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1.
Pediatr Blood Cancer ; 71(6): e30945, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38462769

ABSTRACT

Consistent with studies showing a high prevalence of the Duffy null phenotype among healthy Black Americans, this retrospective study found that Duffy null was present in >75% of a young and contemporary cohort of children with sickle cell disease (SCD) in the United States. Despite the potential for this phenotype to impact absolute neutrophil counts, hydroxyurea (HU) dosing, and outcomes, it was not associated with being prescribed a lower HU dose or having increased acute SCD visits early in the HU treatment course. Future studies are needed to confirm these findings in older children with SCD.


Subject(s)
Anemia, Sickle Cell , Antisickling Agents , Duffy Blood-Group System , Hydroxyurea , Humans , Hydroxyurea/therapeutic use , Hydroxyurea/administration & dosage , Anemia, Sickle Cell/drug therapy , Anemia, Sickle Cell/epidemiology , Male , Female , Retrospective Studies , Child, Preschool , United States/epidemiology , Child , Duffy Blood-Group System/genetics , Prevalence , Antisickling Agents/therapeutic use , Infant , Receptors, Cell Surface/genetics , Adolescent
2.
J Extra Corpor Technol ; 55(4): 189-193, 2023 Dec.
Article in English | MEDLINE | ID: mdl-38099633

ABSTRACT

BACKGROUND: Highly sensitized pediatric patients awaiting heart transplantation experience longer wait times and thus higher waitlist mortality. Similarly, children less than 2 years of age have increased waitlist times and mortality when compared to their older peers. To improve the likelihood of successful transplantation in these patients, various strategies have been utilized, including peri-operative plasmapheresis. However, limited data exists comparing plasmapheresis techniques for antibody reduction. This study's aim was to compare the in vitro magnitude of isohemagglutinin titers (IT) and human leukocyte antigen (HLA) antibody removal and the time required between membrane-based plasmapheresis (MP) and centrifuge-based plasmapheresis (CP) incorporated into the extracorporeal (EC) circuit. METHODS: Two MP (Prismaflex) and two CP (Spectra Optia, Terumo BCT) circuits were incorporated into four separate EC circuits primed with high titer, highly sensitized type O donor whole blood. Assays were performed to determine baseline IT and anti-HLA antibodies and then at 30-minute increments until completion of the run (two plasma volume exchanges) at two hours. RESULTS: There was a decrease in anti-A and anti-B IgM and IgG titers with both MP and CP. Mean anti-A and anti-B titer reduction was by 4.625 titers (93.7% change) and 4.375 titers (93.8% change) using MP and CP, respectively. At 2 h of apheresis, CP reduced 62.5% of all ITs to ≤ 1:4, while MP reduced 50% of ITs to ≤ 1:4. Additionally, reduction of anti-HLA class II antibody to mean fluorescence intensity (MFI) <3000 was achieved with both MP and CP. At 2 h of apheresis, CP reduced MFI by 2-3.5 fold and MP reduced MFI by 1.7-2.5 fold. Both demonstrated similar hemolytic and thrombotic profiles. CONCLUSIONS: In this in vitro plasmapheresis model of IT and anti-HLA antibody reduction, both MP and CP incorporated into the EC circuit can be used quickly and effectively to reduce circulating antibodies. While CP may have some greater efficiency, further study is necessary to verify this in vivo.


Subject(s)
Heart Transplantation , Hemagglutinins , Humans , Child , HLA Antigens , Plasmapheresis , Heart Transplantation/methods , Graft Rejection/prevention & control
3.
Am J Clin Pathol ; 160(1): 72-77, 2023 07 05.
Article in English | MEDLINE | ID: mdl-37405408

ABSTRACT

OBJECTIVES: The aim of our investigation was to establish normal pediatric reference intervals (PRIs) for rotational thromboelastometry (ROTEM) Delta assays in a representative group of healthy children, 0 to 18 years of age, at our institution. METHODS: This was a prospective study of healthy pediatric patients undergoing elective minor surgery requiring placement of an intravenous cannula. The sample size for patients was 20 per age group of either sex from 5 different age groups based on coagulation system maturity: 0 to 6 or fewer months, more than 6 to 12 or fewer months, more than 1 year to 5 or fewer years, more than 5 to 11 or fewer years, and more than 11 to 18 or fewer years. ROTEM Delta assays assessed include the EXTEM, INTEM, and FIBTEM. RESULTS: We defined 2 sets of ROTEM PRIs for our patient population: one for patients 11 years or younger and one for children more than 11 years of age. For those 11 years or younger, the PRIs were derived from the 2.5th and 97.5th percentiles from the 0 to 11 age groups. For those older than 11 years, previously published adult reference intervals validated internally with adult normal samples were used. CONCLUSIONS: The 2 sets of PRIs were embedded into our electronic medical record, allowing clinicians to easily interpret their patient's ROTEM results against age-verified reference ranges, enabling them to make informed transfusion decisions.


Subject(s)
Blood Coagulation , Thrombelastography , Adult , Humans , Child , Infant , Reference Values , Prospective Studies , Blood Coagulation Tests/methods
4.
J Trauma Acute Care Surg ; 94(1S Suppl 1): S2-S10, 2023 01 01.
Article in English | MEDLINE | ID: mdl-36245074

ABSTRACT

ABSTRACT: Hemorrhagic shock in pediatric trauma patients remains a challenging yet preventable cause of death. There is little high-quality evidence available to guide specific aspects of hemorrhage control and specific resuscitation practices in this population. We sought to generate clinical recommendations, expert consensus, and good practice statements to aid providers in care for these difficult patients.The Pediatric Traumatic Hemorrhagic Shock Consensus Conference process included systematic reviews related to six subtopics and one consensus meeting. A panel of 16 consensus multidisciplinary committee members evaluated the literature related to 6 specific topics: (1) blood products and fluid resuscitation for hemostatic resuscitation, (2) utilization of prehospital blood products, (3) use of hemostatic adjuncts, (4) tourniquet use, (5) prehospital airway and blood pressure management, and (6) conventional coagulation tests or thromboelastography-guided resuscitation. A total of 21 recommendations are detailed in this article: 2 clinical recommendations, 14 expert consensus statements, and 5 good practice statements. The statement, the panel's voting outcome, and the rationale for each statement intend to give pediatric trauma providers the latest evidence and guidance to care for pediatric trauma patients experiencing hemorrhagic shock. With a broad multidisciplinary representation, the Pediatric Traumatic Hemorrhagic Shock Consensus Conference systematically evaluated the literature and developed clinical recommendations, expert consensus, and good practice statements concerning topics in traumatically injured pediatric patients with hemorrhagic shock.


Subject(s)
Hemostatics , Shock, Hemorrhagic , Child , Humans , Shock, Hemorrhagic/therapy , Resuscitation , Shock, Traumatic , Fluid Therapy
5.
J Trauma Acute Care Surg ; 94(1S Suppl 1): S41-S49, 2023 01 01.
Article in English | MEDLINE | ID: mdl-36221169

ABSTRACT

ABSTRACT: Transfusion of blood products to a hemorrhaging pediatric trauma patient requires seamless partnership and communication between trauma, emergency department, critical care, and transfusion team members. To avoid confusion and delays, understanding of blood banking principles and mutually agreed upon procedures and policies must be regularly updated as knowledge evolves. Because pediatric patients require specialized considerations distinct from those in adults, this brief review covers transfusion principles, policies, and procedures specific to the resuscitation of pediatric trauma patients.


Subject(s)
Blood Banking , Wounds and Injuries , Child , Humans , Blood Transfusion/methods , Emergency Service, Hospital , Hemorrhage/etiology , Hemorrhage/therapy , Wounds and Injuries/complications , Wounds and Injuries/therapy
6.
J Trauma Acute Care Surg ; 94(1S Suppl 1): S11-S18, 2023 01 01.
Article in English | MEDLINE | ID: mdl-36203242

ABSTRACT

BACKGROUND: Traumatic injury is the leading cause of death in children and adolescents. Hemorrhagic shock remains a common and preventable cause of death in the pediatric trauma patients. A paucity of high-quality evidence is available to guide specific aspects of hemorrhage control in this population. We sought to identify high-priority research topics for the care of pediatric trauma patients in hemorrhagic shock. METHODS: A panel of 16 consensus multidisciplinary committee members from the Pediatric Traumatic Hemorrhagic Shock Consensus Conference developed research priorities for addressing knowledge gaps in the care of injured children and adolescents in hemorrhagic shock. These ideas were informed by a systematic review of topics in this area and a discussion of these areas in the consensus conference. Research priorities were synthesized along themes and prioritized by anonymous voting. RESULTS: Eleven research priorities that warrant additional investigation were identified by the consensus committee. Areas of proposed study included well-designed clinical trials and evaluations, including increasing the speed and accuracy of identifying and treating hemorrhagic shock, defining the role of whole blood and tranexamic acid use, and assessment of the utility and appropriate use of viscoelastic techniques during early resuscitation. The committee recommended the need to standardize essential definitions, data elements, and data collection to facilitate research in this area. CONCLUSION: Research gaps remain in many areas related to the care of hemorrhagic shock after pediatric injury. Addressing these gaps is needed to develop improved evidence-based recommendations for the care of pediatric trauma patients in hemorrhagic shock.


Subject(s)
Shock, Hemorrhagic , Adolescent , Child , Humans , Shock, Hemorrhagic/etiology , Shock, Hemorrhagic/therapy , Resuscitation/methods , Shock, Traumatic , Research
7.
J Neurosurg Case Lessons ; 4(25)2022 Dec 19.
Article in English | MEDLINE | ID: mdl-36536526

ABSTRACT

BACKGROUND: Chondroblastomas (CBs) are rare benign bone tumors that are often difficult to treat because of their locations. CBs can be even more challenging to successfully manage when they present alongside aneurysmal bone cyst (ABC)-like changes. To minimize operative morbidity, especially in hard-to-reach lesions, percutaneous approaches for both lesions have been individually described. We present a skull base CB with associated ABC-like changes treated by combining two different previously described percutaneous modalities. OBSERVATIONS: The authors report successful percutaneous treatment of a skull base CB with adjacent ABC-like changes in a 17-year-old male. The CB was treated with radiofrequency ablation (RFA) and the adjacent ABC area with doxycycline sclerotherapy. After 3 years of follow-up, there has been no clinical or radiological evidence of recurrence. LESSONS: CBs occur in the skull base and, as elsewhere in the body, can be associated with ABC-like changes. Successful percutaneous treatment of such a CB with ABC-like changes is possible by combining previously described techniques of RFA and doxycycline sclerotherapy.

8.
J Pediatr Surg ; 56(6): 1120-1125, 2021 Jun.
Article in English | MEDLINE | ID: mdl-33745740

ABSTRACT

BACKGROUND/PURPOSE: Thyroid Imaging Reporting and Data System (TI-RADS) is validated in adults but not yet in children. The purpose of this study was to determine the sensitivity, specificity, and accuracy of TI-RADS in predicting thyroid malignancy for pediatric nodules, and to compare the diagnostic accuracy to the current American Thyroid Association (ATA) guidelines. METHODS: A single institution retrospective review was performed of patients younger than 21 years who underwent thyroid nodule fine needle aspiration biopsy (FNAB). Two radiologists were blinded to the pathology and independently classified all biopsied thyroid nodules based on TI-RADS. ATA and TI-RADS guidelines were analyzed to determine the diagnostic sensitivity and specificity of both scoring systems. RESULTS: 115 patients (median age 15.5 years, 90 females) with 138 nodules were scored using TI-RADS. There was moderate inter-rater agreement between radiologists (Kappa = 0.51; p < 0.0001). Evaluating several potential TI-RADS criteria, 23.2%-68.1% of nodules were recommended for FNAB, compared to 82.6% of nodules using ATA guidelines. Using TI-RADS ≥ 3 (without size cutoff) as an indication for FNAB had 100% sensitivity with no missed suspicious or malignant nodules on cytology or pathology. CONCLUSIONS: Using TI-RADS for diagnostic management of pediatric thyroid nodules improves accuracy in predicting malignancy.


Subject(s)
Thyroid Nodule , Adolescent , Adult , Biopsy, Fine-Needle , Child , Female , Humans , Retrospective Studies , Thyroid Nodule/diagnostic imaging , Ultrasonography , United States
9.
Clin Chem ; 67(10): 1432-1433, 2021 10 01.
Article in English | MEDLINE | ID: mdl-36103291
10.
J Pediatr Hematol Oncol ; 43(5): e702-e706, 2021 07 01.
Article in English | MEDLINE | ID: mdl-32941297

ABSTRACT

Adolescent and young adult (AYA) patients with Ewing sarcoma have inferior survival compared with pediatric patients even when treated with similar regimens. Investigation into specific explanations is lacking. A retrospective chart review of Ewing sarcoma patients at a single institution was performed, and 104 patients were identified, 45 were 15 to 39 years of age (AYA cohort) and 59 younger than 15 years (pediatric cohort). AYA patients demonstrated more metastatic disease (50% vs. 24%, P=0.009), peripheral tumor location (64% vs. 41%, P=0.025), percentage of male patients (76% vs. 51%; P=0.010), and tumor size ≥5 cm (93% vs. 70%, P=0.016) than pediatric patients. Five-year overall survival was 77.7% and 53.0% and event-free survival was 68.7% and 40.6% for pediatric versus AYA, respectively. Similar rates of toxicity and chemotherapeutic dose adjustments were demonstrated. In this cohort, increased AYA patient mortality appears to be related to disease characteristics rather than treatment-related differences.


Subject(s)
Sarcoma, Ewing/epidemiology , Adolescent , Adult , Age Factors , Female , Humans , Male , Prognosis , Retrospective Studies , Sarcoma, Ewing/diagnosis , Sarcoma, Ewing/therapy , Survival Analysis , Young Adult
11.
BMJ Case Rep ; 13(12)2020 Dec 13.
Article in English | MEDLINE | ID: mdl-33318272

ABSTRACT

Viscoelastic monitoring (VEM) tools, such as rotational thrombelastometry, have been used extensively to measure coagulopathy in adults but have received less attention in paediatric care. The presented case involves a 5-year-old boy who was brought to the emergency department after a motor vehicle collision with a Glasgow Coma Scale score of 6T and extensive injuries, including a subdural hematoma. VEM was used to monitor the patient's coagulopathy and to inform treatment measures by allowing real-time visualisation of the patient's coagulation status. VEM was additionally used to direct blood product replacement in preparation for neurosurgical intervention, and 4-factor prothrombin complex concentrate (PCC) was used to help reverse the coagulopathy. The patient underwent successful hemicraniectomy after improvement of his coagulopathy. In paediatrics, VEM and PCC are increasingly being used for post-trauma coagulopathy, and this case highlights their potential promise and the need for further research.


Subject(s)
Blood Coagulation Disorders/diagnosis , Blood Coagulation Disorders/drug therapy , Blood Coagulation Factors/therapeutic use , Brain Injuries, Traumatic/complications , Hematoma, Subdural/complications , Monitoring, Physiologic/methods , Accidents, Traffic , Blood Coagulation Disorders/etiology , Brain Edema/complications , Brain Edema/diagnosis , Brain Edema/surgery , Brain Injuries, Traumatic/diagnosis , Brain Injuries, Traumatic/surgery , Child, Preschool , Craniotomy , Hematoma, Subdural/diagnosis , Hematoma, Subdural/surgery , Humans , Male
12.
Pediatrics ; 145(4)2020 04.
Article in English | MEDLINE | ID: mdl-32217740

ABSTRACT

Idiopathic acute eosinophilic pneumonia is a rare and potentially life-threatening condition that is defined by bilateral pulmonary infiltrates and fever in the presence of pulmonary eosinophilia. It often presents acutely in previously healthy individuals and can be difficult to distinguish from infectious pneumonia. Although the exact etiology of idiopathic acute eosinophilic pneumonia remains unknown, an acute hypersensitivity reaction to an inhaled antigen is suggested, which is further supported by recent public health risks of vaping (electronic cigarette) use and the development of lung disease. In this case, a patient with a year-long history of vaping in conjunction with tetrahydrocannabinol cartridge use who was diagnosed with idiopathic acute eosinophilic pneumonia with associated bilateral hilar lymphadenopathy is described.


Subject(s)
Dronabinol/toxicity , Lymphadenopathy/etiology , Psychotropic Drugs/toxicity , Pulmonary Eosinophilia/etiology , Vaping/adverse effects , Adolescent , Adrenal Cortex Hormones/therapeutic use , Electronic Nicotine Delivery Systems , Female , Humans , Lymphadenopathy/diagnosis , Lymphadenopathy/drug therapy , Pulmonary Eosinophilia/diagnosis , Pulmonary Eosinophilia/diagnostic imaging , Pulmonary Eosinophilia/drug therapy
13.
Pediatr Crit Care Med ; 21(8): e475-e484, 2020 08.
Article in English | MEDLINE | ID: mdl-32195902

ABSTRACT

OBJECTIVE: To test the hypothesis that early RBC transfusion is associated with duration of organ dysfunction in critically ill septic children. DESIGN: Secondary analysis of a single-center prospective observational study. Multivariable negative binomial regression was used to determine relationships between RBC transfusion within 48 hours of sepsis onset and number of days in 14 with organ dysfunction, or with multiple organ dysfunction syndrome. SETTING: A PICU at a quaternary care children's hospital. PATIENTS: Children less than 18 years old with severe sepsis/septic shock by consensus criteria were included. Patients with RBC transfusion prior to sepsis onset and those on extracorporeal membrane oxygenation support within 48 hours of sepsis onset were excluded. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Ninety-four patients were included. Median age was 6 years (0-13 yr); 61% were male. Seventy-eight percentage had septic shock, and 41 (44%) were transfused RBC within 48 hours of sepsis onset (early RBC transfusion). On multivariable analyses, early RBC transfusion was independently associated with 44% greater organ dysfunction days (adjusted relative risk, 1.44 [1.04-2.]; p = 0.03), although risk differed by severity of illness (interaction p = 0.004) and by shock severity (interaction p = 0.04 for Vasoactive Inotrope Score and 0.03 for shock index). Relative risks for multiple organ dysfunction syndrome days varied by shock severity (interaction p = 0.008 for Vasoactive Inotrope Score and 0.01 for shock index). Risks associated with early RBC transfusion were highest for the children with the lowest shock severities. CONCLUSIONS: In agreement with previous studies, early RBC transfusion was independently associated with longer duration of organ dysfunction. Ours is among the first studies to document different transfusion-associated risks based on clinically available measures of shock severity, demonstrating greater transfusion-associated risks in children with less severe shock. Larger multicenter studies to verify these interaction effects are essential to plan much-needed RBC transfusion trials for critically ill septic children.


Subject(s)
Sepsis , Shock, Septic , Shock , Adolescent , Child , Critical Illness , Female , Humans , Male , Multiple Organ Failure/etiology , Sepsis/complications , Sepsis/therapy , Shock, Septic/therapy
14.
World Neurosurg X ; 5: 100061, 2020 Jan.
Article in English | MEDLINE | ID: mdl-31660538

ABSTRACT

BACKGROUND: A giant cell tumor (GCT) of bone is a benign, locally aggressive tumor that is often challenging to treat. When complete resection is not possible, curettage with or without adjuvants is the most common treatment. The high frequency of local recurrence and risk of injury to adjacent structures can limit this surgical approach, especially with skull and spine lesions. CASE DESCRIPTION: We report 2 cases of axial skeleton GCTs, 1 in the skull of a 58-year-old woman in whom operative management failed, who experienced local recurrence, and 1 in the cervical spine of an 8-year-old girl that grew extracompartmentally to surround her brachial plexus. Both patients were referred to us because of the surgically challenging nature of their tumors. After completion of the same percutaneous doxycycline sclerotherapy protocol previously described for aneurysmal bone cysts (ABCs), both patients were considered cured and were able to return to normal activities without loss of pretreatment function. After 4 and 10 years of follow-up, respectively, there has been no tumor recurrence in either patient. CONCLUSIONS: We successfully treated 2 patients with very challenging axial skeleton GCTs using a percutaneous doxycycline sclerotherapy protocol previously shown to have success with ABCs. We believe that this minimally invasive procedure should be considered a potential alternative treatment for GCTs, especially axial skeleton lesions, which may not be easily approached with standard surgical techniques.

15.
Lung ; 198(1): 157-161, 2020 02.
Article in English | MEDLINE | ID: mdl-31807920

ABSTRACT

The contribution of T-cells after lung transplant (LTx) remains controversial with no current consensus of their role concerning chronic lung allograft dysfunction. Using flow cytometry to assess T-cell subsets of bronchoalveolar lavage fluid (BALF) in 16 cystic fibrosis (CF) LTx recipients, we identified a decline in CD4+ T-cell frequency and an increase in CD8+ T-cell frequency in patients who developed severe bronchiolitis obliterans syndrome (BOS) (N = 10) when comparing baseline (6 months post-LTx) and follow-up (most recent bronchoscopy-clinical or surveillance per protocol). Comparing BOS to No BOS cohorts, significant differences were found in CD4+ T-cell frequency [17.4 (12.5, 28.2) vs 46.6 (44.4, 48.4), p = 0.003] and CD8+ T-cell frequency [65.6 (62.8, 75.3) vs 39.2 (32.2, 43.3), p = 0.014], respectively. The mean difference of the CD4:CD8 ratio was 0.87 units lower (95% CI - 1.44 to - 0.30, p = 0.006) than patients without BOS, while the median difference of the CD4:CD8 ratio was 0.92 units lower (95% CI - 1.83 to - 0.009, p = 0.048). Therefore, our results suggest that T-cell profiles measured through flow cytometry of BALF in the CF LTx population are associated with the development of severe BOS. Further work is needed in larger patient populations to validate our findings and to determine if this is useful for recipients who underwent LTx for other indications.


Subject(s)
Bronchiolitis Obliterans/immunology , Cystic Fibrosis/surgery , Lung Transplantation , Postoperative Complications/immunology , T-Lymphocyte Subsets/immunology , T-Lymphocytes/immunology , Adolescent , Adult , Bronchiolitis Obliterans/epidemiology , Bronchoalveolar Lavage Fluid/cytology , CD3 Complex/immunology , CD4-CD8 Ratio , CD4-Positive T-Lymphocytes/immunology , CD8-Positive T-Lymphocytes/immunology , Case-Control Studies , Female , Graft Rejection/prevention & control , Humans , Immunophenotyping , Immunosuppressive Agents/therapeutic use , Male , Postoperative Complications/epidemiology , Severity of Illness Index , Young Adult
16.
Biol Blood Marrow Transplant ; 26(3): 546-552, 2020 03.
Article in English | MEDLINE | ID: mdl-31756537

ABSTRACT

We compared the incidence of refractory thrombocytopenia (RT) and platelet transfusion requirements (PTR) in 35 children who developed veno-occlusive disease (VOD) with 35 matched control subjects who underwent hematopoietic stem cell transplant but did not develop VOD. RT developed in 100% of the VOD patients, at a median of 8 days before VOD diagnosis, as compared with 71.5% of the control group. VOD patients required more platelet transfusions than control subjects (median PTR, 6.9 mL/kg [range, .57 to 17.59] versus 3.57 mL/kg [range, 0 to 14.63], respectively) with a statistically significant difference (P < .0001). The number of days with platelet requirements was significantly higher for VOD patients as compared with control subjects (median 68% versus 39%, P =< .0001). The PTR peaked at ~12 mL/kg/day, 2 days before VOD diagnosis, whereas the PTR in the control population was 5 mL/kg/day. The positive predictive value of developing VOD was 88.9% (95% confidence interval, 66.5% to 97%) in patients who were given >7 mL/kg/day of platelets during the at-risk period of days +3 to +13 after transplant. For patients who received >8 mL/kg/day of platelets, the positive predictive value of developing VOD was 86.7% (95% confidence interval, 61.2% to 96.4%). There was no difference in the PTR in patients with mild to moderate VOD as compared with severe VOD; however, the PTR was higher in patients whose VOD did not resolve. The median daily PTR after the diagnosis of VOD in 17 patients who got defibrotide as compared with those who did not get defibrotide was 6.04 mL/kg and 5.72 mL/kg, respectively, but the difference was not statistically significant (P = .56). On univariate and multivariate analysis use of intravenous immunoglobulin was significantly associated with VOD (P = .0088) but was not significantly associated with fatal VOD. In conclusion, RT occurs in 100% of patients at a median of 8 days before VOD diagnosis. VOD should be suspected in any patient with RT after the exclusion of other causes of consumptive thrombocytopenia, especially if they require >7 mL/kg/day of platelets.


Subject(s)
Hematopoietic Stem Cell Transplantation , Hepatic Veno-Occlusive Disease , Thrombocytopenia , Child , Hematopoietic Stem Cell Transplantation/adverse effects , Hepatic Veno-Occlusive Disease/diagnosis , Hepatic Veno-Occlusive Disease/etiology , Humans , Polydeoxyribonucleotides , Thrombocytopenia/diagnosis , Thrombocytopenia/etiology , Transplantation Conditioning
17.
Arch Pathol Lab Med ; 143(10): 1259-1264, 2019 10.
Article in English | MEDLINE | ID: mdl-30969156

ABSTRACT

CONTEXT.­: Cystoisospora belli is an intracellular parasite associated with gastrointestinal disease in immunocompromised hosts. Although infection has been classically associated with intestinal disease, studies have identified Cystoisospora in the gallbladder of immunocompetent patients based on hematoxylin-eosin morphology. Recently, the identity of this histologic finding as Cystoisospora has been questioned based on negative results of nucleic acid studies. OBJECTIVE.­: To determine the prevalence of this histologic feature in pediatric patients, we retrospectively reviewed all cholecystectomy specimens from a pediatric hospital during a 24-month period. DESIGN.­: In 180 cholecystectomy specimens, we identified 11 cases (6.1%) with classical histologic features previously described to represent Cystoisospora organisms. To further investigate these structures, we retrieved tissue from paraffin-embedded blocks and performed electron microscopy. RESULTS.­: Ultrastructural examination identified ovoid perinuclear cytoplasmic structures composed of dense fibrillar aggregates rather than organisms. Patients with positive cases were similar in age to controls (positive cases: mean patient age 13.4 years [range, 2-23 years]; negative cases: mean patient age 14.7 years [range, 12 weeks-31 years]; P = .35). There was no significant association of this finding with cholelithiasis (54.5% versus 65.1%, P = .52), cholesterolosis (0% versus 22.5%, P = .12), acute cholecystitis (9.1% versus 10.1%, P > .99), or chronic cholecystitis (45.5% versus 66.3%, P = .20). CONCLUSIONS.­: To our knowledge, this is the first positive identification of these structures as cytoplasmic fibrillar aggregates rather than parasitic inclusions by ultrastructural examination, and the first study of this histologic finding in pediatric cholecystectomies.


Subject(s)
Gallbladder Diseases/diagnostic imaging , Inclusion Bodies/ultrastructure , Adolescent , Adult , Child , Child, Preschool , Cholecystectomy , Epithelium/diagnostic imaging , Gallbladder/diagnostic imaging , Humans , Immunocompromised Host , Infant , Retrospective Studies , Young Adult
18.
Pathol Res Pract ; 215(5): 880-884, 2019 May.
Article in English | MEDLINE | ID: mdl-30711197

ABSTRACT

Papillary thyroid carcinoma (PTC) is the most common differentiated thyroid cancer in children; and the follicular variant is the second most common variant after the classic subtype. The histological appearance of follicular variant of papillary thyroid cancer (FVPTC), can be mimicked by benign follicular nodules. Pediatric pathologists encountering such lesions with FVPTC-like appearance may err on diagnosing the benign lesions as malignant. In adult patients, several immunohistochemical markers have emerged recently as a useful adjunct to distinguish differentiated thyroid carcinomas from benign follicular lesions. We undertook an inter-institutional retrospective study to establish the diagnostic utility of immunohistochemical staining for HBME-1, Galectin-3 and CD56 in differentiating FVPTC from its benign mimics, follicular adenoma and adenomatoid nodules, in children. Our specific aim of the project was to define the sensitivity and specificity of the three antibodies in FVPTC. Based on institutional diagnoses, a total of 66 cases were obtained: 32 FVPTC and 34 benign follicular nodules that comprised of 23 follicular adenoma and 11 adenomatoid nodules. Five investigators, who were blinded to the original diagnoses, independently reviewed the slides following pre-determined criteria and semi-quantitatively scoring the immunohistochemical staining. The immunohistochemical staining revealed that a combination of positive HBME-1 and negative CD56 result gave 100% specificity and positive predictive value in distinguishing FVPTC from benign follicular nodules. However, the antibody combination suffered from a lower sensitivity (50%). We used a cutoff of 25% positivity of tumor cells in determining positivity of tumor cells to an antibody. In conclusion, our study found a very high specificity and strong positive predictive value for the combination of HBME-1 and CD56 immunohistochemical stains in distinguishing FVPTC from benign follicular lesions.


Subject(s)
Biomarkers, Tumor/analysis , CD56 Antigen/biosynthesis , Thyroid Cancer, Papillary/diagnosis , Thyroid Neoplasms/diagnosis , Adolescent , Biomarkers, Tumor/biosynthesis , CD56 Antigen/analysis , Child , Child, Preschool , Female , Humans , Immunohistochemistry/methods , Infant , Male , Retrospective Studies , Sensitivity and Specificity
19.
J Pediatr Hematol Oncol ; 41(4): e206-e209, 2019 May.
Article in English | MEDLINE | ID: mdl-30531598

ABSTRACT

Myoepithelial carcinomas (MC) represent aggressive tumors that occur in a myriad of ages and anatomic locations. The rarity and histologic similarity with other tumors make them difficult to diagnosis. We report an extremely rare case of a right ventricular outflow tract mass identified to be an intracardiac MC in a 4-month-old male infant. Pathology revealed an EWS-KLF15 translocation. Treatment included gross total resection and intensive chemotherapy. Recurrent cardiac mass with brain metastasis was seen 16 months after primary diagnosis. We describe the rarity of intracardiac MC in pediatric patients and the challenges encountered in the multimodal management of this patient.


Subject(s)
Heart Neoplasms/pathology , Myoepithelioma/pathology , Fatal Outcome , Heart Neoplasms/genetics , Heart Neoplasms/therapy , Humans , Infant , Kruppel-Like Transcription Factors/genetics , Male , Myoepithelioma/genetics , Myoepithelioma/therapy , Nuclear Proteins/genetics , Oncogene Fusion , RNA-Binding Protein EWS/genetics
20.
J Extra Corpor Technol ; 50(2): 94-98, 2018 06.
Article in English | MEDLINE | ID: mdl-29921987

ABSTRACT

Hemolysis is a known consequence of extracorporeal membrane oxygenation (ECMO) resulting from shear force within the different components of the extracorporeal circuit. The primary aim of this study was to evaluate the EOS PMP™ oxygenator for generation of plasma free hemoglobin (PfHg) over 24 hours at nominal operating range flow rates. The EOS ECMO™ (LivaNova, Inc.; formerly Sorin, Arvada, CO) is equipped with a plasma tight polymethylpentene (PMP) hollow fiber oxygenator. We hypothesized that PfHg generation would be elevated in circuits with higher flow rates, because of the significant pressure drop across the oxygenator according to manufacturer provided flow charts. Generated PfHg concentrations were compared with PfHg concentrations from blood not exposed to an ECMO circuit. The secondary aim was to evaluate circuit flow-rate-induced changes in platelet count and platelet function over 24 hours. Circuits contained a CentriMag® (St. Jude Medical, St. Paul, MN) blood pump and an EOS ECMO PMP™ oxygenator. Circuits in triplicate were run continuously for 24 hours at three flow rates [1, 3, and 5 liters per minute {LPM}]. PfHg was analyzed at baseline, 6, 12, 18, and 24 hours. Platelet count and function were measured at baseline and 24 hours. Concentrations of PfHg at baseline for circuits operating at 1, 3, and 5 LPM were 24.4 ± 4.0, 38.4 ± 28.6, and 26.7 ± 6.9 mg/dL, respectively. PfHg concentrations after 24 hours were statistically compared for the three flow rates using analysis of variance; PfHg concentrations at 1 LPM (181.4 ± 29.1 mg/dL), 3 LPM (145.9 ± 8.7 mg/dL), and 5 LPM (100.1 ± 111.3 mg/dL) circuits. The F-test was not statistically significant (p = .632), indicating that PfHg generation at 24 hours was similar among the three flow rates. Excessive hemolysis using PfHg levels in the EOS PMP™ membrane oxygenator was not observed.


Subject(s)
Extracorporeal Membrane Oxygenation , Hemoglobins , Oxygenators, Membrane , Extracorporeal Membrane Oxygenation/instrumentation , Extracorporeal Membrane Oxygenation/methods , Hemoglobins/analysis , Hemoglobins/chemistry , Hemoglobins/metabolism , Humans , Platelet Function Tests
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