Long-Term Outcomes in Patients with Sickle Cell Disease and Frequent Vaso-Occlusive Crises

Prasad, R.; Hasan, Syed; Castro, Oswaldo; Perlin, Elliott; Kim, Kyung-Sook

doi:10.1097/00000441-200303000-00001

Cited by 24 publications

(19 citation statements)

References 12 publications

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“…This trend has been observed in previous research where amelioration and even a cessation in crises have occurred in patients over the age of 40 years [14]. Other studies have examined mortality in populations of severely ill patients but we had no patients in the hospital for 100 days and therefore cannot compare our data to those studies [21]. A larger study of patients alive and over 40 will be necessary to further investigate this issue.…”

Section: Admissions and Crisesmentioning

confidence: 58%

“…Two groups of SS patients were selected on the basis of their age: the older group consisted of 40 patients (20 male, 20 female) over 40 years of age (41-67 years); and the younger group consisted of 40 patients (25 male, 15 female) under 30 years of age (21)(22)(23)(24)(25)(26)(27)(28)(29). The distribution of patients is demonstrated in Fig.…”

Section: Laboratory Determinantsmentioning

confidence: 99%

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The older sickle cell patient

2004

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The lifespan of sickle cell patients has increased substantially. We chose to compare the attributes of older sickle cell patients with younger ones within the confines of a single institutional study serving the local Bronx population. Laboratory and clinical assessments from 40 patients with sickle cell disease over 40 years of age were compared to 40 patients under 30 years of age. When the older group was compared to the younger group, hemoglobin, indirect bilirubin, and platelet counts were significantly lower. Creatinine clearances were lower and BUN levels were higher in the older group. Although the prevalence of radiographically measured cardiomegaly was significantly higher in the older group, systolic and diastolic blood pressures remained unchanged. The number of crises and number of admissions were higher in the younger group than in the older, but there was wide variation, and no significant differences could be detected; days spent in hospital over the previous 2 years did not differ. No statistically significant differences were observed for absolute reticulocyte count, MCV, % HbF, white cell count, liver function tests, or oxygen saturation. Comparing genders within age groups, younger males had higher hemoglobin levels and absolute reticulocyte counts than younger females, while only the older females had higher HbF levels than the corresponding males. Lower levels of hemoglobin associated with older age may reflect decreased hematopoietic potential that may in part be due to decreased renal function. Lower platelet counts in older sickle cell patients may be a consequence of this decreased hematopoietic potential but may also represent a secondary survival benefit effect in sickle cell anemia. Am.

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Section: Admissions and Crisesmentioning

confidence: 58%

Section: Laboratory Determinantsmentioning

confidence: 99%

The older sickle cell patient

2004

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“…The prognosis of SCD patients is not known; however, individuals with SCD have a severe clinical course with acute pain and often require hospitalization for vaso-occlusive crises and other complications of the disease. 29,30 Although all of the patients evaluated in our clinic were well maintained by our hematology clinic, 72% of them had been hospitalized during the past 2 years, and the mean number of days spent in hospital per year was 28 days. Seventy-eight percent of these patients had a mean of seven crises per person during the past 2 years.…”

Section: Discussionmentioning

confidence: 97%

“…28 Pulmonary hypertension, acute chest syndrome, and sepsis have been reported as reasons for sudden death in adult patients. 28,30 Another sudden death risk arises from excessive exercise; reduction in pH and temperature at tissue level facilitates oxygen delivery during exercise, and these changes lead to higher concentrations of deoxygenated hemoglobin. 31 Infections and sepsis remain the most common causes of death and vaso-occlusive crises for patients with SCD, and dental or periodontal infections 4 may play a part in precipitating a crisis.…”

Section: Discussionmentioning

confidence: 99%

Dental and periodontal health status of subjects with sickle cell disease

Güzeldemir

Toygar

Boğa

et al. 2011

Journal of Dental Sciences

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Background/purpose: Sickle cell disease (SCD) is a chronic, hereditary, autosomal recessive disorder. The pathophysiology of SCD is thought to result from polymerization of hemoglobin S in red blood cells under hypoxic conditions, which results in vaso-occlusion. The aim of this study was to determine the periodontal and dental health status of patients with SCD. Materials and methods: Fifty-five SCD patients and 41 healthy individuals were evaluated. Detailed medical and dental histories were taken, and a record made of dental status (missing teeth, restorations, impacted teeth, root canal treatment), periodontal status [plaque index (PI), gingival index (GI), probing depth (PD), bleeding on probing (BOP)], alveolar bone level (ABL), mandibular cortex index, and bone quality index. Results: Two hundred and six teeth were missing, and a total of 195 teeth had restorations. Between-group differences existed for the PI, GI, and BOP; these variables were higher in patients than in the healthy individuals (P < 0.0001). No between-group differences existed for PD. In patients, there was a positive correlation between PD and BOP (P < 0.0001; r Z 0.657), PD and GI (P Z 0.02; r Z 0.299), PD and PI (P Z 0.01; r Z 0.343); BOP and GI (P < 0.0001; r Z 0.503), BOP and PI (P < 0.0001; r Z 0.496); and GI and PI (P Z 0.003; r Z 0.388). The ABL in patients was found to be similar to that of the general population with an unknown periodontal condition.

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“…Concise presentation of data highlighting the increased risk for mortality associated with frequent hospitalizations for symptomatic SCD as well as the long-term benefits of HU would be useful reminders for community H/Os [8,33]. Furthermore, the utility of HU for patients with recurrent or severe acute chest syndrome deserves emphasis.…”

Section: Discussionmentioning

confidence: 99%

Hydroxyurea therapy for sickle cell disease in community‐based practices: A survey of Florida and North Carolina hematologists/oncologists

et al. 2005

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Little is known about patterns of hydroxyurea (HU) use by community-based hematologist/oncologists (H/Os) for the treatment of sickle cell disease (SCD). Determination of these practice patterns pertaining to adult SCD patients was the focus of this study. A self-administered survey was mailed to H/Os in two southeastern states. Replies were received from 70% of eligible physicians. This study focuses on responses from 184 community H/Os and a comparison group of 30 university-based/affiliated H/Os providing ongoing care for at least 3 SCD patients/month. The majority of community H/O respondents saw less than 3 SCD patients/month. HU was prescribed by more than half (55%) of community H/Os in at least 10% of their patients. The most common reasons cited for prescribing HU include frequent painful crises (76%), chronic pain with frequent narcotic use (58%), and acute chest syndrome (43%). Although the majority of community H/Os care for few patients with SCD, the reported indications for HU were consistent with currently accepted recommendations. However, community H/Os reported acute chest syndrome, stroke, and pulmonary hypertension as indications for HU less often than the academic H/O group. Barriers to wider use of HU include physician concerns about carcinogenic potential, doubts about HU effectiveness, perceived patient apprehension about adverse effects, concern about lack of contraceptive use, and patient compliance. Further resources should focus on updating physicians on recently published material supporting the effectiveness of HU in symptomatic SCD as well as providing management guidelines to optimize the use of HU. Am.

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Long-Term Outcomes in Patients with Sickle Cell Disease and Frequent Vaso-Occlusive Crises

Cited by 24 publications

References 12 publications

The older sickle cell patient

The older sickle cell patient

Dental and periodontal health status of subjects with sickle cell disease

Hydroxyurea therapy for sickle cell disease in community‐based practices: A survey of Florida and North Carolina hematologists/oncologists

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