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Pediatric Reports

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https://www.readbyqxmd.com/read/30363636/surgical-approach-to-giant-ovarian-masses-in-adolescents-technical-considerations
#1
Giorgio Persano, Elisa Severi, Noemi Cantone, Filippo Incerti, Enrico Ciardini, Bruno Noccioli
Ovarian neoplasms arising from the surface epithelium are rare in the pediatric population; their knowledge is therefore limited and the appropriate management is poorly defined. We describe our experience and suggest our surgical approach to adolescents affected by voluminous ovarian masses. Two 15-year-old adolescents were admitted to our institution in 2017 for multilobulated, fluid-filled masses measuring over 30 cm arising from the ovaries. The cystic component was drained intraoperatively with a spillage-free technique, consisting in the application of a sterile autoadhesive transparent drape on the cyst and the insertion of a 12 Ch pleural drain, secured with a purse-string suture...
September 25, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/30363617/recurrent-supraventricular-tachycardia-and-necrotizing-enterocolitis-a-causative-role-or-a-simple-association-a-case-report-and-literature-review
#2
Ghassan Nakib, Sameer Sajwani, Zahreddin Abusalah, Abdelazeim Abdallah, Nada Ibrahim, Aseel Fattah, Rossana Bussani, Valeria Calcaterra, Gloria Pelizzo
Insufficiency in mesenteric flow is a risk factor for the development of necrotizing enterocolitis (NEC). Recurrent episodes of supraventricular tachycardia (SVT) can lead to gut ischemia, with subsequent reperfusion injury. We present a term infant who developed NEC at 37 days of life after refractory SVT and reverted to sinus rhythm on day 23 and 25 of life. Resected stenotic ileum and transverse colon demonstrated inflammation with ischemia. This is the first case of NEC following SVT in a term infant without a congenital morphologic abnormality...
September 25, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/30069296/a-case-of-atypical-systemic-primary-carnitine-deficiency-in-saudi-arabia
#3
Abdulrahman Alghamdi, Hani Almalki, Aiman Shawli, Rahaf Waggass, Fahad Hakami
Systemic primary carnitine deficiency (SPCD) is an autosomal recessive inborn error of fatty acid metabolism caused by a defect in the transporter responsible for moving carnitine across plasma membrane. The clinical features of SPCD vary widely based on the age of onset and organs involved. During infancy, patients might show episodes of hypoketotic hypoglycemia, hepatomegaly, elevated transaminases, and hyperammonemia. Skeletal myopathy, elevated creatine kinase, and cardiomyopathy are the main manifestations in children with SPCD, while in adults, the disorder is usually manifested as cardiomyopathy, arrhythmias, or fatigability...
May 24, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/30069295/rapunzel-syndrome-how-to-orient-the-diagnosis
#4
Enrico Finale, Piergiorgio Franceschini, Cesare Danesino, Michelangelo Barbaglia, Andrea Guala
Rapunzel syndrome is a rare form of tricobezoar with a tail extending from the stomach into the small bowel; surgical removal is generally required. About 60 cases have been reported and described in the literature since 1968. We present the case of an 8-year-old girl who, during the course of a genetic consultation, was initially assigned with a clinical suspicion of ectodermal dysplasia. Surgical intervention, which resulted in the extraction of a tricobezoar of enormous size and extension, led us to the diagnosis of Rapunzel syndrome...
May 24, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/30069294/takayasu-arteritis-with-an-initial-presentation-of-chronic-monoarthritis-mimicking-oligoarticular-juvenile-idiopathic-arthritis
#5
Maynart Sukharomana, Sorawit Viravan, Nuntawan Piyaphanee, Sirirat Charuvanij
Patients with Takayasu arteritis (TA) generally present with non-specific symptoms that, if unrecognized and untreated, may develop vessel stenosis and/or aneurysm. There is limited data regarding chronic monoarthritis as the initial presentation in children with TA. We report a 6-yearold girl diagnosed and treated as oligoarticular juvenile idiopathic arthritis (JIA). She later developed stroke with malignant hypertension and was definitively diagnosed with TA. She additionally developed proteinuria secondary to focal segmental glomerulosclerosis...
May 24, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/30069293/waardenburg-syndrome-with-isolated-deficiency-of-myenteric-ganglion-cells-at-the-sigmoid-colon-and-rectum
#6
Shun Watanabe, Shotaro Matsudera, Takeshi Yamaguchi, Yukiko Tani, Kei Ogino, Masanobu Nakajima, Satoru Yamaguchi, Kinro Sasaki, Hiroshi Suzumura, Takashi Tsuchioka
Waardenburg syndrome (WS) has the characteristic clinical features caused by the embryologic abnormality of neural crest cells. WS patients sometimes suffer from functional intestinal obstruction. When it is Hirschsprung disease (HD), the WS is diagnosed as type 4 WS. We report a case of WS which did not have myenteric ganglion cells in the sigmoid colon and rectum. Whether to diagnosis this case as type 1 or 4 WS is controversial. Moreover, this is the third report which has peristalsis failure caused by abnormal myenteric plexus...
May 24, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/29721248/isolated-tubal-torsion-a-rare-cause-of-acute-abdomen-in-childhood
#7
Berat Dilek Demirel, Sertac Hancioglu, Unal Bicakci, Ender Ariturk, Ferit Bernay
Isolated fallopian tube torsion is a rare cause of acute abdomen mostly seen in women of reproductive age. It is often diagnosed during surgery. In this study we aimed to present a case of isolated fallopian tube torsion in a child. An 11-year-old girl presented with abdominal pain. With the help of radiological evaluation we diagnosed. It should be kept in mind that even if ovarian blood flow is normal in girls with abdominal pain, it may be an isolated tubular torsion and requires immediate intervention.
March 22, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/29721247/anesthesia-in-mowat-wilson-syndrome-information-on-11-italian-patients
#8
Marianna Spunton, Livia Garavelli, Paola Cerutti Mainardi, Uta Emmig, Enrico Finale, Andrea Guala
Mowat-Wilson syndrome is a genetic disease caused by heterozygous mutations or deletions of the ZEB2 gene and characterized by typical clinical features. The congenital malformations typical of this syndrome call for early diagnostic and surgical procedures requiring general anesthesia, but few information about the anesthesiology management of such patients is available. We enrolled 11 families of patients with Mowat-Wilson syndrome who had undergone surgical or diagnostic procedures requiring general anesthesia, and sent them a retrospective questionnaire including 16 open questions about the procedures...
March 22, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/29721246/acute-encephalopathy-with-biphasic-seizures-and-late-reduced-diffusion-associated-with-streptococcus-sanguinis-sepsis
#9
Hitoshi Awaguni, Jun Shinozuka, Shin-Ichiro Tanaka, Sayaka Kadowaki, Shigeru Makino, Rikken Maruyama, Yosuke Shigematsu, Kenji Hamaoka, Shinsaku Imashuku
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) develops in association with systemic as well as central nervous system (CNS) viral or bacterial infections. AESD is most often noted with influenza or human herpesvirus 6 infection in previously healthy infants. However, AESD has also been reported in an infant with developmental retardation and in a mentally and motor-disabled adolescent. Here, we report the case of a 4- year-old female with significant development delay due to spinal muscular atrophy, who developed AESD during Streptococcus sanguinis sepsis with no apparent CNS infection...
March 22, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/29721245/an-infantile-alantoaxial-dislocation-with-patent-foramen-ovale-managed-with-titanium-cabling-and-allogenic-bone-grafts
#10
Seidu A Richard, Zhi Gang Lan, Xiao Yang, Siqing Huang
Atlantoaxial dislocation is a disorder that is characterized with loss of stability of the atlas and axis (C1-C2) with consequential loss of usual articulation. Although this condition is very common, no one has reported a case as young as our patients. We present a 7-month infant with bilateral paralysis of the lower limbs for four (4) months with no history of trauma. Computer tomographic (CT-scan) imaging revealed alantoaxial dislocation with severe cervical spinal cord compression. The odontoid process is displaced outwardly with no bone destruction...
March 22, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/29721244/epidemiology-of-vertebral-fractures-in-pediatric-and-adolescent-patients
#11
Dominik Saul, Klaus Dresing
Spinal injuries in children and adolescents are rare injuries, but consequences for the growing skeleton can be devastating. Knowledge of accident causes, clinical symptoms and diagnostics should be part of every trauma department treating these patients. We retrospectively analyzed patients with radiographically proven vertebral fractures of the spine. After clinical examination and tentative diagnosis the fractures and injuries were proven with conventional X-ray, computed tomography (CT) scans or magnetic resonance imaging (MRI)...
March 22, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/29383223/a-boy-with-recurrent-swelling-of-the-jaw
#12
Lien Haverals, Marjolein Mattheij, Esther Hoppenreijs, Stefaan Bergé, Annemieke van der Weij
No abstract text is available yet for this article.
November 21, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/29383222/zika-virus-infection-in-a-pediatric-patient-with-acute-gastrointestinal-involvement
#13
Svetoslav Slavov, Alessandra Matsuno, Aparecida Yamamoto, Katia Otaguiri, Maria Cervi, Dimas Covas, Simone Kashima
No abstract text is available yet for this article.
November 21, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/29383221/zinc-supplementation-enhances-linear-growth-in-school-aged-children-a-randomized-controlled-trial
#14
Sanguansak Rerksuppaphol, Lakkana Rerksuppaphol
No abstract text is available yet for this article.
November 21, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/29383220/pectus-updates-and-special-considerations-in-marfan-syndrome
#15
Stephanie Fraser, Anne Child, Ian Hunt
No abstract text is available yet for this article.
November 21, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/29383219/initiation-of-complementary-feeding-and-associated-factors-among-children-of-age-6-23-months-in-sodo-town-southern-ethiopia-cross-sectional-study
#16
Tefera Chane, Shimelash Bitew, Tesfa Mekonnen, Wubalem Fekadu
No abstract text is available yet for this article.
November 21, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/29285340/46-xy-ovotesticular-disorders-of-sex-development-a-therapeutic-challenge
#17
Maria-Grazia Scarpa, Massimo Di Grazia, Gianluca Tornese
No abstract text is available yet for this article.
November 21, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/29285339/medium-chain-acyl-coa-dehydrogenase-deficiency-in-a-premature-infant
#18
Steven F Dobrowolski, Lina Ghaloul-Gonzalez, Jerry Vockley
Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is identified by newborn screening (NBS). The natural history of MCADD includes metabolic decompensation with hypoglycemia, hyperammonemia, seizures, coma, and death. NBS enables expectant management thus severe symptoms are rare in managed patients. We report premature birth of an MCADD affected infant and resultant management challenges. Nutritional support advanced from parenteral nutrition at 24 hours to enteral feeds. A NBS sample was collected day 2, positive results for MCADD was reported day six, and diagnostic tests were performed day seven...
November 21, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/29081936/the-role-of-interleukin-6-in-the-early-diagnosis-of-sepsis-in-premature-infants
#19
Mehrdad Mirzarahimi, Manouchehr Barak, Abdolkarim Eslami, Afsaneh Enteshari-Moghaddam
Septicemia is one of the major causes of mortality and morbidity in newborns. The aim of this study was to investigate the role of interleukin-6 (IL-6) in the early diagnosis of Sepsis in premature newborns. This cross-sectional study was conducted on preterm infants admitted to NICU of Hospital Buali in Ardabil city, Iran. C-reactive protein (CRP), IL-6 and complete blood count tests have been done in baseline, third and seventh day. Collected data analyzed by one-sample t-test, repeated measures and ANOVA in SPSS...
October 6, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/29081935/clinical-heterogeneity-in-a-family-with-dkc1-mutation-dyskeratosis-congenita-and-hoyeraal-hreidarsson-syndrome-in-first-cousins
#20
Cristina Olivieri, Anna Mondino, Matteo Chinello, Alessandra Risso, Enrico Finale, Marina Lanciotti, Andrea Guala
Dyskeratosis congenita (DC) is an inherited bone marrow failure disorder characterized by mucocutaneous features (skin pigmentation, nail dystrophy and oral leukoplakia), pulmonary fibrosis, hematologic and solid malignancies. Its severe form, recognized as Hoyeraal-Hreidarsson syndrome (HHS), also includes cerebellar hypoplasia, microcephaly, developmental delay and prenatal growth retardation. In literature phenotypic variability among DC patients sharing the same mutation is wellknown. To our knowledge this report describes for the first time a family of DC patients, characterized by a member with features of classic DC and another one with some features of HHS, both with the same mutation in DKC1 ...
October 6, 2017: Pediatric Reports
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