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Journal of Surgical Case Reports May 2023Secondary chronic osteomyelitis (SCO) is caused by bacterial infection leading to progressive jawbone destruction. Antibiotics are used as first-line treatment; surgical...
Secondary chronic osteomyelitis (SCO) is caused by bacterial infection leading to progressive jawbone destruction. Antibiotics are used as first-line treatment; surgical therapy is usually extensive and may not provide a cure. Bisphosphonates have been reported to be successful in patients with primary nonbacterial osteomyelitis, and literature indicates promising results in SCO. A 38-year-old patient presented with a progressive mandible-destruction 17 years after wisdom tooth extraction. Several treatment attempts have been unsuccessful. Seeking a second opinion, the patient was then interdisciplinary treated with 90 mg of intravenous pamidronate every four weeks, three times. The patient did not experience side effects presenting a considerable improvement in mouth opening and reporting a dissolvent of signs of pain or infection. The MRI showed edema reduction and decreased contrast uptake. Therefore, bisphosphonate treatment of secondary chronic osteomyelitis of the jaw is a safe and effective option in selected cases after unsuccessful first- and second-line therapy.
PubMed: 37251250
DOI: 10.1093/jscr/rjad270 -
JPMA. the Journal of the Pakistan... Dec 2022Neonatal severe hyperparathyroidism (NSHPT) is a rare genetic disorder that presents within the first six months of life. We present the case report of a male child who...
Neonatal severe hyperparathyroidism (NSHPT) is a rare genetic disorder that presents within the first six months of life. We present the case report of a male child who presented to us in the first month of his life with symptoms of lethargy, constipation, and reluctance to feed. One sibling of the child had died earlier with similar symptoms in the first six months of life. Upon physical examination, the child was lethargic, dehydrated, had bradycardia with hyperreflexia. Serum electrolyte analysis showed hypercalcaemia and hypophosphataemia. Further workup revealed elevated serum parathyroid hormone levels and Calcium sensing receptor (CaSR) gene mutation in autosomal recessive patterns. The father was discovered to be heterozygous for the same mutation but is asymptomatic. Diagnosis of neonatal severe hyperparathyroidism was made and the child was managed medically with intravenous fluids, Furosemide, Pamidronate, and Cinacalcet. On inconsistent response to medical therapy, he underwent total parathyroidectomy with auto transplantation of half of the left lower parathyroid gland. Postoperatively, the child is being managed on oral calcium and Alpha Calcidiol supplementation and is doing well.
Topics: Infant, Newborn; Child; Humans; Male; Hyperparathyroidism, Primary; Cinacalcet; Mutation; Heterozygote; Hypercalcemia; Calcium
PubMed: 37246686
DOI: 10.47391/JPMA.4195 -
The Cochrane Database of Systematic... May 2023Osteoporosis is characterized by low bone mass and micro-architectural deterioration of bone tissue leading to increased bone fragility. In people with... (Review)
Review
BACKGROUND
Osteoporosis is characterized by low bone mass and micro-architectural deterioration of bone tissue leading to increased bone fragility. In people with beta-thalassaemia, osteoporosis represents an important cause of morbidity and is due to a number of factors. First, ineffective erythropoiesis causes bone marrow expansion, leading to reduced trabecular bone tissue with cortical thinning. Second, excessive iron loading causes endocrine dysfunction, leading to increased bone turnover. Lastly, disease complications can result in physical inactivity, with a subsequent reduction in optimal bone mineralization. Treatments for osteoporosis in people with beta-thalassaemia include bisphosphonates (e.g. clodronate, pamidronate, alendronate; with or without hormone replacement therapy (HRT)), calcitonin, calcium, zinc supplementation, hydroxyurea, and HRT alone (for preventing hypogonadism). Denosumab, a fully human monoclonal antibody, inhibits bone resorption and increases bone mineral density (BMD). Finally, strontium ranelate simultaneously promotes bone formation and inhibits bone resorption, thus contributing to a net gain in BMD, increased bone strength, and reduced fracture risk. This is an update of a previously published Cochrane Review.
OBJECTIVES
To review the evidence on the efficacy and safety of treatment for osteoporosis in people with beta-thalassaemia.
SEARCH METHODS
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register, which includes references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also searched online trial registries. Date of most recent search: 4 August 2022.
SELECTION CRITERIA
Randomized controlled trials (RCTs) in people with beta-thalassaemia with: a BMD Z score below -2 standard deviations (SDs) for children aged under 15 years, adult males (aged 15 to 50 years) and premenopausal females aged over 15 years; or a BMD T score below -2.5 SDs for postmenopausal females and males aged over 50 years.
DATA COLLECTION AND ANALYSIS
Two review authors assessed the eligibility and risk of bias of the included RCTs, and extracted and analysed data. We assessed the certainty of the evidence using GRADE.
MAIN RESULTS
We included six RCTs (298 participants). Active interventions included bisphosphonates (3 trials, 169 participants), zinc supplementation (1 trial, 42 participants), denosumab (1 trial, 63 participants), and strontium ranelate (1 trial, 24 participants). The certainty of the evidence ranged from moderate to very low and was downgraded mainly due to concerns surrounding imprecision (low participant numbers), but also risk of bias issues related to randomization, allocation concealment, and blinding. Bisphosphonates versus placebo or no treatment Two RCTs compared bisphosphonates to placebo or no treatment. After two years, one trial (25 participants) found that alendronate and clodronate may increase BMD Z score compared to placebo at the femoral neck (mean difference (MD) 0.40, 95% confidence interval (CI) 0.22 to 0.58) and the lumbar spine (MD 0.14, 95% CI 0.05 to 0.23). One trial (118 participants) reported that neridronate compared to no treatment may increase BMD at the lumbar spine and total hip at six and 12 months; for the femoral neck, the study found increased BMD in the neridronate group at 12 months only. All results were of very low-certainty. There were no major adverse effects of treatment. Participants in the neridronate group reported less back pain; we considered this representative of improved quality of life (QoL), though the certainty of the evidence was very low. One participant in the neridronate trial (116 participants) sustained multiple fractures as a result of a traffic accident. No trials reported BMD at the wrist or mobility. Different doses of bisphosphonate compared One 12-month trial (26 participants) assessed different doses of pamidronate (60 mg versus 30 mg) and found a difference in BMD Z score favouring the 60 mg dose at the lumbar spine (MD 0.43, 95% CI 0.10 to 0.76) and forearm (MD 0.87, 95% CI 0.23 to 1.51), but no difference at the femoral neck (very low-certainty evidence). This trial did not report fracture incidence, mobility, QoL, or adverse effects of treatment. Zinc versus placebo One trial (42 participants) showed zinc supplementation probably increased BMD Z score compared to placebo at the lumbar spine after 12 months (MD 0.15, 95% CI 0.10 to 0.20; 37 participants) and 18 months (MD 0.34, 95% CI 0.28 to 0.40; 32 participants); the same was true for BMD at the hip after 12 months (MD 0.15, 95% CI 0.11 to 0.19; 37 participants) and 18 months (MD 0.26, 95% CI 0.21 to 0.31; 32 participants). The evidence for these results was of moderate certainty. The trial did not report BMD at the wrist, fracture incidence, mobility, QoL, or adverse effects of treatment. Denosumab versus placebo Based on one trial (63 participants), we are unsure about the effect of denosumab on BMD Z score at the lumbar spine, femoral neck, and wrist joint after 12 months compared to placebo (low-certainty evidence). This trial did not report fracture incidence, mobility, QoL, or adverse effects of treatment, but the investigators reported a reduction in bone pain measured on a visual analogue scale in the denosumab group after 12 months of treatment compared to placebo (MD -2.40 cm, 95% CI -3.80 to -1.00). Strontium ranelate One trial (24 participants) only narratively reported an increase in BMD Z score at the lumbar spine in the intervention group and no corresponding change in the control group (very low-certainty evidence). This trial also found a reduction in back pain measured on a visual analogue scale after 24 months in the strontium ranelate group compared to the placebo group (MD -0.70 cm (95% CI -1.30 to -0.10); we considered this measure representative of improved quality of life.
AUTHORS' CONCLUSIONS
Bisphosphonates may increase BMD at the femoral neck, lumbar spine, and forearm compared to placebo after two years' therapy. Zinc supplementation probably increases BMD at the lumbar spine and hip after 12 months. Denosumab may make little or no difference to BMD, and we are uncertain about the effect of strontium on BMD. We recommend further long-term RCTs on different bisphosphonates and zinc supplementation therapies in people with beta-thalassaemia-associated osteoporosis.
Topics: Adult; Child; Female; Male; Humans; Middle Aged; beta-Thalassemia; Alendronate; Pamidronate; Clodronic Acid; Denosumab; Osteoporosis; Diphosphonates; Fractures, Bone
PubMed: 37159055
DOI: 10.1002/14651858.CD010429.pub3 -
Turkish Archives of Pediatrics May 2023Primary osteoporosis is a rare and essential problem in childhood that can cause severe skeletal deformities. We aimed to reveal the spectrum of primary osteoporosis and...
OBJECTIVE
Primary osteoporosis is a rare and essential problem in childhood that can cause severe skeletal deformities. We aimed to reveal the spectrum of primary osteoporosis and assess the effectiveness and safety of bisphosphonates in increasing bone mineral density and reducing fractures.
MATERIALS AND METHODS
Patients with primary osteoporosis who received at least one course of pamidronate or zoledronic acid were included in the study. Patients were divided into 2 groups, osteogenesis imperfecta and non-osteogenesis imperfecta subjects. We evaluated bone densitometer parameters, activation scores, pain status, deformity status, and the number of fractures per year in all patients.
RESULTS
Of the 31 patients, 21 with osteogenesis imperfect, 3 patients with spondyloocular syndromes, 2 with Bruck Syndrome, and 5 with idiopathic juvenile osteoporosis were included. A total of 21 patients had received pamidronate treatment, while only 4 received zoledronic acid, and 6 of them switched from pamidronate to zoledronic acid. At the end of the treatment, the mean bone mineral density height-adjusted Z-score increased from -3.39 ± 1.30 to -0.95 ± 1.34. The number of fractures per year decreased from 2.28 ± 2.67 to 0.29 ± 0.69. The activation score increased from 2.81 ± 1.47 to 3.16 ± 1.48. The pain decreased significantly. There was no difference in bone mineral density increase in patients treated with pamidronate or zoledronic acid.
CONCLUSION
Those with osteogenesis imperfecta were diagnosed at an earlier age with severe deformity and fractures. Pamidronate and zoledronic acid increased bone mineral density in all types of primary osteoporosis.
PubMed: 37144266
DOI: 10.5152/TurkArchPediatr.2023.22248 -
Medicina 2023Complex regional pain syndrome (CRPS), also known as Sudeck syndrome, is a chronic painful condition usually affecting the limbs after trauma or surgery. Its...
INTRODUCTION
Complex regional pain syndrome (CRPS), also known as Sudeck syndrome, is a chronic painful condition usually affecting the limbs after trauma or surgery. Its presentation is heterogeneous and its physiopathology, diagnosis and treatment remain controversial. The objective of this study was to analyze a group of patients with this rare syndrome, describing in detail the results of the dual energy X-ray absorptiometry (DXA) and the response to bisphosphonate treatment.
METHOD
We retrospectively analyzed 54 patients with CRPS, taking into account their demographic features, inciting events and diagnostic tests. As regards treatment, we analyzed the results and adverse events of the use of bisphosphonates Results: We found a female predominance (74%), with 55 ± 13 years. The most common inciting event was trauma (59%), followed by surgery. The difference in bone mineral density between the affected limb and the healthy one was 12 to 15%. Forty-four patients were treated with bisphosphonates (pamidronate, ibandronate, zoledronic acid) and showed a clinical improvement, mainly in terms of pain intensity. Only six patients presented with adverse events, like pseudoflu syndrome and acute phase symptoms.
CONCLUSION
In our cohort, lower limbs CRPS predominantly affects middle aged women. DXA scans are tests used to quantify bone loss and the response to treatment. The use of bisphosphonates is an interesting therapeutic option to improve clinical symptoms in most patients. Future prospective randomized studies will be needed to confirm our results.
Topics: Middle Aged; Humans; Female; Male; Retrospective Studies; Diphosphonates; Complex Regional Pain Syndromes; Pamidronate; Lower Extremity
PubMed: 37094194
DOI: No ID Found -
ACS Medicinal Chemistry Letters Apr 2023Organophosphonic compounds are distinctive among natural products in terms of stability and mimicry. Numerous synthetic organophosphonic compounds, including pamidronic...
Organophosphonic compounds are distinctive among natural products in terms of stability and mimicry. Numerous synthetic organophosphonic compounds, including pamidronic acid, fosmidromycin, and zoledronic acid, are approved drugs. DNA encoded library technology (DELT) is a well-established platform for identifying small molecule recognition to target protein of interest (POI). Therefore, it is imperative to create an efficient procedure for the on-DNA synthesis of α-hydroxy phosphonates for DEL builds.
PubMed: 37077381
DOI: 10.1021/acsmedchemlett.3c00022 -
Revue Medicale de Liege Apr 2023At least 80 % of persons with cystic fibrosis are pancreatic insufficient and benefit from daily supplementation with fat-soluble vitamins (ADEK). Magistral formulations...
At least 80 % of persons with cystic fibrosis are pancreatic insufficient and benefit from daily supplementation with fat-soluble vitamins (ADEK). Magistral formulations offer ideal flexibility for prescriptions tailored to vitamin A, D and E blood levels. However, they expose to human errors, mainly leading to vitamin D intoxication whose clinical features are related to hypercalcaemia. Symptoms are mostly digestive (vomiting, constipation, abdominal pain …) and, less frequently, renal (nycturia …) complaints. When symptoms and/or serum calcium levels ≥ 14 mg/100 ml are present, prompt management is required. Besides interruption of supplementation, rapid intravenous hyperhydration (saline) is essential. Once hydration has been restored, and still under close biological supervision, a loop diuretic (furosemide) may be used but the drug of choice to achieve rapid normalization of blood calcium levels will often be intravenous pamidronate. Normalization of serum vitamin 25(OH)-D levels may take several months but the prognosis is very good. In Belgium, the very late reimbursement of a fixed combination of fat-soluble vitamins (Dekas®) meeting the standards of the pharmaceutical industry is expected to reduce the incidence of these intoxications, at the price, however, of less flexible prescription.
Topics: Child; Humans; Vitamin D; Calcium; Cystic Fibrosis; Vitamins; Vitamin A
PubMed: 37067832
DOI: No ID Found -
Journal of Orthopaedic Case Reports Dec 2022Osteofibrous dysplasia is a fibro-osseous benign lesion of childhood and infancy that are commonly seen in the anterior shin of the tibia. Osteofibrous dysplasia in the...
INTRODUCTION
Osteofibrous dysplasia is a fibro-osseous benign lesion of childhood and infancy that are commonly seen in the anterior shin of the tibia. Osteofibrous dysplasia in the clavicle is rare and in this study, we reported a case of osteofibrous dysplasia arising in the midshaft of the clavicle.
CASE REPORT
An 11-year boy presented with complaints of pain and swelling over his left clavicle and was unable to do overhead abduction following a fall while playing 2 years back. Initially, the patient was diagnosed with a left clavicle fracture and was treated conservatively. The pain subsided after 3 months. The patient had re-injury after 6 months, following which pain and swelling of the left clavicle were gradually progressive. On examination, there was a diffuse swelling extending from the medial end to the lateral end of the left clavicle, which was tender, and bony-hard in consistency. The range of movements of the left shoulder was painful and terminally limited. A percutaneous core-needle biopsy was done, suggestive of a benign fibro-osseous lesion. An open biopsy was done from the tumor-normal bone junction, and caseous materials were found inside the medullary canal, the microscopic finding shows fibroblastic proliferation and osteoblastic proliferation laying down the woven bone. We treated the case with intravenous pamidronate injection in 6 months intervals for 2 years. The patient improved symptomatically achieving a full range of movements of the affected shoulder with good radiological consideration of the lesion.
CONCLUSION
Osteofibrous dysplasia is uncommonly seen in the clavicle, and if it is seen, it may mimic osteomyelitis clinically. It should be differentiated from other lesions by radiological, histopathological, and immunohistochemistry findings.
PubMed: 37056607
DOI: 10.13107/jocr.2022.v12.i12.3454