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https://www.readbyqxmd.com/read/27872411/osteoporosis-in-pediatric-liver-transplantation
#1
Walid Faraj, Ghina El Nounou, Abdallah Abou Al Naaj, Nancy Nakhoul, Ali Haydar, Mohammad Khalife
Liver transplantation provides an important, often life-saving treatment for end-stage liver disease. Osteoporosis post-liver transplantation has been described in adults; however, this has not been described in the pediatric population to date. We present a case of a 13-year-old female patient who underwent an orthotopic liver transplant for cryptogenic liver cirrhosis. Her immunosuppressants were tacrolimus and prednisone. Four months posttransplant, she started complaining of bilateral lower limb pain and limping while walking, progressing to a point where she was almost immobile...
December 2016: Progress in Transplantation
https://www.readbyqxmd.com/read/27824794/balloon-kyphoplasty-for-managing-intractable-pain-in-pediatric-pathologic-vertebral-fractures
#2
Jane S Hoashi, Stefanie M Thomas, Ryan C Goodwin, David P Gurd, Rabi Hanna, Thomas E Kuivila
BACKGROUND: Vertebral compression fractures are a common result of osteoporosis and osteopenia secondary to steroid use and chemotherapy treatment. Balloon kyphoplasty is a treatment option with good to excellent results well described in adults. Although a few recent studies have been published regarding the use of kyphoplasty in children, no formal indication exists for the pediatric population. The purpose of this study is to describe the outcomes of 3 chronically ill children with intractable pain from vertebral compression fractures, managed with kyphoplasty...
November 7, 2016: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/27821253/bone-mineral-density-in-children-with-idiopathic-nephrotic-syndrome
#3
Ghada Mohamed El-Mashad, Mahmoud Ahmed El-Hawy, Sally Mohamed El-Hefnawy, Sanaa Mansour Mohamed
OBJECTIVES: To assess bone mineral density (BMD) in children with idiopathic nephrotic syndrome (NS) and normal glomerular filtration rate (GFR). METHODS: Cross-sectional case-control study carried out on 50 children: 25 cases of NS (16 steroid-sensitive [SSNS] and nine steroid-resistant [SRNS] under follow up in the pediatric nephrology unit of Menoufia University Hospital, which is tertiary care center, were compared to 25 healthy controls with matched age and sex...
November 5, 2016: Jornal de Pediatria
https://www.readbyqxmd.com/read/27820879/heparin-for-the-treatment-of-thrombosis-in-neonates
#4
REVIEW
Olga Romantsik, Matteo Bruschettini, Simona Zappettini, Luca Antonio Ramenghi, Maria Grazia Calevo
BACKGROUND: Among pediatric patients, newborns are at highest risk of developing thromboembolism. Neonatal thromboembolic (TE) events may consist of both venous and arterial thromboses and often iatrogenic complications (eg, central catheterization). Treatment guidelines for pediatric patients with TE events most often are extrapolated from the literature regarding adults. Options for the management of neonatal TE events include expectant management; nitroglycerin ointment; thrombolytic therapy or anticoagulant therapy, or a combination of the two; and surgery...
November 7, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27813341/pharmacological-and-biological-therapeutic-strategies-for-osteogenesis-imperfecta
#5
Ronit Marom, Yi-Chien Lee, Ingo Grafe, Brendan Lee
Osteogenesis imperfecta (OI) is a connective tissue disorder characterized by bone fragility, low bone mass, and bone deformities. The majority of cases are caused by autosomal dominant pathogenic variants in the COL1A1 and COL1A2 genes that encode type I collagen, the major component of the bone matrix. The remaining cases are caused by autosomal recessively or dominantly inherited mutations in genes that are involved in the post-translational modification of type I collagen, act as type I collagen chaperones, or are members of the signaling pathways that regulate bone homeostasis...
November 3, 2016: American Journal of Medical Genetics. Part C, Seminars in Medical Genetics
https://www.readbyqxmd.com/read/27771681/ibandronate-in-the-treatment-of-pediatric-osteoporosis
#6
Stepan Kutilek, Ivana Plasilova, Jan Langer
We administered oral ibandronate (once a month) to 7 children (6 boys) with low bone mineral density and prevalent low energy fractures. We observed a significant increase (17%) in bone density after one year and additional 3% increase after second year. No further fractures occurred.
October 8, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/27755346/extra-intestinal-manifestations-of-celiac-disease-effectiveness-of-the-gluten-free-diet
#7
Hilary Jericho, Naire Sansotta, Stefano Guandalini
OBJECTIVE: To evaluate the effectiveness of the GFD on extra-intestinal symptoms in pediatric and adult celiac populations at the University of Chicago (UofC). METHODS: We conducted a retrospective chart review of the UofC Celiac Center clinic charts from January 2002 to October 2014. Demographics, serologic testing, intestinal biopsies, and extra-intestinal symptoms at presentation, 12, 24, and greater than 24 months were recorded. Extra-intestinal symptoms included: abnormal liver enzymes, arthralgia/arthritis, dermatitis herpetiformis (DH), alopecia, fatigue, headache, anemia, stomatitis, myalgias, psychiatric disorders, rashes, seizures, neuropathy, short stature, delayed puberty, osteoporosis and infertility...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27742529/association-of-gross-motor-function-classification-system-level-and-school-attendance-with-bone-mineral-density-in-patients-with-cerebral-palsy
#8
Ki Jin Jung, Soon-Sun Kwon, Chin Youb Chung, Kyoung Min Lee, Ki Hyuk Sung, Byung Chae Cho, Myoung Ki Chung, Seung Jun Moon, Jaeyoung Kim, Moon Seok Park
The present study aimed to evaluate bone mineral density (BMD) in children and adolescents with cerebral palsy (CP) and to critically analyze the effects of a variety of factors, particularly the Gross Motor Function Classification System (GMFCS) level, the Caregiver Priorities and Child Health Index of Life with Disabilities questionnaire, and the Pediatric Outcomes Data Collection Instrument (PODCI), on BMD. Fifty patients with CP who underwent dual-energy X-ray absorptiometry were included. Collected data included the extent of involvement, muscle tone, demographic data, factors determined through chart review, and laboratory results...
October 11, 2016: Journal of Clinical Densitometry
https://www.readbyqxmd.com/read/27669735/bone-densitometry-in-children-and-adolescents
#9
Laura K Bachrach, Catherine M Gordon
Concerns about bone health and potential fragility in children and adolescents have led to a high interest in bone densitometry. Pediatric patients with genetic and acquired chronic diseases, immobility, and inadequate nutrition may fail to achieve expected gains in bone size, mass, and strength, leaving them vulnerable to fracture. In older adults, bone densitometry has been shown to predict fracture risk and reflect response to therapy. The role of densitometry in the management of children at risk of bone fragility is less clear...
September 26, 2016: Pediatrics
https://www.readbyqxmd.com/read/27665261/implementation-and-adherence-to-osteoporosis-screening-guidelines-among-coeliac-disease-patients
#10
Prashant Singh, John J Garber
BACKGROUND: There are no studies evaluating the implementation of American Gastroenterological Association (AGA) guidelines on osteoporosis screening in coeliac disease. AIMS: To investigate implementation of osteoporosis screening guidelines in coeliac disease patients and determine how often bone mineral density (BMD) assessment leads to therapeutic intervention. METHODS: We screened all patients with biopsy-proven coeliac disease at our center from 2003 to 2013 and collected data on indication and results of dual energy X-ray absorptiometry scanning (DXA) and therapeutic interventions...
September 5, 2016: Digestive and Liver Disease
https://www.readbyqxmd.com/read/27603884/-teenager
#11
Nelly Altamirano-Bustamante, Myriam M Altamirano-Bustamante
The bone mass peak is the maximum bone quantity to be achieved through bone modeling. About 40% of the total bone mass is achieved at puberty; therefore, adolescence is critical on the skeletal development. This paper is about the transfunctional analysis of nutrition, mineral metabolism, endocrinology and life style in adolescence. Core factors to achieve the maximum potential of bone modeling through puberty and prevent osteoporosis from a pediatric stage are addressed.
September 2016: Gaceta Médica de México
https://www.readbyqxmd.com/read/27579163/genetics-of-pediatric-bone-strength
#12
REVIEW
Jonathan A Mitchell, Diana L Cousminer, Babette S Zemel, Struan F A Grant, Alessandra Chesi
Osteoporosis is one of the most common chronic forms of disability in postmenopausal women and represents a major health burden around the world. Bone fragility is affected by bone mineral density (BMD), and, one of the most important factors in preventing osteoporosis is optimizing peak bone mass, which is achieved during growth in childhood and adolescence. BMD is a complex trait resulting from environmental and genetic factors. Genome-wide association studies have discovered robust genetic signals influencing BMD in adults, and similar studies have also been conducted to investigate the genetics of BMD in the pediatric setting...
2016: BoneKEy Reports
https://www.readbyqxmd.com/read/27574779/evaluation-of-bone-mineral-density-in-patients-with-type-1-gaucher-disease-in-argentina
#13
M S Larroudé, G Aguilar, I Rossi, G Drelichman, N Fernandez Escobar, N Basack, M Slago, A Schenone, A Fynn, M F Cuello, R Fernandez, A Ruiz, P Reichel, N Guelbert, H Robledo, N Watman, M Bolesina, G Elena, S E Veber, G Pujal, G Galvan, J J Chain, A Arizo, J Bietti, M Aznar, M Dragosky, M Marquez, L Feldman, K Muller, S Zirone, G Buchovsky, V Lanza, I Fernandez, R Jaureguiberry, M A Barbieri, A Maro, G Zarate, G Fernandez, M Rapetti, A Degano, G Kantor, A Albina, M Alvarez Bollea, H Arrocena, V Bacciedoni, F Del Rio
The purpose of this study was to evaluate the frequency of osteoporosis (OP) in patients with Gaucher disease (GD) in Argentina. GD patients from 28 centers were consecutively included from April 2012 to 2014. Bone mineral density (BMD) was determined by dual X-ray absorptiometry in the lumbar spine and the femoral neck or the total proximal femur for patients ≥20 yr of age, and by whole-body scan in the lumbar spine in patients <20 yr of age. In children, mineral density was calculated using the chronological age and Z height...
August 26, 2016: Journal of Clinical Densitometry
https://www.readbyqxmd.com/read/27555069/osteoporosis-in-pediatric-liver-transplantation
#14
Walid Faraj, Ghina El Nounou, Abdallah Abou Al Naaj, Nancy Nakhoul, Ali Haydar, Mohammad Khalife
Liver transplantation provides an important, often life-saving treatment for end-stage liver disease. Osteoporosis post-liver transplantation has been described in adults; however, this has not been described in the pediatric population to date. We present a case of a 13-year-old female patient who underwent an orthotopic liver transplant for cryptogenic liver cirrhosis. Her immunosuppressants were tacrolimus and prednisone. Four months posttransplant, she started complaining of bilateral lower limb pain and limping while walking, progressing to a point where she was almost immobile...
August 22, 2016: Progress in Transplantation
https://www.readbyqxmd.com/read/27435427/informing-evidence-based-clinical-practice-guidelines-for-children-with-cerebral-palsy-at-risk-of-osteoporosis-an-update
#15
REVIEW
Sezgi Ozel, Lauren Switzer, Alex Macintosh, Darcy Fehlings
AIM: To investigate the impact of new evidence for weight-bearing, bisphosphonates, and vitamin D and calcium interventions, towards updating the systematic review and clinical practice guidelines for osteoporosis in children with cerebral palsy (CP) published in 2011. METHOD: Computer-assisted literature searches were conducted for articles published from 2010 to 2016. Searches focused on children with CP functioning at Gross Motor Function Classification System levels III to V and limited to weight-bearing activities, bisphosphonates, and vitamin D and/or calcium supplementation...
September 2016: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/27355856/recommendations-of-the-neuroendocrinology-department-of-the-brazilian-society-of-endocrinology-and-metabolism-for-the-diagnosis-of-cushing-s-disease-in-brazil
#16
Márcio Carlos Machado, Maria Candida Barisson Vilares Fragoso, Ayrton Custódio Moreira, César Luiz Boguszewski, Leonardo Vieira, Luciana A Naves, Lucio Vilar, Luiz Antônio de Araújo, Mauro A Czepielewski, Monica R Gadelha, Nina Rosa Castro Musolino, Paulo Augusto C Miranda, Marcello Delano Bronstein, Antônio Ribeiro-Oliveira
Although it is a rare condition, the accurate diagnosis and treatment of Cushing's disease is important due to its higher morbidity and mortality compared to the general population, which is attributed to cardiovascular diseases, diabetes mellitus and infections. Screening for hypercortisolism is recommended for patients who present multiple and progressive clinical signs and symptoms, especially those who are considered to be more specific to Cushing's syndrome, abnormal findings relative to age (e.g., spinal osteoporosis and high blood pressure in young patients), weight gain associated with reduced growth rate in the pediatric population and for those with adrenal incidentalomas...
June 2016: Archives of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27297606/enhanced-wnt-signaling-improves-bone-mass-and-strength-but-not-brittleness-in-the-col1a1-mov13-mouse-model-of-type-i-osteogenesis-imperfecta
#17
Christina M Jacobsen, Marissa A Schwartz, Heather J Roberts, Kyung-Eun Lim, Lyudmila Spevak, Adele L Boskey, David Zurakowski, Alexander G Robling, Matthew L Warman
Osteogenesis Imperfecta (OI) comprises a group of genetic skeletal fragility disorders. The mildest form of OI, Osteogenesis Imperfecta type I, is frequently caused by haploinsufficiency mutations in COL1A1, the gene encoding the α1(I) chain of type 1 collagen. Children with OI type I have a 95-fold higher fracture rate compared to unaffected children. Therapies for OI type I in the pediatric population are limited to anti-catabolic agents. In adults with osteoporosis, anabolic therapies that enhance Wnt signaling in bone improve bone mass, and ongoing clinical trials are determining if these therapies also reduce fracture risk...
September 2016: Bone
https://www.readbyqxmd.com/read/27150561/neonatal-transient-hypophosphatemic-hypercalciuric-rickets-in-dizygous-twins-a-role-for-maternal-alendronate-therapy-before-pregnancy-or-antireflux-medications
#18
M Gerin, G Jambon, A Fouque-Aubert, C Raybaud, P Cochat, O Claris, J Bacchetta
BACKGROUND: Bisphosphonates (BP) are sometimes used in children and young women, but their use requires expertise and caution due to the relative lack of long-term efficacy and safety data. CLINICAL CASES: We report on two dizygotic male twins with a past of mild prematurity who presented at the age of 2 months with moderate clinical craniotabes, hypophosphatemia, normal circulating calcium, severe hypercalciuria, and low parathyroid hormone levels. Following supplementation with oral phosphorus and native vitamin D, the clinical and biological abnormalities disappeared within 2 months...
September 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/27125514/the-management-of-osteoporosis-in-children
#19
REVIEW
L M Ward, V N Konji, J Ma
This article reviews the manifestations and risk factors associated with osteoporosis in childhood, the definition of osteoporosis and recommendations for monitoring and prevention. As well, this article discusses when a child should be considered a candidate for osteoporosis therapy, which agents should be prescribed, duration of therapy and side effects. There has been significant progress in our understanding of risk factors and the natural history of osteoporosis in children over the past number of years...
July 2016: Osteoporosis International
https://www.readbyqxmd.com/read/27117933/body-composition-analysis-by-dual-energy-x-ray-absorptiometry-in-young-preschool-children
#20
F Lifshitz, J P Hecht, E F Bermúdez, C A Gamba, J M Reinoso, P L Casavalle, S M Friedman, P N Rodriguez
BACKGROUND/OBJECTIVES: Dual-energy X-ray absorptiometry (DXA) is considered a specific method for measuring body composition to assess obesity and osteoporosis, although few studies have been conducted in preschool children. The aim of this study was to provide sex - and age-specific references for bone mineral density (BMD), bone mineral content (BMC), fat mass (FM) and fat-free mass (FFM) normative data for children aged 2 to <6 years. SUBJECTS/METHODS: One hundred and eighty seven healthy white children from Buenos Aires City suburbs, Argentina, were studied by the Lunar DPX-L DXA, pediatric software: BMC less head (g), BMD (g/cm(2)), FM (%) and FFM (g)...
April 27, 2016: European Journal of Clinical Nutrition
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