Assessment of Matrix Metalloprotease - 7 (MMP7) Immunohistochemistry in Biliary Atresia and Other Pediatric Cholestatic Liver Diseases.

Background and aims: Biliary atresia (BA) is a progressive fibro-obliterative cholangiopathy. The histopathological diagnosis is often challenging and an immunohistochemical marker is often sought as an adjunct. We evaluated MMP7 immunohistochemistry in BA and other non-BA pediatric cholestatic liver diseases. Materials and methods: MMP7 immunohistochemistry was applied in 5 age-matched normal control, 23 cases of BA and 43 cases of non-BA pediatric cholestasis including 16 cases of choledochal cyst (CC), and a multiplication score was obtained by multiplying the intensity and percentage positivity in the cholangiocytes. Results: BA showed a high mean MMP7 multiplication score which was significantly different from the normal control and other non-BA pediatric cholestatic diseases including CC (p value < 0.001). The sensitivity, specificity, positive, and negative predictive values of MMP7 immunohistochemistry were 91.3%, 93.02%, 87.5%, and 95.2% respectively. Conclusion: MMP7 immunohistochemistry may be an adjunct to histomorphology in BA.

Idiopathic Pericardial Ossification Causing Chronic Constrictive Pericarditis.

BACKGROUND: Chronic constrictive pericarditis (CCP) is usually caused by the fibroinflammatory reaction of the visceral and parietal pericardium that encase the heart. The cause of CCP is various including tuberculosis, trauma, prior surgery, radiation, and malignancy. MATERIAL AND METHODS: We examined the pericardiectomy specimen of a case of CCP in a 17-year-old boy. RESULTS: The histopathology of the pericardium revealed pericardial ossification bony remodeling and hematopoiesis within the intertrabecular marrow spaces. No granulomatous or neoplastic etiology was identified. CONCLUSION: Idiopathic pericardial ossification can cause CCP in pediatric patients.

Transient Abnormal Myelopoiesis: An Abnormal Course and the Efficacy of Delayed Treatment.

Transient abnormal myelopoiesis (TAM) is observed in a few neonates with Down syndrome. While a large proportion undergo complete remission without any treatment, some of them can develop myeloid leukemia of Down syndrome (ML-DS) in the future. Without proper treatment, mortality can be high. Here we have described an interesting and difficult-to-treat case of a neonatal with Down syndrome who presented with anemia, thrombocytopenia, and 75% blasts. We came across multiple challenges in treatment due to severe pneumonia.

Gingival hyperplasia: An initial oral manifestation of acute myeloid leukemia.

Various systemic diseases can manifest oral signs and symptoms early, which may be crucial for diagnosis and outlining the treatment plan. This case report highlights the presentation of acute leukemia (a malignancy of white blood cells) in a young female. An 11-year-old girl presented with gingival overgrowth and bleeding from the gingiva, weakness, and recent history of weight loss. A detailed workup consisting of complete blood count, bone marrow examination, flow cytometric immunophenotyping, cytogenetics, and molecular studies were carried out. The investigations confirmed the infiltration of blast cells of myelomonocytic origin, and a confirmatory diagnosis of acute myeloid leukemia (French-American-British classification M5) was made. The patient was put on induction chemotherapy and responded well. She developed febrile neutropenia following chemotherapy, which was managed conservatively. Gingival overgrowth subsided after the chemotherapy, and at the time of discharge, she was asymptomatic and hemodynamically stable. The oral health-care professionals must recognize that gingival overgrowth/enlargement may represent an initial manifestation of an underlying systematic disease.