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ABSTRACT: Approximately 30% of pediatric acute lymphoblastic leukemia patients present with musculoskeletal symptoms and are often referred first to a pediatric rheumatologist. We examined the survival and causes of death of these patients presenting to a pediatric rheumatologist and compared the rates with that reported in the hematology-oncology literature.
We used the Pediatric Rheumatology Disease Registry, including 49,023 patients from 62 centers, newly diagnosed between 1992 and 2001. Identifiers were matched with the Social Security Death Index censored for March 2005. Deaths were confirmed by death certificates, referring physicians, and medical records. Causes of death were derived by chart review or from the death certificate.
There were 7 deaths of 89 patients (7.9%, 95% confidence interval: 3.9%-15.4%) with acute lymphoblastic leukemia with a 5-year survival rate of 95.5% (88.3 to 98.3) and 10-year survival rate of 89.8% (79.0% to 95.2%). The causes of death were sepsis (bacterial and/or fungal) in 4 (57%) patients, the disease in 2 (29%) and post bone-marrow transplantation in 1 (14%).
The overall survival of patients with acute lymphoblastic leukemia seen first by pediatric rheumatologists is higher than the range reported in the pediatric oncology literature for the same period of diagnosis.
Journal of Pediatric Hematology/Oncology 05/2011; 33(6):424-8. · 1.16 Impact Factor
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ABSTRACT: The purpose of our study was to evaluate HPV vaccine acceptance among parents and guardians of children aged 0-10 years.
Prospective questionnaire study.
Cleveland Clinic Children's Hospital.
Parents and guardians of children aged 0-10 years.
Brief HPV vaccine educational intervention.
Desire for child to get HPV vaccine.
We enrolled 81 participants in the study; 70 (86%) were female, and 39 (49%) were Caucasian. Prior to receiving an educational fact sheet about HPV and the HPV vaccine, only 49% of participants reported that they wanted their young child to receive the HPV vaccine when it becomes available. After receiving the fact sheet, this number increased to 70%, suggesting that a simple educational intervention could significantly affect vaccine acceptance in this population (P = .001). Other significant results of this study included that HPV vaccination would receive greater acceptance if the participants believed that it can prevent HPV infection in their child (P = .0024), it was perceived to be safe (P = .0005), and if the vaccine were recommended by a physician (P < .0001). Participants' attitudes about HPV vaccination were not affected by concerns over whether receiving the vaccine might mean the child is more likely to have sex or to have multiple sexual partners.
Our results suggest that if it were approved for children aged 0-10 years, the HPV vaccine would be accepted by the parents and guardians provided they received adequate educational information about it.
Journal of pediatric and adolescent gynecology 04/2011; 24(2):66-70. · 0.90 Impact Factor
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ABSTRACT: The authors describe the demographics, clinical presentation, investigation, treatment, and outcomes of pediatric patients with Guillain-Barré syndrome. They identified 35 pediatric patients with Guillain-Barré syndrome presenting to a tertiary academic center over a 20-year period. The most common presenting symptoms were paresthesias (54%), weakness (49%), and myalgias (49%). Sensation was affected in 54% of patients, and hyporeflexia or areflexia was present in 94% of patients. Cranial nerve dysfunction (46%) and autonomic involvement (eg, changes in blood pressure, pulse, bowel/bladder control, or priapism; 46%) were also common. Autonomic dysfunction, cranial nerve involvement, and albuminocytological dissociation were significantly associated with a decreased time to nadir, the point when symptoms peaked (P = .015, .007, and .005, respectively). Although not statistically significant, treatment with plasmapheresis had a better success rate than intravenous immunoglobulin. The authors' results will help to further delineate the clinical picture of Guillain-Barré syndrome in children and refine treatment strategies.
Journal of child neurology 12/2010; 25(12):1504-10. · 1.59 Impact Factor
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ABSTRACT: Pulmonary vein stenosis (PVS) complicating pulmonary vein isolation (PVI) can progress to total pulmonary vein occlusion (PVO). Little is known about the accuracy of noninvasive diagnosis and treatment of PVO.
The purpose of this study was to study the diagnostic accuracy of noninvasive testing and the feasibility and outcome of percutaneous intervention for PVO.
Computed tomography (CT)-diagnosed and angiographically confirmed PVOs were identified from percutaneous interventions for PVS complicating PVI between December 2000 and December 2008. Diagnostic accuracy of CT combined with lung perfusion scan was studied. Outcome of percutaneous intervention was reviewed.
CT diagnosed "PVO" in 53 PVs, with only 20 of 53 determined angiographically to be totally occluded. True PVO had lower perfusion (4.0%) compared with CT-diagnosed "PVO" (7.3%, P = .024). Recanalization was attempted in 9 and successful in 8. Of the 8 patients, 7 were dilated with 4.5- to 7-mm balloons and 1 was stented primarily (7 mm). At repeat catheterization 2.9 +/- 0.8 months later, 6 of 7 pulmonary veins (PVs) were stented to 5 to 10 mm. At follow-up of 11.3 +/- 8.7 months, all but 1 PV remained patent (mean diameter 6.9 +/- 1.7 mm). Flow to the lung quadrant increased from 5.6% before recanalization to 12.2% at last follow-up (P = .016). Symptoms improved in all but one patient.
PVO is overestimated by CT. Quantification of lung perfusion improves diagnostic accuracy, but angiography remains the gold standard. Recanalization of PVO can be attempted when a remnant of the PV is visible. Good mid-term patency rates and improved perfusion were observed with a two-stage approach of initial dilation and subsequent stenting. Longer follow-up and larger numbers of patients are needed to better understand when to intervene for PVO.
Heart rhythm: the official journal of the Heart Rhythm Society 03/2010; 7(9):1233-9. · 4.56 Impact Factor
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ABSTRACT: To describe mortality rates, causes of death, and potential mortality risk factors in pediatric rheumatic diseases in the US.
We used the Indianapolis Pediatric Rheumatology Disease Registry, which includes 49,023 patients from 62 centers who were newly diagnosed between 1992 and 2001. Identifiers were matched with the Social Security Death Index censored for March 2005. Deaths were confirmed by death certificates, referring physicians, and medical records. Causes of death were derived by chart review or from the death certificate. Standardized mortality ratios (SMRs) and 95% confidence intervals (95% CIs) were determined.
After excluding patients with malignancy, 110 deaths among 48,885 patients (0.23%) were confirmed. Patients had been followed up for a mean +/- SD of 7.9 +/- 2.7 years. The SMR of the entire cohort was significantly decreased (0.65 [95% CI 0.53-0.78]), with differences in patients followed up for > or =9 years. The SMR was significantly greater for systemic lupus erythematosus (3.06 [95% CI 1.78-4.90]) and dermatomyositis (2.64 [95% CI 0.86-6.17]) but not for systemic juvenile rheumatoid arthritis (1.8 [95% CI 0.66-3.92]). The SMR was significantly decreased in pain syndromes (0.41 [95% CI 0.21-0.72]). Causes of death were related to the rheumatic diagnosis (including complications) in 39 patients (35%), treatment complications in 11 (10%), non-natural causes in 25 (23%), background disease in 23 (21%), and were unknown in 12 patients (11%). Rheumatic diagnoses, age at diagnosis, sex, and early use of systemic steroids and methotrexate were significantly associated with the risk of death.
Our findings indicate that the overall mortality rate for pediatric rheumatic diseases was not increased. Even for the diseases and conditions associated with increased mortality, mortality rates were significantly lower than those reported in previous studies.
Arthritis & Rheumatism 02/2010; 62(2):599-608. · 7.87 Impact Factor
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ABSTRACT: Pulmonary vein stenosis (PVS) is a rare but significant complication of pulmonary vein isolation (PVI). Dilation and stent angioplasty have been described but not compared.
All percutaneous interventions for PVS complicating PVI between December 2000 and March 2007 were reviewed. Acute success, defined as post-intervention stenosis <or=30%, and long-term outcome of dilation versus stent angioplasty were compared. Freedom from restenosis was defined as freedom from repeat intervention. Overall outcome for all interventions was examined. We studied 34 patients with 55 stenotic veins followed for a mean of 25 months. Dilation was performed in 39 veins and stenting in 40 veins (16 primarily, 24 after dilation restenosis). Acute success and restenosis rates were 42% and 72% for dilation versus 95% (P < 0.001) and 33% for stenting. Time to restenosis was greater for stent angioplasty (P = 0.003). Stents >or=10 mm in diameter had lower restenosis than smaller stents. Risk factors for restenosis included small reference vessel diameter and longer time from PVI to intervention for PVS. All but two patients experienced improvement (n = 10) or resolution of symptoms (n = 22). The mean percent stenosis decreased from 82% to 21% for the entire cohort and mean flow to the lung quadrant increased from 10% to 17%.
Stent angioplasty results in less restenosis than dilation, particularly for stents >or=10 mm. Early referral may improve long-term patency by minimizing reference vessel atrophy. Most patients with PVS post-PVI can be improved symptomatically with catheter intervention.
Journal of Cardiovascular Electrophysiology 03/2008; 19(7):673-8. · 3.06 Impact Factor
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ABSTRACT: To examine the relationship of early human embryonic development with the level of reactive oxygen species (ROS) in the culture media on the first day (day 1 ROS) after insemination.
A prospective study.
Patients undergoing assisted reproduction in a teaching hospital.
Patients undergoing conventional IVF (n = 104; 115 cycles) and intracytoplasmic sperm injection (ICSI) (n = 91; 96 cycles) were included. Both fertilization and early cultures were performed in human tubal fluid with 5% serum substitute supplement.
Day 1 ROS levels in the central well (sample) and the outer well (control) of each embryo culture dish were measured after overnight incubation by chemiluminescence assay using luminol as the probe.
Fertilization rate and embryo quality at day 3 and 5 were recorded for each cycle. Age, parity, and demographic features were also compared.
High day 1 ROS levels in culture media were associated with low blastocyst rate, low fertilization rate, low cleavage rate, and high embryonic fragmentation with ICSI but not with conventional IVF. High day 1 ROS levels in culture media were associated with lower pregnancy rates in both IVF and ICSI cycles.
Reactive oxygen species generated in culture media by day 1 may be an important biochemical marker for early embryonic growth. Increased embryonic fragmentation and slow cleavage rate may be partially attributed to early exposure of embryos to high ROS levels in ICSI cycles. Differential growth of ICSI embryos incubated under identical conditions may be in part due to differences in ROS levels of the culture medium surrounding these embryos.
Fertility and Sterility 10/2004; 82(3):593-600. · 3.56 Impact Factor
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ABSTRACT: The 700 Ultrex (American Medical Systems, Minnetonka, Minnesota) is the only penile prosthesis capable of length and girth expansion. Early experience with the 700 Ultrex showed an increased mechanical failure rate compared with the 700CX, mostly secondary to cylinder failure. In 1993 the Ultrex cylinders were modified. We examined the performance of the Ultrex device before and after modification.
We compared our results with the Ultrex prosthesis before (group 1) and after (group 2) the 1993 modification. We implanted 239 devices from October 1989 to December 1999. A total of 26 patients have died. Followup was obtained on the results of 137 of the remaining 213 implants (64%), including 85 pre-modification devices in 85 patients and 52 post-modification devices in 51, via a mailed questionnaire, telephone survey or chart review. The questionnaire and survey included a 5-point satisfaction scale. Groups 1 and 2 were compared in regard to 3 end points, namely cylinder, mechanical and overall failure.
Followup was less than 1 to 136 months (median 92, 25th to 75th percentiles 43 to 108) in group 1 and less than 1 to 92 months (median 46, 25th to 75th percentiles 21 to 75) in group 2. The 5-year Kaplan-Meier estimates of overall, mechanical and cylinder survival in groups 1 and 2 were 64.7%, 70.7% and 80.2%, and 77.7% (p = 0.23), 93.7% (p = 0.017) and 96.2% (p = 0.008), respectively. Overall satisfaction was similarly high in groups 1 and 2 (mean 3.9 and 4 points).
On long-term followup the 1993 modification of the Ultrex cylinders appears to have significantly decreased the propensity of cylinder failure of the pre-modification device.
The Journal of Urology 07/2002; 167(6):2502-6. · 3.75 Impact Factor
