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The authors aim to identify criteria for the diagnosis of Intestinal Visceral Myopathy (IVM), results were compared with ultrastructural studies (US). Six IVM patients and 7 paediatric control cases (without gastrointestinal diseases) were studied. One case was a typical Megacystis-Mycrocolon-Intestinal Hypoperistalsis Syndrome (MMIHS). The diagnostic path included: Rectal Suction Biopsy, One-Trocar Transumbilical Laparoscopic intestinal full-thickness Biopsy technique (OTTLB). Pathological analysis included anti-alpha smooth muscle actin staining, and US study of intestinal biopsies. IVM histological examination demonstrated thinning of longitudinal muscle layer. The ratio circular/longitudinal thickness was evaluated in all samples, in cases this ratio presented a mean value = 2.91; in controls a mean value = 1.472 (p = 0.0002). Ultrastructural diagnosis revealed variable myofibrils density in smooth muscle cells, irregularity of sarcolemma membranes, interstitial fibrosis, and myofiber disarray. The Authors concluded that in IVM, Circular/longitudinal thickness ratio and alpha smooth muscle actin staining can be used as significant tools to address the diagnosis.

Diagnostic Criteria of Pediatric Intestinal Myopathies / Lombardi, Laura; Bruder, Elisabeth; Pio, Luca; Nozza, Paolo; Thai, Elena; Lerone, Margherita; Del Rossi, Carmine; Mattioli, Girolamo; Silini, Enrico Maria; Paraboschi, Irene; Martucciello, Giuseppe. - In: JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION. - ISSN 0277-2116. - 66:3(2018), pp. 383-386. [10.1097/MPG.0000000000001727]

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