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1.
J Ultrasound Med ; 33(11): 1981-9, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25336486

RESUMEN

OBJECTIVES: The purpose of this study was to assess the role of transperineal sonography in assessment of pathologic changes to the anal sphincter complex in patients with chronic anal fissures. METHODS: We conducted a prospective case-control study of 100 consecutive patients of any age and both sexes with chronic anal fissures who presented to a colorectal clinic between January 2012 and August 2013 (group A) and 50 healthy volunteers (group B). RESULTS: The most common patterns of radiologic changes to anal sphincters associated with chronic anal fissures were circumferential thickening of the anal sphincter complex in 5 patients (5%), circumferential thickening of the internal anal sphincter in 3 patients (3%), preferential thickening of the internal anal sphincter at the 6-o'clock position in 80 patients (80%) and the 12-o'clock position in 7 patients (7%), preferential thickening of the internal and external anal sphincters in 3 patients (3%), and thinning of the internal anal sphincter in 2 patients (2%). CONCLUSIONS: Chronic anal fissures cause differential thickening of both internal and external anal sphincters, with a trend toward increased thickness in relation to the site of the fissure. Routine preoperative transperineal sonography for patients with chronic anal fissures is recommended, and it is mandatory in high-risk patients.


Asunto(s)
Canal Anal/diagnóstico por imagen , Fisura Anal/diagnóstico por imagen , Interpretación de Imagen Asistida por Computador/métodos , Perineo/diagnóstico por imagen , Adolescente , Adulto , Estudios de Casos y Controles , Enfermedad Crónica , Femenino , Humanos , Masculino , Persona de Mediana Edad , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Ultrasonografía , Adulto Joven
2.
Middle East J Anaesthesiol ; 22(4): 407-12, 2014 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25007695

RESUMEN

BACKGROUND: The aim of the current study is to compare the accuracy of the ultrasound (US) versus clinical assessment for determination of the spinal level, using X-ray as the Gold Standard for control. METHODS: 200 patients were randomized into two equal groups. Patients in the Clinical Group were examined by landmarks to assess the Assumed Clinical Tuffier's Line, and then by fluoroscopy to determine the True Clinical Tuffier's Line. Patients in the Ultrasound Group were examined by the ultrasound to determine the Ultrasound Tuffier's Line. The results of both groups were compared in relation to the plain X-ray, done for each patient, which determined the Radiological Tuffier's Line. RESULTS: In the Clinical Group, the True Clinical Tuffier's line met the Assumed Tuffier's line in only 12% of the patients. In the remaining patients, wrong leveling ranged from one space above in 80% to 2 spaces above in 7% and in 1% of patients the line was at L2. In the Ultrasound Group, wrong leveling occurred in 22% of patients. The Ultrasound misidentification was less than one level in 17% and one level in 5% of patients. Ultrasound examination had a true limitation of 2% of patients. CONCLUSION: Ultrasound examination of the spine is recommended in patients planned for spinal anesthesia, as it is superior to clinical assessment in identification of the interspinous levels. This will decrease the hazard of spinal cord trauma.


Asunto(s)
Anestesia Raquidea/métodos , Vértebras Lumbares/diagnóstico por imagen , Adulto , Femenino , Fluoroscopía/métodos , Humanos , Región Lumbosacra/diagnóstico por imagen , Masculino , Estudios Prospectivos , Reproducibilidad de los Resultados , Ultrasonografía
3.
Pediatr Endocrinol Rev ; 11(2): 167-80, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24575552

RESUMEN

In recent years, the issue of osteopenia/osteoporosis in children, adolescents and young adults with thalassaemia major (TM) has attracted much attention because it is a prominent cause of morbidity despite adequate transfusion and iron chelation therapy. The reported frequency of osteoporosis, even in well treated TM patients varies from 13.6% to 50% with an additional 45% affected by osteopenia. The pathogenesis of TM-induced osteoporosis is multifactorial. Genetic and acquired factors play role in demineralization of bones in thalassemia. Osteoporosis is characterized by low bone mass and disruption of bone architecture, resulting in reduced bone strength and increased risk of fractures. The significant predictors of fracture prevalence include male gender, hypothyroidism, age, lack of spontaneous puberty in females, active hepatitis, heart disease and diabetes. The early identification of osteopenia and osteoporosis is of paramount importance. This is because delayed diagnosis and inadequate treatment have led to severe osteoporosis, skeletal abnormalities, fractures, spinal deformities, nerve compression and growth failure. dequate hormonal replacement, has been posponed, Effective iron chelation adequate hormonal replacement, improvement of hemoglobin levels, calcium and vitamin D administration and physical activity are currently the main measures for the management of the disease. The use of bisphosphonates in TM patients with osteoporosis is increasing and their positive effect in improving bone mineral density is encouraging. The recommendations of the International Network on Growth Disorders and Endocrine Complications in Thalassaemia (I-CET) for diagnosis and management of osteoporosis in TM are also briefly included in this review.


Asunto(s)
Monitoreo Fisiológico/métodos , Osteoporosis/etiología , Osteoporosis/terapia , Talasemia beta/complicaciones , Talasemia beta/terapia , Adolescente , Adulto , Densidad Ósea , Niño , Femenino , Fracturas Óseas/epidemiología , Fracturas Óseas/etiología , Fracturas Óseas/prevención & control , Humanos , Masculino , Osteoporosis/epidemiología , Factores de Riesgo , Adulto Joven , Talasemia beta/epidemiología
4.
Acta Biomed ; 94(3): e2023177, 2023 06 14.
Artículo en Inglés | MEDLINE | ID: mdl-37326259

RESUMEN

Symptomatic avascular necrosis (AVN) imposes a higher risk for acute care consumption in adults living with SCD. Symptomatic AVN, have higher rates of visits to the emergency department, higher rates of admissions, and longer lengths of stay in hospitals. Properly timed diagnosis and early interventions can reduce morbidity and enhance the quality of life in these patients. Vaso-occlusion secondary to sickling leads to osteonecrosis of the joint/bone (AVN, dactylitis) and invites infection (osteomyelitis and septic arthritis). Understanding and awareness of the imaging features related to this major morbidity complication are essential for early diagnosis and prompt management. In about half of the patients with SCD, AVN can lead to chronic pain, particularly in the head of the femur and humerus. Humeral and femoral head AVN tend to be linked with each other.  Vertebral bone compression and collapse secondary to AVN have also been reported.  The diagnosis of AVN must be accurate, as the condition is complex requiring specific treatment according to the grade of bone and joint involvement. There are several classifications or staging systems used for grading bone and joint involvement. Knowledge of the image patterns and grade of affection in different joints and bones and the degree of progression of AVN lesions can markedly improve management decisions on AVN-specific surgical versus non-surgical interventions and improve patient outcomes. The aim of this report is to summarize the different imaging techniques and their role in the proper/early diagnosis and follow up of patients with AVN with detailed examples of the common sites involved.


Asunto(s)
Anemia de Células Falciformes , Necrosis de la Cabeza Femoral , Adulto , Humanos , Calidad de Vida , Radiografía , Necrosis de la Cabeza Femoral/diagnóstico por imagen , Necrosis de la Cabeza Femoral/etiología , Necrosis de la Cabeza Femoral/terapia , Húmero , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/diagnóstico por imagen
5.
Acta Biomed ; 92(6): e2021333, 2022 01 19.
Artículo en Inglés | MEDLINE | ID: mdl-35075088

RESUMEN

INTRODUCTION: Early puberty (EP) in girls is defined as the onset of thelarche that begins after 6 years and before 8 years and/or acceleration in the tempo of pubertal development. The stage of puberty and the ovarian volume at presentation and the effect of treatment with GnRH analogue (GnRHa) on final adult height are still debated. PATIENTS AND METHODS: We analyzed the data of 22 girls, who presented early and fast puberty (FEP). The clinical stage of puberty, hormonal levels and the ovarian volume (OV) (measured by ovarian ultra-sonography) at presentation were studied. We recorded the effects of 3 years treatment with GnRHa on their growth in relation to their mid parental height, pubertal progression, and bone maturation. RESULTS AND CONCLUSION: GnRHa therapy decreased the fast progress of puberty, skeletal maturation, and GV/year. It was successful in increasing the predicted final adult height comparable to or surpassing their mid-parenteral height. A larger OV at presentation was associated with reduced Ht-SDS after 3 years of GnRHa treatment. Clearly, a definitive evaluation of the efficacy of GnRHa as treatment for EFP in girls will require expanded and concerted studies.


Asunto(s)
Hormona Liberadora de Gonadotropina , Pubertad Precoz , Estatura , Desarrollo Óseo , Femenino , Humanos , Pubertad , Pubertad Precoz/tratamiento farmacológico
6.
Case Rep Rheumatol ; 2022: 4647227, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35685894

RESUMEN

Statin-induced necrotizing autoimmune myopathy is an immune-mediated necrotizing myopathy related to the use of statins. It is a very rare disease, which usually presents with proximal muscle weakness and frank elevation in creatine kinase levels. Stopping statin and the use of immunosuppressive therapy are considered the mainstay therapy. Use of steroids in patients with inflammatory myopathy can be complicated by steroid-induced myopathy. Herein, we present a case of a 55-year-old patient with statin-induced necrotizing autoimmune myopathy based on the presence of proximal muscle weakness, magnetic resonance findings, suggestive muscle biopsy features, and positive anti-HMGCR autoantibodies. The patient was treated with triple immunosuppressive therapy with a particularly good response to intravenous immunoglobulin. This report highlights the importance of timely diagnosis and early use of combined immunosuppressive therapy to improve patients' outcome affected by this rare disease.

7.
J Trop Pediatr ; 57(4): 303-6, 2011 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-20427425

RESUMEN

We recorded the manifestations of severe vitamin D deficiency (VDD) in 40 adolescents before and 3 and 6 months after treatment with a mega dose of cholecalciferol (10 000 IU kg(-1), max 600 000 IU). Significant improvement of symptoms related to VDD was reported in 34/40. Three months after the injection, serus calcium, phosphate, alkaline phosphatase and parathormone were normal in all adolescents with VDD with 25-hydroxyvitamin D (25OHD) level = or >20 ng ml(-1). After 6 months, the majority had 25OHD level <20 ng ml(-1). Two patterns of radiological changes have been recorded with complete healing achieved in all patients after a year of therapy. A mega dose of cholecalciferol is an effective therapy for treatment of VDD in adolescents for 3 months but not for 6 months. Radiographs of the ends of long bones are still valuable tool for diagnosis and follow-up of these patients.


Asunto(s)
Conservadores de la Densidad Ósea/uso terapéutico , Colecalciferol/uso terapéutico , Deficiencia de Vitamina D/tratamiento farmacológico , Adolescente , Fosfatasa Alcalina/sangre , Artralgia/etiología , Dolor de Espalda/etiología , Biomarcadores/sangre , Conservadores de la Densidad Ósea/administración & dosificación , Calcio/sangre , Colecalciferol/administración & dosificación , Femenino , Humanos , Inyecciones Intramusculares , Rodilla/diagnóstico por imagen , Masculino , Debilidad Muscular/etiología , Hormona Paratiroidea/sangre , Fósforo/sangre , Estudios Prospectivos , Qatar , Radiografía , Medición de Riesgo , Factores de Riesgo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Deficiencia de Vitamina D/sangre , Deficiencia de Vitamina D/complicaciones , Deficiencia de Vitamina D/diagnóstico , Caminata , Muñeca/diagnóstico por imagen
8.
Acta Cytol ; 54(3): 325-7, 2010.
Artículo en Inglés | MEDLINE | ID: mdl-20518420

RESUMEN

BACKGROUND: Filarial worms lodge anywhere, from body fluids to subcutaneous tissue, as various case reports prove. CASE: A 21-year-old Nepali male presented with bilateral groin swellings. Imaging studies showed dilated retroperitoneal lymphatics forming a mass, suggestive of lymphangioma or lymphangioleiomyoma. Fine needle aspiration (FNA) of the retroperitoneal mass revealed microfilariae of Wuchereria bancrofti. CONCLUSION: This is the first reported case of retroperitoneal filarial lymphangiectasia diagnosed on FNA, thus stressing the importance of FNA as a simple yet effective diagnostic tool.


Asunto(s)
Filariasis/diagnóstico , Espacio Retroperitoneal/parasitología , Wuchereria bancrofti/aislamiento & purificación , Animales , Biopsia con Aguja Fina , Diagnóstico Diferencial , Humanos , Linfangioma/diagnóstico , Linfangiomioma/diagnóstico , Masculino , Adulto Joven
9.
J Trop Pediatr ; 56(1): 19-26, 2010 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-19506025

RESUMEN

OBJECTIVES: Was to investigate the effect of treatment with an IM injection, a mega dose of vitamin D3 (10,000 IU/kg) on the clinical, biochemical and radiological parameters of 40 rachitic children with vitamin D deficiency (VDD) over a period of 3 months. DESIGN: In this prospective study we evaluated the clinical, biochemical and radiological responses of an IM injection of cholecalciferol (10,000 IU/kg) for 3 months. RESULTS: At presentation, the most frequent manifestations were enlarged wrist joints, hypotonia, irritability, cranial bossing, wide anterior fontanel, bow legs, delayed teething and walking and Harrison's sulcus with chest rosaries. Short stature (length SDS < -2) was recorded in 30% of patients. Craniotabes and hypocalcemic tetany were the least common presentations. In VDD children the most frequent biochemical abnormality was high alkaline phosphatase (ALP) (100%), followed by low phosphate (PO(4)) (75%) and low calcium (Ca) (12.5%). One month after treatment, serum Ca, PO(4) and 25(OH)D concentrations were normal. Three months after the injection, serum level of ALP and parathormone (PTH) decreased to normal. The majority of patients (87.5%) had serum 25(OH)D level >or= 20 ng/ml, but some (12.5%) had level <20 ng/ml. Hypercalcemia was not recorded in any patient during the 3-month-period. Significant cure of all symptoms and signs related to vitamin D deficiency had been achieved in all children. Leg bowing showed significant improvement in all patients but was still evident in one third. Complete healing of the radiological evidence of rickets was achieved in 95% of all children. CONCLUSION: An IM injection of a mega dose of cholecalciferol is a safe and effective therapy for treatment of VDD rickets in infants and toddlers with normalization of all the biochemical parameters and healing of radiological manifestations. Measurement of serum 25(OH)D level is highly recommended in all short children with a clear need for a general vitamin D supplementation for all infants and young children in Qatar.


Asunto(s)
Conservadores de la Densidad Ósea/administración & dosificación , Colecalciferol/administración & dosificación , Raquitismo/tratamiento farmacológico , Deficiencia de Vitamina D/tratamiento farmacológico , Vitaminas/administración & dosificación , Fosfatasa Alcalina/sangre , Calcio/sangre , Relación Dosis-Respuesta a Droga , Femenino , Humanos , Lactante , Inyecciones Intramusculares , Masculino , Hormona Paratiroidea/sangre , Fosfatos/sangre , Estudios Prospectivos , Qatar , Raquitismo/diagnóstico , Resultado del Tratamiento , Vitamina D/análogos & derivados , Deficiencia de Vitamina D/sangre
10.
Acta Biomed ; 91(4): e2020172, 2020 11 10.
Artículo en Inglés | MEDLINE | ID: mdl-33525220

RESUMEN

BACKGROUND: Plain chest radiograph (CXR), although less sensitive than chest CT, is usually the first-line imaging modality used for patients with symptomatic SARS-CoV-2 infection. The relation between radiological changes in CXR and clinical severity of the disease in symptomatic patients with COVID 19 has not been fully studied and there is no scoring system for the severity of the lung involvement, using the plain CXR. AIM OF THE STUDY: Current COVID-19 radiological literature is dominated by CT and a detailed description CXR appearances in relation to the disease time course is lacking. We propose an easy scoring system (CO X-RADS) to describe the severity of chest involvement in symptomatic COVID 19 patients using CXR and to correlate the radiological changes with the clinical severity of the disease. PATIENTS AND METHODS: The clinical manifestations and CXR findings were recorded in 500 symptomatic COVID-19 positive patients who were admitted to Hamad Medical Corporation (HMC) COVID-19 designated facility Center from January to June 2020. The severity and outcome of the disease included: intensive care unit admission, need for oxygen therapy, mechanical ventilation. and mortality rate. RESULTS: Most of our symptomatic patients (86.8%) had mild and moderate clinical manifestations. The remaining 13.2% had severe manifestations, including: fever, persistent dry cough, shortness of breath, dyspnea, abdominal and generalized body pains. Based on our radiological scoring system (0 to 10) patients were distributed according to their CXR findings into different categories and according to our suggested (CO X-RADS) severity system into five categories (0 to IV). Patients with mild clinical manifestations showed low scoring in CXR (score 0 up to 4) and they represented 72% of our patients. Patients with moderately severe clinical manifestations showed mainly GGO (scoring 5 and 6) and represented about 14.8% of patients. Patients presented with severe clinical manifestations had obvious lung consolidations at the time of presentation with CXR scoring system ≥ 7 and represented about 13.2% of patients. CONCLUSION: We proposed a simple CXR reporting scoring system (CO X-RADS) to categorize COVID-19 patients according to their radiological severity. This radiological score was correlated well with the clinical severity score of patients. We encourage other centers to test this scoring system in correlation with the clinical status of patients.


Asunto(s)
COVID-19/diagnóstico por imagen , Índice de Severidad de la Enfermedad , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Radiografía Torácica , Estudios Retrospectivos
11.
J Trop Pediatr ; 54(5): 347-9, 2008 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-18450819

RESUMEN

We studied growth hormone (GH) stimulation and insulin-like growth factor -I (IGF-I) generation tests in 15 children with neglected congenital hypothyroidism (CH) (age = 6.4 +/- 4.2 years) and measured their growth parameters for >1 years after starting thyroxine (T4) replacement. One year after treatment, height SDS (HtSDS) increased from -4.3 +/- 2.5 to -2.7 +/- 2.3. Peak GH response to clonidine increased from 3.2 +/- 1.2 ng ml(-1) to 7.62 +/- 1.38 ng ml(-1) after treatments. Basal and peak IGF-I response to GH increased from (34.66 +/- 17.3 ng ml(-1) and 58.4 +/- 36.99 ng ml(-1), respectively) before treatment to (130.6 +/- 97.8 ng ml(-1) and 193.75 +/- 122.5 ng ml(-1), respectively). HtSDS increments were correlated significantly with basal free T4 concentrations (r = 0.622, P < 0.01). In summary, after long period of hypothyroidism, T4 replacement produced significant, although incomplete, catch-up growth through a partial recovery of GH- IGF-I axis.


Asunto(s)
Estatura/efectos de los fármacos , Hipotiroidismo Congénito/sangre , Hipotiroidismo Congénito/tratamiento farmacológico , Hormona de Crecimiento Humana/sangre , Factor I del Crecimiento Similar a la Insulina/metabolismo , Tiroxina/uso terapéutico , Índice de Masa Corporal , Estudios de Casos y Controles , Niño , Preescolar , Egipto , Crecimiento/efectos de los fármacos , Hormona de Crecimiento Humana/metabolismo , Humanos , Estudios Retrospectivos , Resultado del Tratamiento
12.
Pediatr Endocrinol Rev ; 6 Suppl 1: 155-7, 2008 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-19337171

RESUMEN

A 16-year-old boy with transfusion-dependent thalassemia major presented with tetany, numbness, bone pain, short stature and pubertal delay. His height SDS score=-2.6, BMI=22.4, spleen was palpable 5 cm and liver 7 cm below the costal margins. The cardio-vascular examination was normal. Laboratory investigations showed a hemoglobin level (8 g/dL), hypocalcemia, hyperphosphatemia and elevated alkaline phosphatase (ALP) with serum 25-OH D below 3 ng/ml and a normal magnesium level. Serum parathyroid hormone (PTH) level was lower (21 pg/mL; normal 16-70 pg/mL) than expected for the degree of hypocalcemia. Serum ferritin concentration was 4442 ug/L, insulin-like growth factor I (IGF-I) was 31 microg/L (normal 122- 286 microg/L), free T4 was 13.1 microg/dL, TSH 1.2 mIU/ml. These results revealed a combined vitamin D-parathyroid defect. Peak growth hormone (GH) responses to clonidine and glucagon tests were 7.6 ng/ml and 6.2 ng/ml, respectively. Serum LH and FSH concentrations were below 0.5 U/L and testosterone was below 10 ng/dl. Radiographs revealed osteopenia of the phalanges and long bones and DXA scanning revealed low BMD Z-score of the femoral neck and 4th and 5th lumbar spines. MRI showed evidence of hemosiderin deposition in the pituitary. The patient was started on oral daily calcium carbonate (1500 mg elemental calcium) and vitamin D2 (calciferol) 25,000 IU/day and intensive iron chelation therapy. A low dose of IM testosterone enanthate (1 mg/kg/month) was injected for 6 months. Follow-up after 4, 8 and 12 months revealed normal Ca, PO4, ALP, and 25-OH D concentrations and disappearance of spasms and numbness and increased growth velocity. In conclusion, investigating calcium homeostasis at regular intervals and early management of any abnormality can preclude the occurrence of complications.


Asunto(s)
Hipoparatiroidismo/complicaciones , Hipopituitarismo/complicaciones , Talasemia beta/complicaciones , Adolescente , Calcio/administración & dosificación , Calcio/metabolismo , Ergocalciferoles/administración & dosificación , Ergocalciferoles/metabolismo , Humanos , Hipoparatiroidismo/metabolismo , Hipoparatiroidismo/terapia , Hipopituitarismo/metabolismo , Hipopituitarismo/terapia , Quelantes del Hierro/uso terapéutico , Masculino , Talasemia beta/metabolismo , Talasemia beta/terapia
13.
Pediatr Endocrinol Rev ; 6 Suppl 1: 123-6, 2008 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-19337165

RESUMEN

The modern radiologist is unlikely to encounter the classic radiographic features of thalassemia other than in teaching files. The main pathological change that leads to radiological skeletal changes in beta thalassemia major is extensive marrow proliferation. The severity of the skeletal responses is related to the type of thalassemia, the extent and duration of the disease, the type of treatment and the volume of blood transfusions given to the patient, as well as the side effects of transfusion-chelation therapy, and also depends on the bone involved. The radiographic features can be divided into those affecting the skeleton (axial and appendicular) and those occurring extra-medullary. Axial skeletal changes mainly include skull and facial bones, paranasal sinuses, vertebral bodies, weight-bearing bones, while appendicular skeleton manifestations are more pronounced in peripheral bones, mainly hands and feet as well as ribs. Patients on repeated blood transfusions and iron-chelation therapy may demonstrate variable range of manifestations than the scope of untreated patients.


Asunto(s)
Huesos/patología , Talasemia beta/patología , Huesos/diagnóstico por imagen , Humanos , Quelantes del Hierro/efectos adversos , Radiografía , Reacción a la Transfusión , Talasemia beta/diagnóstico por imagen , Talasemia beta/terapia
14.
Metabolism ; 80: 66-79, 2018 03.
Artículo en Inglés | MEDLINE | ID: mdl-28987275

RESUMEN

Bone disorders in patients with thalassemia major (TM) and intermedia (TI) constitute complex conditions that result from various factors affecting the growing skeleton. Although much progress has been made in our understanding of the natural history, pathogenesis and clinical manifestations of ß- and δß-thalassemia, bone manifestations remain a puzzle for the clinician. In this review, we outline the key points in the current literature on the pathogenesis and management of bone disease in patients with TM and TI who were conventionally treated in recent decades with frequent blood transfusions and iron chelation. Prevention, early recognition and treatment are the most effective strategies for the management of bone disease in these patients. However, further studies are required to maintain optimal bone health for both TM and TI patients. Studying bone disease in patients with non-transfusion dependent TI, which seems to worsen considerably with age, is important to delineate the effect of the disease itself on bone health without the intervening factors of transfusions, iron intoxication and chelation.


Asunto(s)
Enfermedades Óseas/etiología , Talasemia beta/complicaciones , Animales , Enfermedades Óseas/genética , Enfermedades Óseas/patología , Enfermedades Óseas/terapia , Remodelación Ósea , Humanos , Talasemia beta/genética , Talasemia beta/patología , Talasemia beta/terapia
15.
Acta Biomed ; 87(2): 121-31, 2016 09 13.
Artículo en Inglés | MEDLINE | ID: mdl-27648992

RESUMEN

Unaccompanied minors refer to immigrants who are under the age of 18 and are not under the care of a parent or legal guardian. Age assessment is used in Europe mainly to establish whether or not an individual is under 18 years of age and therefore eligible for protection under the United Nations' Convention on the Rights of the Child (UN - CRC). EU Member States use a combination of techniques to determine the age of a minor and to certify minor status, including interviews and documentation, physical examinations (anthropometric assessment; sexual maturity assessment; dental observation); psychological and sociological assessment; radiological tests (carpal, dental or collarbone x-rays). All such techniques are criticized as they are often arbitrary, do not take into account ethnic variations, and are based on reference materials that are outdated, invasive and may procure harm to the individuals whose age is assessed. They also generate a margin of error that makes them inaccurate to use. There is a debate about the risks and ethics associated with the use of X-rays for non-medical purposes versus the benefits of more accurate age assessments in the interest of justice. It appears that in European countries many individuals carrying out age assessment do not have sufficient training or are not sufficiently independent enough to be carrying out such assessments. Moreover, there is a lack of standardized approach between countries or even within the same country. Only some countries clearly indicate a margin of error in the results of age assessment examinations but there is no consensus - within and among countries - about the width of such margins in relation to each exams applied. It has been advised that the expert report should give the degree of age probability to allow Magistrate to interpret the age assessment results on the 'balance of probabilities' and give the detainee the right to the rule of the 'benefit of the doubt'. It also addresses concerns rested in the convention of the Rights of the Child.


Asunto(s)
Determinación de la Edad por el Esqueleto , Determinación de la Edad por los Dientes , Emigrantes e Inmigrantes , Menores , Adolescente , Desarrollo Óseo , Huesos/diagnóstico por imagen , Niño , Europa (Continente) , Humanos , Examen Físico , Probabilidad
16.
Case Rep Pediatr ; 2014: 903421, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-24511404

RESUMEN

Splenic abscess is one of the rarest complications of the UVC in a newborn and it is hypothesized that it could be due to an infection or trauma caused by a catheter. The case that is being reported presented with abdominal distension and recurrent desaturation with suspicion of neonatal sepsis versus necrotizing enterocolitis. However, the final diagnosis was splenic abscess as a complication of an inappropriate UVC insertion which was discovered by abdominal ultrasound. The patient was given broad spectrum antibiotics empirically and the symptoms were resolved without any surgical intervention. Such cases and controlled studies need to be reported in order to identify further causes and risk factors associated with splenic abscess in a patient with UVC which can eventually help us adopt preventive strategies to avoid such complications.

17.
Indian J Endocrinol Metab ; 18(4): 546-51, 2014 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25143915

RESUMEN

INTRODUCTION: Osteoporosis represents the second most common cause of endocrinopathy in patients with beta thalassemia major (BTM). Some drugs proved effective to reduce vertebral and non-vertebral fracture risk. Denosumab is a fully human monoclonal antibody to the receptor activator of nuclear factor kappa B ligand (RANKL), a member of the tumor necrosis factor receptor superfamily essential for osteoclastogenesis. The efficacy and safety of denosumab in BTM-induced osteoporosis has not been tested. OBJECTIVE: To evaluate the efficacy and safety of anti-RANKL on the biochemical and radiological parameters of bone mineralization in patients with BTM-induced osteoporosis. DESIGN: The study population was selected using the random sampling method from the patient's database of our thalassemia clinic. Transfusion-dependent BTM patients above 18 years with no history of treatment with bisphosphonates were randomly selected. Bone mineral density (BMD) of the lumbar spine (LS) and right femoral neck (FN) were measured by dual energy X-ray absorption (DEXA) scan using a calibrated method. Independent factors likely to be associated with low bone mass were determined and included in the analysis to ascertain possible associations. PATIENTS AND METHODS: We studied 30 patients with BTM-induced osteoporosis as per World Health Organization criteria (T Score of less than - 1.0 being defined as osteopenic and a T Score of less than - 2.5 being referred as osteoporotic). 19 males and 11 females aged between 18 and 32 years, with full pubertal development (Tanner's stage 5) at the time of the study. Their mean serum ferritin concentration was 3557 ng ± 1488 ng/ml. Every patient underwent DEXA scan as a baseline and after 12 months of denosumab therapy. Biochemical evaluation including serum concentrations of creatinine, Na, K, calcium, phosphorus, parathormone, bone specific alkaline phosphatase and type 1 collagen carboxy telopetide (ICCT) using enzyme-linked immunosorbent assay (Nordic Bioscience Diagnostics A/S) was done at baseline, after a month and then every 3 months for 12 months after starting denosumab. 60 mg of denosumab was administered subcutaneously twice yearly for a year. The mean BMD T Scores at baseline were -2.7 at the LS and -2.1 at the FN. RESULTS: Denosumab therapy for a year was associated with a significant increase in BMD of 9.2% (95% confidence interval [CI], 8.2-10.1) at the LS and 6.0% (95% CI, 5.2-6.7) at the FN. Denosumab treatment decreased serum ICCT levels by 56% at 1 month and normalized them in all patients at 1 year. Significant correlations were found between BMD T Score before and 1 year after denosumab in LS (r = 0.752, P < 0.001) and FN (r = 0.758 P < 0.001), respectively. The most common side effects were pain in the back and extremities (12%) and nausea (10%). Asymptomatic hypocalcaemia occurred in two patients. CONCLUSION: Denosumab therapy for a year significantly increased BMD density at LS and FN of patients with BTM and was associated with a rapid and sustained reduction in ICCT levels. Further studies are required to confirm long-term effects of this therapy.

18.
Indian J Endocrinol Metab ; 17(4): 697-703, 2013 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-23961489

RESUMEN

INTRODUCTION: The Clinical and radiological manifestations of newborns with severe VDD have not been studied well. MATERIALS AND METHODS: We studied the clinical, biochemical, and radiological manifestations of 10 full-term (FT) newborns (6: M, 4: F) infant presented to with symptomatic hypocalcemia (seizure) secondary to vitamin D deficiency (VDD) during the first 10 days of life are described. All were exclusively breastfed since birth. All their mothers have low 25 hydroxy vitamin D (25OHD) level <10 ng/mL and were not taking vitamin supplements during pregnancy. RESULTS: FT newborns with hypocalcemia secondary to VDD presented with generalized convulsions (10/10) and craniotabes (8/10), but none had rachitic chest rosaries or joint broadening. Cranial ultrasonographic evaluation was normal. Serum 25OHD concentrations were low in these newborns (13.2 ± 3.8 ng/mL) and their mothers (8.1 ± 1.5 ng/mL). A total of 60% of them had increased parathormone (PTH) concentrations (>60 ng/mL) and 60% had decreased magnesium (Mg) concentrations (<0.7 mmol/L). Their alkaline phosphatase (ALP) concentrations were significantly higher than normal newborns. All other laboratory results (liver function tests, urea and electrolytes, C reactive protein, lumbar puncture, blood culture, and lactate) were normal. In all patients, seizures ceased within 2 days of starting treatment with alphacalcidol and calcium. Radiological manifestations included metaphyseal band of relative lucency (osteopenia), just under the line of provisional calcification, within distal radius (7/10), femur (4/10), and tibia (3/10), mild cupping and haziness of distal radius (2/10). DISCUSSION: Newborns with VDD had significantly lower serum calcium, ALP and PTH and higher phosphate concentrations, compared to older infants with VDD rickets. In newborns with VDD, serum calcium levels were correlated significantly with 25OHD (r = 0.597, P < 0.001), Mg concentrations (r = 0.436, P < 0.001) and negatively with ALP concentrations (r = -0.451, P < 0.001). Serum PTH concentrations were correlated significantly with serum Mg (r = 0.78, P < 0.0001) but not with serum calcium (r = -0.103, P = 0.3) or 25OHD (r = -0.03, P = 0.7) concentrations. CONCLUSION: The clinical, biochemical, and radiological manifestations of VDD in newborns indicate that they are less adapted to VDD compared to older infants. VD supplementation for mothers and newborns should be considered to avoid short-term complications of VDD in the neonatal period and on the growing infants especially in countries with high prevalence of VDD.

19.
Indian J Endocrinol Metab ; 17(1): 8-18, 2013 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-23776848

RESUMEN

The current management of thalassemia includes regular transfusion programs and chelation therapy. It is important that physicians be aware that endocrine abnormalities frequently develop mainly in those patients with significant iron overload due to poor compliance to treatment, particularly after the age of 10 years. Since the quality of life of thalassemia patients is a fundamental aim, it is vital to monitor carefully their growth and pubertal development in order to detect abnormalities and to initiate appropriate and early treatment. Abnormalities should be identified and treatment initiated in consultation with a pediatric or an adult endocrinologist and managed accordingly. Appropriate management shall put in consideration many factors such as age, severity of iron overload, presence of chronic liver disease, thrombophilia status, and the presence of psychological problems. All these issues must be discussed by the physician in charge of the patient's care, the endocrinologist and the patient himself. Because any progress in research in the field of early diagnosis and management of growth disorders and endocrine complications in thalassemia should be passed on to and applied adequately to all those suffering from the disease, on the 8 May 2009 in Ferrara, the International Network on Endocrine Complications in Thalassemia (I-CET) was founded in order to transmit the latest information on these disorders to the treating physicians. The I-CET position statement outlined in this document applies to patients with transfusion-dependent thalassemia major to help physicians to anticipate, diagnose, and manage these complications properly.

20.
Cases J ; 2: 9153, 2009 Dec 07.
Artículo en Inglés | MEDLINE | ID: mdl-20062670

RESUMEN

Acute mesenteric ischemia is a serious acute abdominal condition requiring early diagnosis and intervention to improve the outcome. Although transmural acute bowel infarction represents about 1% of all cases of acute abdomen, it has a higher annual mortality rate than colon cancer. It tends to affect the colon in segmental fashion, mostly the splenic flexure and rectosigmoid portions of the colon. Isolated ischemia of the right side of the colon is rarely reported, especially in association with shock. Diagnosis of acute colonics ischemia is challenging as it may easily be confused with other non ischemic conditions both clinically and radiologically. Surgical resection is still the main curative approach. We present a case of segmental terminal ileum, cecum and part of ascending colon infarction due to isolated IleoColic artery thrombosis.

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