scholarly journals Lost bones: differential diagnosis of acro-osteolysis seen by the pediatric rheumatologist

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Elizaveta Limenis ◽  
Jennifer Stimec ◽  
Peter Kannu ◽  
Ronald M. Laxer
Keyword(s):  
Differential Diagnosis ◽  
Pediatric Population ◽  
Genetic Disorders ◽  
Spinal Dysraphism ◽  
Radiographic Finding ◽  
Hypertrophic Osteoarthropathy ◽  
Local Trauma ◽  
Key Terms ◽  
Year 2000 ◽  
Distinguishing Features

Abstract Introduction Acro-osteolysis is a radiographic finding which refers to bone resorption of the distal phalanges. Acro-osteolysis is associated with various conditions and its presence should prompt the clinician to search for the underlying etiology. The aim of this review is to discuss disorders with which acro-osteolysis is associated and their distinguishing features, with a focus on the pediatric population. Methods A targeted literature review was performed using the term “acro-osteolysis” in combination with other key terms. The primary search results were supplemented using reference citations. Articles published prior to the year 2000 were included if they described additional associations not encountered in the more recent literature. Results Genetic disorders (particularly primary hypertrophic osteoarthropathy and skeletal dysplasias) and rheumatic diseases (particularly psoriatic arthritis and systemic sclerosis) are the most frequently encountered conditions associated with acro-osteolysis in children. Hyperparathyroidism, neuropathy, local trauma and thermal injury, and spinal dysraphism should also be included in the differential diagnosis. Conclusion Although acro-osteolysis is uncommon, its presence should prompt the clinician to consider a differential diagnosis based on clinical and radiographic features.

scholarly journals Paranasal Mass in a Healthy Male Toddler

2021 ◽  
pp. 014556132110079
Author(s):  
Melonie Anne Phillips ◽  
Meredith Lind ◽  
Gerd McGwire ◽  
Diana Rodriguez ◽  
Suzanna Logan
Keyword(s):  
Differential Diagnosis ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Age Group ◽  
Significant Morbidity ◽  
Healthy Male ◽  
Neck Tumors ◽  
Complete Surgical Resection ◽  
Maxilla And Mandible ◽  
Benign Mass

Head and neck tumors are rare in pediatric patients but should be kept in the differential when a patient presents with a new swelling or mass. One of these tumors is a myxoma, which is an insidiously growing, benign mass originating from the mesenchyme. They most commonly arise in the myocardium but can also develop in facial structures, particularly in the maxilla and mandible. When arising in facial structures, ocular, respiratory, and digestive systems can be affected based on local invasion. Complete surgical resection is curative but can lead to significant morbidity as well. Here, we present a case of a 15-month-old toddler presenting with a paranasal mass, which was ultimately diagnosed as a maxillary myxoma. This tumor is very rare in the pediatric population, especially in the toddler age-group, reminding clinicians to broaden the differential diagnosis when a patient’s course is atypical.

scholarly journals The spectrum and prevalence of genetic pathology among children and adolescents of the northern districts of kharkiv region

2019 ◽  
Keyword(s):  
Children And Adolescents ◽  
Human Genetics ◽  
Pediatric Population ◽  
Genetic Disorders ◽  
Single Gene ◽  
Modern Human ◽  
Chromosomal Disorders ◽  
Healthcare Facilities ◽  
Single Gene Disorders ◽  
Epidemiological Characteristics

The spectrum and prevalence of genetic pathology among the population of a certain region are determined by the founder effect and microevolution factors and, therefore, are not always comparable in different countries. The study of these indicators is an important trend of modern human genetics. The purpose of the research was to study genetic and epidemiological characteristics of the pediatric population of two northern districts of the Kharkiv region, Ukraine: Bogodukhiv and Vovchansk. Total number of children aged 0–17 was 6896 in Bogodukhiv district, and 7891 in Vovchansk district on 01/01/2016. The medical records of 307 patients were analyzed in healthcare facilities of both districts and the city of Kharkiv. The subject of the study was the cases of single-gene and chromosomal diseases. The burden of genetic disorders among children and adolescents was 0.30% in both districts. The prevalence of single-gene diseases in these districts was 0.24% in Bogodukhiv district and 0.25% in Vovchansk district. There were 9 and 12 single-gene disorders with different modes of inheritance, respectively. Only two of them were common in the districts: congenital hypothyroidism and sensorineural hearing loss. The incidence of the latter is 1:985 in Bogodukhiv district and 1:1578 in Vovchansk district. Chromosomal pathology was detected in 0.06% of the patients in Bogodukhiv district and 0.05% in Vovchansk district. Down syndrome was the only nosological form of chromosomal disorders in both districts. For other five areas of Kharkiv region, the prevalence of genetic pathology ranges from 0.36% in Izyum district to 0.47% in Balakliia and Blyzniuky as have been previously reported. The incidence of single-gene disorders is 0.27% in Izyum and 0.39% in Blyzniuky, while the incidence of chromosomal disorders varies from 0.07% in Zmiiv to 0.13% in Krasnohrad. Thus, the spectrum and prevalence of single-gene and chromosomal pathology in Bogodukhiv and Vovchansk districts correspond to those in other districts of Kharkiv region and most European countries.

Ivy sign: a diagnostic and prognostic biomarker for pediatric moyamoya

2021 ◽  
pp. 1-9
Keyword(s):  
Blood Flow ◽  
Cerebral Artery ◽  
Pediatric Patients ◽  
Clinical Decision Making ◽  
Prognostic Biomarker ◽  
Pediatric Population ◽  
Radiographic Finding ◽  
Clinical Decision ◽  
Stage Iii ◽  
Postoperative Stroke

OBJECTIVE Ivy sign is a radiographic finding on FLAIR MRI sequences and is associated with slow cortical blood flow in moyamoya. Limited data exist on the utility of the ivy sign as a diagnostic and prognostic tool in pediatric patients, particularly outside of Asian populations. The authors aimed to investigate a modified grading scale with which to characterize the prevalence and extent of the ivy sign in children with moyamoya and evaluate its efficacy as a biomarker in predicting postoperative outcomes, including stroke risk. METHODS Pre- and postoperative clinical and radiographic data of all pediatric patients (21 years of age or younger) who underwent surgery for moyamoya disease or moyamoya syndrome at two major tertiary referral centers in the US and Israel, between July 2009 and August 2019, were retrospectively reviewed. Ivy sign scores were correlated to Suzuki stage, Matsushima grade, and postoperative stroke rate to quantify the diagnostic and prognostic utility of ivy sign. RESULTS A total of 171 hemispheres in 107 patients were included. The median age at the time of surgery was 9 years (range 3 months–21 years). The ivy sign was most frequently encountered in association with Suzuki stage III or IV disease in all vascular territories, including the anterior cerebral artery (53.7%), middle cerebral artery (56.3%), and posterior cerebral artery (47.5%) territories. Following surgical revascularization, 85% of hemispheres with Matsushima grade A demonstrated a concomitant, statistically significant reduction in ivy sign scores (OR 5.3, 95% CI 1.4–20.0; p = 0.013). Postoperatively, revascularized hemispheres that exhibited ivy sign score decreases had significantly lower rates of postoperative stroke (3.4%) compared with hemispheres that demonstrated no reversal of the ivy sign (16.1%) (OR 5.5, 95% CI 1.5–21.0; p = 0.008). CONCLUSIONS This is the largest study to date that focuses on the role of the ivy sign in pediatric moyamoya. These data demonstrate that the ivy sign was present in approximately half the pediatric patients with moyamoya with Suzuki stage III or IV disease, when blood flow was most unstable. The authors found that reversal of the ivy sign provided both radiographic and clinical utility as a prognostic biomarker postoperatively, given the statistically significant association with both better Matsushima grades and a fivefold reduction in postoperative stroke rates. These findings can help inform clinical decision-making, and they have particular value in the pediatric population, as the ability to minimize additional radiographic evaluations and tailor radiographic surveillance is requisite.

Introduction to Cultural Psychology

2020 ◽  
pp. 1-37
Author(s):  
Robyn M. Holmes
Keyword(s):  
Cross Cultural Psychology ◽  
Cultural Psychology ◽  
Real Life ◽  
Cultural Relativism ◽  
Indigenous Psychology ◽  
Individualism And Collectivism ◽  
Key Terms ◽  
Distinguishing Features

Chapter 1 explores the field of cultural psychology, the concept of culture, why we should study culture, and other disciplines that study culture. It provides definitions and distinguishing features for the terms culture, nationality, and ethnicity. It discusses the fields that study culture including cross-cultural psychology, cultural psychology, indigenous psychology, anthropology, cultural studies, and sociology. It explains ways to think about culture and constructs such as cultural universal and culture-specific, emics and etics, ethnocentrism and cultural relativism, and individualism and collectivism. It examines why we should study culture and the applied value of cultural psychology in real-life settings such as school, the workplace, and clinical contexts. This chapter includes a case study, Culture Across Disciplines box, chapter summary, key terms, a What Do Other Disciplines Do? section, thought-provoking questions, and class and experiential activities.

scholarly journals Intradural Lipoma at the Craniocervical Junction Presenting with Progressing Hemiparesis: A Case Report

2019 ◽  
Vol 11 (2) ◽  
pp. 183-188
Author(s):  
Takuro Inoue ◽  
Hisao Hirai ◽  
Ayako Shima ◽  
Fumio Suzuki ◽  
Masayuki Matsuda ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Pediatric Population ◽  
Adult Population ◽  
Spinal Dysraphism ◽  
Craniocervical Junction ◽  
Suboccipital Craniotomy ◽  
Benign Nature ◽  
Intradural Lipoma ◽  
Radiological Studies ◽  
Dorsal Medulla

Intradural spinal lipomas are rare in an adult population. They are mostly asymptomatic and usually associated with spinal dysraphism in a pediatric population. We report a rare case of spinal lipoma without dysraphism and with progressing hemiparesis. A 60-year-old woman had incidental lipoma at the craniocervical junction observed for more than 5 years. Recently, she developed right-sided hemiparesis and sensory disturbance. Radiological studies revealed a large lipoma compressing the dorsal medulla and C1–C2 spinal cord. Standard midline suboccipital craniotomy and C1 laminectomy were performed, and the lipoma was removed subtotally. The lipoma showed severe adhesion to the dorsal medulla and C1 spinal cord; therefore, the excision was limited as internal debulking. Her neurological deficit subsided within 6 months after the decompressive surgery. Considering the benign nature of lipoma, internal decompression is a reasonable management for this lesion.

Isolated cerebellar Rosai-Dorfman granuloma mimicking Lhermitte-Duclos disease

2009 ◽  
Vol 4 (2) ◽  
pp. 118-120 ◽  
Author(s):  
James M. Johnston ◽  
David D. Limbrick ◽  
Wilson Z. Ray ◽  
Stephanie Brown ◽  
Joshua Shimony ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Histological Examination ◽  
Pediatric Population ◽  
Cerebellar Lesion ◽  
Presumptive Diagnosis ◽  
Suboccipital Craniotomy ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder that rarely involves the CNS. Rosai-Dorfman disease is exceedingly rare in the pediatric population and has never been observed in the cerebellum of a child. The authors present the case of a 14-year-old male with a cerebellar lesion having radiographic characteristics of Lhermitte-Duclos disease. After a period of observation with a presumptive diagnosis of Lhermitte-Duclos disease, the child underwent suboccipital craniotomy and resection of the lesion due to continuous suboccipital headaches. Histological examination of the tissue demonstrated RDD. The published literature on RDD is reviewed with an emphasis on differential diagnosis.

scholarly journals Unilateral Hemiparesis with Thoracic Epidural in an Adolescent

2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Rosalie F. Tassone ◽  
Christian Seefelder ◽  
Navil F. Sethna
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Epidural Anesthesia ◽  
Pediatric Population ◽  
Motor Blockade ◽  
Neural Blockade ◽  
Epidural Catheter Placement ◽  
Thoracic Epidural ◽  
Thoracic Epidural Catheter ◽  
Correct Location

Objective. Unilateral sensory and motor blockade is known to occur with epidural anesthesia but is rarely reported in children. The differential diagnosis should include the presence of a midline epidural septum.Case Report. We describe a case of a 16-year-old adolescent who developed repeated complete unilateral extensive epidural sensory and motor blockade with Horner’s syndrome after thoracic epidural catheter placement. This unusual presentation of complete hemibody neural blockade has not been reported in the pediatric population. Maneuvers to improve contralateral uniform neural blockade were unsuccessful. An epidurogram was performed to ascertain the correct location of the catheter within the epidural space and presence of sagittal compartmentalization.Conclusion. This case report highlights a less frequently reported reason for unilateral sensory and motor blockade with epidural anesthesia in children. The presence of a midline epidural septum should be considered in the differential diagnosis of unilateral epidural blockade.

scholarly journals Schwannoma Localized Retroperitoneally in a 14-Year-Old Boy

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Hasan Cayirli ◽  
Halil Ibrahim Tanriverdi ◽  
Ali Aykan Ozguven ◽  
Cuneyt Gunsar ◽  
Betul Ersoy ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Adrenal Gland ◽  
Adrenal Mass ◽  
Pediatric Population ◽  
Pediatric Case ◽  
Adrenal Gland Tumors ◽  
Retroperitoneal Schwannoma

Schwannomas usually occur in adults being between the second and fifth decades, and such neoplasms are extremely rare in a pediatric population. In addition, they are not normally found in the retroperitoneal region. Here, we present a pediatric case of a retroperitoneal schwannoma in an adrenal location where the tumor was not able to be preoperatively differentiated from other benign or malign adrenal gland tumors. In our opinion, this tumor can be included in the differential diagnosis of a nonfunctioning retroperitoneal adrenal mass in children.

scholarly journals Diagnostic Approach to Macrocephaly in Children

2022 ◽  
Vol 9 ◽  
Author(s):  
Andrea Accogli ◽  
Ana Filipa Geraldo ◽  
Gianluca Piccolo ◽  
Antonella Riva ◽  
Marcello Scala ◽  
...  
Keyword(s):  
Pediatric Population ◽  
Genetic Disorders ◽  
Correct Diagnosis ◽  
Clinical Aspects ◽  
Healthy Population ◽  
Pathological Conditions ◽  
Large Head ◽  
Subarachnoid Spaces ◽  
The Mean ◽  
Size Evaluation

Macrocephaly affects up to 5% of the pediatric population and is defined as an abnormally large head with an occipitofrontal circumference (OFC) >2 standard deviations (SD) above the mean for a given age and sex. Taking into account that about 2–3% of the healthy population has an OFC between 2 and 3 SD, macrocephaly is considered as “clinically relevant” when OFC is above 3 SD. This implies the urgent need for a diagnostic workflow to use in the clinical setting to dissect the several causes of increased OFC, from the benign form of familial macrocephaly and the Benign enlargement of subarachnoid spaces (BESS) to many pathological conditions, including genetic disorders. Moreover, macrocephaly should be differentiated by megalencephaly (MEG), which refers exclusively to brain overgrowth, exceeding twice the SD (3SD—“clinically relevant” megalencephaly). While macrocephaly can be isolated and benign or may be the first indication of an underlying congenital, genetic, or acquired disorder, megalencephaly is most likely due to a genetic cause. Apart from the head size evaluation, a detailed family and personal history, neuroimaging, and a careful clinical evaluation are crucial to reach the correct diagnosis. In this review, we seek to underline the clinical aspects of macrocephaly and megalencephaly, emphasizing the main differential diagnosis with a major focus on common genetic disorders. We thus provide a clinico-radiological algorithm to guide pediatricians in the assessment of children with macrocephaly.

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