Charu Subramony

Pulmonary thrombotic arteriopathy in patients with sickle cell disease.

BACKGROUND Shortened life expectancy due to pulmonary hypertension (PH) is seen in 5% to 10% of patients with sickle cell disease. The principal factors suspected of causing PH are pulmonary thromboemboli (PE) and in situ arterial thrombosis. OBJECTIVE To investigate the possible role that PE or in situ arterial thrombosis play in the development of PH in sickle cell disease. METHODS Autopsies of 12 patients with sickle cell disease were correlated with clinical data from medical records. RESULTS Right ventricular hypertrophy was present in 9 of 12 patients. Six patients with right ventricular hypertrophy had thrombi in large elastic pulmonary arteries. All patients with elastic artery thrombi had fresh or organized thrombi in small muscular pulmonary arteries. Hypertensive small arterial changes were present in 5 of these 6 patients. Six patients showed no thrombi in elastic arteries. Among these 6 patients, 3 had right ventricular hypertrophy and recent and organized thrombi, as well as hypertensive changes in small arteries. One of these 3 patients demonstrated plexiform-like lesions and fibrinoid necrosis of small arteries. Three patients without right ventricular hypertrophy had pneumonia or pulmonary edema with no identifiable pulmonary artery pathology. CONCLUSIONS Arterial thrombosis with PH and cor pulmonale was regarded as the cause of death among most of these patients. Elastic artery thrombi are pulmonary thromboemboli, but pulmonary thromboemboli are always associated with widespread thrombosis of small arteries. Widespread thrombosis of small arteries alone was associated with PH in some cases. This finding suggests that pulmonary thromboemboli may be a late complication of PH and cor pulmonale and that an in situ thrombotic arteriopathy underlies the development of PH in most patients with sickle cell disease.

Human Immunodeficiency Virus-Associated Gastrointestinal Disease: Common Endoscopic Biopsy Diagnoses

The gastrointestinal (GI) tract is a major site of disease in HIV infection: almost half of HIV-infected patients present with GI symptoms, and almost all patients develop GI complications. GI symptoms such as anorexia, weight loss, dysphagia, odynophagia, abdominal pain, and diarrhea are frequent and usually nonspecific among these patients. Endoscopy is the diagnostic test of choice for most HIV-associated GI diseases, as endoscopic and histopathologic evaluation can render diagnoses in patients with non-specific symptoms. In the past three decades, studies have elucidated a variety of HIV-associated inflammatory, infectious, and neoplastic GI diseases, often with specific predilection for various sites. HIV-associated esophageal disease, for example, commonly includes candidiasis, cytomegalovirus (CMV) and herpes simplex virus (HSV) infection, Kaposis sarcoma (KS), and idiopathic ulceration. Gastric disease, though less common than esophageal disease, frequently involves CMV, Mycobacterium avium-intracellulare (MAI), and neoplasia (KS, lymphoma). Small bowel biopsies and intestinal aspirates from HIV-infected patients often show HIV enteropathy, MAI, protozoa (Giardia, Isospora, Cryptosporidia, amebae, Microsporidia), and helminths (Strongyloides stercoralis). Colorectal biopsies demonstrate viral (CMV, HSV), bacterial (Clostridia, Salmonella, Shigella, Campylobacter), fungal (cryptococcosis, histoplasmosis), and neoplastic (KS, lymphoma) processes. Herein, we review HIV-associated GI pathology, with emphasis on common endoscopic biopsy diagnoses.

Retroperitoneal mucinous cystadenoma.

Primary retroperitoneal mucinous cystadenoma is an uncommon tumor found exclusively in women. Herein, we describe a patient who had resection of a large retroperitoneal cystic mass. Histologic, immunohistochemical, and electron microscopic examination of the lining epithelial cells showed features of mesothelial cells in addition to ovarian mucinous cystadenoma. These findings suggest that these tumors arise from inclusions of mesothelial cells and subsequent mucinous metaplasia of the lining cells to form a cystadenoma. Estrogen receptors may be implicated in tumor promotion, explaining the occurrence exclusively in women.

Hepatic iron overload in children with sickle cell anemia on chronic transfusion therapy.

Hepatic iron overload is a serious complication of chronic transfusion therapy in patients with sickle cell disease (SCD). No firm consensus has been reached with regard to correlation between hepatic iron content (HIC) and variables including age, number of transfusions, and serum iron makers. Also, the role of HIC in determining hepatic injury is not well established. There is scarcity of data on chronically transfused children with SCD and no other confounding liver pathology. We aimed to further explore relationships between these variables in a cohort of children with SCD on chronic transfusion therapy naive to chelation. Liver biopsies obtained before starting chelation therapy from 27 children with sickle cell anemia receiving chronic transfusion therapy were evaluated for histologic scoring and determination of HIC. Average serum ferritin and iron saturation values were determined for 6 months before biopsy. Duration and total volume of transfusion were obtained from the medical records. All children were negative for human immunodeficiency virus, hepatitis B virus, and hepatitis C virus infections. Mean age at biopsy was 10.95±3.34 years. Mean duration and total volume of transfusions were 50.0±26.6 months and 17.4±9.6 L, respectively. Pearson product-moment bivariate correlation coefficients indicated significant correlations between HIC and histologic iron score, serum ferritin, iron saturation, age, and transfusion volume. After adjusting for transfusion volume, a significant correlation was only seen between HIC and transfusion volume. Mean HIC was 21.8±10.4 mg/g dry weight, with fibrosis observed in 10 patients and lobular inflammation in 9. HIC was higher in biopsies with fibrosis (28.2±3.8 mg/g) than biopsies without fibrosis (17.6±18.3 mg/g; P=0.012). HIC did not differ between biopsies with lobular inflammation (25.5±4.0 mg/g) and biopsies without inflammation (19.9±2.5 mg/g; P=0.22). These findings show that transfusion volume provides more insight on hepatic iron overload than serum iron markers.

Fatal Enteritis Necroticans (Pigbel) in a Diabetic Adult

Enteritis necroticans is a segmental necrotizing infection of the jejunum and ileum caused by Clostridium perfringens, Type C. The disease occurs sporadically in parts of Asia, Africa, and the South Pacific, where it primarily affects children with severe protein malnutrition. The disease is extremely rare in developed countries, where it has been seen primarily in diabetics. Two cases have previously been reported in the United States, one in a child with poorly controlled Type 1 diabetes. A 66-year-old woman with a 12-year history of Type 2 diabetes mellitus developed severe abdominal pain and bloody diarrhea after eating a meal of turkey sausage. She died unattended at home. An autopsy showed peritonitis and segmental necrosis of the jejunum and ileum. Microscopic examination showed Gram-positive club-shaped bacilli consistent with Clostridia coating a necrotic mucosa. Products of cpa and cpb genes of C. perfringens, Type C were identified in the necrotic jejunum by polymerase chain reaction amplification.

Liver Surface Nodularity Quantification from Routine CT Images as a Biomarker for Detection and Evaluation of Cirrhosis

Purpose To determine the accuracy, reproducibility, and intra- and interobserver agreement of a computer-based quantitative method to measure liver surface nodularity (LSN) from routine computed tomographic (CT) images as a biomarker for detection and evaluation of cirrhosis. Materials and Methods For this institutional review board-approved HIPAA-compliant retrospective study, adult patients with healthy livers (n = 24) or various stages of hepatitis C virus-induced chronic liver disease (n = 70) with routine nonenhanced and portal venous phase contrast agent-enhanced liver CT imaging with thick-section (5.0 mm) and thin-section (1.25-1.50 mm) axial images obtained between January 1, 2006, and March 31, 2011, were identified from the electronic medical records. A computer algorithm was developed to measure LSN and derive a score. LSN scores, splenic volume, and the ratio of left lateral segment (LLS) to total liver volume (TLV) were measured from the same multiphasic liver CT examinations. Accuracy for differentiating cirrhotic from noncirrhotic livers was assessed by area under the receiver operating characteristic curve. Intra- and interobserver agreement was assessed by intraclass correlation coefficient. Results Median LSN scores from nonenhanced thick-section CT images in cirrhotic livers (3.16; 56 livers) were significantly higher than in noncirrhotic livers (2.11; 38 livers; P < .001). LSN scores from the four CT imaging types (94 patients for each type) were very strongly correlated (range of Spearman r, 0.929-0.960). LSN scores from portal venous phase contrast-enhanced thick-section CT images had significantly higher accuracy (area under the receiver operating characteristic curve, 0.929) than splenic volume (area under the receiver operating characteristic curve, 0.835) or LLS-to-TLV ratio measurements (area under the receiver operating characteristic curve, 0.753) for differentiating cirrhotic from noncirrhotic livers (P = .038 and .003, respectively; n = 94). Intra- and interobserver agreements that used nonenhanced thick CT images were very good (intraclass correlation coefficient, 0.963 and 0.899, respectively). Conclusion Quantitative measurement of LSN on routine CT images accurately differentiated cirrhotic from noncirrhotic livers and was highly reproducible. (©) RSNA, 2016 Online supplemental material is available for this article.

Solitary rectal ulcer: a rare cause of gastrointestinal bleeding in an adolescent with hemophilia A.

Solitary rectal ulcer syndrome (SRUS) is a rarely reported condition in children. The typical presentation is one of anorectal pain with passage of blood and mucus per rectum in the setting of defecation abnormalities. Diagnosis is made via endoscopy and biopsy. Solitary rectal ulcer syndrome alone is usually a benign condition; however, significant morbidity can occur if complicated by underlying disease states. We report an adolescent with hemophilia A and SRUS who presented with a rectal bleed that required blood transfusion.

Postinfectious gastroparesis related to autonomic failure: a case report

Abstract  Background and aim:  Severe dysautonomia may be secondary to viral infections, resulting in impaired autoimmune, cardiovascular, urinary and digestive dysfunction. Herein, we present a case of a 31‐year‐old white female patient who had severe gastroparesis related to autonomic failure following an episode of acute gastroenteritis. This seems to be the first report providing thorough assessment of the enteric and autonomic nervous system by analysis of full‐thickness small intestinal biopsies, cardiovagal testing and autopsy.

Follicular carcinoma arising in ectopic thyroid tissue: Case report with fine‐needle aspiration findings

A 32‐yr‐old woman was found to have a 1 × 2 cm mass in the soft tissue of the neck 1 cm lateral to the left lobe of the thyroid gland. A fine‐needle aspiration biopsy showed a follicular neoplasm. The excised mass showed a follicular carcinoma arising in lateral ectopic thyroid tissue. Subsequent excision of the thyroid gland and pathological examination showed no primary carcinoma in the gland. This report illustrates a case of primary follicular carcinoma arising in a lateral ectopic thyroid tissue in the neck. Diagn. Cytopathol. 16:39–41, 1997.

Age-related differences in granulomatous gastritis: a retrospective, clinicopathological analysis

Background Granulomatous gastritis (GG) is an uncommon pathological finding that may accompany systemic disease, infections, foreign body reaction, malignancy or vasculitis, but may also be an isolated finding. Clinical and pathological features of GG have been systematically evaluated in adults but not children. Objectives To compare clinical and pathological features of GG in adults and children, and also determine the prevalence of GG in children from a single centre. Methods A retrospective analysis of 23 children and 23 adults with GG was conducted. Demographic and clinical information was recorded for each patient. Gastric biopsy specimens were evaluated for the presence of gastritis, infectious organisms, and number and location of the granulomas. Results Children were a mean±SD age of 12.5±3.0 years, had a male predominance, and were most often Caucasian. Adults were a mean±SD age of 49.2±13.2 years, had a female predominance, and were most often African–American. Primary diagnoses were Crohns disease in children, and sarcoidosis and isolated GG in adults. In both groups, granulomas were most often located in the antrum, with no difference in the number of granulomas per biopsy between children and adults. All biopsy specimens were negative for acid-fast bacilli and fungal organisms; Helicobacter pylori infection was uncommon. Overall prevalence of GG in children in this study was 1.7% for all diagnostic upper endoscopies. Conclusion Differences in aetiology of GG between children and adults reflect age-specific disease states. Gender differences can be partially explained by gender differences intrinsic to the underlying aetiology. Irrespective of the underlying aetiology, the number and location of granulomas are similar in children and adults.