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).

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.

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.

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.

Hepatocellular carcinoma in children and adolescents: results from the Pediatric Oncology Group and the Children's Cancer Group intergroup study.

PURPOSE: To determine surgical resectability, event-free survival (EFS), and toxicity in children with hepatocellular carcinoma (HCC) randomized to treatment with either cisplatin (CDDP), vincristine, and fluorouracil (regimen A) or CDDP and continuous-infusion doxorubicin (regimen B). PATIENTS AND METHODS: Forty-six patients were enrolled onto Pediatric Intergroup Hepatoma Protocol INT-0098 (Pediatric Oncology Group (POG) 8945/Children's Cancer Group (CCG) 8881). After initial surgery or biopsy, children with stage I (n = 8), stage III (n = 25), and stage IV (n = 13) HCC were randomly assigned to receive regimen A (n = 20) or regimen B (n = 26). RESULTS: For the entire cohort, the 5-year EFS estimate was 19% (SD = 6%). Patients with stage I, III, and IV had 5-year EFS estimates of 88% (SD = 12%), 8% (SD = 5%), and 0%, respectively. Five-year EFS estimates were 20% (SD = 9%) and 19% (SD = 8%) for patients on regimens A and B, respectively (P =.78), with a relative risk of 1.2 (95% confidence interval, 0.60 to 2.3) for regimen B when compared with regimen A. Outcome was similar for either regimen within disease stages. Events occurred before postinduction surgery I in 18 (47%) of 38 patients with stage III or IV disease, and tumor resection was possible in two (10%) of the remaining 20 children with advanced-stage disease after chemotherapy. CONCLUSION: Children with initially resectable HCC have a good prognosis and may benefit from the use of adjuvant chemotherapy. Outcome was uniformly poor for children with advanced-stage disease treated with either regimen. New therapeutic strategies are needed for the treatment of advanced-stage pediatric HCC.

The diagnosis and management of endocrine tumors causing hypertension in children.

In contrast to that in adults, hypertension in children is frequently amenable to surgical therapy. With advancing techniques in imaging, surgery, and anesthesia, the outcomes of surgery are excellent for children with endocrine tumors causing hypertension.

Using a pediatric database to drive quality improvement.

To survive in today's competitive market, children's hospitals must make informed decisions on cost reduction and resource optimization, while maintaining the highest quality of care. The Pediatric Health Information System (PHIS) is designed to assist children's hospitals and physicians in making these types of decisions. PHIS is a detailed comparative database that gives participating hospitals and clinicians the ability to assess and improve the resources required for patient care. PHIS data are used to compare hospital performance against clinical guidelines for the purpose of identifying opportunities that can lead to quality improvement and development of clinical benchmarks among peers. There is broad interest in the use of guidelines for clinical benchmarking because of rising health care costs, increasing demand for care, and more expensive technologies. PHIS continues to be an invaluable tool in assisting hospitals in their everyday efforts to improve efficacy and performance.

Guiding practice improvements in pediatric surgery using multidisciplinary clinical pathways.

Clinical pathways guide the evaluation and care of patient populations and can advance the strategic initiatives of the organization as well. To meet external and internal demands, this organization developed pathways to reduce variability in practice, integrate available evidence into care delivery, improve care coordination and documentation, increase efficiencies, and provide outcomes data to consumers and payers. The authors highlight processes and outcomes of some of the organization's surgical pathways, including guidelines for children with second degree burns grafted with TransCyte, those children hospitalized for tonsillectomy or adenoidectomy, appendectomies, atrial and ventricular septal defects, and percutaneous endoscopic gastrostomy or surgical gastrostomy procedures.

Laparoscopy is useful in the diagnosis and management of foramen of Morgagni hernia in children.

Foramen of Morgagni hernia is a rare form of congenital diaphragmatic hernia. We report the use of laparoscopy to diagnose and manage suspected foramen of Morgagni hernias in children. Cardiophrenic masses were seen on chest radiography in two asymptomatic children aged 1 and 3 years. Further radiologic workup was nondiagnostic. Laparoscopy was performed in both children, and the foramen of Morgagni hernias were immediately identified. Laparoscopic repair was performed on one of the children. Both children had uneventful postoperative courses, were discharged home early, and had no evidence of recurrence at the 6-month follow-up examination. Laparoscopy is a safe and effective technique to diagnose and potentially repair foramen of Morgagni hernias. We recommend the use of this modality as the primary diagnostic evaluation in infants and children with suspected foramen of Morgagni hernias.

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.

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.

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.

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.

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.