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1.
Acta méd. costarric ; 65(3): 155-159, jul.-sep. 2023. graf
Article in Spanish | LILACS, SaludCR | ID: biblio-1556694

ABSTRACT

Resumen La drepanocitosis o anemia de células falciformes es una hemoglobinopatía hereditaria que se transmite de forma autosómica recesiva, la cual está asociada con episodios de dolor agudo y daño progresivo a órganos blanco que producen un gran impacto en la esperanza y la calidad de vida de quienes la padecen. La necrosis avascular de la cabeza del fémur es una complicación bien conocida en los pacientes con drepanocitosis presente hasta en un 25 % de los pacientes adultos y tanto en un 12 % de los pacientes pediátricos. A continuación, se presenta el caso de un paciente masculino de 9 años con drepanocitosis, quien en cita de control refirió 15 días de coxalgia y claudicación de la marcha. Tras realizar estudios radiológicos se documentó una necrosis avascular de la cabeza del fémur, convirtiéndose de esta manera en el primer caso documentado en el Hospital Nacional de Niños de Costa Rica de un paciente pediátrico con una necrosis avascular de la cadera del fémur como complicación de su drepanocitosis.


Sickle cell disease is an autosomic recessive hereditary hemoglobin disorder, associated with episodes of acute pain and progressive organ damage with great impact on a patient's life expectancy and quality of life. Avascular necrosis of the femoral head is a well-known complication of sickle cell disease affecting almost 25% of adult patients and just in 12% of pediatric patients. Below is the case of a 9-year-old male patient with sickle cell disease, who on a control appointment reported 15 days of hip pain and gait claudication. After performing radiological studies, avascular necrosis of the femoral head was diagnosed, becoming the first case documented at the National Children Hospital of Costa Rica of avascular necrosis of the femoral head as a complication in a pediatric patient with sickle cell disease.


Subject(s)
Humans , Male , Child , Femur Head Necrosis/diagnosis , Anemia, Sickle Cell/complications , Costa Rica
2.
Rev. Bras. Ortop. (Online) ; 53(5): 537-542, Sept.-Oct. 2018. tab, graf
Article in English | LILACS | ID: biblio-977883

ABSTRACT

ABSTRACT Objectives: This study is aimed at evaluating whether core decompression of the femoral head in the early stages of femoral head osteonecrosis improves patients' subjective perception of pain and avoids the progression of the disease to a femoral head collapse and a final indication of total hip arthroplasty. Methods: Eighteen patients (30 hips) in the early stages of the disease (Ficat and Arlet 1 and 2A) were evaluated through clinical, radiological, risk factor maintenance, and by the functional Merle D'Aubigné, and Postel score before and after core decompression of the femoral head. Results: There was an improvement of symptoms up to the sixth month in 83.3% of the hips evaluated through the Merle D'Aubigné and Postel score. However, 73.3% of the cases evolved with femoral head collapse, and in 50%, total hip arthroplasty was indicated regardless of whether or not the risk factors were maintained. Conclusions: Core decompression of the femoral head improves patients' pain early in the initial stages of the pathology. However, it does not alter the prognosis and the ultimate indication of total hip arthroplasty in the final stages of the disease.


RESUMO Objetivos: O presente estudo tem como objetivo avaliar se a descompressão simples da cabeça femoral nos estágios iniciais da osteonecrose da cabeça femoral melhora a percepção subjetiva da dor dos pacientes e se evita a progressão da doença para o colapso da cabeça femoral e a indicação final de artroplastia total do quadril. Métodos: Foram avaliados 18 pacientes (30 quadris) em estágios iniciais da doença (Ficat e Arlet 1 e 2 A) por critérios clínicos, radiológicos, manutenção dos fatores de risco e pela escala funcional de Merle D'Aubigné e Postel antes e após a descompressão simples da cabeça femoral. Resultados: Houve melhoria dos sintomas precocemente (até o sexto mês) em 83,3% dos quadris avaliados pela escala de Merle D'Aubigné e Postel. No entanto, 73,3% dos casos evoluíram com colapso da cabeça femoral e em 50% deles foi indicada artroplastia total do quadril, independentemente da manutenção ou não dos fatores de risco. Conclusões: A descompressão simples da cabeça femoral melhora a dor dos pacientes precocemente nos estágios iniciais da patologia. Entretanto, não altera o prognóstico da doença e a indicação final de artroplastia total do quadril nos estágios finais da doença.


Subject(s)
Humans , Male , Female , Treatment Outcome , Femur Head Necrosis/diagnosis , Femur Head Necrosis/physiopathology , Femur Head Necrosis/pathology
3.
Int Orthop ; 42(7): 1527-1534, 2018 07.
Article in English | MEDLINE | ID: mdl-29582115

ABSTRACT

PURPOSE: The aim of this study was to describe the clinical features of osteonecrosis (ON) in sickle cell disease (SCD) patients in Bahia, a Northeast state with the highest prevalence of the disease in Brazil. METHODS: Between 2006 and 2017, 283 cases of osteonecrosis in SCD patients were enrolled to analyse the age at diagnosis, genotype, gender, pain, distribution of the lesions and disease staging. MRI and radiograph were obtained at the participation. RESULTS: Of the 283 SCD cases, 120 (42.4%) were haemoglobin SS genotype while 163 (57.6%) were SC genotype. Two hundred and forty-six cases were bilateral and 37 were unilateral, with an average age at diagnosis of 33.7 (range 10-67) years. The most frequent identified ON site not only was the hip (74.6%), but also affected shoulder, knee and ankle. Most cases presented at early stage I (172, 60.8%) disease. No significant differences on the features of osteonecrosis were identified between haemoglobin SS and haemoglobin SC cases. CONCLUSIONS: Given the relatively high prevalence of bilateral osteonecrosis at early stages, painful symptoms and rather late age at diagnosis, SCD patients should have radiological examination of their joints more often in order to prevent severe functional disability and increase patient's life quality.


Subject(s)
Anemia, Sickle Cell/complications , Femur Head Necrosis/complications , Adolescent , Adult , Aged , Brazil , Child , Cross-Sectional Studies , Female , Femur Head Necrosis/diagnosis , Femur Head Necrosis/epidemiology , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Prevalence , Young Adult
4.
Rev. cuba. ortop. traumatol ; 29(1): 62-66, ene.-jun. 2015. ilus
Article in Spanish | CUMED | ID: cum-64430

ABSTRACT

La necrosis aséptica de la cabeza femoral (NACF) es la muerte de tejido óseo en la cabeza del fémur debido a inadecuado riego sanguíneo, la misma se ha asociado a diversas causas. En la osteonecrosis el grado de destrucción tanto de la arquitectura ósea como de la red de aporte sanguíneo a la cabeza del fémur se afecta en extremo y en muchos casos es necesaria la cirugía como las osteotomías valguizantes, varizantes, Sugioka y otras). En este estudio se utilizó la descompresión central asociada a la terapia celular. Se presenta un paciente masculino de 46 años, con antecedentes de Diabetes Mellitus, bebedor habitual, al cual se le diagnosticó de NACF de cadera izquierda mediante tomografía computarizada, y posteriormente se le realizó cirugía con perforaciones por vía lateral trocantérica (Descompresión Central) y administración de células mononucleares autólogas. El paciente fue dado de alta a las 48 h de operado, con limitación del apoyo los primeros 10 días, y después deambulación asistida. Se le realizaron radiografías a los 6 meses del implante. Después de un año de operado se le da alta y se indica la reincorporación del paciente a sus actividades sociales habituales, no constatando progresión de la osteonecrosis, y logrando así la deambulación sin asistencia(AU)


Aseptic necrosis of the femoral head (AVN-FH) is the death of bone tissue in the femoral head because of inadequate blood supply; it has been associated with various causes. The degree of destruction of bone architecture and the blood supply network to the femoral head is extremely affected. In many cases, surgery is necessary as valgus osteotomies, varus, Sugioka and others. Core decompression associated with cell therapy was used in this study. A male patient aged 46 is presented here. this patient has history of Diabetes Mellitus, regular alcohol consumption, he was diagnosed with aseptic necrosis of the femoral head left hip with CT scan, and he subsequently underwent surgery by perforated lateral approach trochanteric (Central Decompression) and administration of autologous mononuclear cells. The patient was discharged 48 hours after surgery, with limited support during the first 10 days, and then assisted ambulation. Radiographs were performed at 6 months after implantation. After a year of surgery the patient is discharged and he is instructed to return to his usual social activities, noting no progression of osteonecrosis, thus, non-assisted ambulation is achieved(AU)


Subject(s)
Humans , Male , Middle Aged , Femur Head Necrosis/diagnosis , Osteonecrosis/surgery , Osteotomy/methods , Prostheses and Implants , Magnetic Resonance Spectroscopy
5.
Rev. cuba. ortop. traumatol ; 29(1): 62-66, ene.-jun. 2015. ilus
Article in Spanish | LILACS, CUMED | ID: lil-762765

ABSTRACT

La necrosis aséptica de la cabeza femoral (NACF) es la muerte de tejido óseo en la cabeza del fémur debido a inadecuado riego sanguíneo, la misma se ha asociado a diversas causas. En la osteonecrosis el grado de destrucción tanto de la arquitectura ósea como de la red de aporte sanguíneo a la cabeza del fémur se afecta en extremo y en muchos casos es necesaria la cirugía como las osteotomías valguizantes, varizantes, Sugioka y otras). En este estudio se utilizó la descompresión central asociada a la terapia celular. Se presenta un paciente masculino de 46 años, con antecedentes de Diabetes Mellitus, bebedor habitual, al cual se le diagnosticó de NACF de cadera izquierda mediante tomografía computarizada, y posteriormente se le realizó cirugía con perforaciones por vía lateral trocantérica (Descompresión Central) y administración de células mononucleares autólogas. El paciente fue dado de alta a las 48 h de operado, con limitación del apoyo los primeros 10 días, y después deambulación asistida. Se le realizaron radiografías a los 6 meses del implante. Después de un año de operado se le da alta y se indica la reincorporación del paciente a sus actividades sociales habituales, no constatando progresión de la osteonecrosis, y logrando así la deambulación sin asistencia(AU)


Aseptic necrosis of the femoral head (AVN-FH) is the death of bone tissue in the femoral head because of inadequate blood supply; it has been associated with various causes. The degree of destruction of bone architecture and the blood supply network to the femoral head is extremely affected. In many cases, surgery is necessary as valgus osteotomies, varus, Sugioka and others. Core decompression associated with cell therapy was used in this study. A male patient aged 46 is presented here. this patient has history of Diabetes Mellitus, regular alcohol consumption, he was diagnosed with aseptic necrosis of the femoral head left hip with CT scan, and he subsequently underwent surgery by perforated lateral approach trochanteric (Central Decompression) and administration of autologous mononuclear cells. The patient was discharged 48 hours after surgery, with limited support during the first 10 days, and then assisted ambulation. Radiographs were performed at 6 months after implantation. After a year of surgery the patient is discharged and he is instructed to return to his usual social activities, noting no progression of osteonecrosis, thus, non-assisted ambulation is achieved(AU)


Subject(s)
Humans , Male , Middle Aged , Osteonecrosis/surgery , Osteotomy/methods , Prostheses and Implants , Femur Head Necrosis/diagnosis , Magnetic Resonance Spectroscopy
6.
J Pediatr Orthop ; 35(4): 358-62, 2015 Jun.
Article in English | MEDLINE | ID: mdl-25075885

ABSTRACT

BACKGROUND: Redislocation after an open reduction for develpmental dislocation of the hip is relatively common. The purpose of this study was to determine if the use of a transarticular pin (TAP) is safe and effective in maintaining reduction. METHODS: A total of 578 patients (645 hips) were reviewed after an open reduction, mean age at the time of surgery was 2.1 years. In 621 cases a smooth Kirschner wire was placed across the joint. The rates of redislocation, avascular necrosis (AVN), and other complications were determined. AVN was classified according to Kalamchi et al. Outcome was determined at a minimum of 6 years using the Severin classification. RESULTS: Redislocation occurred in 27 cases (4.1% rate), 24 had a TAP (3.8%) and 3 did not (12.5%). AVN was observed in 127 cases (19.7%), it was type I in 73 cases, type II in 38 cases, type III in 14 cases, and type IV in 2 cases; AVN was seen in 123 cases which had a TAP (19.8%) and 4 cases which did not (16.7%). Analyzing pin placement: when it was in the inferior third of the neck the rate of AVN was 15.2% (32/211), in the middle third it was 21.7% (71/326), and in the superior third it was 28.6% (24/84). According to the Severin classification for the hips with a TAP, 496 were type I or II (79.8%), 113 were type III (18.2%), 10 were type IV (1.6%), and 2 were type V (0.3%); in the group without a TAP 19 cases were type I or II (79.2%), 4 were type III (16.7%), and 1 was type IV. CONCLUSIONS: The use of a TAP was effective in maintaining reduction and was not associated with significant morbidity. Placing the pin in the inferior third of the neck was associated with the lowest rate of AVN. LEVEL OF EVIDENCE: Level IV--therapeutic.


Subject(s)
Femur Head Necrosis , Hip Dislocation, Congenital , Orthopedic Procedures , Osteonecrosis , Postoperative Complications/diagnosis , Bone Nails , Child, Preschool , Female , Femur Head Necrosis/diagnosis , Femur Head Necrosis/etiology , Hip Dislocation, Congenital/diagnosis , Hip Dislocation, Congenital/surgery , Humans , Male , Mexico , Orthopedic Procedures/adverse effects , Orthopedic Procedures/instrumentation , Orthopedic Procedures/methods , Osteonecrosis/diagnosis , Osteonecrosis/etiology , Retrospective Studies , Treatment Outcome
7.
Genet Mol Res ; 13(4): 8342-52, 2014 Oct 20.
Article in English | MEDLINE | ID: mdl-25366728

ABSTRACT

This study evaluated the outcomes of using porous tantalum rods for the treatment of osteonecrosis of the femoral head (ONFH). We performed core decompression and inserted porous tantalum implants in 149 patients (168 consecutive hips) with ONFH. Hips had large (65), medium (64), or small (39) lesions; 63 lesions were lateral, 68 were central, and 35 were medial. Conversion to total hip arthroplasty (THA) was the end point of this survey. A total of 130 cases (138 hips) were followed. The mean follow-up time was 38.46 ± 5.76 months; 43 hips (31%) were converted to or needed THA. Of the 43 hips requiring THA, 33 had large lesions, including 1 medial, 3 central, and 29 lateral lesions; 9 had medium, lateral lesions, and 1 hip had a small, lateral lesion. Bone grafting was used in 59 hips, with 3 hips failing; 40 of 79 hips without bone grafts failed. The sum distances between the tops of the rods and the lateral lesion boundaries (SDTL, mm) were measured in anteroposterior and lateral radiographs. In the failure and spared groups, the average SDTLs were 7.65 ± 2.759 and 0.83 ± 2.286 mm, respectively. The survival of porous tantalum rods used for treating early-stage ONFH was affected by the size and location of the lesion, whether or not a bone graft was used, as well as the distance between top of the rod and the lateral boundary of the lesion.


Subject(s)
Femur Head Necrosis/therapy , Prostheses and Implants , Tantalum/therapeutic use , Adult , Female , Femur Head Necrosis/diagnosis , Femur Head Necrosis/etiology , Follow-Up Studies , Humans , Male , Tantalum/chemistry , Treatment Failure , Treatment Outcome
8.
Acta Ortop Mex ; 27(4): 265-72, 2013.
Article in Spanish | MEDLINE | ID: mdl-24707618

ABSTRACT

Avascular necrosis of the femoral head is a frequent condition with an insidious presentation; it is initially asymptomatic and thus it is seldom diagnosed at the early stages. The purpose of this review is to update the status of the diagnosis and non-surgical treatment of avascular necrosis of the femoral head. This paper analyzes the natural history of the disease as well as the prognostic importance of early diagnosis and the identification of the extension and location of osteonecrosis. It also discusses the different nonsurgical treatment methods such as: high energy extracorporeal shock waves, electromagnetic pulsed fields, hyperbaric oxygen therapy, drugs, physical therapy, and regenerative medicine. The conclusion is that no method is effective in the advanced stages and there is great hope of treating this condition without surgery; however, progress needs to be made in research, particularly in the use of external biophysical agents and regenerative medicine. The gold standard of treatment is still surgical decompression of the femoral head at the stages in which no collapse has occurred. The prognosis apparently improves with the use of biophysical and regenerative medicine adjuvants.


Subject(s)
Femur Head Necrosis/therapy , Femur Head Necrosis/diagnosis , Humans
9.
Cir Cir ; 80(6): 556-61, 2012.
Article in Spanish | MEDLINE | ID: mdl-23336152

ABSTRACT

BACKGROUND: sacral insufficiency fractures are a cause of debilitating pain in the elderly. These fractures were first described as a clinical entity in 1982. The bone in these patients is structurally weakened and often associated with diseases such as osteoporosis, cancer and immunological processes. This translates into back and pelvic pain unrelated to trauma. These fractures are usually caused by fatigue in most cases. Bone scans and MRI are the imaging studies with the most sensitivity for detecting sacral insufficiency. CLINICAL CASES: two patients with sacral insufficiency fractures who were studied by MRI and bone scan, in whom the diagnosis of sacral insufficiency was made, were treated by sacroplasty. CONCLUSIONS: sacral insufficiency is an underdiagnosed disease, caused by wide range of diseases, mainly osteoporosis. The studies of choice for diagnosis are MRI and bone scans. Techniques, such as percutaneous sacroplasty, produce significant improvements in pain scores and seem a suitable alternative for managing this disease.


Subject(s)
Fractures, Stress/complications , Low Back Pain/etiology , Sacrum/injuries , Spinal Fractures/complications , Accidental Falls , Aged , Bone Diseases, Metabolic/complications , Bone Diseases, Metabolic/diagnosis , Bone Diseases, Metabolic/diagnostic imaging , Female , Femur Head Necrosis/complications , Femur Head Necrosis/diagnosis , Femur Head Necrosis/surgery , Fractures, Spontaneous/complications , Fractures, Spontaneous/diagnosis , Fractures, Spontaneous/diagnostic imaging , Fractures, Stress/diagnosis , Fractures, Stress/diagnostic imaging , Fractures, Stress/surgery , Humans , Imaging, Three-Dimensional , Intervertebral Disc Displacement/complications , Intervertebral Disc Displacement/diagnosis , Lumbar Vertebrae/pathology , Magnetic Resonance Imaging , Positron-Emission Tomography , Sacrum/diagnostic imaging , Sacrum/surgery , Spinal Fractures/diagnosis , Spinal Fractures/diagnostic imaging , Spinal Fractures/surgery , Spinal Stenosis/complications , Spinal Stenosis/diagnosis , Spondylolisthesis/complications , Spondylolisthesis/diagnosis , Spondylolisthesis/diagnostic imaging , Tomography, X-Ray Computed , Vertebroplasty
10.
Orthop Traumatol Surg Res ; 95(2): 134-8, 2009 Apr.
Article in English | MEDLINE | ID: mdl-19285936

ABSTRACT

INTRODUCTION: Osteonecrosis of the femoral head (ONFH) is a frequent complication of sickle-cell disease. Numerous studies have demonstrated increased intraosseous pressure (IOP) in idiopathic necrosis and necrosis secondary to corticotherapy or alcohol poisoning. Several reports have testified to the clinical interest of decompression by drilling which, when performed in the early course of the pathology, can arrest or slow evolution. To the best of our knowledge, no studies have reported IOP increase in sickle-cell ONFH. The present study sought to show that intraosseous hyperpressure plays a role in the physiopathology of sickle-cell, like idiopathic, ONFH. MATERIALS AND METHODS: Sixteen intraosseous pressure (IOP) measurements were taken: eight in adult sickle-cell disease patients, four in sickle-cell trait carrying ONFH patients (AS) and four in non-sickle-cell ONFH patients (AA). Arterial blood-pressure equipment with bone-puncture needle was used to measure IOP in the great trochanter body. Three IOP measurements were made after zero calibration: before drilling (direct pressure: IOP-1), after hyperpressure test but before drilling (IOP-2), and after drilling (IOP-3). RESULTS: The present, admittedly short, series displayed elevated predrilling IOP-1 and IOP-2, reduced after drilling (IOP-3). Abnormal IOP fell after drilling performed for evolutive symptomatic ONFH. Significant differences in IOP-1 and IOP-2 were found, these being higher in the "sickle-cell disease" and "sickle-cell trait carriers" groups (p<0.05). Only in the sickle-cell groups was there a significant correlation between pain score and hyperpressure level, with significantly reduced pain after drilling. DISCUSSION: The elevated IOP levels found in symptomatic sickle-cell hips were comparable to those reported in the literature. Ischemia due to femoral head sinusoid occlusion by falciform globules with secondary intraosseous hyperpressure is the cause of the pain and of the onset and evolution of ONFH. The drilling tunnel acts as a safety valve, achieving real decompression of the segment involved and immediate postoperative reduction in or disappearance of pain. CONCLUSION: Measuring pressure is of diagnostic interest in sickle-cell disease patients with symptomatic hips. Manometry can be performed independently of surgery, under local anesthesia, and provides early confirmation of ONFH in geographic regions in which MRI is not readily available. It can be carried out very straightforwardly, without pressure sensor, using a simple water column (physiological saline) and three-way tap. Peroperative comparison of IOP-1 and IOP-3 is a means of assessing the effectiveness of decompression drilling.


Subject(s)
Anemia, Sickle Cell/diagnosis , Femur Head Necrosis/diagnosis , Femur Head Necrosis/surgery , Adolescent , Adult , Age Factors , Anemia, Sickle Cell/complications , Case-Control Studies , Decompression, Surgical/methods , Disease Progression , Female , Femur Head Necrosis/etiology , Follow-Up Studies , Hip Joint/physiopathology , Humans , Male , Manometry/methods , Middle Aged , Pressure/adverse effects , Reference Values , Risk Assessment , Severity of Illness Index , Sex Factors , Sickle Cell Trait/complications , Sickle Cell Trait/diagnosis , Treatment Outcome , Young Adult
11.
J Bone Joint Surg Br ; 90(2): 228-31, 2008 Feb.
Article in English | MEDLINE | ID: mdl-18256094

ABSTRACT

Metal meshes are used in revision surgery of the hip to contain impacted bone grafts in cases with cortical or calcar defects in order to provide rotational stability to the stem. However, the viability of bone allografts under these metal meshes has been uncertain. We describe the histological appearances of biopsies obtained from impacted bone allografts to the calcar contained by a metal mesh in two femoral reconstructions which needed further surgery at 24 and 33 months after the revision procedure. A line of osteoid and viable new bone was observed on the surface of necrotic trabeculae. Active bone marrow between these trabeculae showed necrotic areas in some medullary spaces with reparative fibrous tissue and isolated reactive lymphocytes. This is interpreted as reparative changes after revascularisation of the cancellous allografts. These pathological findings are similar to those reported in allografts contained by cortical host bone and support the hypothesis that incorporation of morcellised bone under metal meshes is not affected by these devices.


Subject(s)
Anti-Bacterial Agents/administration & dosage , Arthroplasty, Replacement, Hip/adverse effects , Femur Head Necrosis/diagnosis , Prosthesis-Related Infections/diagnosis , Vancomycin/administration & dosage , Acetabulum/diagnostic imaging , Acetabulum/microbiology , Adult , Aged , Biopsy , Female , Femur Head Necrosis/microbiology , Humans , Male , Radiography , Staphylococcal Infections/prevention & control , Surgical Mesh , Transplantation, Homologous
12.
J Pediatr ; 151(2): 197-201, 2007 Aug.
Article in English | MEDLINE | ID: mdl-17643778

ABSTRACT

OBJECTIVE: To document the effects of interrupting enzyme replacement therapy (ERT) for at least 1 year in a group of children with type 1 Gaucher disease. STUDY DESIGN: All children with type 1 Gaucher disease who were treated at 2 pediatric centers and who were required to suspend ERT for at least 1 year were studied before, during, and after treatment interruption. Hemoglobin and platelet levels, organomegaly, growth, and bone manifestations were monitored. RESULTS: Five of 32 children experienced treatment interruptions. Before ERT, all children had splenomegaly, 4 children had hepatomegaly, 4 children had growth retardation, 3 children had skeletal manifestations, 3 children had thrombocytopenia, and 1 child had anemia. After 1 to 7 years of ERT, all children were growing normally, none had skeletal manifestations, organomegaly had decreased or disappeared, and hematologic features had improved. After 15 to 36 months of ERT interruption, splenomegaly recurred or worsened in all children, hepatomegaly and hematologic features recurred or worsened in 4 children, serious bone manifestations developed in 4 children, and 3 children experienced growth retardation. After at least 11 months of resumed ERT in 4 children, 2 had hepatomegaly, 2 had splenomegaly, and all had persistent skeletal manifestations. CONCLUSION: Interruption of ERT in children with type 1 Gaucher disease should be avoided because it can cause recurrent organomegaly, growth delays, and skeletal manifestations that do not resolve after treatment reinstatement.


Subject(s)
Gaucher Disease/diagnosis , Gaucher Disease/drug therapy , Glucosylceramidase/administration & dosage , Treatment Refusal , Adolescent , Child , Child, Preschool , Cohort Studies , Dose-Response Relationship, Drug , Drug Administration Schedule , Enzyme Therapy , Female , Femur Head Necrosis/diagnosis , Femur Head Necrosis/epidemiology , Follow-Up Studies , Hemoglobins/analysis , Hepatomegaly/diagnosis , Hepatomegaly/epidemiology , Humans , Infant , Male , Retrospective Studies , Risk Assessment , Thrombocytopenia/diagnosis , Thrombocytopenia/epidemiology
13.
Arq. ciênc. vet. zool. UNIPAR ; 9(2): 111-115, jul.-dez. 2006. ilus, tab
Article in Portuguese | LILACS | ID: lil-453732

ABSTRACT

O trabalho teve como objetivo, descrever a técnica da artroplastia excisional pelo acesso dorso-caudal em 16 cães com necrose asséptica da cabeça do fêmur, como uma nova alternativa terapêutica cirúrgica para o alívio do paciente traumatizado. A necrose asséptica da cabeça femoral, também conhecida como Legg-Calvé-Perthes, apresenta em todos os casos, o osso da cabeça e colo femorais com necrose e deformação. Acomete normalmente animais de pequeno porte, apresentando ao exame clínico claudicação gradual do membro afetado, devido à dor articular. Ao exame radiográfico observam-se focos de diminuição da densidade óssea na cabeça e colo femorais, e em casos mais graves presença de osteófitos na margem acetabular dorsal. Foram atendidos 16 cães (7 Poodle; 4 Pinscher; 1 Pequinês; 1 Beagle; 1 Yorkshire Terrier; 2 Sem Raça Definida) no Hospital Veterinário da Universidade Norte do Paraná (UNOPAR), Arapongas, PR, com diagnóstico de necrose asséptica da cabeça do fêmur. O tratamento preconizado foi cirúrgico, através da artroplastia por excisão da cabeça e colo femorais com abordagem dorso-caudal, e o pós-operatório constituíram-se de antibioticoterapia por 3 dias, curativo tópico até a cicatrização da ferida cirúrgica. A administração de DMSO (Dimesol®) na dose de 0,8 mL/Kg diluído em 125 mL de solução fisiológica de NaCl a 0,9%, durante 4 dias, repouso nos primeiros 7 dias e volta gradativa ao exercício. O retorno ao uso do membro afetado e o desaparecimento total da claudicação foram observados 20 dias após a cirurgia.


The aim of this study is to describe the excisional arthroplasty technique through dorsocaudal access in 16 dogs with aseptic necrosis of the femoral head as a new therapeutic alternative for the relief of traumatized patients. The femoral head necrosis, also known as Legg-Calvé-Perthes, presents, in all cases, both the bone of the femoral head and the neck with necrosis and deformation. Small animals are usually affected thus presenting a gradual level of lameness upon the painful member due to joint pain in the clinical exam. Traits of bone density loss in the femoral head and neck, and, in severe cases, the occurrence of osteophyte in the dorsal border of acetabular appear in the radiographical exam. Sixteen dogs (7 Poodle; 4 Pinscher; 1 Pekinese; 1 Beagle; 1 Yorkshire Terrier; 2 mongrel dogs) presenting aseptic necrosis of the femoral head were assisted in the Veterinary Hospital of the Universidade do Norte do Paraná (UNOPAR) in Arapongas–PR . The surgery was carried out through the excision arthroplasty of the head and femoral neck with a dorsocaudal approach. The postoperative period consisted of antibioticotherapy for 3 days, topical curative until the surgical wound healing. The administration of 0,8 ml/kg DMSO (Dimesol®) diluted into 125 ml of NaCL 0.9% physiological solution for 4 days; a rest cure in the first 7 days followed by a gradual return to the exercise. Returning to use the affected member as well as the total disappearance of the lameness level was observed 20 days after the surgery.


El trabajo tuvo como objetivo, describir la técnica de la artroplastía por escisión dorsal en 16 perros con necrosis aséptica de la cabeza femoral como una nueva alternativa terapéutica de la cirugía para el alivio del paciente traumatizado. La necrosis aséptica de la cabeza femoral, también conocida como Legg-Calvé-Perthes, presenta en todos los casos, el hueso de la cabeza y cuello femoral con necrosis y deformaciones. Acomete normalmente a los animales de pequeño porte, presentando en el examen clínico claudicación gradual del miembro afectado, debido al dolor articular. En el examen radiográfico se observa focos de disminución de la densidad ósea en la cabeza y en el cuello femoral, y en los casos más graves, la presencia de osteófitos en la borda del acetábulo dorsal. Fueron atendidos 16 perros [7 Poodles, 4 pinscher, 1 pequinés, 1 Beagle, 1 Yorkshire Terrier, 2 Sin Raza Definida (SRD)] en el Hospital Veterinario de la Universidad Norte del Paraná (UNOPAR) - Arapongas-PR con diagnóstico de necrosis aséptica de la cabeza femoral. El tratamiento proconizado fue la cirugía a através de la artroplastía por escisión de la cabeza y el cuello femoral con abordaje dorso-caudal y el posoperatorio se constituyó de antibiótico terapia por 3 (tres) días, curativo tópico hasta la cicatrización de la herida quirúrgica, administración de DMSO (Dimesol®) en la dosis de 0,8 ml/kg diluído en 125 ml de solución fisiológica de NaCL a 0.9% por 4 (cuatro) días, reposo en los primeros 7 (siete) días y vuelta gradual a los ejercicios. El retorno al uso del miembro afectado y el desaparecimiento total de la claudicación fue observado 20 (veinte) días después de la cirugía.


Subject(s)
Animals , Arthroplasty/methods , Arthroplasty/veterinary , Dogs , Femur Head Necrosis/diagnosis , Femur Head Necrosis/veterinary
14.
Arq. ciênc. vet. zool. UNIPAR ; 9(2): 111-115, jul.-dez.2006. ilus, tab
Article in Portuguese | VETINDEX | ID: vti-3209

ABSTRACT

O trabalho teve como objetivo, descrever a técnica da artroplastia excisional pelo acesso dorso-caudal em 16 cães com necrose asséptica da cabeça do fêmur, como uma nova alternativa terapêutica cirúrgica para o alívio do paciente traumatizado. A necrose asséptica da cabeça femoral, também conhecida como Legg-Calvé-Perthes, apresenta em todos os casos, o osso da cabeça e colo femorais com necrose e deformação. Acomete normalmente animais de pequeno porte, apresentando ao exame clínico claudicação gradual do membro afetado, devido à dor articular. Ao exame radiográfico observam-se focos de diminuição da densidade óssea na cabeça e colo femorais, e em casos mais graves presença de osteófitos na margem acetabular dorsal. Foram atendidos 16 cães (7 Poodle; 4 Pinscher; 1 Pequinês; 1 Beagle; 1 Yorkshire Terrier; 2 Sem Raça Definida) no Hospital Veterinário da Universidade Norte do Paraná (UNOPAR), Arapongas, PR, com diagnóstico de necrose asséptica da cabeça do fêmur. O tratamento preconizado foi cirúrgico, através da artroplastia por excisão da cabeça e colo femorais com abordagem dorso-caudal, e o pós-operatório constituíram-se de antibioticoterapia por 3 dias, curativo tópico até a cicatrização da ferida cirúrgica. A administração de DMSO (Dimesol®) na dose de 0,8 mL/Kg diluído em 125 mL de solução fisiológica de NaCl a 0,9%, durante 4 dias, repouso nos primeiros 7 dias e volta gradativa ao exercício. O retorno ao uso do membro afetado e o desaparecimento total da claudicação foram observados 20 dias após a cirurgia.(AU)


The aim of this study is to describe the excisional arthroplasty technique through dorsocaudal access in 16 dogs with aseptic necrosis of the femoral head as a new therapeutic alternative for the relief of traumatized patients. The femoral head necrosis, also known as Legg-Calvé-Perthes, presents, in all cases, both the bone of the femoral head and the neck with necrosis and deformation. Small animals are usually affected thus presenting a gradual level of lameness upon the painful member due to joint pain in the clinical exam. Traits of bone density loss in the femoral head and neck, and, in severe cases, the occurrence of osteophyte in the dorsal border of acetabular appear in the radiographical exam. Sixteen dogs (7 Poodle; 4 Pinscher; 1 Pekinese; 1 Beagle; 1 Yorkshire Terrier; 2 mongrel dogs) presenting aseptic necrosis of the femoral head were assisted in the Veterinary Hospital of the Universidade do Norte do Paraná (UNOPAR) in Arapongas–PR . The surgery was carried out through the excision arthroplasty of the head and femoral neck with a dorsocaudal approach. The postoperative period consisted of antibioticotherapy for 3 days, topical curative until the surgical wound healing. The administration of 0,8 ml/kg DMSO (Dimesol®) diluted into 125 ml of NaCL 0.9% physiological solution for 4 days; a rest cure in the first 7 days followed by a gradual return to the exercise. Returning to use the affected member as well as the total disappearance of the lameness level was observed 20 days after the surgery.(AU)


El trabajo tuvo como objetivo, describir la técnica de la artroplastía por escisión dorsal en 16 perros con necrosis aséptica de la cabeza femoral como una nueva alternativa terapéutica de la cirugía para el alivio del paciente traumatizado. La necrosis aséptica de la cabeza femoral, también conocida como Legg-Calvé-Perthes, presenta en todos los casos, el hueso de la cabeza y cuello femoral con necrosis y deformaciones. Acomete normalmente a los animales de pequeño porte, presentando en el examen clínico claudicación gradual del miembro afectado, debido al dolor articular. En el examen radiográfico se observa focos de disminución de la densidad ósea en la cabeza y en el cuello femoral, y en los casos más graves, la presencia de osteófitos en la borda del acetábulo dorsal. Fueron atendidos 16 perros [7 Poodles, 4 pinscher, 1 pequinés, 1 Beagle, 1 Yorkshire Terrier, 2 Sin Raza Definida (SRD)] en el Hospital Veterinario de la Universidad Norte del Paraná (UNOPAR) - Arapongas-PR con diagnóstico de necrosis aséptica de la cabeza femoral. El tratamiento proconizado fue la cirugía a através de la artroplastía por escisión de la cabeza y el cuello femoral con abordaje dorso-caudal y el posoperatorio se constituyó de antibiótico terapia por 3 (tres) días, curativo tópico hasta la cicatrización de la herida quirúrgica, administración de DMSO (Dimesol®) en la dosis de 0,8 ml/kg diluído en 125 ml de solución fisiológica de NaCL a 0.9% por 4 (cuatro) días, reposo en los primeros 7 (siete) días y vuelta gradual a los ejercicios. El retorno al uso del miembro afectado y el desaparecimiento total de la claudicación fue observado 20 (veinte) días después de la cirugía.(AU)


Subject(s)
Animals , Arthroplasty/methods , Arthroplasty/veterinary , Femur Head Necrosis/diagnosis , Femur Head Necrosis/veterinary , Dogs
15.
Rev. cuba. ortop. traumatol ; 19(1)2005. ilus, tab, graf
Article in Spanish | CUMED | ID: cum-26213

ABSTRACT

Se realizó una revisión bibliográfica de actualización sobre el controvertido tema de la osteonecrosis de cabeza femoral para profundizar en el uso de las clasificaciones tanto cualitativas como cuantitativas de la afección y enfatizar en su importancia diagnóstica y terapéutica. Se abordó la utilidad de la resonancia magnética nuclear y la gammagrafía ósea en el diagnóstico y control evolutivo del paciente. Se describe la afección acetabular propuesta por diferentes autores(AU)


Subject(s)
Humans , Femur Head Necrosis/classification , Femur Head Necrosis/diagnosis , Femur Head Necrosis , Magnetic Resonance Spectroscopy/methods
16.
Rev. cuba. ortop. traumatol ; 19(1)2005. ilus, tab, graf
Article in Spanish | LILACS | ID: lil-418698

ABSTRACT

Se realizó una revisión bibliográfica de actualización sobre el controvertido tema de la osteonecrosis de cabeza femoral para profundizar en el uso de las clasificaciones tanto cualitativas como cuantitativas de la afección y enfatizar en su importancia diagnóstica y terapéutica. Se abordó la utilidad de la resonancia magnética nuclear y la gammagrafía ósea en el diagnóstico y control evolutivo del paciente. Se describe la afección acetabular propuesta por diferentes autores


Subject(s)
Humans , Magnetic Resonance Spectroscopy/methods , Femur Head Necrosis/classification , Femur Head Necrosis/diagnosis , Femur Head Necrosis
17.
Rev Med Chil ; 132(8): 947-54, 2004 Aug.
Article in Spanish | MEDLINE | ID: mdl-15478296

ABSTRACT

BACKGROUND: Bone marrow edema syndrome (BMES) is a rare clinical condition. Its etiology is unknown and it can be seen in different locations. In the case of the hip, avascular necrosis is the main differential diagnosis. AIM: To present our experience of BMES of the hip and a review of the literature. PATIENTS AND METHODS: Retrospective analysis of clinical records of patients with hip pain that met clinical, radiological, and magnetic resonance imaging criteria for BMES. Clinical outcome and clinical and radiological follow up are presented. RESULTS: Two men and two women (one of them pregnant) aged 42, 48, 36 and 26 years old, fulfilled criteria. Treatment included limited weight bearing, non steroidal antiinflammatory drugs, intranasal calcitonin and physical therapy. Complete remission of symptoms was observed within five to seven months. At an average follow up of 36 months, all patients presented complete function of the hip, returning to their previous activity levels, with no new episodes of BMES. CONCLUSIONS: It is important to be aware of this condition as part of the differential diagnosis of hip pain to avoid aggressive and unnecessary diagnostic and therapeutic procedures.


Subject(s)
Bone Marrow Diseases/diagnosis , Edema/diagnosis , Hip Joint , Adult , Bone Diseases, Metabolic/diagnosis , Diagnosis, Differential , Female , Femur Head Necrosis/diagnosis , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Pregnancy , Syndrome
18.
Rev. méd. Chile ; 132(8): 947-954, ago. 2004. ilus, tab
Article in Spanish | LILACS | ID: lil-384190

ABSTRACT

Background: Bone marrow edema syndrome (BMES) is a rare clinical condition. Its etiology is unknown and it can be seen in different locations. In the case of the hip, avascular necrosis is the main differential diagnosis. Aim: To present our experience of BMES of the hip and a review of the literature. Patients and methods: Retrospective analysis of clinical records of patients with hip pain that met clinical, radiological, and magnetic resonance imaging criteria for BMES. Clinical outcome and clinical and radiological follow up are presented. Results: Two men and two women (one of them pregnant) aged 42, 48, 36 and 26 years old, fulfilled criteria. Treatment included limited weight bearing, non steroidal antiinflammatory drugs, intranasal calcitonin and physical therapy. Complete remission of symptoms was observed within five to seven months. At an average follow up of 36 months, all patients presented complete function of the hip, returning to their previous activity levels, with no new episodes of BMES. Conclusions: It is important to be aware of this condition as part of the differential diagnosis of hip pain to avoid aggressive and unnecessary diagnostic and therapeutic procedures (Rev Méd Chile 2004; 132: 947-54).


Subject(s)
Adult , Female , Humans , Male , Middle Aged , Pregnancy , Bone Marrow Diseases/diagnosis , Edema/diagnosis , Hip Joint , Bone Diseases, Metabolic/diagnosis , Diagnosis, Differential , Femur Head Necrosis/diagnosis , Magnetic Resonance Imaging , Syndrome
19.
La Paz; s.n; ene. 2004. 55 p. ilus. (BO).
Monography in Spanish | LILACS, LIBOCS, LIBOSP | ID: lil-364145

ABSTRACT

La enfermedad de Perthes es una patologia osteoarticular que afecta a la población infantil, sus secelas alteran el desarrollo dela cabeza del fémur y la relación de ésta cone l acetábulo será anormal, lo que predispone a una degeneración del cartílago articular, dando como resultado una artrosis prematura; por lo tanto los ovimientos del abducción, educción y rotación de la cadera estarán restringidos, produceindo una limitación funcional con claudicación en la march que, en mucos casos, acarrea trastornos psicológicos en losniños. Eisten diferentes tipos de tratamiento ortopédicos y quirúrgicos, que en ningún caso restauran completamente la anatomía y la función de la articulación de la cadera. Esto conduce a realizar una revisión de todos los tratamientos ortopédicos y quirúrgicos que se efectuán. Así miso, se revisa las nuevas propuestas etiológicas, los exámenes complemtarios y el pronóstico a que éstos conducen. El proposito de este estudio es que los especialistas que tratan a niños con enfermedad de Perthes conozcan todas las opciones que existen en la actualidad para tratar esta enfermedad, a fin de disminuir las secuelas y las consecencias que conlleva.


Subject(s)
Orthopedics , Traumatology , Legg-Calve-Perthes Disease , Femur , Femur Head/pathology , Femur Head Necrosis/diagnosis
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