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PubMed1994

Symptomatic cataplexy in pontomedullary lesions.

Cataplexy is a cardinal manifestation of the narcolepsy syndrome. Although symptomatic narcolepsy is well described, isolated cataplexy is extremely rare. We reviewed clinical and radiologic data in two patients with isolated symptomatic cataplexy and associated CNS disease. In an HLA-DR2-positive patient with chronic progressive MS, we confirmed cataplexy by observation of reported spells. MRI revealed diffuse white-matter lesions involving the medial medulla, pons, and subcortical white matter; protriptyline provided symptomatic relief. A second patient with a pontomedullary pilocytic astrocytoma developed infrequent but recurrent cataplectic attacks in association with sleep fragmentation due to nocturnal cough and nausea. MRI revealed an enhancing lesion involving the dorsal pons and medulla. Genetic predisposition and sleep fragmentation may precipitate symptomatic cataplexy in patients with structural pontomedullary lesions.

Adult↗
PubMed1995

Comparison and meta-analysis of randomized trials of endarterectomy for symptomatic carotid artery stenosis.

OBJECTIVE: Comparison and meta-analysis of randomized trials of carotid endarterectomy for symptomatic stenosis of the extracranial carotid artery. BACKGROUND: Randomized trials (North American Symptomatic Carotid Endarterectomy Trial [NASCET], the European Carotid Surgery Trial [ECST], and the VA Cooperative Study [VACS]) each show that carotid endarterectomy improves outcomes in selected symptomatic patients with high-grade extracranial carotid artery stenosis. Direct comparisons among the studies have not been possible because of differing methodologies, endpoints, and formats of data reporting. DESIGN/METHODS: Data for specified endpoints and corresponding person-years at risk were obtained for each trial. The rates of nonfatal stroke, nonfatal myocardial infarction, or death for surgically or medically treated patients in the perioperative period (30 days) and thereafter were compared (both including and excluding perioperative events) and then combined using meta-analytic techniques. Data from NASCET and ECST were also analyzed for differences in outcomes by sex. RESULTS: Event rate estimates (with 95% confidence intervals [95% CI]) for the first 30 days (events per person-year, primarily nonfatal stroke) for medically treated patients were 0.44 (0.22 to 0.76) for NASCET, 0.15 (0.04 to 0.38) for ECST, and 0.27 (0.03 to 0.96) for VACS. For surgically treated patients, the corresponding rates (per person-year) were 0.78 (0.49 to 1.19), 0.63 (0.41 to 0.94), and 1.27 (0.58 to 2.43). Event rates per year after 30 days were higher for medically treated patients (0.20 [0.16 to 0.25] versus 0.08 [0.05 to 0.11] for NASCET; 0.12 [0.10 to 0.15] versus 0.07 [0.06 to 0.09] for ECST; and 0.15 [0.07 to 0.25] versus 0.07 [0.03 to 0.16] for VACS). There were no significant differences among the trials, with an overall benefit for surgical therapy (risk ratio estimate, RR = 0.67, 95% CI = 0.54 to 0.83). There were no significant sex-based differences between NASCET and ECST and the overall benefit was not significantly different for men and women (RR = 0.58, 95% CI = 0.45 to 0.74 for men; RR = 0.84, 95% CI = 0.57 to 1.25 for women). CONCLUSIONS: Adjusting for primary endpoints and duration of follow-up, carotid endarterectomy has a similar benefit for symptomatic patients across trials and a similar benefit for men and women.

Aged↗
PubMed1997

Risk factors for status epilepticus in children with symptomatic epilepsy.

The purpose of this study was to determine risk factors for status epilepticus (SE) in children with symptomatic epilepsy through a retrospective case-control study. Patients (44 children with a prior diagnosis of symptomatic epilepsy experiencing one or more episodes of SE between January 1, 1991, and June 1, 1995) were matched for age at follow-up to controls (88 children with symptomatic epilepsy without SE during that interval) and medical records were reviewed. Patients and controls did not differ in etiology or in age at epilepsy onset (1 year 5 months [SD, 2 years 3 months] versus 1 year 3 months [SD, 1 year 5 months]). Univariate analysis revealed significant associations between SE and several factors, including history of a first seizure as SE, partial epilepsy, partial seizures with secondary generalization, focal paroxysmal abnormalities and focal background abnormalities on EEG, and generalized abnormalities on neuroimaging. Groups did not differ in the proportion of patients on polypharmacy or with subtherapeutic serum antiepileptic drug levels, but there was a trend for greater use of phenobarbital in patients (57% versus 38%, odds ratio (OR) = 2.6, p = 0.057). With multiple logistic regression, four factors emerged as independent predictors of SE risk: focal background EEG abnormalities (OR = 6.51, p = 0.0005), partial seizures with secondary generalization (OR = 4.61, p = 0.0021), first seizure as SE (OR = 3.99, p = 0.034), and generalized abnormalities on neuroimaging (OR = 2.85, p = 0.034). These four factors are indicators of a higher risk of SE in children with symptomatic epilepsy.

Case-Control Studies↗
PubMed2006

Outcomes of surgery for resection of regions of symptomatic radiation injury after stereotactic radiosurgery for arteriovenous malformations.

OBJECTIVE: Although radiation injury after stereotactic radiosurgery (SRS), including radiation necrosis (RN), is often treated with surgical resection, detailed outcome data are lacking after resection of symptomatic radiation-injured regions with imaging characteristics suspicious for RN after SRS for arteriovenous malformations (AVM). We present outcomes in seven such patients. METHODS: We conducted a retrospective chart review of seven patients with AVMs of Spetzler-Martin Grades II (n = 1), III (n = 2), and IV (n = 4) who underwent helium ion, proton beam, or gamma knife SRS and required resection of RN-suspicious tissue 1 to 24 months after post-SRS symptom onset. Postoperative outcomes included Karnofsky Performance Scale (KPS) score and time to symptomatic improvement. RESULTS: Symptomatic improvement required at least 9 months in the three patients with large regions suspicious for RN (>or=4 cm), whereas of four patients with smaller regions (<4 cm), three showed improvement within 2 months (P < 0.05). The remaining patient, who showed no benefit, underwent resection 2 years after the onset of RN symptoms (compared with <or= 8 mo for the other six patients). Surgery improved KPS scores in four patients with a preoperative KPS score of 50 or lower, but not in three patients with preoperative KPS scores greater than 70 (P < 0.05). Outcomes were not consistently associated with AVM size or location, SRS treatment volume or dose, associated aneurysm, or residual AVM. CONCLUSION: After SRS for AVMs, resection of symptomatic RN-suspicious tissue areas is beneficial in reversing neurological deficits and improving KPS scores in selected patients. Times to improvement were longer when larger areas were resected. Delaying such surgery after symptom onset may negatively affect improvement.

Adult↗
PubMed2006

Early and intermediate-term outcomes with drug-eluting stents in high-risk patients with symptomatic intracranial stenosis.

OBJECTIVE: To report the 1-month and intermediate-term results of treatment of symptomatic intracranial stenosis using drug-eluting stents. BACKGROUND: Patients with intracranial stenosis who are at high risk because of either high-grade stenosis or medication failure may have an annual risk of recurrent ischemic events in excess of 40%. Drug-eluting stents may reduce the rate of ischemic events in patients with a low restenosis rate. METHODS: We determined rates of technical success (defined as reduction of target lesion to stenosis <30%) and 1-month major stroke or death in patients with symptomatic intracranial stenosis (> or =70% and/or medication failure). Patients' clinical and follow-up information during a mean period of 14.3 +/- 7 months were obtained. Kaplan-Meier analysis was performed to determine the rate of major stroke-free survival during 12 months. RESULTS: There were 18 patients (mean age, 58 +/- 16 yr; 12 were men) treated with either a sirolimus-eluting stent (n = 14) or a paclitaxel-eluting stent (n = 4) for stenosis located in the: intracranial internal carotid artery (n = 6), proximal middle cerebral artery (n = 4), intracranial vertebral artery (n = 4), vertebrobasilar junction (n = 2), or basilar artery (n = 2). There was one major stroke and no death observed in the 1-month follow-up. At the 6-month follow-up examination, no major stroke or death was observed. Major stroke-free survival was 86% (+/-standard error of 9%) at 12 months after the procedure. One symptomatic angiographic restenosis was observed during the follow-up period. CONCLUSION: A low rate of major stroke or death was observed after treatment of symptomatic intracranial stenosis using drug-eluting stents in high-risk patients.

Blood Vessel Prosthesis↗
PubMed1989

Assessment of silent and symptomatic ischemia in daily activity by an ambulatory ventricular function monitor.

We have assessed the sequential changes in left ventricular function and ECG in 39 patients with coronary artery disease (CAD) using an ambulatory ventricular function monitor. Following radionuclide ventriculography, the instrument detectors were placed over the region of the left ventricle and lung, and beat-to-beat left ventricular time activity curve and modified V5 ECG data were continuously monitored for up to 6 hours (mean 2.5 hours), while the patient performed various daily activities. Thirty-six episodes of transient ejection fraction (EF) decrease (6-18%) were recorded in 16 patients, 12 were symptomatic and 24 asymptomatic. ST segment changes were recorded in 6 of the 12 symptomatic and 5 of the 24 asymptomatic episodes. In 10 of the 12 symptomatic episodes, EF began to drop 30 to 90 seconds before the onset of the symptoms. Of 14 episodes of significant ST segment depression in five patients, 11 were associated with a significant EF decrease. We conclude that the combination of left ventricular function and ECG monitoring is a promising means for determining incidence of silent and symptomatic ischemia and for severity of ischemia in patients with CAD.

Activities of Daily Living↗
PubMed1999

Symptomatic anterior uveitis in HIV-positive patients.

Symptomatic anterior uveitis is rare in HIV-positive patients. The uveitis associated with cytomegalovirus retinitis (CMVR), the commonest ocular manifestation in AIDS patients, is rarely symptomatic and patients do not typically present with a red painful eye in conjunction with blurred vision. In this article we report 12 cases of symptomatic anterior uveitis in HIV-positive patients and discuss the aetiology. The case notes of all HIV-positive patients presenting to the eye department with symptoms of uveitis over a 4-year period were studied retrospectively. The notes were analysed for age, sex, race, risk factors of HIV, features of the uveitis, concurrent disease and CD4 counts. Only 12 patients were identified to have symptomatic uveitis out of a total 172 patients. There were 9 males and 3 females with an average age of 35 years. None of these patients were taking either rifabutin, protease inhibitors or cidofovir. Seven out of the 12 patients had granulomatous uveitis. Of these 7 patients, 4 had CD4 counts over 200 and no other concurrent illness whilst 3 patients, with CD4 counts between 130-200, were subsequently found to have an underlying aetiology namely lymphoma, tuberculosis and candida. The remaining 5 patients, all with CD4 counts below 40, had a history of systemic illness with herpes zoster preceding the onset of the uveitis. HIV-positive patients with symptoms of uveitis do not have active CMVR and the ophthalmologist must search for other causes such as tuberculosis or lymphoma in those with granulomatous uveitis or herpes zoster in those with non-granulomatous uveitis. The CD4 count may be helpful.

AIDS-Related Opportunistic Infections↗
PubMed2002

The effect of house dust mite sensitization on lung size and airway caliber in symptomatic and nonsymptomatic preadolescent children: a community-based study in Poland.

There are conflicting reports on the effects of atopy on lung function. The purpose of this study was to compare the effects of house mite (HM) atopy on lung function in subsamples of 12-year-old symptomatic and nonsymptomatic preadolescent children taken from the community sample. An additional objective of this study was to identify possible environmental determinants of HM skin reaction. We obtained questionnaire data on respiratory symptoms and skin-prick tests and performed spirometry on a subsample of 311 children of a birth cohort of children who have been followed over 3 years. Multivariate regression analysis showed progressive decrement of lung function indices (forced vital capacity, forced expiratory volume in 1 sec, and forced expiratory flow, midexpiratory phase) with increasing degree of HM atopy reflected by the skin reaction to HM allergens. The apparent association between the level of HM atopy and the lung function indices was highly significant in symptomatic individuals but insignificant in nonsymptomatic subjects. HM sensitization was significantly associated with mother's atopy. It occurred predominantly in boys and was related to the use of coal or gas for house heating. The effect of allergen sensitization on lung size and airway caliber confined to symptomatic subjects may reflect the inflammatory status of bronchial airways in the symptomatic subjects.

Air Pollution, Indoor↗
PubMed2006

Mammography in symptomatic women attending a rapid diagnosis breast clinic: a prospective study.

INTRODUCTION: We determined whether it is safe to avoid mammograms in a group of symptomatic women with a non-suspicious history and clinical examination. PATIENTS AND METHODS: Symptomatic women aged 35 years or over newly referred to a rapid-diagnosis breast clinic underwent mammography on arrival in the clinic. A breast radiologist reported on the mammograms. An experienced clinician who was unaware of the mammogram findings examined patients and decided whether a mammogram was indicated or not. If not, a management plan was formulated. Mammogram findings were then provided to the clinician and any change to the original management plan as a result of mammography was recorded. RESULTS: In two-thirds (67%) of 218 patients, the clinician felt a mammogram was indicated. Half (46%) of these mammograms showed an abnormality; of these abnormal mammograms, 41% were breast cancer. Among the third (n = 71) of mammograms felt not to be indicated, 3 showed abnormalities of which 2 were breast cancer. One cancer was not suspected clinically or mammographically but was diagnosed on cyto/histopathological assessment. CONCLUSIONS: A significant proportion of patients attending a symptomatic breast clinic have a non-suspicious history and normal clinical findings on examination. However, even in this group avoiding mammograms risks missing clinically occult breast cancers. It would appear sensible to offer mammograms to all symptomatic women over 35 years of age.

Adult↗
PubMed1998

A potential role for glucagon in the treatment of drug-induced symptomatic bradycardia.

Nine cases of symptomatic bradycardia are presented in which treatment with intravenous glucagon was administered when atropine failed to improve the patient's condition significantly. Although the cause often was not obvious at presentation, all nine subjects took oral medications that could have contributed to the development of symptomatic bradycardia. Eight of nine patients demonstrated clinical improvement 5 to 10 min after glucagon administration, which was consistent with its peak clinical action. Beta-blockers, calcium channel blockers, and digoxin were ultimately thought to have contributed to the majority of these presentations. This report suggests that glucagon may have a role in the treatment of symptomatic bradycardia, particularly in the presence of beta-adrenergic blockade and perhaps calcium channel blockade. Furthermore, the results in these cases suggest that future clinical trials should not be limited to drug-induced symptomatic bradycardia.

Adrenergic beta-Antagonists↗
PubMed2002

Symptomatic gastroesophageal reflux in subjects with a breathing sleep disorder.

STUDY OBJECTIVES: A link between gastroesophageal reflux (GER) and obstructive sleep apnea (OSA) has been suggested; however, the prevalence and frequency of symptomatic GER and the influence of OSA severity on GER are not known. DESIGN AND PATIENTS: Two hundred seventy-one subjects referred for overnight sleep studies were investigated for subjects with a breathing sleep disorder, occurrence of symptomatic GER, potential risk factors for both conditions, and comorbidity using a validated questionnaire. RESULTS: Overall, 160 of the 228 respondents (73%; 135 subjects with OSA and 93 subjects who snore) reported GER-related symptoms, with heartburn and/or acid regurgitation being the leading symptoms. No evidence of a difference in the occurrence of symptomatic GER between subjects who snore and subjects with OSA was observed (odds ratio [OR], 1.21; 95% confidence interval [CI], 0.7 to 2.1). Furthermore, the occurrence of reflux symptoms was not influenced by the severity of OSA (OR per 10 4% arterial oxygen saturation [SaO(2)] dips per hour, 0.98; 95% CI, 0.8 to 1.1). Self-reported comorbidity was higher in subjects with OSA compared with subjects who snore (p = 0.02), but none of the potential risks produced an association with the presence of reflux symptoms in this sample of patients with a breathing sleep disorder. CONCLUSION: We conclude that symptomatic GER is common in subjects with a breathing sleep disorder, but there was no difference between those with OSA and subjects who snore.

Female↗
PubMed1989

Left ventricular dysfunction in symptomatic mitral valve prolapse.

Idiopathic MVP is characterized by a late systolic click or murmur from myxomatous mitral valvular dysfunction. It may be complicated by atypical chest pain, ventricular arrhythmias, and ECG changes that can mimic the symptoms of coronary artery disease. We prospectively performed radionuclide cineangiograms before and after stress tests in MVP patients with chest pain compared with asymptomatic MVP patients and symptomatic normal control patients. In ten patients with MVP, chest pain, and normal coronary anatomy, the LVEF remained essentially unchanged (increase of -0.5 +/- 4 percent) after exercise. In ten patients with MVP and no chest pain and in nine with normal cardiovascular system and chest pain, the exercise LVEF increased by 11.5 +/- 2 percent (p less than 0.05) and 17.4 +/- 3 percent (p less than 0.005), respectively. The resting LVEF was significantly lower (p less than 0.02) in the symptomatic MVP patients (59 +/- 3 percent) than in the asymptomatic MVP (76 +/- 5 percent) or symptomatic normal patient control subjects (70 +/- 3 percent). Patients with MVP and chest pain had a lower resting LVEF and an abnormal left ventricular functional response to exercise compared with asymptomatic MVP patients or symptomatic normal subjects. Therefore, exercise radionuclide ventriculography may not adequately differentiate between chest pain due to MVP or coronary artery disease.

Cineangiography↗
PubMed2006

Hypoadiponectinemia is associated with symptomatic atherosclerotic peripheral arterial disease.

There is growing evidence that adiponectin, an adipocytokine with anti-inflammatory and antiatherogenic properties, is involved in the development of atherosclerosis. The aim of the present study was to examine whether serum levels of adiponectin were associated with symptomatic atherosclerotic peripheral arterial disease (PAD). Serum concentrations of adiponectin were measured in 433 patients with symptomatic PAD and 433 controls from the Linz Peripheral Arterial Disease (LIPAD) study. Cases and controls were matched for age, sex and diabetes mellitus. The median serum level of adiponectin was significantly lower in PAD patients than in control subjects (9.5 vs. 10.8 mg/L; p=0.014). After adjustment for several possible confounding variables using multivariable logistic regression, odds ratios for symptomatic PAD were 0.95 (95% CI, 0.64-1.42; p=0.080) and 0.59 (95% CI, 0.36-0.97; p=0.037) in the second and third tertiles for adiponectin serum concentrations, respectively, compared with the first tertile. Low serum levels of adiponectin were associated with the presence of symptomatic atherosclerotic PAD, independent of traditional and non-traditional risk factors, suggesting that hypoadiponectinemia may be a marker for systemic atherosclerotic disease.

Adiponectin↗
PubMed2004

Pulmonary vein isolation by robotic-enhanced thoracoscopy for symptomatic paroxysmal atrial fibrillation.

BACKGROUND: Pulmonary vein isolation (PVI) has been shown to be effective treatment of patients with symptomatic paroxysmal atrial fibrillation (PAF). The percutaneous approach is currently the technique of choice. Unfortunately, this procedure has limitations and complications that lead to fluctuating success rates. We explored an alternative technique of robotic-enhanced, closed-chest PVI with an endoscopic microwave-based catheter. METHODS: Seven symptomatic PAF patients were included in the study. The pulmonary veins were isolated through right (only) robotic-enhanced thoracoscopy on the beating heart. RESULTS: Six patients underwent successful endoscopic PVI. In 1 patient the operation was converted into small right thoracotomy. Operative assessment of the ablation line showed a successful electric block in every patient. Three months after the procedure, the first 5 patients were in permanent sinus rhythm. The 2 other patients had AF but had less frequent and less symptomatic episodes compared with the preoperative situation. CONCLUSIONS: On the basis of this preliminary experience, we believe that in the near future endoscopic right-chest robotic-enhanced PVI on the beating heart may become a valid option in the treatment of symptomatic PAF patients. This procedure allows for more-reproducible ablation lines and may avoid many of the pitfalls and drawbacks of the percutaneous approach. Therefore this technique deserves larger prospective evaluation in the treatment of AF.

Atrial Fibrillation↗
PubMed1997

Neuroradiographic findings in the newborn period and long-term outcome in children with symptomatic congenital cytomegalovirus infection.

OBJECTIVE: To determine whether newborn cranial computed tomographic (CT) scan abnormalities predict an adverse neurodevelopmental outcome in children with symptomatic congenital cytomegalovirus (CMV) infection and to examine the association between clinical findings at birth and imaging abnormalities. METHODS: The data from 56 children with symptomatic congenital CMV infection who underwent cranial CT scans as newborns and were enrolled in a long-term follow-up study were analyzed. The incidence of sequelae was compared between the groups of children with normal and abnormal imaging studies. The relationship between CT scan results and other newborn findings was also examined. RESULTS: Abnormal CT scans were noted in 70% of subjects; intracerebral calcification was the most frequent finding. Most of the children with an abnormal newborn CT scan (90%) developed at least one sequela, compared with 29% of those with a normal study. Only 1 child with a normal CT scan had an IQ < 70, in contrast to 59% of those with imaging abnormalities. In addition, almost half of the children with CT abnormalities had an IQ < 50 compared with none of those with a normal CT scan. Newborn CT abnormalities were also associated with an abnormal hearing screen at birth and hearing loss on follow-up. None of the neonatal neurologic findings were predictive of an abnormal CT scan. CONCLUSION: In neonates with symptomatic congenital CMV infection, a cranial CT scan is a good predictor of an adverse neurodevelopmental outcome. In addition, newborn clinical and laboratory findings did not predict neuroradiographic abnormalities in neonates with symptomatic congenital CMV infection.

Brain↗
PubMedDate not supplied

[Incidental and symptomatic kidney cancer: pathological features and survival].

BACKGROUND: Patients with early diagnosis of renal cell carcinoma (CCR) have higher chance of cure following surgical treatment. This study was set to compare the pathological characteristics between the surgical specimens and the survival of the patients with incidental and symptomatic CCR. METHODS: One hundred and fifteen patients with sporadic CCR were studied retrospectively following nephrectomy and divided into two groups. Group 1; 59 patients with incidental diagnosis and Group 2; 56 symptomatic patients. The mean age of the patients was 59 years, with 86 men and 29 women. Radical nephrectomy was performed in 96 patients and the conservative surgery was performed in the remaining 19. Comparison parameters included pathological outcome, specifically nuclear grade, pathological stage, size of the tumor and presence of microvascular invasion intratumoral and patients survival. RESULTS: Comparison between the two groups confirmed that the incidental tumors have smaller nuclear grade (p=0.003), smaller size (p=0.001), smaller incidence of microvascular invasion (p<0.001) and lower stage (p<0.001). Disease specific survival and recurrence free survival of the incidental group were statistically higher than the symptomatic group (p<0.001). CONCLUSION: Incidentally discovered CCR have more favorable pathological characteristics, the patients have disease free survival when compared to symptomatic CCR.

Adolescent↗
PubMed2000

Can angiotensin-converting enzyme inhibitors protect against symptomatic radiation pneumonitis?

This study was designed to determine whether patients taking angiotensin-converting enzyme (ACE) inhibitors while receiving radiation therapy for lung cancer are protected from developing symptomatic radiation pneumonitis. The records of 213 eligible patients receiving thoracic irradiation for lung cancer with curative intent at Duke University Medical Center from 1994-1997 were reviewed. Of the 213 patients, 26 (12.2%) were on ACE inhibitors (usually for the management of hypertension) during radiotherapy (group 1); the remaining 187 patients (group 2) were not. Patients were irradiated, with fields shaped to protect normal tissues, with total doses of 50-80 Gy. After treatment, patients were generally followed every 3 months for 2 years, then every 6 months thereafter. Symptomatic radiation pneumonitis was scored according to modified National Cancer Institute Common Toxicity Criteria (i.e., radiographic changes alone were not sufficient for the diagnosis of pneumonitis). There was no difference in the incidence of pneumonitis between the two groups (P = 0.75). Fifteen percent of the patients on ACE inhibitors (group 1) developed symptomatic radiation-induced lung injury compared to 12% of the patients not receiving these drugs (group 2). Although patients in group 1 tended to develop pneumonitis slightly sooner than did patients in group 2, this difference also was not significant (P = 0. 8). Within the dose range prescribed for treating hypertension, ACE inhibitors do not appear to either decrease the incidence or delay the onset of symptomatic radiation pneumonitis among lung cancer patients receiving thoracic irradiation.

Adult↗
PubMed2003

The natural history of symptomatic osteonecrosis in adults with sickle-cell disease.

BACKGROUND: Adult patients with sickle-cell disease are at risk for the development of osteonecrosis of the hip. However, there is little information in the literature about the rate of progression of osteonecrosis once symptoms begin. The purpose of this study was to evaluate the natural history of the symptomatic hip in adult patients with osteonecrosis and sickle-cell disease. METHODS: Ninety-two symptomatic hips in sixty-four consecutive adult patients with sickle-cell disease were initially evaluated between 1980 and 1987. Sixty symptomatic hips had radiographic evidence of osteonecrosis at the initial evaluation: forty-three were classified as stage II; two, as stage III; and fifteen, as stage IV, according to the system of Steinberg et al. The other thirty-two hips had lesions (stage I) that were evident only on magnetic resonance imaging. All patients were evaluated after a mean duration of follow-up of seventeen years. RESULTS: Of the seventy-five hips without collapse of the femoral head at the initial evaluation, sixty-five demonstrated collapse within five years after the diagnosis. The average time between the diagnosis and collapse was forty-two months for stage-I hips and thirty months for stage-II hips. At the most recent follow-up examination, ninety hips had had collapse of the femoral head and eighty-eight of the ninety-two hips had had surgery because of intractable pain. CONCLUSIONS: Symptomatic osteonecrosis of the hip in sickle-cell disease has a high likelihood of leading to femoral head collapse, necessitating surgical intervention. When osteonecrosis develops, the deterioration is rapid and, in most patients, operative intervention is necessary because of intractable pain. LEVEL OF EVIDENCE: Prognostic study, Level II-1 (retrospective study). See Instructions to Authors for a complete description of levels of evidence.

Adolescent↗