The Constriction Ring of Amniotic Band Syndrome Reveals Idiosyncrasies of Wound Repair in Infancy

ABSTRACTWe hypothesized that the constriction rings in Amniotic Band Syndrome (ABS) are the sequelae of localized mechanical injury. Typical scarring pattern was examined in skin tissue of ABS origin, containing an amniotic band constriction, for collagen and elastin distribution, and the ratio of collagen I to collagen III (CI:CIII). A skin sample from an extra finger was the control. In the ABS specimens, sub-epidermal structures were intact and present throughout, and collagen I exhibited a normal basket-weave pattern. At the site of constriction in both ABS samples, reticular dermis elastin fibers were fragmented and papillary dermis elastin fibers were absent. In the control tissue, the reticular dermis contained relatively thick, branching fibers of elastin, and papillary dermis elastin was present.The elastin fragmentation at the constriction ring indicates localized elastin remodeling in response to injury. The absence of elastin in the papillary dermis of the constriction likely indicates a localized disruption in elastin formation. The formation and deposition of collagen and the presence of sub-epidermal structures favor a non-scarring phenotype, while the organization of elastin favors that of localized scarring.Summary StatementA rare, fetal model of healing after constrictive injury of skin, in which elastin fragmentation at the constriction injury indicates remodeling explained by the differential expression of elastin during gestation.

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Matrix protein organization near the constriction ring of amniotic band syndrome reveals idiosyncrasies of wound repair in infancy

Journal of the American Academy of Dermatology ◽

10.1016/j.jaad.2017.04.645 ◽

2017 ◽

Vol 76 (6) ◽

pp. AB166

Keyword(s):

Wound Repair ◽

Matrix Protein ◽

Amniotic Band ◽

Constriction Ring ◽

Amniotic Band Syndrome

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SURGICAL TREATMENT OUTCOME OF AMNIOTIC BAND SYNDROME (ABS) INVOLVING THE FINGER AND LEG WITH INFECTION OF A FOUR-MONTHS MALE CHILD: A CASE REPORT

Biomedika ◽

10.23917/biomedika.v12i2.10562 ◽

2020 ◽

Vol 12 (2) ◽

pp. 90-97

Author(s):

Hendra Cahya Kumara ◽

Pamudji Utomo ◽

Umar Kharisma Islami ◽

Adhitya Indra Pradhana

Keyword(s):

Treatment Outcome ◽

Surgical Treatment ◽

Daily Living ◽

Proximal Phalanx ◽

Middle Finger ◽

Amniotic Band ◽

Left Hand ◽

Constriction Ring ◽

Knee Amputation ◽

Amniotic Band Syndrome

ABSTRACTAmniotic band syndrome is an uncommon congenital disorder without any genetic or hereditary predisposition factor. It involves fetal entrapment in strands of amniotic tissue and causes an array of deletions and deformations. The aim of this article was to report surgical treatment outcome of amniotic band syndrome finger and infected intrauterine leg amputation. A case of four months male child with complaint of incomplete formation and constricting band on his left leg was reported. On the stump of left leg initially there was a small lump. Over time the size of the stump grew bigger and infected wound appeared. Other deformities were constricting band on proximal phalanx of the middle finger of left hand, acrosyndactyly of first and second toe of right foot and congenital scrotalis hernia. We performed a release surgery of constriction ring band of proximal phalanx of the middle finger of left hand with z-plasty incision and below knee amputation for left leg. We followed up patient in one year after operation. The patient complained no pain and no sign of infection. Patient could walk normally and independently with good activity daily living. We concluded that procedure with z-plasty incision had good result and avoided morbidity. Below knee amputation procedure and application of suitable prosthesis provided satisfying outcome on patient activity daily living and ambulation. Key words: Amniotic band syndrome, intrauterine amputation, z-plasty, infectioABSTRAKSindrom amniotic band merupakan kelainan bawaan yang jarang terjadi dan tanpa adanya kecenderungan faktor genetik atau keturunan. Kelainan ini diakibatkan terlilitnya janin dalam untaian jaringan amnion dan menyebabkan berbagai jenis kehilangan dan kecacatan. Tujuan dari artikel ini adalah melaporkan luaran tatalaksana operasi sindrom amniotic band pada jari tangan dan amputasi pada tungkai yang terinfeksi intrauterine. Pasien adalah seorang anak laki-laki empat bulan dengan amputasi tungkai bawah kiri disertai constriction band pada bagian proksimal. Pada ujung tungkai bawah kiri didapatkan benjolan dengan ukuran minimal yang makin lama ukuran sisi distal menjadi lebih besar dan tampak luka dengan nanah. Kelainan bentuk lainnya adalah constricting band pada phalanx proksimal jari tengah tangan kiri, acrosyndactyly jari kaki pertama dan kedua kaki kanan serta congenital hernia skrotalis. Kami melakukan operasi release constriction ring band phalanx proximal jari tengah tangan kiri dengan irisan z-plasty dan below knee amputation tungkai kiri. Follow up pasien setelah operasi satu tahun. Tidak ada keluhan nyeri, tidak ada tanda infeksi, pasien dapat berjalan normal dan aktivitas sehari-hari dengan mandiri. Kesimpulan bahwa prosedur dengan irisan z-plasty memberikan hasil yang baik dan menghidari morbiditas pasien. Prosedur below knee amputation dan penggunaan prosthesis yang tepat memberikan hasil yang memuaskan pada aktivitas sehari-hari dan ambulasi pasien. Kata kunci: Sindrome amniotic band, amputasi intrauterine, z-plasty, infeksi

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106 MMP-1, TIMP-1, collagen I and collagen III genes expression in myocardium from patients with obstructive HCM

European Journal of Heart Failure Supplements ◽

10.1016/s1567-4215(05)80064-5 ◽

2005 ◽

Vol 4 (1) ◽

pp. 25-25

Keyword(s):

Collagen I ◽

Genes Expression ◽

Collagen Iii

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Inhibition of Lysine 63 Ubiquitination Prevents the Progression of Renal Fibrosis in Diabetic DBA/2J Mice

International Journal of Molecular Sciences ◽

10.3390/ijms22105194 ◽

2021 ◽

Vol 22 (10) ◽

pp. 5194

Author(s):

Paola Pontrelli ◽

Francesca Conserva ◽

Rossella Menghini ◽

Michele Rossini ◽

Alessandra Stasi ◽

...

Keyword(s):

Diabetic Nephropathy ◽

Epithelial To Mesenchymal Transition ◽

Selective Inhibition ◽

Collagen I ◽

Protein Accumulation ◽

Mesenchymal Transition ◽

Collagen Iii ◽

Proximal Tubular Epithelial Cells

Diabetic nephropathy (DN) is the most frequent cause of end-stage renal disease. Tubulointerstitial accumulation of lysine 63 (K63)-ubiquitinated (Ub) proteins is involved in the progression of DN fibrosis and correlates with urinary miR-27b-3p downregulation. We explored the renoprotective effect of an inhibitor of K63-Ub (NSC697923), alone or in combination with the ACE-inhibitor ramipril, in vitro and in vivo. Proximal tubular epithelial cells and diabetic DBA/2J mice were treated with NSC697923 and/or ramipril. K63-Ub protein accumulation along with α-SMA, collagen I and III, FSP-1, vimentin, p16INK4A expression, SA-α Gal staining, Sirius Red, and PAS staining were measured. Finally, we measured the urinary albumin to creatinine ratio (uACR), and urinary miR-27b-3p expression in mice. NSC697923, both alone and in association with ramipril, in vitro and in vivo inhibited hyperglycemia-induced epithelial to mesenchymal transition by significantly reducing K63-Ub proteins, α-SMA, collagen I, vimentin, FSP-1 expression, and collagen III along with tubulointerstitial and glomerular fibrosis. Treated mice also showed recovery of urinary miR-27b-3p and restored expression of p16INK4A. Moreover, NSC697923 in combination with ramipril demonstrated a trend in the reduction of uACR. In conclusion, we suggest that selective inhibition of K63-Ub, when combined with the conventional treatment with ACE inhibitors, might represent a novel treatment strategy to prevent the progression of fibrosis and proteinuria in diabetic nephropathy and we propose miR-27b-3p as a biomarker of treatment efficacy.

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