disease, paucity of intrahepatic bile ducts, autoimmune hepatitis, genetic diseases, idiopathic juvenile osteoporosis, disuse osteoporosis, beta-thalassemia major, survivors of acute lymphoblastic leukemia, liver transplantation, calcium deficiency, and nutritional or X-linked hypophosphatemic rickets. Amplitude-dependent speed of sound through the distal end of the first phalangeal diaphysis of the last four fingers of the hand was measured by an ultrasound device. In the majority of patients cortical area to total area ratio by metacarpal radiogrammetry (n = 120) and lumbar bone mineral density (BMD) by dual-energy x-ray absorptiometry (n = 99) were also assessed. In patients with X-linked hypophosphatemic rickets radial BMD by single-photon absorptiometry instead of lumbar BMD was measured. Mean values of amplitude-dependent speed of sound, cortical area to total area ratio, lumbar BMDarea, or lumbar BMD corrected for bone sizes estimated by a mathematical model (BMDvolume), as well as mean values of radial BMD in patients with X-linked hypophosphatemic rickets, expressed as z score, were significantly reduced (p < 0.0001) in comparison with their reference values (-1.7 +/- 1.0, -2.0 +/- 0.9, -3.0 +/- 1.3, -1.9 +/- 1.0, -2.7 +/- 0.7, respectively). A positive relationship was found between amplitude-dependent speed of sound and cortical area to total area ratio (r = 0.90, p < 0.0001), lumbar BMDarea (r = 0.62, p < 0.0001), or lumbar BMDvolume (r = 0.66, p < 0.0001). Fifty-two patients (38.5%) had suffered fractures in the 6 mo preceding the bone measurements, the radial distal metaphysis being the most frequent fracture site (28.8%). Mean values of amplitude-dependent speed of sound, cortical area to total area ratio, lumbar BMDarea, or lumbar BMDvolume, expressed as z score, of fractured patients were significantly lower (p < 0.0001) than those of fracture-free patients (-2.2 +/- 1.0 and -1.4 +/- 0.8, -2.6 +/- 0.9 and -1.7 +/- 0.7, -3.5 +/- 1.2 and -2.5 +/- 1.0, -2.5 +/- 1.0 and -1.3 +/- 0.7, respectively). Phalangeal quantitative ultrasound may be a useful method to assess bone quality and fracture risk in children and adolescents with bone and mineral disorders.     

Efficacy and safety of oral alendronate treatment in children and adolescents with osteoporosis

OBJECTIVES: To evaluate the efficacy and safety of oral alendronate on bone mineral density (BMD) in children and adolescents with osteoporosis. METHODS: Oral alendronate was given to 22 patients with an average age of 13.3 +/- 3.9 years (range 4.3-19 years) and BMD z-score < or = -2. Thirteen of them were treated with steroids. Dual-energy X-ray absorptiometry (DEXA) was used to measure lumbar vertebral BMD before and 14 +/- 7.75 months after treatment. Auxological, biochemical (Ca, P, alkaline phosphatase [ALP]) and densitometric parameters before and after treatment were compared for all patients. Responses of the patients taking steroids were compared with those who did not. RESULTS: Post-treatment z-scores of patients were significantly higher than basal values (p < 0.001). Average annual BMD increase with treatment was 32.74 +/- 52.57% (5.17 to 255.42%). Z-score and annual BMD increase in patients taking no steroids were significantly higher than the others (p = 0.020 and p = 0.006, respectively). Post-treatment serum ALP levels were significantly lower than their pretreatment levels (p = 0.007). After 1 year of treatment, osteoporosis completely recovered in six patients (28.6%), improved to osteopenia levels in seven patients (33.3%), continued although the z-score was increased in six patients (28.6%), and worsened in two patients (9.5%). There was no significant difference between the height standard deviation scores (SDS) of patients before and after treatment (p = 0.022). No side effect was observed due to alendronate treatment during the study. CONCLUSION: It is concluded that oral alendronate increases BMD without any side effects in osteoporotic children and adolescents, and it is cheaper and is easier to use than i.v. bisphosphonates.