Severe Coronavirus Disease-2019 in Children and Young Adults in the Washington, DC, Metropolitan Region.

Despite worldwide spread of severe acute respiratory syndrome coronavirus-2, few publications have reported the potential for severe disease in the pediatric population. We report 177 infected children and young adults, including 44 hospitalized and 9 critically ill patients, with a comparison of patient characteristics between infected hospitalized and nonhospitalized cohorts, as well as critically ill and noncritically ill cohorts. Children <1 year and adolescents and young adults >15 years of age were over-represented among hospitalized patients (P = .07). Adolescents and young adults were over-represented among the critically ill cohort (P = .02).

Surgical correction of an arteriovenous fistula in a ring-tailed lemur (Lemur catta).

A 10-y-old ovariohysterectomized ring-tailed lemur (Lemur catta) was presented for exacerbation of respiratory signs. The lemur had a history of multiple examinations for various problems, including traumatic lacerations and recurrent perivulvar dermatitis. Examination revealed abnormal lung sounds and a femoral arteriovenous fistula with a palpable thrill and auscultable bruit in the right inguinal area. A diagnosis of congestive heart failure was made on the basis of exam findings, radiography, abdominal ultrasonography, and echocardiography. The lemur was maintained on furosemide until surgical ligation of the fistula was performed. Postoperative examination confirmed successful closure of the fistula and resolution of the signs of heart failure. Arteriovenous fistulas are abnormal connections between an artery and a vein that bypass the capillary bed. Large arteriovenous fistulas may result in decreased peripheral resistance and an increase in cardiac output with consequent cardiomegaly and high output heart failure. This lemur's high-flow arteriovenous fistula with secondary heart failure may have been iatrogenically induced during blood collection by prior femoral venipuncture. To our knowledge, this report is the first description of an arteriovenous fistula in a prosimian. Successful surgical correction of suspected iatrogenic femoral arteriovenous fistulas in a cynomolgus monkey (Macaca fascicularis) and a rhesus macaque (Macaca mulatta) have been reported previously. Arteriovenous fistula formation should be considered as a rare potential complication of venipuncture and as a treatable cause of congestive heart failure in lemurs.

A mosaic activating mutation in AKT1 associated with the Proteus syndrome.

BACKGROUND: The Proteus syndrome is characterized by the overgrowth of skin, connective tissue, brain, and other tissues. It has been hypothesized that the syndrome is caused by somatic mosaicism for a mutation that is lethal in the nonmosaic state. METHODS: We performed exome sequencing of DNA from biopsy samples obtained from patients with the Proteus syndrome and compared the resultant DNA sequences with those of unaffected tissues obtained from the same patients. We confirmed and extended an observed association, using a custom restriction-enzyme assay to analyze the DNA in 158 samples from 29 patients with the Proteus syndrome. We then assayed activation of the AKT protein in affected tissues, using phosphorylation-specific antibodies on Western blots. RESULTS: Of 29 patients with the Proteus syndrome, 26 had a somatic activating mutation (c.49G→A, p.Glu17Lys) in the oncogene AKT1, encoding the AKT1 kinase, an enzyme known to mediate processes such as cell proliferation and apoptosis. Tissues and cell lines from patients with the Proteus syndrome harbored admixtures of mutant alleles that ranged from 1% to approximately 50%. Mutant cell lines showed greater AKT phosphorylation than did control cell lines. A pair of single-cell clones that were established from the same starting culture and differed with respect to their mutation status had different levels of AKT phosphorylation. CONCLUSIONS: The Proteus syndrome is caused by a somatic activating mutation in AKT1, proving the hypothesis of somatic mosaicism and implicating activation of the PI3K-AKT pathway in the characteristic clinical findings of overgrowth and tumor susceptibility in this disorder. (Funded by the Intramural Research Program of the National Human Genome Research Institute.).

Embryonal rhabdomyosarcoma in a Rothschild's giraffe (Giraffa camelopardalis rothschildi).

Abstract: A 3-yr-old male Rothschild's giraffe (Giraffa camelopardalis rothschildi) presented for acute swelling caudomedial to the left parietal horn. Following initial diagnostics and supportive treatment, the mass was surgically resected and intralesional chemotherapy was administered. Despite treatment, the giraffe's condition worsened and euthanasia was performed. Gross necropsy revealed neoplastic invasion and destruction of underlying parietal bone, adjacent horn base, and sinuses, and metastases in the tracheobronchial and mandibular lymph nodes and lung. Histologically, the tumor was composed of packets of anaplastic round cells. Immunohistochemical studies further characterized the tumor as an embryonal rhabdomyosarcoma. This is the first reported case of rhabdomyosarcoma in a giraffe.

Pediatric surgery workforce: supply and demand.

INTRODUCTION: Recent studies report a shortage of pediatric surgeons in the United States. We surveyed members of the American Pediatric Surgical Association (APSA) to estimate current workforce and demand and to provide data for workforce planning. METHODS: We conducted a survey of 849 APSA members to provide workforce data on their communities as follows: the number of active, retired, or inactive APSA surgeons; non-APSA fellowship graduates; surgeons without accredited fellowship training; and the estimated demand for additional pediatric surgeons. Internet search engines identified surgeons and practices offering pediatric surgical services. The US Census Metropolitan Statistical Areas (MSAs) defined service areas with populations of 100,000 or more. RESULTS: Of 137 MSAs with APSA members in practice, we obtained data from 113 (83%), with 247 (29%) of 849 surgeons responding. We estimate that the current pediatric surgical workforce consists of 1150 surgeons, with APSA members in active practice (60%) forming the single largest group, followed by general surgeons (21%). The percentage of active APSA surgeons was greater than the percentage of general surgeons in the 50 largest MSAs (76% vs 2%, respectively), whereas the opposite was observed in the smaller MSA ranked more than 51 in population (37% vs 46%, respectively). American Pediatric Surgical Association respondents estimated a national demand for 280 additional pediatric surgeons. Active APSA surgeons plan to delay retirement (8% of respondents) because it would leave their group or community shorthanded; 2% reported that retirement would leave the community without a pediatric surgeon. DISCUSSION: Workforce shortage in pediatric surgery is a problem of number and distribution. Incentives to direct trainees to underserved areas are needed. General surgeons provide pediatric services in many communities. Surgical training should include additional training in pediatric surgery.

Clinical and financial characteristics of pediatric surgery practices.

UNLABELLED: Pediatric surgical practices face many challenges. We wanted to define the clinical practice and financial support among different types of practices as follows: academic, private practice, and employed. METHODS: This study involved an Internet survey of members of the American Pediatric Surgical Association (APSA), comparisons using chi(2) and paired t test analyses. RESULTS: The response rate was 28.7% (233/811), 145 academic, 48 private, and 40 employed. More than 90% received partial to full financial hospital support. Only 7.3% received no outside support, most frequently those in private practices (16.7%; P = .016). More than 90% had resident or fellow coverage. Nearly all practices covered newborn conditions and solid tumors, with differences in pediatric trauma, patent ductus arteriosus, and urologic condition. Transfer out of community was low but increased for specific conditions during the respondents' absence, from 0.4% to 5.2% to 3.4% to 6.9% (P = .001-0.003). A minority of respondents noted that nonpediatric surgeons treated selected pediatric conditions in their communities as follows: inguinal hernia (38.4%), umbilical hernia (42.6%), abscesses (37.5%), and trauma (36.6%). Pediatric surgeons shared call within their group in 86.3%, whereas 5.6% took call alone. Many restricted call by excluding trauma (37.2%), soft tissue infections or appendectomies (21.3% for both), and older children (>12 years, 23.8%). Nearly one fifth (18.9%) expressed interest in having an APSA surgeon serve as a locum tenens in their practices. DISCUSSION: Many pediatric surgeons receive both financial and in-kind subsidies. Although they cover a wide breadth of surgical conditions, many limit the conditions that they treat to reduce call responsibilities. The workforce shortage in pediatric surgery creates call coverage problems that may affect up to 8% of US practices.

Interobserver variation in the assessment of appendiceal perforation.

BACKGROUND: Following an appendectomy, surgeons define appendicitis, for treatment and billing purposes, into one of four categories: normal appendix, acute appendicitis, gangrenous appendicitis, and perforated appendicitis. Treatment of appendicitis is predicated upon classification at the time of visual inspection. Further, this classification often plays a role in the assessment of hospital outcomes. The currently accepted classification system is based solely upon intraoperative surgeon opinion and not objective data. Inconsistent surgeon grading of the severity of appendicitis may have implications in both management and outcomes. OBJECTIVE: The aim of this study was to assess the interobserver variation among surgeons in grading of the inflammatory severity of acute appendicitis as recognized on visual findings at operation. METHODS: A cross-sectional study design. 110 surgeons, and surgical residents were randomly selected. Surgeons were shown images of intraoperative appendicitis and were asked to evaluate the severity of the appendicitis (i.e., normal, inflamed, gangrenous, and perforated). Demographic information regarding the type of practice, hospital setting, and the number of encounters with patients with acute appendicitis were assessed. An Intraclass Correlation Coefficient score, represented by R, was calculated to assess interobserver reliability in grading the inflammatory severity of acute appendicitis. The two-way analysis of variance procedure for multivariate analysis was used for this calculation. RESULTS: The study group consisted of 100 surgeons, 62 practicing surgeons, and 48 surgical trainees. Overall, 79% of the surgeons treated predominantly adults with appendicitis, 18% treated primarily children, and 3% treated both children and adults. Hospital practices included university hospitals (47%), community hospitals (33%), children's hospitals (14%), and others (6%). Overall, there was poor agreement among surgeons in assessing the severity of appendicitis. Among all attending surgeons, the agreement of defining an image as to whether it was perforated or not was 27% (R4 = 0.27). Completion of a general surgery residency did improve the interobserver agreement, when compared with trainees. CONCLUSION: There is poor agreement among surgeons in describing the severity of appendicitis. Treatment protocols based on more accurate assessment and categorization could potentially lead to more favorable, cost-effective outcomes. Further, studies determining efficacy in the diagnosis and treatment of appendicitis should consider observer variability. Future work must attempt to define critical objective assessment points, such as visible discontinuity of the appendix or fecal soilage, to assure a better correlation of findings with prognosis.

Pediatric surgery workforce: population and economic issues.

BACKGROUND: Whether a shortage of pediatric surgeons exists in the United States, such as those observed in the total physician and general surgical workforces, is an important issue that will affect decisions regarding training, credentialing, and reimbursement. Our goal was to update information regarding the demand and supply of pediatric surgeons. METHODS: Online American Pediatric Surgical Association (APSA) membership directory gave numbers of pediatric surgeons and their residence by metropolitan statistical areas (MSA), defined by the US census. Population and economic data were obtained from appropriate US government agencies. RESULTS: There were 835 APSA members and 375 MSA. Eliminated were 86 MSA (with 12 APSA members) with incomplete data, 14 MSA (0 members) with populations less than 100,000, and 25 members with listed locations outside an MSA. The remaining 798 members and 275 MSA comprised the study. The number of APSA members in an MSA correlated closely with MSA population (R(2) = 0.836) and 2006 births (R(2) = 0.767). Metropolitan statistical areas without an APSA member had a smaller population and birth rate than those with one or more members (P = .0001). An MSA with 1 APSA member had a higher population (P = .0003) and births per APSA member ratios (P = .0014) than MSA with 2 and 3 or more members. The presence of a medical school or a pediatric training program had no effect on population or births-to-APSA member ratios. There was no correlation between numbers of APSA members and state GDP or state GDP per capita. We used a low, medium, and high threshold to predict the need for pediatric surgeons based upon population per APSA member +/- 1 SD (272,466 +/- 163,386) to predict a need of 82 to 1344 pediatric surgeons, an increase in the APSA membership by 10% to 168%. CONCLUSION: Based on population estimates and APSA membership, a current shortage of pediatric surgeons exists. Measures should be taken to address this workforce issue.

Incidence of postoperative hyponatremia and complications in critically-ill children treated with hypotonic and normotonic solutions.

OBJECTIVE: To determine the incidence and clinical consequences of postoperative hyponatremia in children. STUDY DESIGN: We performed a retrospective analysis of postoperative admissions to the pediatric intensive care unit (excluding cardiac, neurosurgical, and renal). The incidence of severe (serum sodium < 125 mmol/L or symptoms) and moderate (serum sodium < 130 mmol/L) hyponatremia in children receiving hypotonic (HT) and normotonic (NT) fluids was calculated. RESULTS: Out of a total of 145 children (568 sodium measurements; 116 HT and 29 NT), we identified 16 with hyponatremia (11%). The incidences of moderate (10.3% vs 3.4%, P = .258) and severe (2.6% vs 0%; P = .881) hyponatremia were not significantly different in the HT and NT groups. There were no neurologic sequelae or deaths related to hyponatremia. CONCLUSIONS: In our study group, hyponatremia was common, but morbidity and death were not observed. Careful monitoring of serum sodium level may be responsible for this lack of adverse outcomes. Larger, prospective studies are needed to determine whether the incidence of hyponatremia differs between the HT and NT groups.

Current management of appendicitis.

Appendicitis is the most common surgical disease of the abdomen in children. Pediatric appendicitis varies considerably in its clinical presentation, contributing to delay in diagnosis and increased morbidity. The methods of diagnosis and treatment of appendicitis also vary significantly among clinicians and medical centers according to the patient's clinical status, the medical center's capabilities, and the physician's experience and technical expertise. Recent trends include the increased use of radiologic imaging, minimally invasive and nonoperative treatments, shorter hospital stays, and home antibiotic therapy. Little consensus exists regarding many aspects of the care of the child with complicated appendicitis. This article examines the most debated aspects of the diagnosis and management of the diseased pediatric appendix.

Seminars: controversies in the management of pediatric thyroid malignancy.

Thyroid cancer in children is a rare malignancy with unusual biological behavior. It often presents at advanced stages, yet behaves in a benign manner, when compared to its adult counterpart. Surgeons have debated the risks and benefits of aggressive surgical procedures for over a decade. A consensus treatment plan for childhood thyroid cancer has not been achieved, however radical surgical procedures have, in general, fallen out of favor. The best operative and adjuvant strategy for these children can only be determined when a better understanding of the tumor cell biology and genetics of this cancer is known. This review discusses the current controversies in the management of differentiated childhood thyroid malignancy.

Intensified platinum therapy is an ineffective strategy for improving outcome in pediatric patients with advanced hepatoblastoma.

PURPOSE: The INT-0098 Intergroup Liver Tumor Study demonstrated no statistically significant differences in event-free and overall survival between patients randomized to treatment with either cisplatin + fluorouracil + vincristine (C5V) or cisplatin + doxorubicin. Results from this and other therapeutic trials suggested that cisplatin was the most active agent against hepatoblastoma. To increase the platinum dose-intensity, a novel regimen was developed alternating carboplatin and cisplatin (CC) every 2 weeks. The P9645 study was designed to compare the risk of treatment failure for patients with stage III/IV hepatoblastoma randomized to either C5V or CC. METHODS: C5V was given according to INT-0098 and CC consisted of carboplatin at 700 mg/m2 on day 0 (560 mg/m2 after two cycles) followed by cisplatin 100 mg/m2 on day 14. Granulocyte colony-stimulating factor was used after each CC cycle. All patients received four to six cycles of chemotherapy. RESULTS: From the time the study was opened until the time that random assignment was halted, 56 patients received CC and 53 patients received C5V. The 1-year event-free survival was 37% for patients receiving CC and 57% for those receiving C5V (P = .017). Patients randomly assigned to CC required more blood product support. As a result of a semiannual review by the Children's Oncology Group Data and Safety Monitoring Committee, random assignment was discontinued after 3 years of enrollment because the projected improvement in long-term outcome associated with CC was statistically excluded as a possible outcome of this trial. CONCLUSION: Intensification of therapy by alternating platinum analogs increased the risk of adverse outcome in children with unresectable or metastatic hepatoblastoma.

Transforming a payer-provider relationship from conflict to collaboration.

The current relationship between payers and providers is often adversarial. The growth of managed care has fostered very little cooperation between the payer and provider, even though both are working toward improved patient outcomes. This article describes the process by which a payer and provider were able to transform a contentious relationship into a collaborative patient-centered program. The program demonstrates that through the sharing of retrospective utilization data, improved clinical and financial outcomes can be achieved.

Analysis of head injury admission trends in an urban American pediatric trauma center.

BACKGROUND: Head injury is the leading cause of death in children. Child safety legislation and risk-specific intervention programs have flourished to mitigate the incidence of injury to children. This analysis documents the trend in head injury to children in a specific institution. METHODS: Analysis of 5,003 head injury admissions to a pediatric trauma center over thirteen years was performed. Admission rates were calculated using the appropriate population denominator from census data. Poisson regression analysis was applied to estimate the relative risk of head injury admission by year in different age, sex, mechanism of injury and severity group. RESULTS: There has been a 70% decline in the head injury admission rate since 1989, consistent with regional and national data. The decline was present in all mechanisms of injury and age groups except for less than 1 year of age. The decline in total trauma admissions over the same time period was 50% and the decline in total hospital admission was 10%. CONCLUSION: Pediatric head injury has significantly declined in the last 13 years at a Level I pediatric trauma center.

Hospital- and patient-level characteristics and the risk of appendiceal rupture and negative appendectomy in children.

CONTEXT: The rates of appendiceal rupture and negative appendectomy in children remain high despite efforts to reduce them. Both outcomes are used as measures of hospital quality. Little is known about the factors that influence these rates. OBJECTIVE: To investigate the association between hospital- and patient-level characteristics and the rates of appendiceal rupture and negative appendectomy in children. DESIGN, SETTING, AND PATIENTS: Retrospective review using the Pediatric Health Information System database containing information on 24,411 appendectomies performed on children aged 5 to 17 years at 36 pediatric hospitals in the United States between 1997 and 2002. MAIN OUTCOME MEASURES: Rates of negative appendectomy and appendiceal rupture; the odds ratio (OR) of negative appendectomy and appendiceal rupture by hospital, patient age, race, and health insurance status, and hospital fiscal year and appendectomy volume. Negative appendectomy rate was defined as the number of patients with appendectomy but without appendicitis divided by the total number of appendectomies. RESULTS: The median negative appendectomy rate was 3.06% (range, 1%-12%) and the median appendiceal rupture rate was 35.08% (range, 22%-62%). The adjusted OR for appendiceal rupture was higher in Asian children (1.66; 95% confidence interval [CI], 1.24-2.23) and black children (1.13; 95% CI, 1.01-1.30) compared with white children. Children without health insurance and children with public insurance had increased odds of appendiceal rupture compared with children who had private health insurance (adjusted OR, 1.36; 95% CI, 1.22-1.53 for self-insured; adjusted OR, 1.48; 95% CI, 1.34-1.64 for public insurance). No correlation existed between negative appendectomy rate and race, health insurance status, or hospital appendiceal rupture rate. The negative appendectomy rate improved as the hospital appendectomy volume increased. CONCLUSION: The rate of appendiceal rupture in school-aged children was associated with race and health insurance status and not with negative appendectomy rate and therefore is more likely to be associated with prehospitalization factors such as access to care, quality of care, and patient or physician education.

Fibrolamellar hepatocellular carcinoma in children and adolescents.

BACKGROUND: Children with hepatocellular carcinoma (HCC) were treated on a prospective, randomized trial and were then analyzed to determine whether children with the fibrolamellar (FL) histologic variant of HCC have a more favorable presentation, increased surgical resectability, greater response to therapy, and improved outcome compared with children who have typical HCC. METHODS: Forty-six patients were enrolled on Pediatric Intergroup Hepatoma Protocol INT-0098 (Pediatric Oncology Group Study 8945/Children's Cancer Group Study 8881) between August 1989 and December 1992. After undergoing initial surgery or biopsy, children with Stage I HCC (n = 8 patients), Stage III HCC (n = 25 patients), and Stage IV HCC (n = 13 patients) were assigned randomly, regardless of histology, to receive treatment either with cisplatin, vincristine, and fluorouracil (n = 20 patients) or with cisplatin and continuous-infusion doxorubicin (n = 26 patients). RESULTS: Ten of 46 patients (22%) had the fibrolamellar variant of HCC (FL-HCC). For the entire cohort, the estimated 5-year event free survival (EFS) rate (+/- standard deviation) was 17% +/- 6%. There was no difference in outcome among patients who were treated with either regimen. The 5-year EFS rate for patients with FL-HCC was no different the rate for patients with typical HCC (30% +/- 15% vs. 14% +/- 6%, respectively; P = 0.18), although the median survival was longer in patients with FL-HCC. There was no difference in the number of patients with advanced-stage disease, the incidence of surgical resectability at diagnosis, or the response to treatment between patients with FL-HCC and patients with typical HCC. CONCLUSIONS: Children with FL-HCC do not have a favorable prognosis and do not respond any differently to current therapeutic regimens than patients with typical HCC. Children with initially resectable HCC have a good prognosis irrespective of histologic subtype, whereas outcomes are poor uniformly for children with advanced-stage disease. The use of novel chemotherapeutic agents and the incorporation of other treatment modalities are indicated to improve the dismal survival of pediatric patients with all histologic variants of advanced-stage HCC.

Appendicitis 2000: variability in practice, outcomes, and resource utilization at thirty pediatric hospitals.

BACKGROUND/PURPOSE: To improve clinical results and resource utilization in the care of appendicitis in children, the authors examined the current practice and outcomes of 30 pediatric hospitals. METHODS: The Pediatric Health Information System (PHIS) database consists of comparative data from 30 free-standing Children's hospitals. The study population of 3,393 children was derived from the database by selecting the "Diagnosis Related Group Code" for appendicitis (APRDRGv12 164), ages 0 to 17 years, using discharges between October 1, 1999 and September 30, 2000. Data are expressed as the range and median for individual hospital outcomes. RESULTS: The nonpositive appendectomy rate ranged from 0 to 17% at the 30 hospitals (median, 2.6%). Ruptured appendicitis varied from 20% to 76% (median, 36.5%). The median length of stay (LOS) for nonruptured appendicitis was 2 days (range, 1.4 to 3.1 days), ruptured appendicitis varied from 4.4 to 11 days (median, 6 days). The median readmission rate within 14 days was 4.3% (0 to 10%). Laparoscopic appendectomy varied from 0 to 95% in the 30 hospitals (mean, 31%) The LOS did not vary significantly in laparoscopic versus open for nonruptured (2.3 v 2.0 days) or ruptured appendicitis (5.5 v 6.2 days). Days on antibiotics for ruptured appendicitis ranged from 4.6 to 7.9 days (median, 5.9 days) Children receiving any study varied from 18% to 89% (median, 69%). Ultrasound scan and computed tomography (CT) were comparable in both nonruptured (13% ultrasound scan v 14%) and ruptured appendicitis (14% ultrasound scan v 21% CT). CONCLUSIONS: Significant variability in practice patterns and resource utilization exists in the management of acute appendicitis in pediatric hospitals. Clinical outcomes could be improved by collaborative initiatives to adopt evidence-based best practices.