Recent Publications by CFE Educators

BACKGROUND

Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome with both genetic and acquired causes characterized by elevated cytokine levels, hyperinflammation, and overactivation of lymphocytes and macrophages. It is typically a systemic disease with variable degrees of CNS involvement. Cases with predominantly central nervous system (CNS) involvement are very rare, with the vast majority of these occurring in infants and young children. This report documents a case of adult-onset CNS-HLH involving a middle-aged man.

CASE PRESENTATION

A 55 year-old man developed progressive left hemiparesis and aphasia over the course of several months. Brain MRI showed multifocal, mass-like enhancing lesions with increased susceptibility consistent with blood products. An extensive workup for infectious, autoimmune, and neoplastic etiologies was significant only for a markedly elevated serum ferritin at 1456 ng/mL. Two brain biopsies showed a non-specific inflammatory process. The patient was treated empirically with steroids and plasmapheresis, but he continued to suffer a progressive neurological decline and died one year after onset of neurological symptoms. Autopsy revealed profound histiocytic infiltration, perivascular lymphocytosis, and emperipolesis, compatible with CNS-HLH.

CONCLUSION

This case report describes an exceedingly rare presentation of an adult patient with CNS predominant HLH. This diagnosis should be considered in the differential diagnosis of adults presenting with progressive brain lesions, even in the absence of typical systemic signs of HLH.

Several studies reported an increased risk of thyroid cancer in children and adolescents exposed to radioactive iodines, chiefly iodine-131 ((131)I), after the 1986 Chornobyl (Ukrainian spelling) nuclear power plant accident. The risk of benign thyroid tumors following such radiation exposure is much less well known. We have previously reported a novel finding of significantly increased risk of thyroid follicular adenoma in a screening study of children and adolescents exposed to the Chornobyl fallout in Ukraine. To verify this finding, we analyzed baseline screening data from a cohort of 11,613 individuals aged ≤18 years at the time of the accident in Belarus (mean age at screening = 21 years). All participants had individual (131)I doses estimated from thyroid radioactivity measurements and were screened according to a standardized protocol. We found a significant linear dose response for 38 pathologically confirmed follicular adenoma cases. The excess odds ratio per gray of 2.22 (95% confidence interval: 0.41, 13.1) was similar in males and females but decreased significantly with increasing age at exposure (P 0.01), with the highest radiation risks estimated for those exposed at 2 years of age. Follicular adenoma radiation risks were not significantly modified by most indicators of past and current iodine deficiency. The present study confirms the (131)I-associated increases in risk of follicular adenoma in the Ukrainian population and adds new evidence on the risk increasing with decreasing age at exposure.

Identification of precursors of atrial fibrillation (AF) may lead to early detection and prevent associated morbidity and mortality. This study aimed to examine the association between frequent atrial premature complexes (APCs) and incidence of AF. For this retrospective cohort study, we analyzed Holter recordings obtained from 2000 to 2010 of 1,357 veterans free of AF at baseline. All pertinent data in electronic medical records were reviewed to ascertain baseline characteristics. Holter groups with frequent (≥100/day) and infrequent (100/day) APCs were compared for development of new AF over a median follow-up of 7.5 years. Multivariate Cox regression analyses were performed before and after propensity score matching. Mean age was 64 years with 93% men. Mean body mass index, hemoglobin A1C, low-density lipoprotein, left atrial size, and heart rate were 31.24 kg/m(2), 6.42%, 107.92 mg/dl, 4.26 cm, and 73 beats/min, respectively. AF was noted in 21.8% of patients with frequent APCs compared to 5.6% of those with infrequent APCs. After adjusting for demographics, medication use, co-morbidities, and laboratory and echocardiographic findings, multivariate Cox regression analyses confirmed frequent APCs to be independently associated with higher incidence of AF (hazard ratio [HR] 2.97, 95% confidence interval [CI] 1.85 to 4.80; p 0.001). In propensity-matched groups, this association remained significant (HR 2.87, 95% CI 1.65 to 4.98; p 0.001). Additionally, atrial couplets (≥50/day), atrial bigeminy (≥50/day), frequent runs of ≥3 APCs (≥20 runs/day), and longer runs (≥10 beats/run) were significantly associated with AF (HR 3.11, 3.67, 2.94, and 1.73, respectively, all p 0.05). In conclusion, frequent APCs (≥100/day) are associated with greater risk of AF.

OBJECTIVE

The authors present clinical outcome data and satisfaction of patients who underwent minimally invasive vertebral body corpectomy and cage placement via a mini-open, extreme lateral, transpsoas approach and posterior short-segment instrumentation for lumbar burst fractures.

METHODS

Patients with unstable lumbar burst fractures who underwent corpectomy and anterior column reconstruction via a mini-open, extreme lateral, transpsoas approach with short-segment posterior fixation were reviewed retrospectively. Demographic information, operative parameters, perioperative radiographic measurements, and complications were analyzed. Patient-reported outcome instruments (Oswestry Disability Index [ODI], 12-Item Short Form Health Survey [SF-12]) and an anterior scar-specific patient satisfaction questionnaire were recorded at the latest follow-up.

RESULTS

Twelve patients (7 men, 5 women, average age 42 years, range 22-68 years) met the inclusion criteria. Lumbar corpectomies with anterior column support were performed (L-1, n = 8; L-2, n = 2; L-3, n = 2) and supplemented with short-segment posterior instrumentation (4 open, 8 percutaneous). Four patients had preoperative neurological deficits, all of which improved after surgery. No new neurological complications were noted. The anterior incision on average was 6.4 cm (range 5-8 cm) in length, caused mild pain and disability, and was aesthetically acceptable to the large majority of patients. Three patients required chest tube placement for pleural violation, and 1 patient required reoperation for cage subsidence/hardware failure. Average clinical follow-up was 38 months (range 16-68 months), and average radiographic follow-up was 37 months (range 6-68 months). Preoperative lumbar lordosis and focal lordosis were significantly improved/maintained after surgery. Patients were satisfied with their outcomes, had minimal/moderate disability (average ODI score 20, range 0-52), and had good physical (SF-12 physical component score 41.7% ± 10.4%) and mental health outcomes (SF-12 mental component score 50.2% ± 11.6%) after surgery.

CONCLUSIONS

Anterior corpectomy and cage placement via a mini-open, extreme lateral, transpsoas approach supplemented by short-segment posterior instrumentation is a safe, effective alternative to conventional approaches in the treatment of single-level unstable burst fractures and is associated with excellent functional outcomes and patient satisfaction.

BACKGROUND

The American Thyroid Association (ATA) and National Comprehensive Cancer Network (NCCN) guidelines have discordant recommendations for managing patients with differentiated thyroid cancer (DTC). We hypothesized that physician adherence to either of the 2009 extent of surgery guidelines of the ATA or NCCN was associated with improved survival, and that practice is most standardized nationally when guidelines are concordant.

METHODS

Adult patients undergoing surgery for DTC were included from the National Cancer Database. Multivariable modeling was used to identify factors associated with nonadherence to the 2009 ATA or NCCN guidelines (2010-2011) and hypothetically examine the association of retrospective adherence to guidelines with survival (1998-2006).

RESULTS

A total of 39,687 patients with DTC were included; 2,249 were not treated in accordance with ATA or NCCN guidelines. Factors independently associated with nonadherence were discordance between ATA and NCCN recommendations, black race, and treatment at nonacademic centers (P .01). After adjustment, care not in accordance with either set of guidelines was associated with compromised survival (hazard ratio 1.16, P = .02).

CONCLUSION

A minority of patients received surgery for DTC not aligned with guidelines; nonadherent care was associated with compromised survival. Discordance in recommendations between guidelines is associated with reduction in adherent care, suggesting that standardizing guidelines could decrease confusion, increase adherence, and thereby may improve outcomes.

BACKGROUND

Adrenal venous sampling is an important lateralization study for primary aldosteronism, but inability to cannulate the right adrenal vein is not uncommon and interpreted as a failed study. We challenged this notion by examining whether data from incomplete left-sided adrenal venous sampling could accurately predict lateralization.

METHODS

Sixty-two adrenal venous sampling studies from 2007 to 2014 at 2 tertiary-care institutions were reviewed. For this analysis, data from the right adrenal vein were excluded. The study variable was the aldosterone:cortisol ratio of the left adrenal vein compared with the inferior vena cava (LAV/IVC). Scatterplot analysis identified high and low LAV/IVC cutoffs that predicted accurately unilateral disease in 1 institutional cohort and validated in the second cohort.

RESULTS

Thirty-six studies of adrenal venous sampling were evaluated at the first institution and divided into 3 diagnostic categories: unilateral-left (n = 14), unilateral-right (n = 12), and bilateral (n = 10). Cutoff values of the ratios of LAV/IVC of ≥ 5.5 and ≤ 0.5 accurately predicted left- and right-sided disease, respectively, and were validated in 26 studies from the second institution (100% PPV). The "5.5-0.5 criteria" salvaged accuracy for predicting lateralization in 50% of cases.

CONCLUSION

Even in the setting of failed cannulation of the right adrenal vein, the actual data from the remaining adrenal venous sampling can predict lateralization accurately in many patients. A "failed" adrenal venous sampling study may be of greater predictive utility than believed traditionally.