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Cyclic neutropenia has been rarely associated with chronic inflammation and development of reactive AA amyloidosis. We report a family with cyclic neutropenia with associated renal and thyroid amyloid. A 12-year-old female presented with thyromegaly, recurrent aphthous ulcers, severe neutropenia, and renal failure. Renal and thyroid biopsies revealed abundant amyloid deposition. Presence of a heterozygous ELANE c.358 A&gt;T gene mutation p.I120F variant with autosomal dominant inheritance confirmed the diagnosis of cyclic neutropenia. The patient's father also had neutropenia and amyloidosis with renal failure. We started filgrastim to attenuate neutropenia and thereby reduce chronic inflammation and minimize further amyloid deposition.Glucose-6-phosphatase catalytic subunit 3 (G6PC3) deficiency is a recently identified form of congenital neutropenia associated with developmental anomalies. The severity of neutropenia and the clinical spectrum are highly variable. Aside from infectious complications and extrahematologic features, inflammatory bowel disease and autoinflammatory complications are less frequently observed manifestations. [https://www.selleckchem.com/products/shin1-rz-2994.html RZ-2994] However, amyloidosis has never been reported in G6PC3 deficiency. Here, we present a 12-year-old patient with incidentally discovered neutropenia because of the p.E65A (c.194A&gt;C) variant of the G6PC3 gene. He had recurrent aphthae and abdominal pain episodes, and developed nephrotic-range proteinuria, amyloidosis, and end-stage renal failure during follow-up.<br /><br /> Hypertension (HTN) is a complication of pediatric hematopoietic stem cell transplantation. We report a pediatric stem cell transplant patient who had HTN and adverse event because of amlodipine.<br /><br /> Seven-year-old boy had haploidentical stem cell transplantation with post-transplant cyclophosphamide. He had complete donor chimerism at the end of one month. Amlodipine was started on day +36 for HTN. On day +41, he had petechiae. Platelet function analyzer (PFA)-100 was abnormal. After amlodipine was stopped, petechiae disappeared and PFA-100 returned to normal.<br /><br /> Abnormal PFA-100 and the patient complaints indicated the possibility of amlodipine-induced platelet dysfunction through inhibition of calcium-mediated platelet reactions.<br /><br />Abnormal PFA-100 and the patient complaints indicated the possibility of amlodipine-induced platelet dysfunction through inhibition of calcium-mediated platelet reactions.The severe acute respiratory syndrome coronavirus (SARS-CoV-2) pandemic has disrupted normal health care utilization patterns worldwide, including decreasing emergency department (ED) visits for various medical emergencies. We examined whether this pattern was present in febrile pediatric oncology patients. In this single-center cohort study, we conducted a retrospective chart review of ED visits of febrile pediatric oncology patients during the first 4 months of the global SARS-CoV-2 pandemic and compared those data to the same time periods in the previous 2 years. During the first 5 months of the pandemic, 25 pediatric oncology patients with fever visited our ED; 65 children visited during the same time period in 2018; and 60 visited in 2019. Compared with 2018 and 2019, encounters for 2020 were decreased by 62% and 58%, respectively. A significantly higher percentage of febrile pediatric oncology patients (84%) were admitted to our hospital during the pandemic compared the previous years (58%). Of concern is the possibility that fear of exposure to coronavirus disease-19 (COVID-19) at our health care facility prompted caregivers of pediatric oncology patients to avoid seeking care for their child with fever. Consistent communication with families about the life-threatening nature of fever should be prioritized among pediatric oncology providers.Pancreatic angiosarcoma is an exceedingly rare malignancy accounting for less then 1% of pancreatic neoplasms. A very limited number of pancreatic angiosarcomas have been reported in the literature without any cases described in children. We present the case of a 17-year-old female diagnosed with angiosarcoma of the pancreas following pancreaticoduodenectomy for a pancreatic mass, initially presumed to be a solid pseudopapillary neoplasm of the pancreas. The angiosarcoma was found to have a novel activating internal tandem duplication in the KDR gene (KDR-internal tandem duplication). We discuss the current literature on this disease process. This is the first reported case of pancreatic angiosarcoma in a pediatric patient and the first with an activating KDR-internal tandem duplication.<br /><br /> A prospective multicenter observational study.<br /><br /> To elucidate the efficacy of transcranial motor-evoked potentials (Tc(E)-MEPs) in degenerative cervical myelopathy (DCM) surgery by comparing cervical spondylotic myelopathy (CSM) to cervical ossification of the posterior longitudinal ligament (OPLL) and investigate the timing of Tc(E)-MEPs alerts and types of interventions affecting surgical outcomes.<br /><br /> Although CSM and OPLL are the most commonly encountered diseases of DCM, the benefits of Tc(E)-MEPs for DCM remain unclear and comparisons of these two diseases have not yet been conducted.<br /><br /> We examined the results of Tc(E)-MEPs from 1176 DCM cases (840 CSM /336 OPLL) and compared patients background by disease, preoperative motor deficits, and the type of surgical procedure. We also assessed the efficacy of interventions based on Tc(E)-MEPs alerts. Tc(E)-MEPs alerts were defined as an amplitude reduction of more than 70% below the control waveform. Rescue cases were defined as those in which waveform recovete interventions in response to alerts. The detection of acute onset segmental palsy by Tc(E)-MEPs was partially possible with OPLL, but may still be difficult with CSM. The rescue rate was higher than 50% and appropriate interventions may have prevented postoperative neurological complications.Level of Evidence 3.<br /><br /> Retrospective, randomized, radiographic study assessing age-related changes (ARCs) on lumbar magnetic resonance imaging (MRI) using an ensemble method.<br /><br /> This study proposed to develop a novel reporting method to calculate a predicted "age estimate" for the ARC seen on lumbar MRI.<br /><br /> Lumbar MRI reports include pathological findings but usually not the prevalence data of common findings which has been shown to decrease the need for narcotics in the management of non-specific lower back pain (NSLBP). Comparing the normal age estimation for lumbar spine degenerative changes/ARC on MRI and comparing this to the patient's real age may improve patient outcome in the management of NSLBP.<br /><br /> A total of 60 lumbar MRI were taken from patients aged between 0 and 100 years. Lumbar MRI features reported as associated with age on review of the literature were measured on each MRI and statistically evaluated for correlation with age. Factors found to be associated were then entered into an ensemble model consisting of several machine learning techniques.