Congenital Pilar and Smooth Muscle Nevus

PEDIATRICS ◽  
1987 ◽  
Vol 79 (6) ◽  
pp. 1021-1023
Author(s):  
ANDRE B. GVOZDEN ◽  
NANCY K. BARNETT ◽  
DEBORAH S. SCHRON

Dermatologists and pediatricians are often consulted by parents and other health professionals about the prognosis, management, and aesthetic effect of congenital nevi. In this paper we review the literature and report two new cases of a recently described and possibly underdiagnosed form of congenital lesion, the pilar and smooth muscle nevus. CASE REPORTS Case 1 A 6-month-old girl was evaluated for a skin lesion present since birth on her left flank. At the time of examination, it consisted of a well-defined brownish blue, lightly pigmented, barely visible macule with coarse blond hair, 6 x 7 cm (Fig 1). Upon stroking, slight, transient erection of the hair was appreciated.

2020 ◽  
Vol 11 ◽  
pp. 204209862090961
Author(s):  
Marina A. Malikova

Pharmacovigilance is a field where communication is crucial, and exchange of information is expected to be done in a timely manner. Information from individual case reports is transmitted from pharmaceutical industry and health professionals to the regulatory authorities. The safety profile of a drug is established by analyzing individual cases and aggregate reports. The cumulative information, obtained from these reports, can be used to assist pharmacovigilance professionals in the detection of potential safety signals by monitoring evolving trends. If there is a message identifying concern as potential safety signal, the transmission of individual case reports, as well as cumulative and aggregate reports will occur from pharmaceutical industry to the regulators; and based on their assessments of causality in relationship to the drug, the regulatory decisions will be made. Once regulators confirming a signal as a possible safety alert have made the decision, the decisions and the reasons must be communicated to health professionals, the pharmaceutical industry, and other parties involved (e.g. clinical trials participants, investigators, consumers and medical professionals at post-marketing stage, etc.).


Aquichan ◽  
2020 ◽  
Vol 20 (4) ◽  
pp. 1-13
Author(s):  
Suellen Rodrigues de Oliveira Maier ◽  
João Paulo Ferreira Rodrigues ◽  
Mayara Rocha Siqueira Sudré ◽  
Carina Aparecida Marosti Dessotte

Objective: To verify, based on the literature, what the main cardiac complications are in patients with COVID-19, during treatment in a hospital unit. Materials and methods: An integrative review, carried out by searching for studies in eight national and international databases. The final sample consisted of 16 studies published between January and May 2020, which were subjected to structural evaluation by the following instruments: Case Report Guidelines, for case reports; and Strengthening the Reporting of Observational Studies in Epidemiology, for observational studies. Results: The main cardiac complications were predominantly obstructive events, herein understood as acute myocardial infarction, followed by severe arrhythmic conditions and heart failure, all due to the hyperinflammation condition resulting from viral infection. Conclusions: The knowledge about cardiac complications during COVID-19 treatment has become important, since it can collaborate for the adoption of more effective treatment protocols and guide the assistance offered by health professionals, in order to identify such complications and intervene early.


2018 ◽  
Vol 11 (3) ◽  
pp. 729-734 ◽  
Author(s):  
Montreh Tavakkoli ◽  
Lisa Mueller

Ewing sarcoma is an aggressive mesenchymal malignancy. It is the second most common bone tumor among children and adolescents and less commonly presents as a soft tissue or primary skin lesion. Cutaneous Ewing sarcoma has only been reported in case reports and case series. In this article, we describe a 12-year-old Hispanic female cured of localized, cutaneous Ewing sarcoma (pT1aN0M0) at the 40-month follow-up following surgical resection and adjuvant chemotherapy according to the COG AEWS1031 protocol for Ewing sarcoma of the bone. To our knowledge, this is the first article to provide a potential biological explanation for the differences in the prognosis of Ewing sarcoma of the bone, soft tissue, and skin.


2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Mana Moghadamfalahi ◽  
Daniel S. Metzinger

Adenomyomas are benign tumors composed of smooth muscle and endometrial tissue. These tumors usually arise from the myometrium. Extrauterine adenomyomas are rare with only a few case reports available in the literature. Here, we report an unusual case of multiple adenomyomas in a 39-year-old woman six years after hysterectomy for multiple leiomyomata. To the best of our knowledge, this is the first case of extrauterine adenomyoma presenting as an upper abdominal mass.


1994 ◽  
Vol 1994 (Supplement72) ◽  
pp. 140-146
Author(s):  
Mamoru Fujii ◽  
Katsuhiro Hirakawa ◽  
Koji Yajin ◽  
Takaharu Tatsukawa

2021 ◽  
Vol 15 (58) ◽  
pp. 410-426
Author(s):  
Hermínia Moreira Coelho da Costa

Lesão pulmonar aguda relacionada à transfusão (TRALI –Transfusion Related Acute Lung Injury) é uma complicação grave da transfusão sanguínea que cursa essencialmente com desconforto respiratório durante ou dentro de 6 horas depois de completada a transfusão. Apesar de ser considerada rara e a lesão pulmonar ser geralmente transitória, traz preocupação aos profissionais da área da saúde, visto que está associada à alta morbidade dos pacientes que necessitaram de suporte ventilatório, assim como a alta mortalidade. Sua incidência não está bem estabelecida, muito devido à falta de preparo para identificar os casos suspeitos. Assim, os relatos de casos ganham relevância tanto para contribuir que o diagnóstico desta patologia seja alcançado com mais facilidade quanto para estimular que os casos sejam notificados. O presente relato descreve a ocorrência de TRALI em paciente no segundo dia de pós-operatório de cesárea por pré- eclâmpsia e síndrome Hellp que evoluiu com suspeita de hemoperitônio sendo submetida a laparotomia exploratória (LE).---Transfusion related acute lung injury (TRALI) is a serious complication of blood transfusion that evolves mainly with respiratory distress during or within 6 hours after transfusion. Although considered rare and is usually transient lung injury, she brings concern to health professionals, as it is associated with high morbidity of patients requiring ventilatory support, as well as the high mortality. Its incidence is not well established, much due to lack of preparation to identify suspected cases. Thus, case reports gain relevance to contribute to the diagnosis of this condition is more easily achieved as to stimulate the cases are reported. This report describes the occurrence of TRALI in a patient on the second day after surgery by cesarean preeclampsia and HELLP syndrome who developed suspected hemoperitoneum and underwent exploratory laparotomy (LE).


2018 ◽  
Vol 46 (3-4) ◽  
pp. 159-169 ◽  
Author(s):  
Joshua A. Cuoco ◽  
Christopher M. Busch ◽  
Brendan J. Klein ◽  
Michael J. Benko ◽  
Rachel Stein ◽  
...  

Background: Missense mutations in the gene that codes for smooth muscle actin, ACTA2, cause diffuse smooth muscle dysfunction and a distinct cerebral arteriopathy collectively known as multisystemic smooth muscle dysfunction syndrome (MSMDS). Until recently, ACTA2 cerebral arteriopathy was considered to be a variant of moyamoya disease. However, recent basic science and clinical data have demonstrated that the cerebral arteriopathy caused by mutant ACTA2 exhibits genetic loci, histopathology, neurological sequelae, and radiographic findings unique from moyamoya disease. We conducted a literature review to provide insight into the history, clinical significance, and neurosurgical management of this recently described novel cerebral arteriopathy. Summary: We performed a literature search using PubMed with the key words “ACTA2 mutation,” “ACTA2 cerebral arteriopathy,” and “multisystemic smooth muscle dysfunction syndrome.” Case reports with confirmed ACTA2 mutations and cerebral arteriopathy were included in our review. Our literature search revealed 15 articles (58 cases) of confirmed ACTA2 cerebral arteriopathy. Distinctive features of this arteriopathy included an aberrant internal carotid circulation with dilatation of the proximal segments, occlusive disease at the distal segments, and dolichoectasia. As such, mutant ACTA2 predisposed patients to ischemic strokes as children. Direct and indirect cerebral revascularization procedures are the mainstay treatment options with varying degrees of success. Key Messages: ACTA2 cerebral arteriopathy is a recently described novel cerebrovascular disease seen in patients with MSMDS. Patients currently diagnosed with moyamoya disease who also have dysfunction of smooth muscle organs may benefit from reevaluation by a medical geneticist and ACTA2 genotyping.


1999 ◽  
Vol 123 (9) ◽  
pp. 789-792 ◽  
Author(s):  
Kazuo Watanabe ◽  
Toshimitsu Suzuki

Abstract Objective.—Angiomyolipoma (AML) is a distinctive tumor that usually occurs in the kidney and rarely in the liver. Angiomyolipoma of other sites is extremely rare, and, to our knowledge, only 2 cases have been described in the nasal cavity. We present 2 additional cases of AML of the nasal cavity and discuss the difference between them and renal AML. Case Reports and Pathologic Findings.—Two tumors in a 66-year-old man and an 88-year-old woman without tuberous sclerosis are described. They showed 20-mm, well-circumscribed, polypoid shapes. Histologically, they were composed of mature smooth muscle cells, fat cells, and various-sized blood vessels. In addition, aggregated small lymphocytes were noted. Neither epithelioid smooth muscle cells nor HMB45 immunoreactivity was seen in either case. Conclusions.—The clinicopathologic features of AML of the nasal cavity are distinct from renal and hepatic AML and are common to those of AML arising in the skin and oral and pharyngeal mucosa. The term mucocutaneous angiomyolipoma is thought to be appropriate to express these characteristic tumors.


2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Sunitha Thomas ◽  
Laxmi Radhakrishnan ◽  
Latha Abraham ◽  
Anna Matthai

Angioleiomyomas are benign mesenchymal tumours commonly occurring in the subcutis of extremities. They are typically composed of interlacing fascicles of smooth muscle cells with intersecting vascular channels. Angioleiomyomas of the uterus are rare with only very few case reports available in literature. Herein, we report a case of this rare entity in a 47-year-old woman owing to its highly unusual features of cellular atypia, raised CA-125 levels, and pseudo Meigs syndrome.


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