The Detrimental Impact of End-Stage Kidney Disease Is Not Reflected in Autopsy Reports.

CONTEXT.­: End-stage kidney disease (ESKD) is defined as renal impairment requiring renal replacement therapy to sustain life. With a 1-year mortality of ∼20% to 30%, many die of complications related to this disease. OBJECTIVE.­: To determine the percentage of autopsy cases of decedents with ESKD in which the contribution of ESKD to death is accurately reflected in the final report. DESIGN.­: Autopsy case records were retrospectively reviewed at 4 institutions (Yale New Haven Hospital, University of Chicago Medical Center, University of Illinois at Chicago Hospital, University of Iowa Hospital). Clinical, macroscopic, and microscopic autopsy findings were reviewed, with attention to renal disease findings. RESULTS.­: One hundred sixty decedents with documented ESKD and premortem dialysis who underwent autopsy assessment were identified. ESKD was implicated as a cause of death (CoD) or significant contributing factor in 44 cases (28%), but not in the remaining 116 cases (72%). Cardiovascular disease was the most common CoD in ESKD. There was significant interpathologist variation in the inclusion of ESKD as a CoD across institutions. These rates ranged from 85% correlation (23 of 27 cases), to 13% (4 of 31 and 8 of 62 cases at 2 institutions), and 22.5% (9 of 40 cases) across the 4 participating institutions. CONCLUSIONS.­: The recognition at autopsy of ESKD as a CoD or contributing CoD at autopsy in patients undergoing dialysis remains low (28%). The detrimental impact of ESKD is not reflected in hospital autopsy reports, which carries implications for collection of vital statistics and allocation of research funding for kidney diseases.

Fungemia due to Aureobasidium pullulans.

Aureobasidium pullulans is a yeast-like dematiaceous fungus ubiquitous in nature. It is a rare cause of skin and soft tissue infection, peritonitis, and catheter-related fungemia in certain human hosts. We report a case of recurrent A. pullulans catheter-related fungemia that was successfully treated with caspofungin, posaconazole, and catheter removal.

Gorham Stout disease of the temporal bone with cerebrospinal fluid leak.

Gorham Stout disease (GSD) is a rare disease characterized by the proliferation of endothelial lined vessels and replacement of bone by fibrous tissue. The main imaging features are progressive osteolysis and cortical resorption. Temporal bone involvement is rare but presents as a destructive bone lesion that may be misinterpreted as more common lytic processes in the pediatric population, such as infection or Langerhans cell histiocytosis. GSD of the temporal bone is associated with cerebrospinal fluid (CSF) leaks, may present with otorrhea, and can mimic other causes of ear drainage. Here, we report the clinical course, imaging features, and outcomes of a 3-year-old girl with GSD of the temporal bone presenting with CSF leak initially attributed to infection.

Surgical management of parotid non-tuberculous mycobacteria lymphadenitis in children: A pediatric tertiary-care hospital's experience.

OBJECTIVE: Non-tuberculous mycobacteria (NTM) represents an important etiology of cervicofacial lymphadenitis (CFL) and skin/soft tissue infections in children. It can also affect the salivary glands, including the parotid gland, which is unique due to the presence of intra-salivary lymph nodes. There are no established guidelines for treatment of NTM CFL. NTM lymphadenitis was historically surgically treated; recently the literature supports initial medical treatment. Treatment decisions have been dependent on the extent of disease, preference of providers, and risk of surgical complications. The goal is to report our experience in surgical outcomes of NTM CFL with involvement of the parotid gland after pre-operative medical management. METHODS: A retrospective case series of patients with NTM affecting the parotid gland at a tertiary care pediatric hospital between 2004 and 2020. RESULTS: Seventy-two patients were referred for surgical evaluation of possible parotid NTM. Thirty-three patients underwent surgical excision. Fifteen patients were identified with presumed NTM infection involving the parotid gland. There were twelve females and three males with a mean age of 2.0 years (SD 1.55; range 1-6 days) at the time of surgery. All underwent surgical excision with parotidectomy. The most common pre-operative antimycobacterial therapy used was a combination of clarithromycin and rifampin. All 15 patients had pathological findings consistent with NTM infection (granulomatous lymphadenitis). Forty percent (n = 6) of patients had positive stains with acid-fast bacilli (AFB), with Mycobacterium avium as the most common species (n = 5). The majority of patients, 86.67% (n = 13), had complete resolution of infection after surgery. Clarithromycin and rifampin were the most common post-operative antimycobacterial treatment (mean 81.5 days, SD 110.14, range 2-411 days). The most common complication experienced was acute (<3 months) lower facial nerve paresis (40%, n = 6), but no patient had permanent facial paralysis. CONCLUSION AND RELEVANCE: Parotidectomy is a safe and efficacious treatment in patients with NTM CFL affecting the parotid gland after incomplete resolution with antimycobacterial therapy. Further investigation to optimize duration of antimycobacterial treatment is necessary. We highlight the experience of a high-volume tertiary care pediatric hospital with surgical management of this disease.

Mitochondrial cardiomyopathy and ventricular arrhythmias associated with biallelic variants in C1QBP.

Patients with biallelic mutations in the nuclear-encoded mitochondrial gene C1QBP/p32 have been described with syndromic features and autosomal recessive cardiomyopathy. We describe the clinical course in two siblings who developed cardiomyopathy and ventricular fibrillation in infancy. We provide genomic analysis and clinical-pathologic correlation. Both siblings had profound cardiac failure with ventricular arrhythmia. One child died suddenly. The second sibling survived resuscitation but required extracorporeal cardiopulmonary support and died shortly afterward. On cardiac autopsy, the left ventricle was hypertrophied in both children. Histological examination revealed prominent cardiomyocyte cytoplasmic clearing, and electron microscopy confirmed abnormal mitochondrial structure within cardiomyocytes. DNA sequencing revealed compound heterozygous variants in C1QBP (p.Thr40Asnfs*45 and p.Phe204Leu) in both children. Family segregation analysis demonstrated each variant was inherited from an unaffected, heterozygous parent. Inherited loss of C1QBP/p32 is associated with recessive cardiomyopathy, ventricular fibrillation, and sudden death in early life. Ultrastructural mitochondrial evaluation in the second child was similar to findings in engineered C1qbp-deficient mice. Rapid trio analysis can define rare biallelic variants in genes that may be implicated in sudden death and facilitate medical management and family planning. (184/200).

End-Stage Kidney Disease Is Overlooked as a Proximate Cause of Death at Autopsy.

OBJECTIVES: To determine how often end-stage kidney disease (ESKD) is implicated as a cause of death (COD) at autopsy. METHODS: We searched our autopsy database (2007-2017) using queries "end-stage renal disease," "end-stage kidney disease," "ESRD," "chronic renal disease," and "chronic kidney disease." Final diagnosis and summaries were reviewed to determine if ESKD was appropriately correlated with the COD. Cases in which the COD was unrelated to kidney function were excluded. RESULTS: Eighty-five patients with a history of ESKD and histologic confirmation thereof were identified. Their CODs were cardiovascular (36%), infection/sepsis (41%), pulmonary (6%), gastrointestinal/hepatic (2%), central nervous system (3%), other systemic disease (7%), and unspecified (5%). ESKD was implicated as a contributing COD in 24 (28%) cases. CONCLUSIONS: ESKD is often overlooked at autopsy, particularly in patients with cardiovascular or infectious disease. Accurate documentation of ESKD contributing to mortality is important for education, counseling, record maintenance, and directing research efforts.

Supporting Obesity Prevention in Statewide Quality Rating and Improvement Systems: A Review of State Standards.

INTRODUCTION: A quality rating and improvement system (QRIS) is a fundamental component of most states' early care and education infrastructures. States can use a QRIS to set standards that define high-quality care and award child care providers with a quality rating designation based on how well they meet these standards. The objective of this review was to describe the extent to which states' QRIS standards include obesity prevention content. METHODS: We collected publicly available data on states' QRIS standards. We compared states' QRIS standards with 47 high-impact obesity prevention components in Caring for Our Children: National Health and Safety Performance Standards; Guidelines for Early Care and Education Programs, 3rd Edition, and 6 additional topics based on the Centers for Disease Control and Prevention's Spectrum of Opportunities for Obesity Prevention in the Early Care and Education Setting. RESULTS: Thirty-eight states operated a state-wide QRIS in early 2015. Of those, 27 states' QRIS included obesity prevention standards; 20 states had at least one QRIS standard that aligned with the high-impact obesity prevention components, and 21 states had at least one QRIS standard that aligned with at least one of the 6 additional topics. QRIS standards related to the physical activity high-impact obesity prevention components were the most common, followed by components for screen time, nutrition, and infant feeding. CONCLUSION: The high proportion of states operating a QRIS that included obesity prevention standards, combined with the widespread use of QRISs among states, suggests that a QRIS is a viable way to embed obesity prevention standards into state early care and education systems.

Resonant coherence in photosynthetic electronic energy transfer by site-dependent pigment-protein interactions.

We have numerically examined the effect of site-dependent reorganization on the dynamics of coherent electronic excitation energy transfer in a donor and acceptor pair of photosynthetic pigment-protein complex. Using the quasi-adiabatic propagator path integral method (QUAPI), we have found that a specific proportionality between the site-energy mismatch ϵ and the site-reorganization energy mismatch λ simultaneously increases the length and robustness of the quantum coherence. This behavior is associated with a Rabi type resonance that is manifested in the amplitude and frequency in the coherent portion of the population dynamics. Impact of the resonance on robustness of the coherence under static disorder is also discussed.

Obesity prevention in the early care and education setting: successful initiatives across a spectrum of opportunities.

With an estimated 12.1% of children aged 2-5 years already obese, prevention efforts must target our youngest children. One of the best places to reach young children for such efforts is the early care and education setting (ECE). More than 11 million U.S. children spend an average of 30 hours per week in ECE facilities. Increased attention at the national, state, and community level on the ECE setting for early obesity prevention efforts has sparked a range of innovative efforts. To assist these efforts, CDC developed a technical assistance and training framework - the Spectrum of Opportunities for Obesity Prevention in the ECE setting - which also served as the organizing framework for the Weight of the Nation ECE track. Participants highlighted their efforts at national, state, and local levels pursuing opportunities on the Spectrum, the standards and best practices that had been the emphasis of their efforts, and common steps for developing, implementing, and evaluating initiatives. Strong leadership and collaboration among a broad group of stakeholders; systematic assessment of needs, opportunities and resources; funding sources; and training and professional development were reported to be integral for successful implementation of standards and best practices, and sustainability.

Varicella seroprevalence in the U.S.: data from the National Health and Nutrition Examination Survey, 1999-2004.

OBJECTIVE: We estimated the varicella seroprevalence among the U.S. population aged 6-49 years based on retested National Health and Nutrition Examination Survey (NHANES) specimens collected between 1999 and 2004--originally tested using a method unsuitable for detecting vaccine-induced immunity--and compared it with historical estimates. METHODS: We performed a confirmatory test suitable for detecting vaccine-induced immunity on all available specimens from 6- to 19-year-olds who originally tested negative (n = 633), and on 297 randomly selected specimens that had tested positive. Retest results superseded original results for determining seroprevalence. We assessed seroprevalence for the entire sample aged 6-49 years (n = 16,050) by participant demographic characteristics and compared it with historical estimates (NHANES 1988-1994). RESULTS: The percentage of false-negative results for the original test was higher for specimens from younger children (6-11 years of age: 27.5%; 12-19 years of age: 13.3%) and for specimens collected most recently (2001-2004: 26.0%; 1999-2000: 12.6%). The age-adjusted rate of varicella seroprevalence for 1999-2004 was 93.6% for 6- to 19-year-olds and 98.0% for adults aged 20-49 years compared with 90.0% and 98.1%, respectively, for 1988-1994. We found an increase in seropositivity between the survey periods, from 93.2% to 97.2% (p < 0.001) among 12- to 19-year-olds. For children, non-Hispanic black ethnicity and younger age were associated with lower seroprevalence in both survey periods. CONCLUSIONS: Varicella seroprevalence increased with age among children and was uniformly high in the U.S. adult population between 1999 and 2004. The original testing produced false-negative seroprevalence results among children's specimens collected between 1999 and 2004 from 6- to 19-year-olds.

An outbreak of varicella in elementary school children with two-dose varicella vaccine recipients--Arkansas, 2006.

BACKGROUND: In June 2006, the Advisory Committee on Immunization Practices (ACIP) expanded its June 2005 recommendation for a second dose of varicella vaccine during outbreaks to a recommendation for routine school entry second dose varicella vaccination. In October 2006, the Arkansas Department of Health was notified of a varicella outbreak among students where some received a second dose during an outbreak-related vaccination campaign in February 2006. METHODS: The outbreak was investigated using a school-wide parental survey with a follow-up survey of identified case patients. Vaccination status was verified using state and local immunization records. Limited laboratory testing confirmed circulation of wild-type varicella, including varicella in 2-dose vaccine recipients. RESULTS: Vaccination information was available for 871 (99%) of the 880 children. Varicella vaccination coverage was 97% (2-dose, 39%; 1-dose, 58%). A review of the February vaccination clinic found no deficiencies; lot numbers did not differ between cases and noncases. Varicella was confirmed by PCR in 5 (42%) of 12 lesion specimens and by IgM in 1 (6%) of 16 serum specimens. Varicella was reported in 84 children, including 25 (30%) two-dose and 53 (63%) one-dose recipients. Attack rates among 2-dose recipients (10.4%) and 1-dose recipients (14.6%) were not significantly different (RR: 0.72, 95% CI: 0.44-1.15). All 2-dose recipients and 80% of 1-dose recipients reported having 50 or fewer skin lesions. CONCLUSION: This outbreak is the first to document varicella in both 1- and 2-dose vaccine recipients; both groups had mild disease. The vaccine effectiveness of 1 and 2 doses were similar.

Hospitalizations to treat herpes zoster in older adults: causes and validated rates.

BACKGROUND: The availability of a vaccine for the prevention of herpes zoster has increased interest in methods to measure zoster disease burden. Hospitalizations assigned a zoster diagnosis code have been used as indicators of severe zoster in prior studies. However, a zoster diagnosis code may not be a specific indicator of severe zoster illness, because the code may be assigned to a hospitalization for another cause in a person with coincident zoster. METHODS: To assess the validity of a hospital diagnosis code of zoster as an indicator of hospitalizations that are attributable to zoster, we identified all hospitalizations with a zoster diagnosis code assigned in any position among members of a managed-care organization who were >or=50 years of age during 1992-2004. Of those, we selected a sample of 260 hospitalizations for chart review. RESULTS: Chart reviews were completed for 225 hospitalizations. Sixty-five (29%) were because of zoster or a complication of zoster treatment, and an additional 9 (4%) were because of postherpetic neuralgia or a complication of postherpetic neuralgia treatment. Although the overall age-adjusted rate of hospitalizations with a zoster diagnosis code was 42.5 hospitalizations per 100,000 population per year, the estimated rate of hospitalizations because of zoster, postherpetic neuralgia, or adverse effects of a medication used to treat zoster or postherpetic neuralgia was only 14.0 hospitalizations per 100,000 population per year. CONCLUSIONS: Rates of hospitalizations associated with a zoster diagnosis code will substantially overestimate the burden of hospitalizations attributable to zoster in older adults.

Challenges to implementing second-dose varicella vaccination during an outbreak in the absence of a routine 2-dose vaccination requirement--Maine, 2006.

In June 2005, the Advisory Committee on Immunization Practices (ACIP) recommended administering a second dose of varicella vaccine during outbreaks, supplementing the routine 1-dose requirement. From October 2005 to January 2006, a varicella outbreak occurred in Maine in a highly vaccinated elementary school population. We investigated the outbreak, held a school-based vaccination clinic, and assessed costs in implementing ACIP's outbreak-response recommendation. Parents completed questionnaires and case investigation interviews. Personnel at the Maine Center for Disease Control and Prevention, the school in which the outbreak occurred ("school A"), and physician offices completed economic surveys. Forty-eight cases occurred, with no hospitalizations or deaths. Vaccine effectiveness was 86.6% (95% confidence interval, 82.0%-90.1%). Of 240 eligible students, 132 (55.0%) received second-dose vaccination. Implementing ACIP's outbreak-response recommendation was challenging and cost approximately $26,875. Additionally, the routine 1-dose varicella vaccination policy did not confer adequate population immunity to prevent this outbreak. These findings support ACIP's June 2007 recommendation for a routine 2-dose varicella vaccination program.

Epidemiology of varicella hospitalizations in the United States, 1995-2005.

To describe the impact of the varicella vaccination program on varicella-related hospitalizations (VRHs) in the United States, data from the Varicella Active Surveillance Project (VASP) were used to compare rates of hospitalization and rates of complications among patients hospitalized for varicella-related conditions from 1995 to 2005. Of the 26,290 varicella cases reported between 1995 and 2005, 170 cases resulted in VRHs, including 1 case that resulted in death. Both VRH rates per 100,000 population and complications during VRH per 100,000 population decreased significantly between the early vaccination period (1995-1998) and the middle/late vaccination period (1999-2005). Infants and adults were at highest risk for VRH, and having been vaccinated against varicella was a protective factor. Varicella vaccination may have prevented a significant number of VRHs. The fact that 4 vaccinated children required hospitalization for varicella-related complications demonstrates that 1 dose of varicella vaccine does not prevent serious disease in all cases, even among previously healthy children.

The impact of the varicella vaccination program on herpes zoster epidemiology in the United States: a review.

Speculation that a universal varicella vaccination program might lead to an increase in herpes zoster (HZ) incidence has been supported by modeling studies that assume that exposure to varicella boosts immunity and protects against reactivation of varicella-zoster virus (VZV) as HZ. Such studies predict an increase in HZ incidence until the adult population becomes predominantly composed of individuals with vaccine-induced immunity who do not harbor wild-type VZV. In the United States, a varicella vaccination program was implemented in 1995. Since then, studies monitoring HZ incidence have shown inconsistent findings: 2 studies have shown no increase in overall incidence, whereas 1 study has shown an increase. Studies from Canada and the United Kingdom have shown increasing rates of HZ incidence in the absence of a varicella vaccination program. Data suggest that heretofore unidentified risk factors for HZ also are changing over time. Further studies are needed to identify these factors, to isolate possible additional effects from a varicella vaccination program. Untangling the contribution of these different factors on HZ epidemiology will be challenging.

Knowledge, attitudes, and practices regarding varicella vaccination among health care providers participating in the varicella active surveillance project, Antelope Valley, California, 2005.

Knowledge, attitudes, and practices regarding varicella vaccination and disease were assessed among health care providers participating in the Varicella Active Surveillance Project in Antelope Valley, California, in 2005. Compared with those of a similar survey conducted in 1999, results suggest a reduction in concerns about vaccine safety and efficacy. Routine assessment of adolescents for varicella susceptibility was reported by 87% of respondents, but only 42% reported routine assessment of adults. Several respondents were unaware that disease in a vaccinated person is infectious, and some did not know the vaccination recommendations pertaining to susceptible health care workers, suggesting a need for provider education on these issues.

Validity of self-reported varicella disease history in pregnant women attending prenatal clinics.

OBJECTIVE: The purpose of this study was to assess the validity of self-reported history for varicella disease relative to serological evidence of varicella immunity in pregnant women attending antenatal care at clinics located in two diverse geographical locations in the U.S. (Antelope Valley, California, and Philadelphia) with high varicella vaccination coverage. METHODS: Pregnant women attending prenatal care appointments who needed blood drawn as part of their routine care were eligible to participate. Self-reported varicella disease history was obtained via questionnaire. Varicella serostatus was determined using a whole-cell enzyme-linked immunosorbent assay to test for varicella zoster virus-specific immunoglobulin G (VZV IgG) antibodies. RESULTS: Of the 309 study participants from Antelope Valley and the 528 participants from Philadelphia who self-reported having had chickenpox disease, 308 (99.7%; 95% confidence interval [CI]: 98.2, 100) and 517 (97.9%; 95% CI: 96.3, 99.0), respectively, had serological evidence of immunity to varicella. Only 6.8% (95% CI: 3.9, 11.0) and 17.4% (95% CI: 13.1, 22.5) of women who self-reported having a negative or uncertain varicella disease history in Antelope Valley and Philadelphia, respectively, were seronegative for varicella antibodies. CONCLUSION: Despite the dramatic changes in the epidemiology of varicella that have occurred since 1995 due to the introduction and subsequent widespread use of the varicella vaccine, self-reported history of varicella continues to be a strong predictor of VZV IgG antibodies in pregnant women. Negative or uncertain history remains poorly predictive of negative serostatus.

A population-based study of maternal and perinatal outcomes associated with assisted reproductive technology in Massachusetts.

OBJECTIVE: To assess associations between assisted reproductive technology (ART) and adverse maternal and infant outcomes, with an emphasis on singletons. METHODS: We linked data from the US ART surveillance system with Massachusetts live birth-infant death records data for resident births in 1997-1998 and compared births conceived with ART (N = 3316) with births not conceived with ART or infertility medications (N = 157,066) on: maternal chronic conditions, pregnancy complications, labor and delivery complications, and perinatal and infant outcomes. RESULTS: Overall, ART was strongly associated with numerous adverse outcomes. The magnitude was reduced for several outcomes when analyses were limited to singletons. After further exclusion of maternal subsets with rare ART births (maternal age <20; education