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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of cervical deformity of lambs following dosage with vitamin D was investigated. The lesions of a scoliosis are described together with the kidney lesions. It is suggested that doses of vitamin D normally considered to be non-toxic to sheep may prove to be so under certain conditions.
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PMID:Cervical scoliosis and kidney lesions in sheep following dosage with vitamin D. 126 95

Tadpoles of Rana perezi were kept for 14 weeks in water containing two sublethal levels of the carbamate insecticide ZZ-Aphox or the organophosphate Folidol. Approximate concentrations of their active ingredients were 0.25 and 1 mg/L. Resulting malformations were studied by skeletal analysis and histological and histochemical investigation of te rear limbs of the tadpoles. The pesticides caused the animals to have malformations of the spinal column (scoliosis) and/or limbs (short and thick long bones with the epiphyses grossly twisted). Histochemical study showed differences in the composition of the connective matrix, and microscopic examination of the long bones indicated alterations in the thickness of the uncalcified bone matrix (osteoid) and the presence of abundant vascularised connective tissue in the region of the periosteum. The results confirmed changes in the composition of the connective tissue matrix as the cause of the defects observed in bone formation which are also discussed in relation to vitamin D absorption and calcium homeostasis.
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PMID:Skeletal malformations induced by the insecticides ZZ-Aphox and Folidol during larval development of Rana perezi. 772 46

A 5-year-old girl presented with lower limb deformities, delayed ambulation, short stature, facial dysmorphism and scoliosis. Radiologic examination showed severe anterior and external bowing of the femurs and anterior and internal bowing of the tibia and fibula, with posterior and medial cortical thickening. Square iliac wings, horizontal sacrum and low-set L5 were also seen. The diagnosis of Weismann-Netter, Stuhl syndrome was established with the exclusion of abnormalities in mineral and vitamin D metabolism. This rare skeletal dysplasia should be included in the radiologic differential diagnosis of congenital deformities of the lower extremities.
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PMID:The Weismann-Netter, Stuhl syndrome: a rare pediatric skeletal dysplasia. 776 Nov 60

A 35-year-old woman with neurofibromatosis 1 and thoracic kyphoscoliosis had incomplete paraplegia. She had a history of hyperparathyroidism due to a parathyroid adenoma which had been excised 4 years previously. Plain radiographs of the spine revealed kyphoscoliosis from the third to sixth thoracic vertebrae. Kyphosis and scoliosis angles were 86 degrees and 28 degrees, respectively. Radiographs of the skull and hands showed radiological changes suggestive of hyperparathyroidism. Laboratory tests showed low-normal serum calcium, hypophosphatemia, elevated serum alkaline phosphatase, and low serum 25-hydroxyvitamin D. Retrospective review of the patient's laboratory data showed that she had osteomalacia at the time of diagnosis of primary hyperparathyroidism. The patient had been treated by anterior and posterior decompression and fusion with posterior instrumentation through a single posterior approach. The postoperative kyphosis and scoliosis angles were 30 degrees and 12 degrees, respectively. Neurological recovery and spinal fusion had been achieved. Osteomalacia responded well to vitamin D therapy. This is the first case of coexisting neurofibromatosis 1, primary hyperparathyroidism due to parathyroid adenoma and osteomalacia to be reported in the literature. The osteomalacia in this patient could be related to primary hyperparathyroidism, and not to neurofibromatosis 1. A drop in melatonin level after parathyroidectomy may have been the cause of spinal curvature progression in this patient.
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PMID:The association of neurofibromatosis 1 and spinal deformity with primary hyperparathyroidism and osteomalacia: might melatonin have a role? 1148 9

We report a case of Kallmann's syndrome (KS) in a previously untreated 30-year-old Caucasian male, admitted to our Endocrinology Department, presenting with hypogonadotropic hypogonadism and hypoosmia, and reporting a history of rickets in early childhood and a rapid growth pattern. On admission, his main complaints were back-pain and a decreased tolerance to physical exercise. The patient gave no family history of hypogonadism or hypoosmia, and his case was assumed to be sporadic KS. On physical examination hypogonadism (micropenis, small testes, no puberty), hypoosmia and severe scoliosis, kyphosis and chest malformations were recorded. No facial hair growth was found nor past voice braking. His skeletal proportions were eunuchoidal, no mid-line defects were found. This case of KS was identified unusually late. Due to patient's anxiety that his physical appearance might change due to therapy, he was referred to a clinical psychologist who confirmed the patient's self-perception as a male. The risk of further bone malformations, progression of osteoporosis and consecutive pathological fractures were the main indications for commencing treatment. Psychological support was provided. Six months of treatment with low doses of hCG (500 IU given i.m. twice a week) had elevated his testosterone levels but they still remained below the lower values of the normal reference range for males. Additional treatment with vitamin D and calcium supplementation was continued. A certain improvement of bone density score was observed. The patient noted a marked pain relief and he willingly complied with the treatment. After six months of therapy hCG dose was increased to 2000 IU given twice a week. We conclude that even at such a late diagnosis of Kallmann's syndrome in a male who accepts his physical appearance and does not wish any treatment in this respect, hormonal therapy is necessary and should be introduced to reduce significant risk of osteoporosis and bone fractures, and also to offset slowly progressing skeletal malformations which result from the lack of epiphyseal fusion.
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PMID:Kallmann's syndrome: skeletal and psychological aspects of late diagnosis. 1459 39

Steroids may have a beneficial effect on the course of Duchenne muscular dystrophy (DMD). However, results vary in different studies. This study consisted of 66 DMD boys who were in the therapy group and 22 DMD boys in the control group. The mean ages were 6.8 +/- 2.1 years (range 2.5-12.5) and 7.0 +/- 1.3 years (range 5.0-9.0), respectively. We assessed muscle strength, 10-m walking, ankle contracture, and loss of independent walking ability age and onset of scoliosis. Treatment regimen was oral prednisolone 0.75 mg/kg on alternate days, plus vitamin D 600-1200 units/day and a calcium-enriched diet. After a follow-up period of 2.75 +/- 1.1 years (range 1.5-5) and when compared with controls, there was a statistically significant change in muscle strength between the two groups after 12 months (P < 0.05). Although 10-m walking time decreased in therapy group (P < 0.05), there was not significance between the groups in the end. Boys in the control group developed significantly less ankle contractures (P < 0.05). None of the therapy group had scoliosis during the follow-up period (mean age 10.8 +/- 1.2 years), whereas seven boys of the control group had scoliosis at a mean age of 11.7 +/- 2 years. Loss of walking ability age was statistically different between groups (P < 0.05). Our results indicate that, alternate-day prednisolone regimen may prolong ambulation and scoliosis can be delayed or prevented.
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PMID:Prednisolone therapy in Duchenne muscular dystrophy prolongs ambulation and prevents scoliosis. 1527 99

We compare the long-term benefits and side effects of deflazacort using two treatment protocols from Naples (N) and Toronto (T). Boys with Duchenne muscular dystrophy between the ages of 8 and 15 years and who had four or more years of deflazacort treatment were reviewed. Diagnostic criteria included males with proximal muscle weakness evident before 5 years, increased serum creatine kinase and genetic testing and/or a muscle biopsy consistent with Duchenne muscular dystrophy. Thirty-seven boys were treated with protocol-N using deflazacort at a dose of 0.6 mg/kg per day for the first 20 days of the month and no deflazacort for the remainder of the month. Boys with osteoporosis received daily vitamin D and calcium. Deflazacort treatment started between 4 and 8 years of age. Thirty-two were treated with protocol-T using deflazacort at a dose of 0.9 mg/kg per day, plus daily vitamin D and calcium. Treatment started between 6 and 8 years of age. All boys were monitored every 4-6 months. The results were compared with age-matched controls in the two groups (19 for protocol-N and 30 for protocol-T). For the boys treated with protocol-N, 97% were ambulatory at 9 years (control, 22%), 35% at 12 years (control, 0%), 25% at 15 years (control, 0%). For the 32 boys treated with protocol-T, 100% were ambulatory at 9 years (control, 48%), 83% at 12 years (control, 0%) and 77% at 15 years (control, 0%). No aids or leg braces were used for ambulation. In boys 13 years and older, a scoliosis of >20 degrees developed in 30% of the boys on protocol-N, 16% on protocol-T and 90% of controls. For protocol-N, no cataracts were observed while in protocol-T, 30% of boys had asymptomatic cataracts that required no treatment. Fractures occurred in 19% (control 16%) of boys on protocol-N and 16% (control, 20%) of boys on protocol-T. This report illustrates: (a) the importance of collaborative studies in developing treatment protocols in Duchenne muscular dystrophy and (b) the long-term beneficial effects of deflazacort treatment in both protocols. However, the protocol-T seems to be more effective and frequently is associated with asymptomatic cataracts.
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PMID:Deflazacort in Duchenne muscular dystrophy: a comparison of two different protocols. 1533 88

Improved survival of orthotopic liver transplantation (OLT) has shifted the focus of patient care to quality of life, including prevention and treatment of pre- and post-transplant complications. End-stage liver failure affects bone length and strength, causing growth failure and hepatic osteodystrophy. Growth failure affects 60% of children assessed for OLT. Optimization of nutrition may prevent further stunting of growth before OLT but is rarely successful. Catch-up growth is observed following steroid withdrawal usually from 18 months post OLT. Whether growth hormone treatment would benefit the 20% of children who fail to regain normal height needs to be tested in randomized controlled trials. Hepatic osteodystrophy in children comprises vitamin D deficiency rickets, low bone mass and fractures caused by malnutrition and malabsorption. Vitamin D deficiency should be treated aggressively with cholecalciferol (D2) or ergocalciferol (D3). The active vitamin D metabolites alphacalcidol or calcitriol are used to increase calcium absorption from the gut but do nothing to replace vitamin D stores. Children before and after OLT have an increased prevalence of fractures of 10-13% and 12-38%, respectively. Most fractures are vertebral, and are related to low spine BMD. They often occur asymptomatically but may also cause chronic pain and later scoliosis. The main risk groups are infants with cholestatic liver disease, and adolescents with later OLT and greater BMI. Fracture prediction in these children is limited. OLT also bears the risk of avascular bone necrosis (4%), and development of scoliosis (13-38%). This paper reviews the literature and presents preventative and therapeutic strategies to improve bone length and strength.
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PMID:Growth and bone health in chronic liver disease and following liver transplantation in children. 2052 40

The goals of the well-child examination in school-aged children (kindergarten through early adolescence) are promoting health, detecting disease, and counseling to prevent injury and future health problems. A complete history should address any concerns from the patient and family and screen for lifestyle habits, including diet, physical activity, daily screen time (e.g., television, computer, video games), hours of sleep per night, dental care, and safety habits. School performance can be used for developmental surveillance. A full physical examination should be performed; however, the U.S. Preventive Services Task Force recommends against routine scoliosis screening and testicular examination. Children should be screened for obesity, which is defined as a body mass index at or above the 95th percentile for age and sex, and resources for comprehensive, intensive behavioral interventions should be provided to children with obesity. Although the evidence is mixed regarding screening for hypertension before 18 years of age, many experts recommend checking blood pressure annually beginning at three years of age. The American Academy of Pediatrics recommends vision and hearing screening annually or every two years in school-aged children. There is insufficient evidence to recommend screening for dyslipidemia in children of any age, or screening for depression before 12 years of age. All children should receive at least 400 IU of vitamin D daily, with higher doses indicated in children with vitamin D deficiency. Children who live in areas with inadequate fluoride in the water (less than 0.6 ppm) should receive a daily fluoride supplement. Age-appropriate immunizations should be given, as well as any missed immunizations.
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PMID:Health maintenance in school-aged children: Part I. History, physical examination, screening, and immunizations. 2204 32

With improved survival of orthotopic liver transplantation (OLT) in children, prevention and treatment of pre- and posttransplant complications have become a major focus of care. End-stage liver failure can cause endocrine complications such as growth failure and hepatic osteodystrophy, and, like other chronic illnesses, also pubertal delay, relative adrenal insufficiency, and the sick euthyroid syndrome. Drug-induced diabetes mellitus post-OLT affects approximately 10% of children. Growth failure is found in 60% of children assessed for OLT. Despite optimisation of nutrition, rarely can further stunting of growth before OLT be prevented. Catch-up growth is usually observed after steroid weaning from 18 months post-OLT. Whether growth hormone treatment would benefit the 20% of children who fail to catch up in height requires testing in randomised controlled trials. Hepatic osteodystrophy in children comprises vitamin D deficiency rickets, low bone mass, and fractures caused by malnutrition and malabsorption. Vitamin D deficiency requires aggressive treatment with ergocalciferol (D2) or cholecalciferol (D3). The active vitamin D metabolites alphacalcidol or calcitriol increase gut calcium absorption but do not replace vitamin D stores. Prevalence of fractures is increased both before OLT (10%-28% of children) and after OLT (12%-38%). Most fractures are vertebral, are associated with low spine bone mineral density, and frequently occur asymptomatically, but they may also cause chronic pain. Fracture prediction in these children is limited. OLT in children is also associated with a greater risk of developing avascular bone necrosis (4%) and scoliosis (13%-38%). This article reviews the literature on endocrine and skeletal complications of liver disease and presents preventive screening recommendations and therapeutic strategies.
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PMID:Endocrine and bone metabolic complications in chronic liver disease and after liver transplantation in children. 2206 32


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