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Pediatric Syndromes

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https://www.readbyqxmd.com/read/29458220/incidence-of-autoimmune-and-related-disorders-after-resolution-of-endogenous-cushing-syndrome-in-children
#1
Christina Tatsi, Meg Keil, Charalampos Lyssikatos, Elena Belyavskaya, Constantine A Stratakis, Maya B Lodish
Glucocorticoids are widely used for immunosuppression in autoimmune diseases. After the resolution of hypercortisolemia, the immune system recovers allowing for autoimmune diseases to manifest. Here we investigated the presence of autoimmune and related diseases that developed after cure of endogenous Cushing syndrome (CS) in children. We identified 129 children who were diagnosed and successfully treated for endogenous CS at the National Institutes of Health from 1997 until 2017, and who were followed for at least 6 months after treatment...
February 19, 2018: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/29457852/successful-management-of-living-donor-liver-transplantation-for-biliary-atresia-with-single-ventricle-physiology-from-peri-transplant-through-total-cavopulmonary-connection-a-case-report
#2
Yohei Yamada, Ken Hoshino, Takayuki Oyanagi, Ryohei Gatayama, Jun Maeda, Nobuyuki Katori, Yasushi Fuchimoto, Taizo Hibi, Masahiro Shinoda, Kentaro Matsubara, Hideaki Obara, Ryo Aeba, Yuko Kitagawa, Hiroyuki Yamagishi, Tatsuo Kuroda
Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8-month-old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology...
February 19, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29457021/difficult-renal-pathological-classification-in-a-case-of-pediatric-nephrotic-syndrome
#3
Hiroshi Yamaguchi, Atsutoshi Shiratori, Taku Nakagawa, Kyoko Kanda, Shigeo Hara, Norishige Yoshikawa, Ryojiro Tanaka
The underlying histopathology is very important in determining patient management, as the histopathology usually has direct repercussions on the treatment response and clinical course. However, the impact of the method used to assess renal biopsies, i.e., light microscopy (LM), immunofluorescence (IF), and electron microscopy (EM), on the occurrence of a difficult biopsy classification in the native kidneys of pediatric nephrotic patients is unknown. A 12-month-old Japanese boy was diagnosed with nephrotic syndrome (NS); he was administered prednisolone (60 mg/m2 /day), and a continuous albumin infusion was started...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29456932/neither-hereditary-periodic-fever-nor-periodic-fever-aphthae-pharingitis-adenitis-undifferentiated-periodic-fever-in-a-tertiary-pediatric-center
#4
Silvia De Pauli, Sara Lega, Serena Pastore, Domenico Leonardo Grasso, Anna Monica Rosaria Bianco, Giovanni Maria Severini, Alberto Tommasini, Andrea Taddio
AIM: To describe the frequency and clinical characteristics of patients with undifferentiated periodic fever (UPF) and to investigate whether a clinical classification of UPF based on the PRINTO-Eurofever score can help predicting the response to treatment and the outcome at follow-up. METHODS: Clinical and therapeutic information of patients with recurrent fever who presented at a single pediatric rheumatology center from January 2006 through April 2016 were retrospectively collected...
February 8, 2018: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/29456211/risk-factors-associated-with-acute-kidney-injury-in-newborns
#5
Emad E Ghobrial, Salma Z Elhouchi, Sarah S Eltatawy, Lilian O Beshara
Acute kidney injury (AKI) in the newborn is a common problem in the neonatal intensive care unit with many underlying factors such as asphyxia, respiratory distress syndrome (RDS), and urogenital anomalies. The aim of this study is to highlight possible risk factors and profile of neonates developing AKI in the Neonatal Intensive Care Unit (NICU) of Cairo University Pediatric Hospital. The study was carried out on 90 neonates (30 patients and 60 controls), among neonates admitted to NICU. The study was done over two months, from January 2015 to March 2015...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29454577/morphological-clues-to-the-appropriate-recognition-of-hereditary-renal-neoplasms
#6
REVIEW
Holger Moch, Riuko Ohashi, Jatin S Gandhi, Mahul B Amin
An important emerging role of the surgical pathologist besides the traditional tasks of establishment of the diagnosis and documentation of prognostic and predictive factors, is to recognize the possibility of a hereditary condition in cases where the histology is suggestive for a familial cancer syndrome. In recent years, the knowledge regarding all of the above roles, including the role of recognition of familial cancer, has particularly expanded in renal neoplasms with the close scrutiny to morphology, molecular correlates and clinical features of the different sub-types of renal cell carcinoma...
February 14, 2018: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/29453782/unusual-spontaneous-porto-systemic-shunt-the-importance-of-diagnosing-non-anatomical-porto-systemic-shunts-to-improve-portal-flow-in-pediatric-living-related-liver-transplantation-case-report
#7
Juan S Rubio, Carolina Rumbo, Pablo A Farinelli, Nicolás Aguirre, Diego A Ramisch, Hugo Paladini, Pablo D Angelo, Pablo Barros Schelotto, Gabriel E Gondolesi
Collateral circulation secondary to liver cirrhosis may cause the development of large PSSs that may steal flow from the main portal circulation. It is important to identify these shunts prior to, or during the transplant surgery because they might cause an insufficient portal flow to the implanted graft. There are few reports of "steal flow syndrome" cases in pediatrics, even in biliary atresia patients that may have portal hypoplasia as an associated malformation. We present a 12-month-old female who received an uneventful LDLT from her mother, and the GRWR was 4...
February 16, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29453776/syndromic-sebaceous-nevus-current-findings
#8
Oumama El Ezzi, Anthony S de Buys Roessingh, Michèle Bigorre, Guillaume Captier
BACKGROUND: Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. In the adult patient, tumors (usually benign) develop from sebaceous nevus. Their surgical excision during childhood can give a better result in terms of the definitive scar. OBJECTIVES: The aim of this study is to analyze our cases of syndromic sebaceous hamartoma, perform a review of the existing literature, and propose guidelines for the therapeutic plan...
February 16, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29453636/assessment-of-oximetry-based-statistical-classifiers-as-simplified-screening-tools-in-the-management-of-childhood-obstructive-sleep-apnea
#9
Andrea Crespo, Daniel Álvarez, Leila Kheirandish-Gozal, Gonzalo C Gutiérrez-Tobal, Ana Cerezo-Hernández, David Gozal, Roberto Hornero, Félix Del Campo
PURPOSE: A variety of statistical models based on overnight oximetry has been proposed to simplify the detection of children with suspected obstructive sleep apnea syndrome (OSAS). Despite the usefulness reported, additional thorough comparative analyses are required. This study was aimed at assessing common binary classification models from oximetry for the detection of childhood OSAS. METHODS: Overnight oximetry recordings from 176 children referred for clinical suspicion of OSAS were acquired during in-lab polysomnography...
February 16, 2018: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/29453589/validity-of-triglyceride-glucose-index-as-an-indicator-for-metabolic-syndrome-in-children-and-adolescents-the-caspian-v-study
#10
Pooneh Angoorani, Ramin Heshmat, Hanieh-Sadat Ejtahed, Mohammad Esmaeil Motlagh, Hasan Ziaodini, Majzoubeh Taheri, Tahereh Aminaee, Azam Goodarzi, Mostafa Qorbani, Roya Kelishadi
PURPOSE: The purpose of this study was to determine the cut-off values of triglycerides and glucose (TyG) index as one of the indirect indices for metabolic syndrome (MetS) in a pediatric population. METHODS: This national study was conducted in 2015 on 14400 students, aged 7-18 years. They were selected by random cluster sampling from 30 provinces of our country during the fifth survey of a national school-based surveillance program. MetS was defined based on the Adult Treatment Panel III (ATP III) criteria modified for the pediatric age group...
February 16, 2018: Eating and Weight Disorders: EWD
https://www.readbyqxmd.com/read/29451804/pediatric-acute-respiratory-distress-syndrome-increase-the-peep
#11
Andrew C McKown, Lorraine B Ware
No abstract text is available yet for this article.
February 16, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29451061/neck-tongue-syndrome-an-underrecognized-childhood-onset-cephalalgia
#12
Nicholas M Allen, Hormos S Dafsari, Elizabeth Wraige, Heinz Jungbluth
Neck-tongue syndrome is a rarely reported headache disorder characterized by occipital and/or upper neck pain triggered by sudden rotatory head movement and accompanied by abnormal sensation and/or posture of the ipsilateral tongue. Although onset is thought to be in childhood, most of the limited number of cases reported so far were adults. Here the authors describe 3 cases, 2 girls and 1 boy, with neck-tongue syndrome. In each child additional headache symptoms occurred, headache improved over time in all, spontaneously in 2 and coinciding with gabapentin treatment in the other...
January 1, 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29450676/comorbid-obstructive-sleep-apnea-and-increased-risk-for-sickle-cell-disease-morbidity
#13
Tal Katz, Jeffrey Schatz, Carla W Roberts
PURPOSE: Sickle cell disease (SCD) imparts an increased risk for obstructive sleep apnea (OSA) in childhood. Studies of pediatric SCD have identified an increased risk for pain and neurologic complications with comorbid OSA. We determined the rate of a broad range of SCD-related medical complications to better characterize the spectrum of SCD complications related to OSA. METHODS: Retrospective chart review at a single hematology clinic identified 641 youth with SCD who received consistent screenings for OSA as part of routine hematological health maintenance visits over an 11-year period...
February 15, 2018: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/29450629/congenital-cervical-kyphosis-in-an-infant-with-ehlers-danlos-syndrome
#14
Andrew J Kobets, Daniel Komlos, John K Houten
BACKGROUND: Ehler-Danlos syndome (EDS) refers to a group of heritable connective tissue disorders; rare manifestations of which are cervical kyphosis and clinical myelopathy. Surgical treatment is described for the deformity in the thoracolumbar spine in adolescents but not for infantile cervical spine. Internal fixation for deformity correction in the infantile cervical spine is challenging due to the diminutive size of the bony anatomy and the lack of spinal instrumentation specifically designed for young children...
February 15, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29449094/safety-and-efficacy-of-small-bowel-polypectomy-using-a-balloon-assisted-enteroscope-in-pediatric-patients-with-peutz-jeghers-syndrome
#15
G Blanco-Velasco, O V Hernández-Mondragón, J M Blancas-Valencia, V Paz-Flores, D Fuentes-Hernández, P Rodríguez-González, B González-Ortíz
INTRODUCTION AND AIMS: Peutz-Jeghers syndrome is an autosomal dominant inherited pathology characterized by gastrointestinal hamartomatous polyps, predominantly in the small bowel, and pigmented mucocutaneous lesions. Guidelines suggest polypectomy with a balloon-assisted enteroscope when polyps are larger than 10mm. Complications in adults can be as high as 6.8%, but there is little information on pediatric populations. Our aim was to describe the safety and efficacy of polypectomy in a group of pediatric patients with Peutz-Jeghers syndrome using balloon-assisted enteroscopy...
February 12, 2018: Revista de Gastroenterología de México
https://www.readbyqxmd.com/read/29449075/cardiac-transplantation-in-children-with-down-syndrome-turner-syndrome-and-other-chromosomal-anomalies-a-multi-institutional-outcomes-analysis
#16
Christopher R Broda, Antonio G Cabrera, Joseph W Rossano, John L Jefferies, Jeffrey A Towbin, Clifford Chin, Pirouz Shamszad
BACKGROUND: The purpose of this study was to describe the prevalence, characteristics, and outcomes in pediatric patients with chromosomal anomalies (CA) undergoing orthotopic heart transplantation (OHT). METHODS: A query of the database of the Pediatric Health Information System, a large administrative and billing database of 43 tertiary children's hospitals, was performed for the Years 2004 to 2016. Pediatric patients who received OHT were analyzed based on presence and type of CA...
January 31, 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29447894/cochlear-implantation-in-pediatric-patients-with-cockayne-syndrome
#17
Renae D Van Wyhe, Claudia V Emery, Robert A Williamson
Cockayne Syndrome (CS) is a rare, autosomal recessive disorder characterized by a spectrum of phenotypic abnormalities, including progressive sensorineural hearing loss (SNHL) that involves both peripheral and central components. To date, a single series of CS patients undergoing cochlear implant (CI) placement has been reported; this study reports on additional previously unreported pediatric CI recipients. Subjective benefits were noted early after activation in both patients, and speech perception scores improved over time as well, varying from 42 to 70% (versus 0-12% previously)...
March 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29446798/life-threatening-headaches-in-children-clinical-approach-and-therapeutic-options
#18
Nagma Dalvi, Lalitha Sivaswamy
Life-threatening headaches in children can present in an apoplectic manner that garners immediate medical attention, or in an insidious, more dangerous form that may go unnoticed for a relatively long period of time. The recognition of certain clinical characteristics that accompany the headache should prompt recognition and referral to an institution equipped with neuroimaging facilities, pediatric neurosurgeons, and neurologists. Thunderclap headaches, which reach a peak within a very short period of time, may be the presenting feature of conditions such as arterial dissection, venous sinus thrombosis, and reversible cerebral vasoconstriction syndrome, which can be addressed by specific pharmacological options instituted in an intensive care setting...
February 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29446797/uncommon-pediatric-primary-headache-disorders
#19
M Cristina Victorio
Migraine and tension-type headaches are common primary headache disorders encountered among children and adolescents presenting to a pediatric clinic. At times, children present with a headache with unusual or peculiar features that can be alarming and perplexing. These can be in the form of a brief stabbing headache with lacrimation in one eye or a continuous headache locked to one side of the head or face. These headache syndromes tend to be more common among adults but, on occasion, are known to occur or have their onset during childhood...
February 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29446794/migraine-variants-in-children
#20
A David Rothner
Migraine in children can manifest in ways that are markedly different from adult migraines. In children, migraine variants are often unaccompanied by headache and include conditions such as cyclic vomiting and abdominal migraine. Children who experience these conditions are often thought to have a disorder of the gastrointestinal tract, and when evaluation is unremarkable they may be diagnosed as having a conversion reaction. Complicated migraines, on the other hand, are often accompanied by focal neurological symptoms such as ataxia, hemiparesis, or altered level of consciousness that evoke great consternation in the examining clinician...
February 1, 2018: Pediatric Annals
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