Question: What is the effect of cyclic pamidronate administration on osteoporosis in children with β-thalassemia major? Finding: The dual-energy x-ray absorptiometry scan findings of children with β-thalassemia major and osteoporosis were improved after pamidronate administration. Meaning: Cyclic pamidronate effectively treated osteoporosis in children with β-thalassemia major. |
∙ The application of 3-dimensional (3D) printing in orthopedic oncology is summarized into bone and tumor modeling, patient-specific instruments (PSIs), custom-made implants, and tissue engineering. ∙ The 3D-printed customized implant is the most central application, while modeling and PSI often play adjunct roles. ∙ Short-term surgical outcomes of custom-made 3D-printed implants are promising. |
Question: Does steroid use for 2–6 weeks in children affect bone mineral content (BMC) or density (BMD)? Finding: Steroid use for 2–6 weeks significantly decreased BMC and BMD of the whole body, total body less the head, lumbar spine, and distal radius. A significant negative correlation was observed among BMD, duration, and cumulative dose. Meaning: Steroid use for 2–6 weeks in children negatively affected BMC and BMD. |
Question: What is the effect of maternal smoking during pregnancy (MSDP) on the risk of bone fractures in the offspring? Finding: After the adjustment for misclassification, MSDP may be associated with a 27% increased risk of bone fracture in the offspring (pooled odds ratio, 1.27; 95% confidence interval, 1.00–1.62; I2=0%; P=0.537) Meaning: Preventive measures and health education programs should be designed and implemented to encourage women to stop smoking, especially during. |
Quadriplegic children with cerebral palsy are more susceptible to osteoporosis because of various risk factors that interfere with bone metabolism. Pamidronate is effective for pediatric osteoporosis, but there are no guidelines for optimal dosage or duration of treatment in quadriplegic children with osteoporosis. We aimed to evaluate the efficacy of low-dose pamidronate treatment in these patients. Ten quadriplegic patients on antiepileptic... |
Chronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by impaired phagocytic function. Hematopoietic stem cell transplantation (HSCT) is a definitive cure for CGD; however, the use of HSCT is limited because of associated problems, including transplantation-related mortality and engraftment failure. We report a case of a patient with CGD who underwent successful HSCT following a targeted busulfan and... |
To compare different regimens of vitamin D with respect to its serum increment levels and bone mineral accrual in vitamin D-deficient children. Children identified as being vitamin D deficient (serum levels<20 ng/mL) were divided into 3 treatment groups by stratified block randomization (group 1, 4,000 IU/day of vitamin D3 plus 50 mg/kg/day calcium for 12 weeks; group 2, 30,000 IU/wk of... |
Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of intermediate malignancy with resemblance to Kaposi sarcoma. It occurs predominantly in pediatric age groups as a cutaneous lesion with focal infiltration into the adjacent soft tissue and bone. Although visceral involvement is very uncommon, several cases with bone, retroperitoneal, or mediastinal involvement have been described. KHE has been reported... |
Inherited bone marrow failure syndrome (IBMFS) encompasses a heterogeneous and complex group of genetic disorders characterized by physical malformations, insufficient blood cell production, and increased risk of malignancies. They often have substantial phenotype overlap, and therefore, genotyping is often a critical means of establishing a diagnosis. Current advances in the field of IBMFSs have identified multiple genes associated with IBMFSs... |
As chemotherapy and other sophisticated treatment strategies evolve and the number of survivors of long-term childhood cancer grows, the long-term complications of treatment and the cancer itself are becoming ever more important. One of the most important but often neglected complications is osteoporosis and increased risk of fracture during and after cancer treatment. Acquisition of optimal peak bone mass and... |
Purpose : Several complications can occur in patients who received bone marrow transplantation (BMT) during childhood and adolescence. This study aims to investigate endocrine dysfunctions after BMT so that better care can be provided to care for long-term survivors of BMT. Methods : One hundred patients (61 males, 39 females) were included in this study. Clinical parameters such as initial diagnosis,... |
Peak bone mass is established predominately during childhood and adolescence. It is an important determinant of future resistance to osteoporosis and fractures to gain bone mass during growth. The issue of low bone density in children and adolescents has recently attracted much attention and the use of pediatric dual-energy X-ray absorptiometry (DXA) is increasing. The process of interpretation of pediatric... |
Purpose : Treatment of precocity with gonadotropin releasing hormone analogue (GnRHa) might theoretically exert a detrimental effect on the bone mass during pubertal development. We investigated the short-term changes in bone mineral density (BMD) during GnRHa treatment and the enhancement in the changes with the co-administration of GnRHa and human growth hormone (hGH). Methods : Forty girls with precocious or... |
The treatment of pediatric patients with chronic renal disease comprises management of nutritional imbalance, fluid, electrolyte, and acid-base disturbances, mineral bone disease, anemia, hypertension, and growth retardation. The treatment also includes administration of appropriate renal replacement therapy, if required. Adequate dietary intake of carbohydrates, fats, and proteins and caloric intake must be encouraged in such patients to ensure proper growth... |
Purpose:The differential diagnosis for a pulmonary nodule is intriguing in cancer patients. Metastasis might be a preferential diagnosis, and yet possibilities of other medical conditions still exist. Pulmonary tuberculosis should be enlisted in the differential diagnosis for a pulmonary nodule in cancer patients in Korea. This study was aimed at analyzing the incidence and clinical features of pulmonary tuberculosis that were misdiagnosed as... |
Purpose : Inhaled corticosteroids (ICS) are used as first-line agents for the treatment of persistent asthma; however, their use is accompanied by apprehension of potential systemic adverse effects. This study aimed to assess the effects of ICS on bone mineral density (BMD) and bone metabolism in children with asthma. Methods : From February 2008 to September 2008, 26 asthmatic children treated... |
Bone pain in a child could be associated with cancer as an initial manifestation of the disease. The childhood malignancies that frequently present bone pain are leukemia, neuroblastoma, and primary bone tumors such as osteosarcoma and Ewing sarcoma. Persistent bone or joint pain associated with swelling, mass, or limitation of motion implies underlying serious causes. Systemic manifestations such as lymphadenopathy,... |
Purpose : The hope that arresting pubertal developement might increase final adult height has led to an attempt to use GnRH agonist (GnRHa) in children with early puberty and poor growth prognosis. We investigated the growth-promoting effect of GnRH agonists with or without growth hormone (GH) in girls with early puberty and decreased predicted adult height (PAH). Methods : Thirty five... |
Purpose : The recent results observed in precocious puberty and the hope that interrupting puberty might increase adult height have led to an attempt to use GnRH agonist(GnRHa) in children with premature puberty and a poor growth prognosis. We aimed to analyze the growth promoting effect of GnRHa in girls with early puberty and low predicted adult height(PAH). Methods : Thirty... |
Purpose : Long-term administration of anticonvulsants in children with epilepsy may cause short stature, hypocalcemia and low bone mineral density. This study was performed for the early detection of abnormal bone metabolism in children with epilepsy on taking anticonvulsants. Methods : Thirty children aged 5 to 16 years who were diagnosed with epilepsy were enrolled in this study. All had taken... |
Isolated relapse of myeloid leukemia as a granulocytic sarcoma(GS) following allogeneic bone marrow transplantation(BMT) is very rare manifestation, and usually associated with a poor prognosis. We report a case of isolated intracranial GS in an infant with myelodysplastic syndrome(MDS) following unrelated BMT. A 7 month-old girl was diagnosed with refractory anemia with excess blasts (RAEB). During observation for a couple... |
Purpose : Chronic administration of anticonvulsants for children with epilepsy may cause multiple abnormalities in bone metabolism. The aim of this study was to evaluate the effects of anticonvulsants on bone mineral density(BMD) in children with epilepsy. Methods : Seventy patients with epilepsy were enrolled in this study. All subjects had taken anticonvulsants for at least 1 year or more.... |
Shwachman-Diamond syndrome(SD syndrome) is a rare genetic disorder chracterized by pancreatic insufficiency, short stature, skeletal abnormalities and bone marrow dysfunction. Exocrine pancreatic insufficiency and neutropenia are the main components of the syndrome. A hallmark of SD syndrome is varying severity of pancreatic dysfunction due to acinar maldevelopment. The hematologic abnormalities associated with SD syndrome include varying cytopenias, marrow aplasia, myelodysplasia... |
Purpose : To evaluate the effect of parenteral nutrition in early neonatal period on bone mineralization at the time of reaching 2 kg body weight in premature infants, bone mineral density(BMD) in infants who received long-term parenteral nutrition in the early neonatal period was measured and compared with infants who received enteral nutrition only. Methods : Twenty low birth weight infants(those... |
Purpose : The object of this study is to evaluate bone metabolism in healthy adolescents according to the progression of puberty. Methods : Forty boys(13.9?.7 years) and 42 girls(12.1?.6 years) were classified by Tanner stage (TS) and bone age. Serum levels of osteocalcin(OC) and bone specific alkaline phosphatase(BALP) were measured as bone formation markers. Serum level of C-terminal telopeptide of type... |
Purpose : The aim of this study is to find a method to diagnose and treat children who showed benign hemophagocytic histiocytes in bone marrow examination. Methods : We analyzed the clinical data of thirty patients retrospectively who showed benign hemophagocytic histiocytes in bone marrow examination from January 1995 to November 2001 at Keimyung University Dong-san Hospital. Bone marrow histiocytes... |
Purpose : Dynamics of bone mineral density(BMD) and bone metabolism in children and adolescents with hyperthyroidism have not been thoroughly investigated. The aim of this study was to study how the improvement of thyroid function with antithyroid treatment influenced bone metabolism and BMD in children and adolescents with hyperthyroidism. Methods : Serum levels of osteocalcin(OC), bone-specific alkaline phosphatase(b-ALP), and carboxyterminal telopeptide... |
A twenty six months-old boy developed hemophagocytic syndrome during the course of Kawasaki disease. Despite the appropriate treatment modalities for Kawasaki disease, he developed thrombocytopenia, hepatomegaly, high-grade fever, hypertriglyceridemia, peripheral gangrene, and evidence of hemophagocytosis in bone marrow biopsy. Although the course was stormy, he responded well to a combination therapy of corticosteroid and etoposide. |
Bone marrow necrosis is a rare complication of a variety of diseases affecting the marrow. The cause and incidence are unknown, and reports of treatment response are rare. We describe a case of relapsed acute mixed type leukemia with bone marrow necrosis. The patient was a 10 year old female diagnosed with acute mixed type leukemia four years ago. She... |
Lymphangiomatosis, a benign tumor consisting of a cluster of dilated lymphatic channels, is very unusual. Most lymphangiomatoses are found in the neck and head area. Less than 5% are diagnosed intraabdominally and they are very infrequently encountered in the retroperitoneal area. Herein, we report a rare case of asymptomatic lymphangiomatosis of a 13 year-old boy during a routine chest... |