Simultaneous Acquired Self-limited Hemophagocytic Lymphohistiocytosis and Kikuchi Necrotizing Lymphadenitis in a 16-Year-Old Teenage Girl: A Case Report and Review of the Literature.

OBJECTIVES: The aim of this study was to increase education and awareness among pediatric practitioners of possibility of simultaneous hemophagocytic lymphohistiocytosis and Kikuchi-Fujimoto disease/Kikuchi disease occurring in the pediatric population and the diagnostic dilemma it can present. We describe a case presentation of acquired and self-limited simultaneous hemophagocytic lymphohistiocytosis and Kikuchi-Fujimoto disease in a 16-year-old in the United States who presented with fevers, night sweats, and joint pain, along with tiredness and decreased appetite along with pancytopenia and elevated lactate dehydrogenase. To the best of our knowledge, simultaneous hemophagocytic lymphohistiocytosis and Kikuchi-Fujimoto in the pediatric population has not been described in North America but remains fairly common in Asia. The literature on both diseases and their simultaneous occurrence is comprehensively reviewed. METHODS: This was a case report and review of the literature. RESULTS: The patient was diagnosed with both hemophagocytic lymphohistiocytosis and Kikuchi-Fujimoto disease based on bone marrow aspiration/biopsy and axillary node biopsy, respectively. Both illnesses resolved completely. CONCLUSIONS: Benign causes of pancytopenia and elevated lactate dehydrogenase exist, but they may not be always straightforward diagnostically. Bone marrow aspiration and lymph node biopsy may be helpful in ascertaining the diagnosis. Hemophagocytic lymphohistiocytosis and Kikuchi-Fujimoto disease may represent a continuum of illness.

Urosepsis and postrenal acute renal failure in a neonate following circumcision with Plastibell device.

Plastibell is one of the three most common devices used for neonatal circumcision in the United States, with a complication rate as low as 1.8%. The Plastibell circumcision device is commonly used under local anesthesia for religious circumcision in male neonates, because of cosmetic reasons and ease of use. Occasionally, instead of falling off, the device may get buried under the skin along the shaft of the penis, thereby obstructing the normal flow of urine. Furthermore, the foreskin of neonates is highly vascularized, and hence, hemorrhage and infection are possible when the skin is cut. Necrosis of penile skin, followed by urethral obstruction and renal failure, is a serious surgical mishap requiring immediate corrective surgery and medical attention. We report a case of fulminant urosepsis, acute renal failure, and pyelonephritis in a 4-day-old male neonate secondary to impaction of a Plastibell circumcision device. Immediate medical management was initiated with fluid resuscitation and mechanical ventilation; thereby correcting life threatening complications. Pediatricians and Emergency Department physicians should be cognizant of the complications from Plastibell circumcision device in order to institute appropriate and timely management in neonates.

Impact of nurse integrated rounds on self-reported comprehension, attitudes, and practices of nurses and resident physicians in a pediatric intensive care unit.

OBJECTIVES: To evaluate the impact of nurse integrated rounds (NIRs) on self-reported comprehension, attitudes, and practices of nurses and resident physicians (RPs) in a pediatric intensive care unit (PICU). MATERIALS AND METHODS: A self-reported comprehension, attitude, and practice survey of RPs and nurses was done prior to (T0), 3 months (T3), and 15 months (T15) after initiation of NIRs in our PICU. Responses were graded on Likert-type scale from 1 to 5. The RPs, attending physicians, and nurses also ranked their overall perception of NIRs during these 3 survey time periods. RESULTS: All 3 components of the surveys showed statistically significant improvement (P < .05) from the T0 to T3 and T15 in RPs and nurses. A complete or almost complete reversal of attitude was noted for most questions in the attitude section in both RPs and nurses when T15 was compared to T0. The overall perception that NIRs was good for patient care also showed significant improvement in the survey of nurses and physicians. CONCLUSIONS: The NIRs are well accepted by nurses and physicians and are accompanied by self-reported improvements in comprehension, attitudes, and practices of nurses and RPs in the PICU.

Early repair of large infant ventricular septal defect despite respiratory syncytial virus-induced respiratory failure with postrepair chylous pericardial effusion requiring pleuropericardial window: a case report and review of the literature.

The surgical correction of congenital cardiac lesions that are complicated by intercurrent respiratory syncytial virus (RSV) pneumonitis has traditionally been deferred for at least 6 to 8 weeks. The presumption is that using cardiopulmonary bypass will increase the risk of postoperative pulmonary complications. We present an infant who developed acute respiratory failure related to RSV pneumonitis and required urgent mechanical ventilation. Cardiac evaluation revealed a large nonrestrictive ventricular septal defect (VSD), aortic arch hypoplasia, normally functioning bicuspid aortic valve, and hemodynamic instability associated with markedly increased pulmonary blood flow. Separation from mechanical ventilation was unsuccessful preoperatively. He underwent VSD repair with cardiopulmonary bypass less than 4 weeks after initial RSV infection. He was extubated successfully within 72 hours of VSD repair. Approximately 6 weeks postoperatively, he developed a circumferential chylous pericardial effusion of unclear etiology--an exceedingly rare complication of VSD repair in early infancy in a non-Down syndrome patient. The chylous effusion was initially managed unsuccessfully with Portogen/Monogen and a percutaneously placed pericardial drain. Two weeks later, he underwent creation of a pleuropericardial window with successful resolution of the chylous effusion. It is of interest to pediatricians to be able to correctly time the repair of congenital heart disease lesions after RSV infection to minimize post-bypass pulmonary complications and yet avoid morbidity from undue delays in repair. In addition, chylopericardium can occur in infants after VSD repair, and dietary modification and catheter drainage may not be adequate.

Asymptomatic complete atrioventricular block in a 13-year-old girl.

Atrioventricular (AV) block is a delay or an interruption in the transmission of an impulse from atria to ventricles due to an anatomic or a functional impairment in the conduction system. Atrioventricular block may be congenital or acquired. Electrocardiographic screening of asymptomatic school-aged children (median, 12.4 years) in Japan found the prevalence of a third-degree AV block to be 2 per 100,000. We report a case of asymptomatic complete AV block of unknown etiology in a 13-year-old child who did not require pacemaker placement. The importance of recognizing an asymptomatic complete AV block in the pediatric population, the classification and controversies of pacemaker placement, and the complications of pacemaker placement are discussed.

Posterior dislocation of sternoclavicular joint encroaching on ipsilateral vessels in 2 pediatric patients.

Dislocation of the sternoclavicular joint (DSCJ) with posterior displacement of the clavicle is uncommon in children. This can lead to ipsilateral compression of the great vessels. Diagnosis may not be apparent on routine radiographs. Axial computed tomographic (CT) scan is the imaging of choice for diagnosis. Reconstruction and 3-dimensional (3D) views on CT scan may be helpful to define the anatomy before surgical reduction. We present 2 patients who had traumatic posterior DSCJ with compromise to their vascular structures. Patient 1 was a 13-year-old boy with posterior DSCJ on his left side that was not apparent on x-ray and that was diagnosed to have encroachment of the medial end of the clavicle on the left brachiocephalic vein on CT scan. Reconstruction and 3D views on CT scan were helpful in defining the anatomy before surgical reduction. The patient underwent emergent open reduction and internal fixation with complete recovery.Patient 2 was a 14-year-old boy with posterior DSCJ on the right side that was apparent on x-ray and was evaluated to have encroachment of the medial end of the clavicle on the right innominate artery with an associated paratracheal hematoma on CT scan. Reconstruction and 3D views on CT scan were also helpful in defining the anatomy before surgical reduction. The patient underwent a successful closed reduction with complete recovery.

Acute lymphoblastic leukemia with hyperleukocytosis, sinus pauses, and hypoxemic respiratory failure in an infant.

A 5-month-old female infant presented with new-onset acute lymphoblastic leukemia and hyperleukocytosis with white blood cell count of 352 × 10(9) cells/L. She developed sinus pauses and hypoxemic respiratory failure. A manual single volume exchange blood transfusion was done with complete resolution of sinus pauses and hypoxemia.

Ipsilateral lobar emphysema in an infant with a repaired congenital diaphragmatic hernia.

OBJECTIVE: To report a case of lobar emphysema in an infant who had a congenital diaphragmatic hernia that was surgically repaired at birth. DESIGN: Case report. SETTING: Pediatric critical care unit in a tertiary care hospital. PATIENT: A 9-month-old infant who had massive hyperinflation of the right lung and respiratory failure. INTERVENTIONS: Confirmation of lobar hyperinflation of the right lung with ventilation and perfusion defects and surgical treatment with lobectomy. MEASUREMENTS AND MAIN RESULTS: Successful treatment of respiratory failure. CONCLUSION: Lobar emphysema can be a rare development in patients who had a congenital diaphragmatic hernia at birth. This can result in respiratory failure and needs surgical intervention.

Small bowel obstruction secondary to strangulation through a defect in the falciform ligament after blunt abdominal trauma in a pediatric patient.

Obstruction caused by strangulation internal hernia secondary to incarceration within the falciform ligament, although rare, has been previously reported in the literature. These cases, however, were unrelated to trauma. We report on the first case in the pediatric literature of a strangulated internal hernia secondary to incarceration in the falciform ligament precipitated by blunt abdominal trauma. A 12-year-old girl presented to the emergency room less than 24 hours after sustaining a kick to the right upper quadrant. She described sharp, nonradiating, right-upper-quadrant abdominal pain, which was associated with nausea and vomiting. A KUB (kidney, ureter, bladder) view showed a paucity of bowel in the right upper quadrant with distended adjacent bowel. An ultrasound showed a small amount of abdominal ascites and a prominent liver. Computed tomography scan revealed a linear hypodensity at the tip of the right lobe of the liver, suggestive of a laceration. Moderate abdominal and pelvic ascites and multiple collapsed small-bowel loops with diffuse wall thickening and poor enhancement were seen in the right upper quadrant. Significantly, pneumatosis was noted, raising the question of obstruction/volvulus and/or bowel ischemia. An exploratory laparotomy revealed incarcerated small bowel herniated into a defect in the falciform ligament, which was resected. The defect was repaired. Seemingly trivial trauma may play a precipitating role in strangulation in a patient who already has a defect in the falciform ligament.

Breathing patterns during vaso-occlusive crisis of sickle cell disease.

STUDY OBJECTIVES: To determine the effect of sickle cell pain and its treatment on patients' breathing patterns, and to compare the effect of thoracic cage pain to pain at other sites. DESIGN: Prospective, observational study. SETTING: Sickle Cell Center Day Hospital. PATIENTS: Twenty-five patients with sickle cell disease admitted to the Sickle Cell Center Day Hospital for treatment of vaso-occlusive crisis (VOC) [10 patients with chest (thoracic cage) pain]. INTERVENTIONS: Breathing patterns were measured by respiratory inductive plethysmography. Tidal breathing data, including respiratory rate, tidal volume (VT), minute ventilation, and the rib cage contribution to VT, were collected at baseline and then following treatment with opioid analgesia. MEASUREMENTS AND RESULTS: The patients with chest pain had smaller V(Ts) at baseline than those with pain at other sites (355 +/- 37 mL vs 508 +/- 141 mL, p = 0.003), and higher respiratory rates (23.2 +/- 8.2 breaths/min vs 17.6 breaths/min, p = 0.03). These differences became insignificant following opioid treatment. Six patients had respiratory alternans (four patients in the chest pain group, and two patients with pain at other sites). All cases of respiratory alternans resolved following opioid administration. CONCLUSIONS: Patients with VOC and chest pain have more shallow, rapid breathing than patients with pain elsewhere. Analgesia reduces these differences. As pain-associated shallow breathing and maldistribution of ventilation may contribute to the pathogenesis of acute chest syndrome, these results support the need for adequate pain relief and monitoring of ventilatory patterns during the treatment of VOC.