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https://www.readbyqxmd.com/read/29334143/childhood-alopecia-areata-data-from-the-national-alopecia-areata-registry
#1
Iris Wohlmuth-Wieser, Joyce S Osei, David Norris, Vera Price, Maria K Hordinsky, Angela Christiano, Madeleine Duvic
BACKGROUND/OBJECTIVES: Alopecia areata may occur at any age and is the third-most-common dermatosis in children. The objective of this study was to investigate the clinical and epidemiologic features of children and adolescents with alopecia areata based on the data of the National Alopecia Areata registry on children and adolescents. METHODS: Two thousand two hundred eighteen children and adolescents with alopecia areata self-enrolled in the National Alopecia Areata Registry and completed a web-based, self-administered, short-intake screening questionnaire (first tier)...
January 15, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29334142/severe-ectropion-in-lamellar-ichthyosis-managed-medically-with-oral-acitretin
#2
Manpreet Singh, Manpreet Kaur, Ripanjeet Kaur, Shakeen Singh
Congenital ectropion is commonly associated with lamellar ichthyosis. Severe eyelid ectropion may cause corneal exposure, keratopathy, and permanent corneal scarring. We report a neonate with severe, bilateral, congenital ectropion and eclabium managed using oral retinoids. Both corneas were protected with topical antibiotics and lubricating eyedrops and eye ointments. At 12-month follow-up, the child was doing well, with no ectropion or corneal opacity.
January 15, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29333844/-success-in-probing-for-congenital-nasolacrimal-duct-obstruction-ten-years-experience
#3
Sofía Bravo-Beltranena, Martín A Zimmermann-Paiz
INTRODUCTION: The congenital nasolacrimal duct obstruction is a common pathology, with low morbidity, but not exempt of complications without the correct diagnosis and management. METHODS: Retrospective study from 2005 to 2015 in patients who were submitted to a probing procedure. We identified age at procedure, gender, laterality and presence or absence of success with the procedure. RESULTS: One hundred thirty-seven eyes were analyzed. The median age was 17 months and the total success rate was 85...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333839/-pa%C3%A3-ister-kiuian-syndrome-in-a-mexican-mestizo-patient-case-report
#4
Paola Mendelsberg-Fishbein, Constanza García-Delgado, Linda B Muñoz-Martínez, Maura Robledo-Cayetano, Leonardo J Mejía-Marín, Luis E Martínez-Barrera, Mabel Cerrillo-Hinojosa, Verónica F Moran-Barroso
Pallister-Killian syndrome is caused by a tetrasomy 12p mosaicism and is characterized by facial dysmorphism, pigmentary skin anomalies, congenital heart defects, diaphragmatic hernia, epilepsy and mental retardation. The diagnosis is complex as the cytogenetic analysis in blood is usually normal, requiring karyotyping in other tissues, therefore the clinical suspicion is critical to guide the diagnostic tests and the patient requires an interdisciplinary clinical evaluation regarding the several manifestation of the syndrome...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333838/solitary-median-maxillary-central-incisor-holoprosencephaly-and-congenital-nasal-pyriform-aperture-stenosis-in-a-premature-infant-case-report
#5
Ozkan Ilhan, Yeliz Pekcevik, Sinem Akbay, Senem A Ozdemir, Seyma Memur, Berat Kanar, Ozgur Kirbiyik, Esra A Ozer
Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities such as holoprosencephaly, nasal cavity anomalies, cleft palate-lip, hypotelorism, microcephaly, and panhypopituitarism. Congenital nasal pyriform aperture stenosis is a lethal cause of neonatal respiratory distress due to narrowing of the pyriform aperture anteriorly and it can be confused with choanal atresia. In this report, we present a newborn infant with solitary median maxillary central incisor syndrome accompanied by other abnormalities including holoprosencephaly, nasal pyriform aperture stenosis, microcephaly and panhypopituitarism...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333836/-phakomatosis-pigmentovascularis-cesioflammea-a-case-report
#6
María C Viada Peláez, Paola C Stefano, Ana Cirio, Andrea B Cervini
Phakomatosis pigmentovascularis (PPV) is a syndrome characterized by the association of a vascular nevus with a congenital pigmented lesion (epidermal nevus, nevus spilus, and dermal melanocytosis). There are different types of PPV according to the pigmentary nevus associated with the vascular malformation. Patients may present only the cutaneous condition or have systemic manifestations, among them, trauma, neurological and ophthalmological disorders. We report the case of a 1-year-old girl who had congenital glaucoma...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333834/-congenital-esophageal-stenosis-diagnosis-and-treatment-cases-review
#7
Enrique J Romero Manteola, Pablo Ravetta, Celeste C Patiño González, Víctor H Defago
Congenital esophageal stenosis is a very rare condition and there is no standard treatment. We report the diagnosis, treatment and outcome of 11 patients with this condition managed at our institution. The most common symptom was dysphagia. The age at diagnosis was between 1 day and 14 years (mean age: 4.7 years). The esophagogram confirmed the diagnosis. Five patients presented associated anomalies. Four patients received surgical treatment and 7 only balloon dilatations. Pathologic examinations showed 3 fibromuscular stenosis and one with tracheobronchial remnants...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333832/an-incidentally-detected-anomalous-origin-of-the-right-coronary-artery-from-the-pulmonary-artery-in-an-infant
#8
Sezen Ugan Atik, Levent Saltik, Kazım Oztarhan, Helen Bornaun
Isolated anomalous origin of the right coronary artery from the main pulmonary artery is a rare congenital anomaly, and few cases have been reported in the pediatric age group. Here in, we report an asymptomatic case of a 2-month-old male infant who has been diagnosed as anomalous origin of the right coronary artery from the main pulmonary artery during the evaluation for cardiac abnormalities. For a suspicion on echocardiography, cardiac catheterization and coronary angiography performed to verify the diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333828/-congenital-horner-syndrome-case-report
#9
Romina B Vera Olivera, Loreley FernándezI, Ana P Arias, Diego Ballarino, Andrea Jara
Horner syndrome is characterized by the following triad of clinical signs: miosis, ptosis and facial anhidrosis. In addition, iris heterochromia, conjunctival injection, facial erythema, congestive nasal mucosa and apparent enophthalmos secondary to the reduction of the palpebral fissure can appear. It is caused by an interruption of the sympathetic pathway that extends from the hypothalamus to the orbit. Because there is no decussation, the signs are homolateral to the lesion. Traditionally, it is classified as congenital and acquired...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333824/eosinophilic-esophagitis-and-esophageal-atresia-coincidence-or-causality
#10
Karen V Stave Salgado, Ana M Rocca
Eosinophilic esophagitis is an immune-mediated chronic disease of the esophagus characterized by symptoms related to esophageal dysfunction and tissue eosinophilia. In the endoscopy, the esophageal mucosa may appear normal or show exudates, rings, edema, furrows, and strictures. Its management is based on elimination diet, topical corticosteroids and/or esophageal dilation. Atresia is the most common congenital alteration of the esophagus; it requires surgical repair and poses potential complications, such as gastroesophageal reflux, strictures, and esophageal dysmotility...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333816/national-health-care-network-for-children-with-oral-clefts-organization-functioning-and-preliminary-outcomes
#11
Agustina Cassinelli, Nadia Pauselli, Agustina Piola, Claudia Martinelli, José L Alves de Azeved, María P Bidondo, Boris Groisman, Pablo Barbero, Rosa Liascovich, Ana Sala
INTRODUCTION: Oral clefts are major congenital anomalies that may affect the lip and/or palate, and that may also involve the nose and nostrils. In Argentina, their prevalence is approximately 15 per 10 000 births. In 2015, the Ministry of Health of Argentina created a national health care network for children with oral clefts in Argentina through the joint work with the National Registry of Congenital Anomalies (Red Nacional de Anomalías Congénitas, RENAC) (coordinating center for the national network) and the SUMAR Program...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333814/morbidity-in-congenital-heart-surgery-in-a-public-hospital-in-argentina
#12
María Althabe, Ricardo Rodríguez R, María Balestrini, Alberto Charroqui, Mariela Krynski, Ana M Lenz, Mercedes Montonati, Guillermo Moreno, María L Pilan, Ricardo Magliola, Pablo García Delucis
OBJECTIVE: To describe the complications associated with heart surgery, compare them to a reference population, and identify mortality risk factors. PATIENTS AND METHODS: Retrospective and descriptive study. All patients who underwent surgery at Hospital Garrahan in the 2013-2015 period were included. Age, weight, procedure, mechanical ventilation, length of stay in days, morbidity, and course were recorded. Renal failure requiring dialysis, neurological deficit, permanent pacemaker, circulatory support, phrenic nerve or vocal cord palsy, reoperation, wound infection, chylothorax, and tracheotomy were considered morbidities...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333813/body-composition-and-energy-expenditure-in-a-population-of-children-and-adolescents-with-myelomeningocele
#13
Carolina Caminiti, Carola Saure, Julieta Weglinski, Fernanda de Castro, Liliana Campmany
INTRODUCTION: Myelomeningocele is a congenital defect that occurs when the neural tube fails to close completely. It causes body composition alterations and a high prevalence of obesity. It is difficult to detect the most adequate indicator for a nutritional diagnosis due to the impossibility of recording accurate anthropometric measurements. OBJECTIVE: To describe body composition, resting energy expenditure and metabolic disorders in a population of patients with myelomeningocele managed at "Hospital Garrahan" by comparing obese patients with myelomeningocele and a control population with multifactorial obesity...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333811/neonatal-mortality-and-associated-factors-in-newborn-infants-admitted-to-a-neonatal-care-unit
#14
Juan C Lona Reyes, René O Pérez Ramírez, Leonardo Llamas Ramos, Larissa M Gómez Ruiz, Edith A Benítez Vázquez, Virginia Rodríguez Patino
INTRODUCTION: The increasing survival rate of preterm infants has altered the epidemiology of neonatal diseases; however, neonatal mortality is still the main component of child mortality. The objective of this study was to evaluate neonatal mortality and associated factors in newborn infants admitted to a neonatal care unit. MATERIAL AND METHODS: Prospective cohort study conducted between January 2016 and January 2017 at Hospital Civil de Guadalajara "Dr. Juan I...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333426/are-you-prepared-for-pancreas-bifidum-a-case-report
#15
Jae Ryong Shim, Sang-Jae Park, Hyung Min Park, Eung Chang Lee, Sung-Sik Han
Pancreas divisum-failure of fusion of the dorsal and ventral pancreatic ducts-is relatively well known as the most common congenital anomaly of the pancreatic duct, of with an incidence approximately 10% of all embryos. And there is a rare anomaly similar to pancreas divisum in which doubled ducts are formed. This condition is a rare developmental anomaly called pancreas bifidum or bifid pancreas or fish tail pancreas. This report describes a patient with pancreas bifidum who had 2 separated ducts within the pancreas from tail to neck but did not have a separated parenchyma...
January 2018: Annals of Surgical Treatment and Research
https://www.readbyqxmd.com/read/29333373/correction-of-congenital-cleft-earlobe-with-front-and-back-flaps
#16
Selman Karaci, Rüştü Köse
Congenital auricular deformities may be either deformational or malformational. Malformational anomalies present with a skin or cartilage shortage. Two cases with congenital cleft earlobe were presented. A new surgical technique using a two-layered repair with front and back flaps were introduced. She waited to begin wearing earrings until six months after the surgery to prevent possible scar contracture. The patients were followed up for a period ranging from 3 to 14 months. The earlobe volume deficiency was replaced, and acceptable scar maturation was obtained...
December 2017: Journal of the Korean Association of Oral and Maxillofacial Surgeons
https://www.readbyqxmd.com/read/29333222/congenital-left-atrial-bands-with-atrial-fibrillation
#17
Won Young Jang, Woohyeun Kim, Eun Jin Park, Jah Yeon Choi, Cheol Ung Choi, Hong Euy Lim, Jin Won Kim, Eung Ju Kim, Seung-Woon Rha, Jin Oh Na
No abstract text is available yet for this article.
December 2017: Journal of Cardiovascular Ultrasound
https://www.readbyqxmd.com/read/29333032/macrodystrophia-lipomatosa-clinico-patho-radiological-correlation
#18
Rakesh Mehra, Richa Bhartiya, Pallavi Agrawal, Nawanita Kumari
Macrodystrophia lipomatosa is a rare congenital nonhereditary developmental anomaly. It is characterized by hamartomatous proliferation of the soft tissue leading to disproportionate enlargement of the limbs and digits. Since it leads to diagnostic dilemma, it has to be differentiated from various other conditions as they differ in course, prognosis, complications, and treatment. Herein, we present two cases with localized gigantism and discuss the various differential diagnoses and need for clinico-patho-radilogical correlation for diagnosis of this rare entity...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29332925/pulmonary-artery-pseudoaneurysm-secondary-to-lung-inf-lammation
#19
Shinichirou Ishimoto, Hiroyuki Sakurai, Ryouta Higure, Riken Kawachi, Mie Shimamura
Pulmonary artery aneurysms (PAA) and pseudoaneurysms (PAP) are caused by infections, vasculitis, trauma, pulmonary hypertension, congenital heart disease, and connective tissue disease. Most cases of such aneurysm occur in the trunk or major branches of the pulmonary artery, while the peripheral type is less common. The treatment modalities are medical therapy, surgery, and percutaneous catheter embolization. The mortality rate associated with rupture is approximately 50%. We encountered a case of a 53-year-old man with a pulmonary artery pseudoaneurysm secondary to pneumonia and cavity formation during chemotherapy for acute myeloid leukemia (AML)...
January 15, 2018: Annals of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29332913/early-extubation-in-the-operating-room-after-congenital-open-heart-surgery
#20
Takuma Fukunishi, Norihiko Oka, Takeshi Yoshii, Kensuke Kobayashi, Nobuyuki Inoue, Tetsuya Horai, Tadashi Kitamura, Hirotsugu Okamoto, Kagami Miyaji
Early extubation in the operating room after congenital open-heart surgery is feasible, but extubation in the intensive care unit after the operation remains common practice at many institutions. The purpose of this study was to evaluate retrospectively the adequacy of our early-extubation strategy and exclusion criteria through analysis based on the Risk Adjustment in Congenital Heart Surgery method (RACHS-1).This retrospective analysis included 359 cases requiring cardiopulmonary bypass (male, 195; female, 164; weight > 3...
January 15, 2018: International Heart Journal
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