Acute rheumatic fever and Takayasu arteritis - A synchronous co-occurrence.

ABSTRACT: Acute rheumatic fever and Takayasu arteritis are examples of autoimmune diseases that commonly affect the cardiovascular system. We report an infrequent co-occurrence of both these diseases in an adolescent male. It may appear that in some individuals, the rheumatic fever may act as a trigger for the development of large vessel vasculitis. This possibility should be considered in patients on follow-up if they develop fresh features of cardiovascular compromise despite appropriate medical, interventional, or surgical therapy for rheumatic heart disease.

Ventricular mural thrombi - An occult occurrence?

Background: Ventricular mural thrombi are rare occurrences and form one of the common mechanical complications of myocardial infarctions. They also occur in patients with nonischemic myocardial disorders and even in those devoid of cardiac diseases. Clinical detection often depends on the size of the thrombi. Aim: This study was aimed at ascertaining the etiopathogenesis of mural thrombi with a clinicopathologic correlation. Materials and Methods: This was a 12-year retrospective observational study reviewing the autopsy records of all cases showing ventricular mural thrombi. The location, size, morphology, and histologic appearances of the thrombi were noted. The cases, depending on the etiology, were categorized into myocardial ischemic (Group 1), myocardial nonischemic (Group 2), and non-myocardial (Group 3) causes. These features were correlated with the clinical settings. Results: There were 93 cases of mural thrombi with an almost equal sex distribution and a mean age of 45.9 years. The cause was ischemic heart disease in most of the patients (Group 1, 46.2%), while 21.5% and 32.3% of patients belonged to Group 2 and Group 3, respectively. Unlike the large and dominantly left ventricular thrombi seen in Group 1, the other two groups frequently had biventricular involvement, location in the intertrabecular spaces, and a high microscopic detection. Clinical diagnosis had been made in only 3.2% of patients. Thromboembolism was noted in other organs in 34.4% of the total cases. Conclusion: Ventricular thrombi should be ruled out in patients with evidence of systemic and/or pulmonary thromboembolism, even in those without any cardiac disorders.

How often are heart diseases correctly diagnosed antemortem in children with fatal illnesses? A retrospective review of medical and autopsy records.

This retrospective study analyzed the concordance level between clinical and autopsy diagnosis of heart diseases over six years. Utilizing the Goldmann classification, the concordance rate was found to be 38.1%. Major discrepancies (Class I and II) were found in 39% cases and minor (Class III and IV) in 22.9% cases.

Pulmonary ossifying carcinoid - MEN in a male?

Pulmonary carcinoid tumors are considered as low-grade neoplasms, seen as centrally located endobronchial masses or as peripheral circumscribed nodules. Calcification or ossification is a known phenomenon, but presentation as large bony mass is extremely uncommon. Herein, we report a case of ossifying bronchial carcinoid along with nodular Hashimoto's thyroiditis as incidental autopsy findings in a 32-year-old patient with a prior recent excision of pituitary macroadenoma. This association suggests the possibility of multiple endocrine neoplasia in this young male.

Pulmonary microvascular metastases in cervical carcinoma: A case series.

Pulmonary microvascular tumor embolism (PMTE), pulmonary tumor thrombotic microangiopathy (PTTM), and lymphangitis carcinomatosis (LC) have an intricate pathophysiology and usually occur with cancers of breast, stomach, and lung. Microvascular pulmonary metastases attributable to cervical cancer are a rarity. Clinical presentation and autopsy findings of patients with microvascular pulmonary metastases in cervical cancers were studied with a review of literature. Four patients (mean age of 55.5 years) with carcinoma cervix showed microvascular metastases. Three of whom presented with respiratory symptoms, and the fourth case was unresponsive on presentation. Each patient succumbed to their illness shortly after admission. Autopsy examination performed on each patient depicted varying combination of PMTE, PTTM, and LC, all with squamous histology. This case series highlights the rare association of carcinoma cervix with the aforementioned microvascular phenomena. Besides, it underscores the sequential mechanism of occurrence of microvascular pulmonary metastasis and the associated guarded prognosis.

How often is coarctation of aorta correctly diagnosed antemortem in children with fatal illnesses? A retrospective review of medical and autopsy records.

This retrospective study analyzed the level of concordance between clinical and autopsy diagnosis of coarctation of aorta over 10 years. Utilizing the Goldmann classification, the concordance rate was found to be 16%. Major discrepancies (Class I and II) were found in 56% cases and minor discrepancies (Class III and IV) in 28% cases.

Fungal fibrosing mediastinitis in pregnancy - Case report with review of literature.

Fibrosing mediastinitis (FM) is characterized by extensive and invasive fibro-inflammatory proliferation, triggered by a delayed hypersensitivity reaction to variety of infective or noninfective stimuli. The infective agents often have a geographic distribution such as Histoplasma capsulatum in North America and Mycobacterium tuberculosis in Asian regions. In few reports, the mediastinitis is caused by fungi, particularly Aspergillus species. We report the first case of possible aspergillous FM in a young pregnant woman.

Pulmonary miliary papillary/micropapillary adenocarcinoma manifesting as carcinomatous encephalitis.

The histological pattern and the associated molecular aberrations have an important bearing in the prognosis of pulmonary adenocarcinomas. Papillary and micropapillary growth patterns with mutations in epidermal growth factor receptor and anaplastic large-cell lymphoma kinase rearrangements have an aggressive clinical course. We report one such cancer in a 49-year-old woman, where it assumed a miliary pattern in the lungs, and the patient presented with neurological symptoms related to carcinomatous encephalitis, an infrequent metastatic manifestation.

Right atrial mural thrombi: An autopsy study of an under-diagnosed complication at an unusual site.

BACKGROUND: Right atrial mural thrombi (RAMT) are often seen in association with cardiac diseases or foreign bodies. Unusual locations at the flutter isthmus and the atrial appendage prompted us to evaluate our 2-year autopsy data on such thrombi. MATERIALS AND METHODS: In the 2-year retrospective autopsy, the clinical and autopsy records of patients with RAMT were reviewed, with particular reference to the presence of central venous catheter (CVC), its site of insertion, its type, material and size, its duration of placement, and the drugs infused through the catheter. RESULTS: Of the 940 autopsies performed in 2 years, RAMT was seen in 24 hearts and was related to an insertion of a CVC in 23 patients (95.8%). The risk and/or associated factors for this complication were tunneled and polyethylene catheters, Intensive Care Unit admission, infused drugs, underlying cardiac diseases, and pregnancy. A noteworthy feature was the location of the thrombi in the flutter isthmus in 16 hearts (66.7%) and atrial appendage in another six hearts. Localized endocarditis/myocarditis and pulmonary thromboembolism were observed in six and four patients, respectively. CONCLUSIONS: This autopsy study, which has a high incidence of catheter-related RAMT, does not reflect the true incidence but reiterates the importance of guided insertion of central venous and prompt recognition of thrombus formation.

Cardiac pathology in chronic alcoholics: a preliminary study.

BACKGROUND: Ethyl alcohol exerts both positive and negative effects on the cardiovascular system. Alcoholic cardiomyopathy, produced by direct or indirect mechanisms, is well-documented. An important, but seldom appreciated effect is an increase in iron deposition in the myocardium, which can add to the cardiac dysfunction. The present study was planned to document the pathological features and iron levels in the cardiac tissue of patients who were chronic alcoholics and correlate these characteristics with the liver pathology and iron content. MATERIALS AND METHODS: An autopsy-based prospective study of 40 consecutive patients compared with ten age matched controls (no history of alcohol intake). Histopathological changes like the morphology of the cardiac myocytes, degree of fibrosis (interstitial, interfiber, perivascular, and replacement), presence of inflammatory cells, increased capillary network, and adipose tissue deposition were noted and graded. These were also correlated with the liver pathology. The iron content in the heart and liver were measured by using calorimetry. RESULTS: All cases had increased epicardial adipose tissue with epicardial and endocardial fibrosis, prominence of interstitial and interfiber fibrosis, myofiber degeneration, and increased capillary network; this was particularly prominent in patients with cirrhosis. Elemental iron level in heart tissue was raised in the cases relative to controls. CONCLUSIONS: Alcohol produces subclinical changes in the myocardium, with an increased iron content, which may be the forerunner for subsequent clinical cardiac dysfunction.

Upper aerodigestive tract cancer and the lung: a tale of two aspirations.

Patients with upper aerodigestive epithelial cancers frequently develop second primary cancers due to common risk factors or develop distant metastases depending on the locoregional status of the primary tumor. In most instances, the organ affected is the lung. Pulmonary spread usually occurs due to hematogenous or lymphatic dissemination. The following is a report of two patients with upper aerodigestive tract squamous cell carcinomas who developed lung metastases due to aspiration, a route not well documented in recent literature.

Idiopathic pulmonary hemosiderosis: alveoli are an answer to anemia.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder (triad of iron-deficiency anemia, hemoptysis, and alveolar infiltrates). A 3-year-old male presented with mild fever, breathlessness, dry cough, and bluish nail discoloration for 8 days. He had required five blood transfusions in the past 1 year (last transfusion was given 4 months ago). He had a respiratory rate of 58/min with respiratory distress, cyanosis, and grade III clubbing. Respiratory system examination was normal. Several previous reports of hemoglobin were as low as 3.6 g/dl with hypochromic and microcytic anemia. There were transient increases in the hemoglobin and normalization of red cell morphology with blood transfusions. Serum iron, G6PD enzyme assay, hemoglobin electrophoresis, the sickling test, Coomb's test, stool and urine analysis, and a Meckel's scan were normal. HIV antibody and dsDNA were negative. The chest radiograph revealed symmetrical patchy infiltrates sparing lung apices (confirmed on high-resolution computed tomography). Lung biopsy diagnosed pulmonary hemosiderosis (interstitial lung disease with hemosiderin-laden macrophages scattered in the alveoli and areas of fibrosis in the alveolar septa). The patient showed marked clinical improvement in 10 days of therapy with prednisolone. IPH should be listed in the differential diagnosis of a child presenting with unexplained hypochromic, microcytic anemia and respiratory symptoms.