Turner syndrome with isochromosome Xq as a cause of granulomatous hepatitis: a case report.

Introduction: Turner syndrome (TS) is the most common sex chromosome abnormality in women, caused by a complete or partial absence of the second sex chromosome. The karyotype 46, X,i(Xq) is the underlying cause in about 10% of the cases of TS. Hepatic abnormalities are frequent in TS. Granulomas are relatively common in liver samples but are very rarely reported in TS. Case presentation: A 15-year-old female with TS attended a consultation for evaluation of elevated liver enzymes. Her chromosomal analysis showed mosaicism 46, X (iso xq)100%. There were no stigmata of chronic liver disease. A liver biopsy showed granulomatous hepatitis. Other causes of hepatic granulomas have been excluded. Ursodeoxycholic acid (UDCA) therapy leads to the normalization of transaminases. Clinical discussion: Although Hepatic involvement is common and mostly asymptomatic in TS, the mechanism of liver injury is not well understood. The hepatic histological changes in these cases are variable and range from minimal abnormalities to nonalcoholic steatohepatitis (NASH), liver architectural changes, and biliary lesions. Hepatic granulomas are associated with a wide range of systemic disorders but are very rarely reported in tuner syndrome. Normalization of liver enzymes after treatment with UDCA was previously reported, but the importance of this approach is to be determined. Conclusion: Granulomatous hepatitis may be associated with TS and may be added to the histological patterns encountered in this disorder.

Intimomedial mucoid degeneration resulting in a dissecting infrarenal abdominal aortic aneurysm in a young Middle Eastern male: a case report.

Introduction and importance: Intimomedial mucoid degeneration is a rare vascular disorder first depicted in 1977. It involves mucin deposition in arterial layers. This will cause elastic tissue degeneration and aneurysm formation. This pathology predominantly affects the aorta. However, it could involve other smaller vessels. Surgical treatment could become complicated by a bleeding diathesis. Therefore, a precise surgical technique is necessary to avoid the ensuing complications. Case presentation: We present the case of a previously healthy 27-year-old Middle Eastern male who presented to our surgical clinic following the incidental discovery of an infrarenal abdominal aortic aneurysm following a blunt trauma to the left flank incurred during a fall. Preoperative radiology unveiled a dissecting an infrarenal aortic aneurysm with a concurrent dissection flap at the left renal artery level. Furthermore, an additional dissection flap was observed at the abdominal aortic bifurcation devoid of thrombosis. Clinical discussion: We planned to perform a bilateral aortoiliac bypass. However, due to the fragility of the artery wall, bleeding diathesis, and the tearing that occurred due to the anastomotic suture, the irreparable tear in the anastomosis complicated the situation, we decided to ligate the aorta and perform an axillary-bi-femoral bypass. Conclusion: Intimomedial mucoid degeneration presenting as a dissecting infrarenal abdominal aortic aneurysm is an exceptionally rare pathology. This underscores the crucial need for extensive epidemiological research to document and raise awareness about these cases. Our literature review confirms that our case is the first documented instance in our country, and this emphasizes the significance of our findings.

Sclerosing pneumocytoma in a 1-year-old girl presenting with massive hemoptysis: A case report.

INTRODUCTION AND IMPORTANCE: Sclerosing pneumocytoma (SP) is a rare benign neoplasm of the lung with peak age incidence in middle aged-women. Here we report, for the first time in the literature, a case of a 1-year-old girl with SP. CASE PRESENTATION: A 1-year-old girl was reported to emergency department for massive hemoptysis. After admission, the patient had a three-days episode of melena, with normal body temperature and generally stable condition. CLINICAL DISCUSSION: Fiberoptic bronchoscopy was normal. MSCT was done along with angiography and Three-Dimensional Reconstruction which revealed a well-circumscribed round mass with well-defined borders located near the vessels in the upper lobe of left lung. Anatomic lingula resection was performed. Hilar node was also resected. The histopathological examination confirmed the presence of SP. Fourteen months postoperatively, the patient was in a good health with no clinical or radiological evidence of recurrence. CONCLUSION: SP is a rare benign tumor which usually presents in middle aged-women asymptomatically or with nonspecific symptoms. We report this case to highlight that SP should be considered in cases of hemoptysis in young children.