Congenital hemihypertrophy and pheochromocytoma,not a coincidental combination?

We describe a 19-year-old female, known to have congenital hemihypertrophy, who presented with bilateral benign pheochromocytoma. This is the second time that this combination has been reported in the literature. We speculate that the combination of congenital hemihypertrophy and pheochromocytoma is not coincidental and could be part of the clinical spectrum of the Beckwith-Wiedemann syndrome. CONCLUSION: in patients with congenital hemihypertrophy, the physician should be aware of the symptoms of pheochromocytoma. Besides screening for abdominal tumours, analysis of plasma and/or urinary catecholamines and/or their metabolites should be considered.     

Pharmacodynamics and pharmacokinetics of esmolol,a short-acting β-blocking agent,in children

Summary Esmolol, a short-acting intravenous cardioselective -blocking agent, was evaluated for age-dependent pharmacodynamic and pharmacokinetic features in 17 young patients (6 months to 14 years). A loading dose (500 g/kg/min) alternating with a maintenance dose (25–200 g/kg/min, titrating by 25 g/kg/min every 4 min) was infused until the heart rate or mean arterial pressure decreased 10%. Cardiac index, left ventricular shortening fraction, and systemic vascular resistance were measured at baseline, peak esmolol effect, and recovery. Serum esmolol concentrations were obtained to determine the half-life and the elimination rate constant.Esmolol reduced the heart rate, blood pressure, shortening fraction, and cardiac index in all patients, but it did not change systemic vascular resistance. Maintenance esmolol dose was 118 ±49 g/kg/min, and the half-life was 2.88±2.67 min. Blood pressure and heart rate returned to normal within 2–16 min, but cardiac index and shortening fraction took longer to recover. There were no statistically significant age-dependent pharmacodynamic effects, but blood pressure decreased prior to heart rate and cardiac index took longer to recovery in patients who weighed15kg. The pharmacokinetic profile in young patients was similar to that of older patients, but the half-life was shorter. The only side effeect was transient nausea and vomiting in one patient. Esmolol is a safe and efficacious -blocking agent in young patients.     

Portal hypertension after combined liver and intestinal transplantation,a diagnostic and therapeutic challenge?

Monbaliu D, Vandersmissen J, De Hertogh G, Van Assche G, Hoffman I, Knops N, Debbaut C, Heye S, Pirenne J, Maleux G. Portal hypertension after combined liver and intestinal transplantation, a diagnostic and therapeutic challenge?
Pediatr Transplantation 2012: 00: 000–000. © 2012 John Wiley & Sons A/S. Abstract: A widely accepted technique to transplant the liver‐bowel bloc is first to perform a piggyback anastomosis of the donor suprahepatic vena cava to the recipient vena cava; second to restore the arterial blood supply through an aortic interposition graft; and third to ensure venous drainage of the native foregut. The venous drainage of the native foregut can be restored through an end‐to‐end portocaval anastomosis between the donor infrahepatic vena cava and the recipient portal vein. Stenosis of this anastomosis can lead to portal hypertension presenting with upper GI congestion, bleeding, and hypersplenism. We report the successful treatment of this complication using an e‐PTFE‐covered stent inserted following balloon angioplasty.     

Is urorectal septum malformation sequence a variant of the vertebral defects,anal atresia,tracheo-oesophageal fistula,renal defects and radial dysplasia association? Report of a case and a review of the literature

The urorectal septum malformation sequence (URSMS) consists of multiple systems anomalies including ambiguous genitalia, the absence of a perineal opening, an imperforate anus, and urogenital, colonic and lumbosacral anomalies. We describe a 3-day-old female infant with characteristic URSMS and review its clinical manifestations, outcomes and putative pathogenesis. We also compare its characteristics with those of the vertebral defects, anal atresia, tracheo-oesophageal fistula, renal defects and radial dysplasia (VATER) association. Conclusion:Although defects of the urorectal septum malformation sequence and the vertebral defects, anal atresia, tracheo-oesophageal fistula, renal defects and radial dysplasia association overlap, we believe that they are separate entities. Differentiating the urorectal septum malformation sequence from vertebral defects, anal atresia, tracheo-oesophageal fistula, renal defects and radial dysplasia association is helpful to develop appropriate clinical investigations and search for the aetiology and pathogenesis of these diseases.     

Mycoplasma pneumoniae, splenic infarct, and transient antiphospholipid antibodies: a new association?

We report 2 children with Mycoplasma pneumoniae pulmonary infection with splenic infarcts and transient antiphospholipid antibodies. This association has not been reported previously.     

Hospital paediatricians’ workflow interruptions,performance, and care quality: a unit-based controlled intervention

Objective

Frequent workflow interruptions jeopardise clinicians’ efficiency and quality of clinical care. We sought to determine the effect of a documentation-assistant intervention. Our hypothesis was that the expected decrease of workflow interruptions enhances paediatricians’ performance and simultaneously improves patients’ perceived quality of care.

Methods

This was a controlled intervention study with data collected before and after the intervention at a University Children’s Hospital. For the intervention, a documentation assistant was assigned to an inpatient ward. The main outcome measures were workflow interruptions, paediatricians’ performance, as well as patients’ perceived quality of care. Workflow interruptions were assessed via standardised expert observations. Paediatricians’ evaluated their performance in terms of productivity, quality, and efficiency. Additionally, standardised patients’ reports on perceived quality of care were collected.

Results

For paediatricians in the intervention ward, workflow interruptions decreased significantly from 5.2 to 3.1 disruption events per working hour (decrease in the control unit was from 3.8 to 3.1). Furthermore, paediatricians reported at follow-up significantly enhanced productivity, quality, and efficiency. Similarly, patients’ ratings of care quality improved significantly over time. In multivariate analyses, we found substantial changes attributable to the intervention: for all three outcomes, we found a significant interaction effect of the intervention over study time.

Conclusions

The intervention streamlined paediatricians’ workflow, improved day-to-day functioning of the ward, and enhanced organisational efficiency and delivery of paediatric care. Future studies should investigate potential influences between the reduction of workflow interruptions, paediatricians’ perceived performance, and patient-related outcomes in quality and efficiency of paediatric care.     

Can you hear me now? Tools for cultivating a culture of respect,value, and appreciation within pediatric hematology,oncology, and cellular therapy

The American Society of Pediatric Hematology/Oncology (ASPHO) conducted a workshop “Can you hear me now? Cultivating a culture of respect, value, and appreciation within pediatric hematology/oncology” at their annual meeting in May 2022 in hopes of exploring how the members can enhance wellness in a climate of increasing diversity. Initiatives in the past have focused on personal care, but it has been widely shown that administrative and institutional driven initiatives are essential to create an environment of wellness. In this interactive workshop, we discovered that 22% of participants felt their institution does not instill a culture of respect. We offered tools to the audience on multiple levels: graceful self-promotion, diversity and inclusion, and leadership perspective on creating a culture of respect to address the individual, local community, and top-down leadership approaches. Here, we offer a summary on the content of the workshop, and expand upon many of the discussion points that were raised during the workshop. We bring forth novel information on each topic individually from diverse points of view, specific to the field of pediatric hematology/oncology (PHO). We aim to highlight the importance of creating a diverse and respectful work environment in PHO in hopes of ensuring motivated, satisfied, and fulfilled healthcare providers who feel appreciated and valued.     

Adolescent immunizations and other clinical preventive services: a needle and a hook?

Advances in technology have led to development of new vaccines for adolescents, but these vaccines will be added to a crowded schedule of recommended adolescent clinical preventive services. We reviewed adolescent clinical preventive health care guidelines and patterns of adolescent clinical preventive service delivery and assessed how new adolescent vaccines might affect health care visits and the delivery of other clinical preventive services. Our analysis suggests that new adolescent immunization recommendations are likely to improve adolescent health, both as a "needle" and a "hook." As a needle, the immunization will enhance an adolescent's health by preventing vaccine-preventable diseases during adolescence and adulthood. It also will likely be a hook to bring adolescents (and their parents) into the clinic for adolescent health care visits, during which other clinical preventive services can be provided. We also speculate that new adolescent immunization recommendations might increase the proportion and quality of other clinical preventive services delivered during health care visits. The factor most likely to diminish the positive influence of immunizations on delivery of other clinical preventive services is the additional visit time required for vaccine counseling and administration. Immunizations may "crowd out" delivery of other clinical preventive services during visits or reduce the quality of the clinical preventive service delivery. Complementary strategies to mitigate these effects might include prioritizing clinical preventive services with a strong evidence base for effectiveness, spreading clinical preventive services out over several visits, and withholding selected clinical preventive services during a visit if the prevention activity is effectively covered at the community level. Studies are needed to evaluate the effect of new immunizations on adolescent preventive health care visits, delivery of clinical preventive services, and health outcomes.     

Gastroschisis reduction using “Applied Alexis”, a wound protector and retractor

We used an Applied Alexis (APPLIED MEDICAL RESOURCES CORP, USA) wound protector and retractor (WPAR) instead of a “spring loaded silo” (SLS) for gastroschisis reduction because SLS is currently unavailable in Japan. Our patient was a 1,707 g female diagnosed prenatally with gastroschisis born at 35 weeks gestation by spontaneous vaginal delivery. Attempted primary closure failed and a WPAR was used as a silo. A piece of the bottom ring of the WPAR was cut out and a smaller ring was made from it, so it would fit into the patient’s abdomen. Eviscerated organs were inserted into the retractor through the bottom ring, and the bottom ring was placed into the abdominal cavity through the gastroschisis defect without suturing to complete the silo. Operating time was 20 minutes. Three days later, all eviscerated organs had been reduced into the peritoneal cavity, the silo was removed, and the gastroschisis defect closed without difficulty. Postoperative recovery was uneventful and the WPAR was an excellent alternative for making a silo.     

Rash, fever, and neck pain in the office: is this a neurologic emergency?

Anticonvulsants, neuroleptics, and antispasticity agents are used with increasing frequency in the pediatric population. Each of the drugs discussed in this article has serious but potentially reversible adverse effects. Pediatric primary care providers must be aware of the potential emergencies associated with the use of these neurologic medications to provide prompt and effective treatment.     

Rectal biopsy for Hirschsprung’s disease: a review of techniques,pathology, and complications

Background

Hirschsprung’s disease (HD) is one of the most common congenital anomalies of colorectal function, affecting approximately 1 in 5000 live births, with a 4:1 male predominance. HD is characterized by aganglionosis that is most often limited to the rectosigmoid, but can extend proximally along the colon and, in rare instances, reach into the small intestine. A clinical history of delayed passage of meconium beyond 48 hours after birth, physical exam findings of abdominal distention and vomiting, and a contrast enema demonstrating a transition zone are highly suggestive of HD.

Data sources

We searched databases including PubMed, Google Scholar, and Scopus for the following key words: Hirschsprung’s disease, rectal biopsy, pathology, ganglion cell, nerve trunk hypertrophy, pediatric constipation, and selected publications written in English that were relevant to the scope of this review.

Results

Based on the data presented in the literature, we reviewed 1) biopsy techniques for the diagnosis of Hirschsprung’s disease, addressed inadequate biopsies, and complications from rectal biopsy, and 2) pathologic and histologic interpretation of biopsy specimens for the diagnosis of Hirschsprung’s disease.

Conclusion

A well-executed rectal biopsy with expert pathologic evaluation of the specimen remains the gold standard for the diagnosis of Hirschsprung’s disease and is the subject of this review.