Connective tissue nevus in Marfan Syndrome successfully treated with intralesional steroid injections

2021 ◽  
Vol 156 (5) ◽  
Author(s):  
Valeria MANFREDA ◽  
Lorenzo CERRONI ◽  
Miriam TEOLI ◽  
Luca BIANCHI ◽  
Alessandro GIUNTA
Keyword(s):  
Connective Tissue ◽  
Marfan Syndrome ◽  
Steroid Injections ◽  
Intralesional Steroid

Banded aggregates containing fibrillin are present in the cartilage matrix adjacent to chondrocytes in individuals affected with scoliosis

1992 ◽  
Vol 50 (1) ◽  
pp. 892-893
Author(s):  
Douglas R. Keene ◽  
Magaret Fairhurst ◽  
Catherine C. Ridgway ◽  
Lynn Y. Sakai
Keyword(s):  
Connective Tissue ◽  
Marfan Syndrome ◽  
Cross Section ◽  
Immunoelectron Microscopy ◽  
Cartilage Matrix ◽  
Negative Stain ◽  
Gene Coding ◽  
Rotary Shadowing

Matrix microfibrils are present in the connective tissue matrices of all tissues. Following standard TEM processing, they appear in cross section as cylindrical fibrils 8-10 nm in diameter, often associated with amorphous elastin. They are also seen in the absence of amorphous elastin, for example in the shallow papillary layer of skin, and also in cartilage matrix (Figure 1). Negative stain and rotary shadowing studies suggest that microfibrils are composed of laterally associated globular structures connected by fine filamentous strands (“ beaded strings”), and that they are extendable. Immunoelectron microscopy has demonstrated that fibrillin, a 350 Kd glycoprotein, is distributed along all microfibrils with a relaxed periodicity of about 54 nm The gene coding for fibrillin has recently been identified and is defective in the Marfan syndrome.

scholarly journals A Case Report of Marfan Syndrome with Literature Review

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Clinical Signs ◽  
Connective Tissue Disorder ◽  
Multiple Organ ◽  
Review Of The Literature ◽  
Organ Systems ◽  
Loin Pain ◽  
Routine Physical Examination

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>

Therapeutic Effect of Superficial Cryotherapy versus Intralesional Corticosteroids injection in Alopecia Areata

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Nour El-dissouki Ibrahim ◽  
Mahira Hamdy Elsayed ◽  
Ahmed Abdel-Fattah Afify
Keyword(s):  
Side Effects ◽  
Alopecia Areata ◽  
Response To Treatment ◽  
High Dose ◽  
Efficacy And Safety ◽  
Steroid Injections ◽  
Intralesional Steroid ◽  
Males And Females ◽  
Intravenous Steroids ◽  
Effects Assessment

Abstract Alopecia areata, one or more round bald patches appear suddenly, most often on the scalp. It can affect males and females at any age. It starts in childhood in about 50%, and before the age of 40 years in 80%. The exact mechanism is not yet understood. There is not yet any reliable cure for alopecia areata. Several topical treatments used for alopecia areata are reported to result in temporary improvement in some people. Their role and efficacy are unknown. Injections of triamcinolone acetonide 2.5–10 mg/ml into patchy scalp, its efficacy is temporary. Oral and pulse intravenous steroids in high dose can lead to temporary regrowth of hair. The sensitisers diphenylcyclopropenone provoke hair growth in treated areas Now, superficial cryotheray is introduced as a possible treatment for Alopecia Areata. With minimial side effects and being less painful. So, this study clarifies the therapeutic efficacy and safety of superficial cryotherapy for treatment of patchy Alopecia areata. Aim of the study The aim of the study is to evaluate and compare the efficacy and safety of Superficial Cryotherapy and Intralesional corticosteroids in the treatment of patchy Alopecia Areata. Patients and methods study included 20 patients complaining of Alopecia Areata. 3 patches of scalp alopecia areata will be randomly subjected to either superficial cryotherapy twice monthly for 3 months (3 cycles, 2-3 seconds) or intralesional steroid injections once monthly for 3 months (triaminoclone acetonide 1:7, 1ml) or intralesional saline (1 ml). Assessment for the response to treatment will be done at one month and three month following treatment by digital photography and by phototrichogram by comparing number of Terminal, and vellus and hair thickness, Also assessment for side effects of therapy will be done. Results Patients treated with TCA showed statistically significant high response compared to patients treated with cryotherapy. Also, side effects assessment showed minimal side effects with treatment with TCA compared to cryotherapy. Conclusion TCA treatment of alopecia areata is much tolerable than treatment with cryotherapy with also better response rates.

scholarly journals Dural ectasia in Marfan syndrome and other hereditary connective tissue disorders: a 10-year follow-up study

2019 ◽  
Vol 19 (8) ◽  
pp. 1412-1421 ◽  
Author(s):  
Tordis Böker ◽  
Thy Thy Vanem ◽  
Are Hugo Pripp ◽  
Svend Rand-Hendriksen ◽  
Benedicte Paus ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Marfan Syndrome ◽  
Connective Tissue Disorders ◽  
Dural Ectasia ◽  
Follow Up Study

A Case Report of Marfan Syndrome with Literature Review

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Clinical Signs ◽  
Connective Tissue Disorder ◽  
Multiple Organ ◽  
Review Of The Literature ◽  
Organ Systems ◽  
Loin Pain ◽  
Routine Physical Examination

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>

The effect of intralesional steroid injections on esophageal strictures and the child as whole: A case series

2020 ◽  
Vol 55 (4) ◽  
pp. 646-650 ◽  
Author(s):  
Chantal A. ten Kate ◽  
John Vlot ◽  
Cornelius E.J. Sloots ◽  
Erica L.T. van den Akker ◽  
Rene M.H. Wijnen
Keyword(s):  
Case Series ◽  
Steroid Injections ◽  
Esophageal Strictures ◽  
Intralesional Steroid
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