PND109 - SICKLE-CELL DISEASE RELATED HOSPITALIZATIONS IN FRANCE : A REGIONAL DESCRIPTIVE ANALYSIS FROM THE FRENCH MEDICAL INFORMATION SYSTEM DATABASE IN 2016

Background Sickle cell disease (SCD) induces chronic haemolytic anaemia and intermittent vaso-occlusion that results in tissue ischaemia causing acute, severe pain episodes that can lead to frequent hospitalizations. These consequences can have repercussions on family, social, school and/or professional life. Here, we present some of the results of the PHEDRE study (Pharmacodépendance Et DREpanocytose—drug dependence and sickle-cell disease), which is the largest study of patients with SCD in France. This paper intends to describe characteristics of the French SCD population. We also aimed to assess the impact of the disease on the lives of patients using objective and subjective variables. Methods The PHEDRE study was a national multicentric observational study. Adults, adolescents and children with a confirmed SCD diagnosis were included in the study by their referring doctor. Then, they were interviewed by phone about their socioeconomic status, about the impact of the disease on their lives and about their analgesic and psychoactive drug use. Results The study population consisted of 872 patients (28% were minors). Seventy-two percent of adults were active, and all minors were in school. Many patients presented criteria of severe SCD. Seventy-five percent were homozygous SS, 15% were double heterozygotes SC and 8% were heterozygotes Sβthal, 87% received specific treatment, 58% were hospitalized at least once for vaso-occlusive crisis in the past 12 months, and the number of analgesic drugs taken averaged 3.8. Seventy-five percent of patients reported academic or professional consequences related to their SCD, and 52% reported social consequences. Conclusions The impact of SCD on patients’ lives can be significant, nevertheless their social integration seems to be maintained. We highlighted respect of recommendations regarding analgesic treatments and only a few patients used tobacco, alcohol or cannabis. Trial registration Clinical Trials, NCT02580565; https://clinicaltrials.gov/ Registered 16 October 2015.

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Epidemiology, Management and Outcome Of Priapism In Children and Adolescents With Sickle Cell Disease: A Pediatric Health Information System Database Analysis

Blood ◽

10.1182/blood.v122.21.1698.1698 ◽

2013 ◽

Vol 122 (21) ◽

pp. 1698-1698 ◽

Cited By ~ 1

Author(s):

Ram Kalpatthi ◽

Brian R Lee ◽

Gerald M Woods ◽

Jignesh Dalal

Keyword(s):

Information System ◽

Sickle Cell Disease ◽

Blood Transfusion ◽

Length Of Stay ◽

Conservative Treatment ◽

Sickle Cell ◽

Health Information System ◽

Neurological Complications ◽

Cell Disease

Abstract Background Priapism is a known but largely understudied complication of sickle cell disease (SCD). The epidemiology of priapism in children and adolescents with SCD is not well characterized especially the pattern of hospitalization. Data are scarce about the role of blood transfusion and surgical therapies in the management of priapism in SCD. Recent expert opinion questions the efficacy of blood transfusion and recommends early urological interventions in these patients (Merritt et al. Can J Emerg Med 2006, Kato GJ. J Sex Med. 2012). As hospitalization consumes lots of economic resources, we studied the trend and outcome of hospitalization for priapism in children with SCD over the last decade. Methods We used the Pediatric Health Information System (PHIS), an electronic database of children's hospitals in the US. Patients ≤ 21 years of age with SCD from 33 hospitals from 2000-2011 were analyzed. SCD, priapism and all related conditions were identified by ICD-9 codes. We examined patient demographics, timing of hospitalizations, treatment details, RBC transfusion (simple and exchange transfusion), and urologic procedures (including aspiration and irrigation of corpus cavernosum, corpora cavernosa-corpus spongiosum shunt and corpora-saphenous shunt) and hospital charges. With the inherent skewness of the length of stay and cost data, we used non-parametric analysis when comparing summary statistics. Given the level of over-dispersion, a negative binomial model was used to determine adjusted length of stay and cost. A p-value <.05 was considered statistically significant. Results From 2000 to 2011, there were 7929 unique male pediatric patients with SCD identified. Among these 465 (5.9%) patients had a diagnosis of priapism and accounted for 1069 priapism related hospitalizations. Overall, the number of new sickle cell patients with priapism getting hospitalized remained stable (Figure 1A). The demographic and baseline characteristics of these patients are shown in Figure 1B. In 63% (673/1069) of the priapism related hospitalizations, patients received conservative treatment whereas blood transfusion, urologic procedures or both were performed in 25.2% (269/1069), 6.6% (71/1069) and 5.2% (56/1069) of the hospitalizations respectively. Five percent (51/1069) of priapism related hospitalizations required ICU care, 1% (15/1069) required mechanical ventilation and there was no in-hospital mortality. Average length of stay for priapism related hospitalization was 3.8 days. Multivariate regression analysis showed that the presence of VOC, ACS, blood transfusion, and urologic procedures were independently associated with increased length of stay and costs (Table 1). Multivariate analysis showed neither transfusion nor urologic procedures were associated with neurological complications observed during priapism related hospitalizations. Conclusions In our largest pediatric in-patient sickle cell cohort, the prevalence of priapism was 5.9%. New inpatient diagnoses of priapism among children with SCD remained constant overtime as previously reported in adult SCD population (Chrouser et al. Am J Surg 2011). Majority of our patients received conservative treatment and urologic procedures were utilized infrequently, again consistent with adult literature. Both blood transfusion and urologic procedures were associated with increased length of stay and costs but not with neurological complications. There is a strong need for prospective, multi-institutional trials to define the role of blood transfusions, surgical procedures and other novel therapies to improve the outcome. Disclosures: No relevant conflicts of interest to declare.

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What Is The Hospital Economic Burden Of Hiv In France? Results from an analysis of the national french medical information system database

Value in Health ◽

10.1016/j.jval.2016.09.381 ◽

2016 ◽

Vol 19 (7) ◽

pp. A412

Author(s):

Y Yazdanpanah ◽

F Bonnet ◽

L de Léotoing ◽

L Finkielsztejn ◽

G Chaize ◽

...

Keyword(s):

Information System ◽

Economic Burden ◽

Medical Information ◽

Medical Information System ◽

System Database

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Neurologic Complications In Pediatric Sickle Cell Patients Hospitalized For Priapism: An Analysis Of The Pediatric Health Information System (PHIS) Database

Blood ◽

10.1182/blood.v122.21.2237.2237 ◽

2013 ◽

Vol 122 (21) ◽

pp. 2237-2237 ◽

Cited By ~ 1

Author(s):

Gary Jones ◽

Brian R Lee ◽

Gerald M Woods ◽

Jignesh Dalal ◽

Ram Kalpatthi

Keyword(s):

Information System ◽

Sickle Cell Disease ◽

Blood Transfusion ◽

Health Information ◽

Sickle Cell ◽

Health Information System ◽

Blood Transfusions ◽

Neurologic Complications ◽

Cell Disease ◽

Pediatric Health

Abstract Background Priapism is a known complication of sickle cell disease (SCD). Though, blood transfusion is often used in the management of SCD-associated priapism (SAP), its safety and efficacy is not known. Transfusion has been associated with neurologic events including ASPEN syndrome (Association of Sickle cell disease, Priapism, Exchange transfusion, and Neurologic events) [Siegel et al. J Urol 1993]. A recent systematic review suggests that transfusion does not appear to be effective in SAP as determined by time to detumescense (Merritt et al. Can J Emerg Med 2006). Our objective is to examine the relationship between blood transfusions and neurologic complications (NCs) in the management of SAP. Methods We reviewed the data from 33 children’s hospitals within the Pediatric Health Information System (PHIS) database from 2000-2011. Patients ≤ 21 years of age with SCD were included for the analysis. SCD, priapism and all related conditions were identified by ICD-9 codes. We abstracted data on demographics, hospitalizations, transfusion, urologic procedures and neurologic changes (including stroke, seizure, headache, weakness, transient-ischemic attacks (TIAs), intracranial hemorrhage, and mental status changes). Fisher’s exact test was used to compare the prevalence of NCs. A p-value <.05 was considered statistically significant. Results From the year 2000 to 2011, a total of 7,929 unique male pediatric patients with SCD were identified. Among these 465 (5.9%) patients with 1,069 hospitalizations were identified as having SAP. The majority of these patients were HbSS (n=447; 96%). The frequency of SAP hospitalizations at various age groups were; 4.7% (0-2 years of age), 11.2% (3-5 years), 38% (6-12 years) and 46% (13-21 years). In the majority of SAP hospitalizations (63%, n=673) neither transfusions nor urologic procedures were performed, whereas blood transfusions, urologic procedures or both were performed in 25.2% (n=269), 6.6% (n=71) and 5.2% (n=56) of SAP hospitalizations respectively. There was no significant difference in the incidence of NCs between the patients who received transfusions and those who did not (4.1% vs. 4.7%, p = NS). Both bivariate (Table 1) and multivariate regression analysis (Table 2) revealed no statistically significant associations between NCs and blood transfusion, urologic procedure, age at hospitalization for priapism, or other SCD co-morbidities (VOC and ACS). Conclusion In our large pediatric sickle cell cohort, NCs associated with priapism were rare. Moreover, there was no significant association between the neurologic complications and blood transfusions or urologic procedures in SAP hospitalizations. Further studies are needed to understand the mechanism behind these neurologic complications and any associated risk factors. Disclosures: No relevant conflicts of interest to declare.

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