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involution syndrome

Brian Z Rayala, Dean S Morrell
Congenital melanocytic nevi (CMN) are hamartomas present at birth that are composed of nevomelanocytes and thought to originate from faulty migration of precursor melanocytes in the neural crest. Classification is based on projected adult size of the lesion. CMN size correlates positively with risk of melanoma and neurocutaneous melanocytosis. Management requires a patient-centered approach that weighs the risks and benefits of and alternatives to complete removal. All children with large and giant CMN, regardless of surgical status, should be monitored closely and undergo periodic skin examination...
February 2017: FP Essentials
R F Ashman, N Singh, P S Lenert
MRL-Fas (lpr/lpr) mice represent an excellent animal model for studying non-malignant lymphoproliferation, regeneration and systemic autoimmunity. Retro-transposon insertion into the second intron of the pro-apoptotic Fas gene appears to be responsible for both lymphoproliferation and autoimmunity, while other genes are more likely to contribute to the regenerative healing characteristic of this mouse strain. Previous studies have shown that neonatal thymectomy can halt the development of abnormal lymphoproliferation...
June 2017: Lupus
V Villamil, N A Méndez Aguirre, T Martínez Menchón, O Girón Vallejo, M Fernández Ibieta, P Y Reyes Ríos, I Martínez Castaño, J Rojas Ticona, A Sánchez Sánchez, L Hernández Sánchez, J I Ruiz Jiménez
INTRODUCTION: Segmental cervico facial hemangiomas are defined as those longer than 5 cm, affecting a specific facial area. These lesions can be eventually associated with the PHACE syndrome. Our aim is to propose neonatal treatment with propranolol, showing its efficacy/safety, given the scarce evidence on its neonatal use. CLINICAL OBSERVATION: After written informed consent, four patients with segmental facial hemangioma were treated with propranolol in the neonatal period...
July 20, 2015: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
Bjorn R Olsen, Agnes D Berendsen, Tatiana Y Besschetnova, Xuchen Duan, Kai Hu
Studies of proliferative hemangiomas have led to the discovery that interactions of endothelial cells with extracellular matrix and/or Vascular Endothelial Growth Factor (VEGF)-A stimulate the expression of VEGFR1, the VEGF decoy receptor, and suppress VEGF-dependent VEGFR2 signalling by a mechanism that requires the matrix-binding receptor Anthrax Toxin Receptor (ANTXR)1, VEGFR2, β1 integrin and the Nuclear Factor of Activated T cells (NFAT). In hemangioma endothelial cells, all these components are present, but are functionally compromised, so that the levels of VEGFR1 are extremely low and VEGFR2 signalling is constitutively active...
August 2016: International Journal of Experimental Pathology
Anahita Dua, Lisa McElroy, Abby Wochinski, Johnny C Hong, David C Cronin
Successful liver transplantation typically results in an immediate decrease in intrahepatic resistance accompanied by an initial increased hepatopedal portal flow. Within a short period of time, the portal hypertension resolves and the variceal shunts involute. However, in situations in which intrahepatic vascular resistance to venous flow remains elevated, significant hepatofugal portal flow may continue through persistent mesenteric shunts. This situation, portal steal, can result in decreased perfusion of the liver graft leading to graft dysfunction, failure, and potentially recipient death...
June 2016: WMJ: Official Publication of the State Medical Society of Wisconsin
R R Osmanov
Abstract The results of endovascular laser ablation (EVLA) under ultrasonographic control in treatment of the lower extremities varicose disease (LEVD) in 189 patients (214 extremities) were analyzed. There was established, that for the trustworthy information obtaining the ultrasonographic duplex angioscanning conduction is necessary. The fibrous involution of a big subcutaneous vein trunk was achieved in (97.7 ± 1.0)% observations, in (1.9 ± 0.9)%--while the clinical signs absence--there were revealed the echo-signs of a reflux recurrence--the "ultrasonographic recurrence", and in one observation--a clinical recurrence of LEVD...
February 2016: Klinichna Khirurhiia
Dhanesheshwar N Lanjewar, Varsha Omprakash Bhatia, Sonali Dhaneshwar Lanjewar
BACKGROUND: Human immunodeficiency virus (HIV) infection in India has now been prevalent over three decades, and an increasing number of children are being affected with HIV. The spectrum of pathologic lesions in children with acquired immunodeficiency syndrome (AIDS) in India has not been well described. MATERIALS AND METHODS: A review of systematically conducted autopsies of 11 (10 boys and 1 girl) children with AIDS is presented. RESULTS: The mode of HIV transmission in 6 children was vertical; in one it was blood transfusion and in 4 children route was presumably vertical as these were children of orphanage...
April 2016: Indian Journal of Pathology & Microbiology
N Harrasser, I Banke, H Gollwitzer, F Wilken, A Toepfer, R von Eisenhart-Rothe, M Hauschild
INTRODUCTION: Gluteal insufficiency is a common and challenging complaint. New concepts in pathobiomechanics and improved clinical understanding of chronic gluteal dysfunction have unmasked gluteus medius (GMed) tears as an underlying cause of enhanced trochanteric pain syndrome (GTPS). These tears are often missed or misdiagnosed as bursitis, but lead to prolonged chronic peritrochanteric pain. Clinic: The clinical signs are often dull pain on the lateral hip aspect, reduced hip abduction strength with positive Trendelenburg testing and a tendency for the leg to external rotation, as the internal rotation strength is reduced...
April 2016: Zeitschrift Für Orthopädie und Unfallchirurgie
Ivo J H M de Vos, Alexander P A Stegmann, Carroll A B Webers, Constance T R M Stumpel
Anterior segment dysgeneses are developmental anomalies of the anterior eye segment that can occur as isolated defects or as part of various syndromes. A subgroup is caused by abnormal embryonic neural crest development. The Axenfeld-Rieger syndrome is an umbrella term for a continuum of anterior segment dysgeneses of neural crest origin, characterized by the presence of the Axenfeld or Rieger eye malformation predisposing for glaucoma. Additionally, other structures of neural crest origin can be variably affected giving rise to a wide spectrum of associated extra-ocular malformations...
March 2017: Ophthalmic Genetics
Atalay Ekin, Cenk Gezer, Cuneyt Eftal Taner, Ulas Solmaz, Naciye Sinem Gezer, Mehmet Ozeren
OBJECTIVES: Our aim was to evaluate the size of the fetal thymus by sonography in pregnancies with intrauterine growth restriction (IUGR) and to search for a possible relationship between a small fetal thymus and adverse perinatal outcomes. METHODS: The transverse diameter of the fetal thymus was prospectively measured in 150 healthy and 143 IUGR fetuses between 24 and 40 weeks' gestation. The fetuses with IUGR were further divided according to normal or abnormal Doppler assessment of the umbilical and middle cerebral arteries and ductus venosus...
March 2016: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
Tomonori Fuke, Tohru Takahashi, Yoshiyuki Yamada, Mitsuhiro Miyashita, Naoji Amano, Masaaki Matsushita
A large number of case studies on Cotard's syndrome have reported that this syndrome develops after repeated episodes of depression in the presenile stage of life. Therefore, it has been defined as a severe type of affective spectrum disorder. This report describes three patients who exhibited symptoms characteristic of Cotard's syndrome, such as negative thoughts and delusions of immortality, in the presenile and senile stages of their lives. They also had a history of long-term treatment for schizophrenia based on a diagnosis in early adulthood...
2015: Seishin Shinkeigaku Zasshi, Psychiatria et Neurologia Japonica
Lillian Abreu Dias, Eduardo H Morizot
PURPOSE: To characterize multiple evanescent white dot syndrome (MEWDS)-like lesions as diagnosed by clinical exam and fluorescein angiography (FA) to build an epidemiological profile of this disease and highlight the most common angiographical aspects. It is important to emphasize the existence of this syndrome and improve patients' information about the natural good course of this disease. METHODS: A cross-sectional study including all FA performed from July 2006 to October 2012 (6,111 exams analyzed) in patients at our ophthalmic emergency department, with analysis of the different angiographic aspects of the MEWDS-diagnosed cases...
July 2015: Arquivos Brasileiros de Oftalmologia
Pieta R Winter, Tinte Itinteang, Philip Leadbitter, Trevor FitzJohn, Swee T Tan
The authors present a case of PHACE(S) (posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects, eye anomalies, and sternal cleft or supraumbilical raphe) syndrome with a right-sided segmental infantile hemangioma, and describe in detail, the associated absent ipsilateral intracranial internal carotid artery and anomalous Circle of Willis. Propranolol therapy led to accelerated, complete involution. Nadolol may reduce the theoretical risk of treating PHACE(S) patients with β-blockers...
June 2015: Journal of Craniofacial Surgery
Ombretta Salvucci, Hidetaka Ohnuki, Dragan Maric, Xu Hou, Xuri Li, Sung Ok Yoon, Marta Segarra, Charles G Eberhart, Amparo Acker-Palmer, Giovanna Tosato
Angiogenesis produces primitive vascular networks that need pruning to yield hierarchically organized and functional vessels. Despite the critical importance of vessel pruning to vessel patterning and function, the mechanisms regulating this process are not clear. Here we show that EphrinB2, a well-known player in angiogenesis, is an essential regulator of endothelial cell death and vessel pruning. This regulation depends upon phosphotyrosine-EphrinB2 signalling repressing c-jun N-terminal kinase 3 activity via STAT1...
2015: Nature Communications
Didier Bessis, Michèle Bigorre, Christine Labrèze
BACKGROUND: Reticular infantile hemangioma (RIH) with minimal or arrested growth (MAG) is an underrecognized variant of infantile hemangioma (IH). OBJECTIVE: We describe a new clinical subtype of RIH-MAG associated with lipoatrophy. METHODS: The medical charts and serial clinical photographs and imaging studies of 53 children given a diagnosis between 2004 and 2013 labeled as "abortive," "minimal or arrested growth," "reticular," or "congenital" hemangiomas were reviewed in the departments of dermatology and infantile plastic surgery of 2 French university hospital centers...
May 2015: Journal of the American Academy of Dermatology
L Viart, J Peltier, T Forzini, C Page, P Foulon, F Saint, E Havet
We report the case of a 35-year-old patient with a syndrome of persistent Müllerian ducts (PMDS) of the female type (group A). The diagnosis was made in adulthood during an infertility workup. Clinical examination revealed an empty scrotum, a normal penis and bilateral inguinal cystic masses. The spermogram found azoospermia. Imaging using MRI and tomotensidometry found the presence of an uterus, two fallopian tubes and two inguinal positions of polycystic testes. A surgical management was performed for surgical testicular biopsy...
March 2015: Morphologie: Bulletin de L'Association des Anatomistes
Allyson A Spence-Shishido, William V Good, Eulalia Baselga, Ilona J Frieden
Infantile hemangiomas are a common vascular birthmark with heterogeneous presentations and unique growth characteristics with early rapid growth and eventual self-involution. Hemangiomas that develop around the eye have the potential for inducing amblyopia by several mechanisms and may eventually result in permanent visual impairment in otherwise healthy infants. Segmental periocular hemangiomas carry the additional risk of associated structural anomalies and PHACE syndrome. In recent years, the treatment of periocular hemangiomas has been revolutionized by the serendipitous discovery of the effectiveness of beta-blockers (systemic and topical), with most experts viewing these as first-line therapies...
March 2015: Clinics in Dermatology
Yael Sciaky-Tamir, Reli Hershkovitz, Moshe Mazor, Ilan Shelef, Offer Erez
The fetal inflammatory response syndrome (FIRS) describes a state of extensive fetal multi organ involvement during chorioamnionitis, and is associated with grave implications on perinatal outcome. The syndrome has been linked to the preterm parturition syndrome and is associated with inflammation/infection processes in most of the fetal organs. The fetal thymus, a major organ in the developing immune system involutes during severe neonatal disease and has been shown to be smaller in fetuses with FIRS. Various methods for imaging of the fetal thymus and measurement are described...
May 2015: Prenatal Diagnosis
D S MacDonald
During the last decade much has changed in our understanding of fibro-osseous lesions (FOLs) of the jaws with regards to their imaging, their nomenclature and classification, and their potential impact on the overall health of the patient. The changes in nomenclature, classification, and the FOLs' potential association with important syndromes are discussed with the assistance of a flowchart. The lesions, fibrous dysplasia (FD), ossifying fibroma (OF), and osseous dysplasia (OD), though with similar histopathology, have very different clinical and radiological presentations, behaviour, and treatment outcomes...
January 2015: Clinical Radiology
Helene S Weibel, Michael H Dahan
BACKGROUND: Premature ovarian failure (POF) is described as estrogen deficiency, amenorrhea, and hypergonadotropinemia in a woman < 40 years old. In a proportion of patients diagnosed with POF, intermittent and unpredictable return of ovarian function can be observed, causing fluctuations of follicle-stimulating hormone (FSH). However, these patients also have return of menstrual cycles. When cycles do not resume, other causes could explain the changes in FSH levels. CASE: A 43-year-old woman with known premature ovarian failure since age 23 and high serum FSH levels was referred for normalization of FSH levels...
September 2014: Journal of Reproductive Medicine
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