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Fetal and Pediatric Pathology

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https://www.readbyqxmd.com/read/28644757/relationships-between-adipokine-profiles-physique-index-and-severity-of-bronchiolitis-in-infancy
#1
Ryou Kawamata, Yuji Gunji, Ayafumi Ozaki, Kei Wakabayashi, Yuka Miyajimaa, Yukifumi Monden, Kei Numazaki, Kazuo Takahashi
INTRODUCTION: Relationships between adipokines, adiposity and severity of acute viral bronchiolitis in infancy have not been elucidated. MATERIALS AND METHODS: We investigated the relationships between three serum adipokines (leptin, adiponectin and TNF-α), physique index (Kaup index) and clinical severity in 13 bronchiolitis infants. Seven healthy infants were enrolled as the control group. We used Modified Pulmonary Index Score (MPIS) to evaluate bronchiolitis severity...
June 23, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28617177/discordant-anomalies-with-combined-features-of-pentalogy-of-cantrell-and-oeis-complex-a-case-report-in-monochorionic-twins
#2
Nique Kunapinun, Jitsupa Treetipsatit
INTRODUCTION: Ventral body wall defects have various manifestations. Among others, pentalogy of Cantrell (PC) and omphalocele exstrophy imperforate anus spinal abnormalities (OEIS) complex are defects that involve upper and lower anterior midline of body wall, respectively. Although both entities are in a spectrum of ventral body wall defects, the combination of PC and OEIS complex has not been described. CASE REPORT: In this report, we describe an unusual case of congenital ventral body wall defect with combined features of PC and OEIS complex, which discordantly occurred in monochorionic monoamniotic twins...
June 15, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28569558/persistent-left-superior-vena-cava-in-fetuses-an-autopsy-series
#3
Deepa Ramakrishnan, Shanthi Chidambarathanu, Lata Murli, John Micheal, Sujatha Jagadeesh, Indrani Suresh, Suresh Seshadri
OBJECTIVE: To review fetal autopsy reports with persistent left superior vena cava (PLSVC) and identify its associations. MATERIALS AND METHODS: Autopsy reports of all fetuses diagnosed with PLSVC in our center from January 2011 to December 2015 were reviewed. Fetuses less than 15 weeks gestational age along with autolyzed and damaged hearts were excluded from the study. The study group was compared with controls during this period. RESULTS: Prenatal ultrasound detection rate of PLSVC was 13...
June 1, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28557647/epidermolysis-bullosa-with-pyloric-atresia-and-aplasia-cutis-in-a-newborn-due-to-homozygous-mutation-in-itgb4
#4
Gozdem Kayki, Davut Bozkaya, Fatih Ozaltin, Diclehan Orhan, Figen Kaymaz, Emine Korkmaz, Sule Yigit
BACKGROUND: Epidermolysis bullosa with pyloric atresia (EB-PA) is an autosomal recessive disorder due to mutations in ITGA6 and/or ITGB4, resulting in altered expression of α6β4 integrin. EB-PA can also occur with aplasia cutis. CASE REPORT: We present a newborn with EB-PA and aplasia cutis, born of consanguineous parents, with a homozygous c.3793+1G>A mutation affecting ITGB4, previously described only in the heterozygous state with other mutations. CONCLUSION: The previously unreported homozygous c...
May 30, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28557592/ameloblastic-fibro-odontoma-of-the-maxilla-in-a-pierre-robin-sequence-patient
#5
Kenneth Kufta, Steve Kang, Faizan Alawi, Anna Moran, Neeraj Panchal
INTRODUCTION: Pierre Robin sequence (PRS) is a rare disorder classically observed as a triad of features including micrognathia, glossoptosis, and upper airway obstruction. It is associated with a syndrome in about 60% of cases. While odontogenic tumors are common findings in patients with familial adenomatous polyposis and nevoid basal cell carcinoma syndromes, PRS has not been found to be consistently associated with any tumors of the jaw. CASE REPORT: The current report aims to describe a patient with PRS who presented with an extensive ameloblastic fibro-odontoma (AFO) of the maxilla...
May 30, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28541826/ini-expressing-epithelioid-sarcoma-with-osteoclastic-giant-cells-in-a-child-a-case-report-with-summary-of-prior-published-cases
#6
Riju Bhattacharyya, Ranajoy Ghosh, Koushik Saha, Uttara Chatterjee
BACKGROUND: Epithelioid sarcoma is a heterogeneous tumor with 2 subtypes, classic and proximal. The proximal variant is more aggressive and occurs in proximal location in young adults. CASE REPORT: We present a proximal epithelioid sarcoma in the leg of an 8 year old girl with rhabdoid morphology and scattered osteoclastic giant cells. Nuclear INI-1 was retained. Despite wide local excision, local recurrence occurred at 8 months. Following re-excision, she developed a chest wall metastasis after 9 months...
May 25, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28481137/angiotensin-converting-enzyme-gene-polymorphism-in-children-with-idiopathic-nephrotic-syndrome-effect-on-biopsy-findings
#7
Maryam Monajemzadeh, Mahshid Hesami, Reza Shahsiah, Mohammad Vasei, Safoora Hooshmand, Parin Tanzifi, Niloofar Hajizadeh, Neamatollah Ataei, Mehryar Mehrkash, Faezeh Javadi Larijani, Mastaneh Moghtaderi, Behnaz Bazargani, Reza Khorvash, Narjes Soleimanifar
OBJECTIVE: Angiotensin converting enzyme (ACE) converts angiotensin I into angiotensin II. The ACE gene shows an I/D polymorphism, which correlates with ACE concentrations. The aim of this study is to evaluate the distribution of the ACE I/D genotype in children with idiopathic nephrotic syndrome (INS) and healthy controls and study the effect of this polymorphism on clinical and pathologic findings. METHODS: ACE gene I/D polymorphism of 104 patients with INS and 119 controls were determined...
May 8, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28481134/the-role-of-oxidative-stress-in-the-clinical-manifestations-of-childhood-asthma
#8
Aleksandra Topic, Djordje Francuski, Aleksandra Nikolic, Katarina Milosevic, Snezana Jovicic, Bojan Markovic, Mirjana Djukic, Dragica Radojkovic
INTRODUCTION: The significance of oxidative stress in pathogenesis of childhood asthma was recognized, but its role in the clinical manifestations of disease is still unclear. MATERIALS AND METHODS: The study was conducted in 96 asthmatic children. The urinary biomarker of oxidative stress, 8-oxo-7,8-dihydro-2-deoxyguanosine (8-oxodG/creatinine) was determined by using HPLC-MS/MS. ELISA was performed to measure myeloperoxidase (MPO) and Cu,Zn- superoxide dismutase (Cu,Zn-SOD) in serum...
May 8, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28481127/fetal-cardiac-impairment-in-nitrofen-induced-congenital-diaphragmatic-hernia-postmortem-microcomputed-tomography-imaging-study
#9
Gloria Pelizzo, Valeria Calcaterra, Claudio Lombardi, Rossana Bussani, Vanessa Zambelli, Annalisa De Silvestri, Ana Custrin, Manuel Belgrano, Floriana Zennaro
INTRODUCTION: We assessed the post-mortem micro-CT utility to evaluate fetal cardiac impairment in nitrofen-induced congenital diaphragmatic hernia (CDH). METHODS: At 9.5d postconception (dpc), pregnant rats were exposed to nitrofen. At +18 and +21dpc, fetuses were harvested by cesarean section. Postmortem micro-CT and autopsy were performed. Fetuses were assigned to three experimental groups: Control group (C), Nitrofen group (N, exposition to nitrofen without CDH), CDH group...
May 8, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28481124/refractory-chylous-ascites-secondary-to-neuroblastoma
#10
Vidya B Pai, Richard Benator, Benjamin Torres
BACKGROUND: Neonatal ascites is a complex condition that often poses a diagnostic dilemma for the clinician. We present a case of neonatal ascites secondary to neuroblastoma. CASE PRESENTATION: Our neonatal patient had congenital and recurrent chylous ascites despite multiple postnatal paracenteses, which resolved with complete resection of a retroperitoneal neoplasm. CONCLUSION: Congenital neuroblastoma may present with chylous ascites, probably due to disruption of the lymphatic vasculature...
May 8, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28453380/lipids-on-the-second-day-in-ischemic-and-normoxemic-term-neonates
#11
Miljana Z Jovandaric, Tatjana V Nikolic, Svetlana J Milenkovic, Biljana S Otaševic, Violeta V Bankovic, Petar I Ivanovski, Milos M Jesic
INTRODUCTION: In hypoxic newborns requiring oxygen, lipid peroxidation affects the peripheral blood lipids. OBJECTIVES: Determine the influence of perinatal oxygen therapy for hypoxia on serum lipid concentrations on the second day of life. MATERIALS AND METHODS: Our study included 50 newborns with perinatal hypoxia requiring oxygen and 50 healthy newborns without oxygen therapy. Arterialized capillary blood was taken for categorization of hypoxia (pO2) after birth in both groups...
April 28, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28453379/pseudoamniotic-band-syndrome-post-fetal-thoracoamniotic-shunting-for-bilateral-hydrothorax
#12
Michelle Han, Yalda Afshar, Andrew H Chon, Emily Scibetta, Rashmi Rao, Ramen H Chmait
INTRODUCTION: Pseudoamniotic band syndrome (PABS) occurs iatrogenically after fetal surgery or amniocentesis due to chorioamniotic membrane separation. Separation of the amnion from the chorion can expand to form fibrous amniotic bands that can envelope fetal limbs or the umbilical cord, with consequences ranging from limb constriction to fetal demise. CASE REPORT: We report a case of bilateral fetal pleural effusions at 27 weeks' gestation treated by bilateral thoracoamniotic shunts...
April 28, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28409691/to-the-editor-concerning-kaya-et-al
#13
Gabriela Guzmán-Navarro, Fabiola Castorena-Torres, Víctor Javier Lara-Díaz
No abstract text is available yet for this article.
April 14, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28574806/free-fatty-acids-of-newborns-from-women-with-gestational-diabetes-mellitus
#14
Miljana Z Jovandaric, Petar I Ivanovski
INTRODUCTION: Fetal macrosomia in gestational diabetes mellitus is contributed to by compensatory fetal mechanisms responding to alterations in maternal metabolism. OBJECTIVES: To compare FFA and blood glucose concentrations of newborns derived from healthy and hyperglycemic mothers. METHODS: Prospective study included two equal groups of term newborns (50) from GDM and healthy mothers. Blood was derived from umbilical and cubital vein of mothers immediately after birth...
June 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28574805/the-development-of-fviii-inhibitors-in-relation-to-il10-gene-polymorphism-in-hemophilia-a-egyptian-pediatric-patients
#15
Hoda Sadek, Ilham Youssry, Nihal Salah Eldeen Ibrahim, Amany Ahmed Abou-Elalla, Gehad Atef, Somaia Mohammed Mousa
BACKGROUND: Development of inhibitors against Factor VIII (FVIII) in hemophilia A patients is a serious complication of therapy. Many cytokines, including interleukin-10 (IL10), may affect inhibitor development; however, literature data are not sufficient to prove this association. The aim of this study was to investigate the relation between FVIII inhibitor formation and IL10-1082A/G polymorphism among Egyptian hemophiliacs. METHODS: Patients were screened for FVIII inhibitors using the Bethesda method...
June 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28524764/lack-of-association-between-stat4-single-nucleotide-polymorphisms-and-iranian-juvenile-rheumatoid-arthritis-patients
#16
Saeed Aslani, Mahdi Mahmoudi, Arash Salmaninejad, Shiva Poursani, Vahid Ziaee, Nima Rezaei
Juvenile rheumatoid arthritis (JRA) is a common chronic systemic autoimmune disease in children. Single nucleotide polymorphisms (SNPs) of signal transducer and activator of transcription 4 (STAT4) gene are suspected to have association with the risk of autoimmune diseases. Previous investigations have indicated that the STAT4 rs7574865 T allele was significantly associated with rheumatoid arthritis. In this study, we aimed to evaluate the association of STAT4 SNPs with JRA in Iranian population. T allele of STAT4 rs7574865 SNP was less frequent in patients than in controls, and the difference was not significant (p = 0...
June 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28481156/association-of-ptpn22-single-nucleotide-polymorphisms-with-celiac-disease
#17
Majid Aflatounian, Arezou Rezaei, Maryam Sadr, Amene Saghazadeh, Nazanin Elhamian, Hengameh Sadeghi, Fatemeh Motevasselian, Fatemeh Farahmand, Gholamhossein Fallahi, Farzaneh Motamed, Mehri Najafi, Nima Rezaei
OBJECTIVES: Celiac disease is a chronic autoimmune disease in which gene-environment interactions cause the immune system to unfavorably react to naturally gluten-containing foods. PTPN22 plays a crucial role in regulating the function of various cells of the immune system, particularly T cells. Polymorphisms of the PTPN22 gene have been associated with many autoimmune diseases. The present genetic association study was conducted to investigate the possible associations between PTPNTT single nucleotide polymorphisms (SNPs) and celiac disease in an Iranian population...
June 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28453372/renal-tubular-mitochondrial-abnormalities-in-complex-ii-iii-respiratory-chain-deficiency
#18
Joel France, Isa Ashoor, Randall Craver
Defects in the respiratory chain may present with a wide spectrum of clinical signs and symptoms. In this "Images in Pathology" discussion we correlate the clinical, histologic, and ultrastructural findings in a 12-year-old male with a complex II/III respiratory chain deficiency and kidney dysfunction.
June 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28394716/lethal-congenital-malformations-in-fetuses-antenatal-ultrasound-or-perinatal-autopsy
#19
Sumit Grover, Bhavna Garg, Neena Sood, Kamaldeep Arora
BACKGROUND: Congenital malformations (CMF) are major causes of fetal demise which can be detected antenatally by Ultrasonography (USG). METHODS: We studied 100 perinatal autopsies for CMF. Sensitivity of USG was determined and accuracy of USG with that of autopsy was compared. RESULTS: At Autopsy 134 individual CMF were seen in 40 cases. The sensitivity of USG in detecting major CMF was 54.47%. A complete agreement between autopsy and USG findings was seen in 13/40 (32...
June 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28394668/use-of-cortisol-and-adrenal-weight-at-pediatric-postmortem
#20
Rebecca Morrison, Jansher Khan, Peter Galloway, Jane McNeilly, Syed Faisal Ahmed, Dawn Penman
We studied the relationship between adrenal weight and postmortem cortisol level in cases of infant death, and examined use of these measurements in adrenal insufficiency. We analyzed procurator-fiscal postmortem reports in the West of Scotland over a three year period. Combined adrenal weight was expressed as percentage of total body weight (%TBW). Of 106 cases, median (5(th), 95(th)) %TBW was 0.056 (0.025, 0.23) and median plasma cortisol was 8.4 ug/dl (1.0, 47.1). There was no correlation between %TBW and plasma cortisol (r = 0...
June 2017: Fetal and Pediatric Pathology
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