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 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 44-year-old women was treated for hyperparathyroidism resulting from parathyroid hyperplasia. Several months later, following a flu-like episode, she developed fever, confusion, abdominal pain, and diffuse petechiae, with severe thrombocytopenia and hemolytic anemia. She died on the 11th day of hospitalization. At autopsy she had multiple endocrine neoplasia type I, with two islet cell tumors, adrenal adenoma, pituitary adenoma, and bronchial carcinoid with liver metastasis. Florid visceral microthrombi involved arterioles and capillaries of the heart, including the conduction system. Brain, kidney, pancreas, adrenal, and portal areas of the liver were also heavily involved, but thrombi were rare in the liver sinusoids and the lungs. PAS-positive subendothelial deposits were demonstrated. In spite of the disseminated malignancy, the morphologic and laboratory findings were inconsistent with disseminated intravascular coagulation (DIC), and supported the clinical diagnosis of TTP. To the best of our knowledge this is the first report association of TTP with MEN and raises the question of a genetic linkage and/or hormonal interaction.
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PMID:Fatal thrombotic thrombocytopenic purpura (TTP) presenting concurrently with metastatic multiple endocrine neoplasia (MEN) type I. 887 34

The ciliated hepatic foregut cyst is an unusual solitary cystic lesion of the liver. In a series of 7 cases of hepatic ciliated cysts, we performed a histological, histochemical, and immunohistochemical study to better define the histogenesis of this rare entity. The patients were 4 women and 3 men, aged 39 to 75 years. Four patients presented with abdominal pain. In 3 cases the cyst was discovered incidentally on ultrasonography. The cysts measured from 1 to 4 cm in diameter. Microscopically, the lining of the columnar epithelium was composed of ciliated cells and mucin secreting goblet cells. The wall was composed of bands of smooth-muscle fibers surrounded by an outer fibrous capsule. The goblet cells stained with PAS, alcian blue, and high-iron diamine. The immunohistochemical study showed that endocrine cells were present within the cyst epithelium, positive for chromogranin, synaptophysin, bombesin, and calcitonin, and negative for serotonin, somatostatin, glucagon, insulin, gastrin, and pancreatic polypeptide. In all the cases, immunoreactivity of some cells for CC10 strongly suggested the presence of Clara cells. Our study shows that the epithelium lining ciliated hepatic foregut cysts has histological, histochemical, and immunohistochemical features similar to those observed in the bronchiolar epithelium. This lesion is a developmental ventral foregut abnormality that could arise from a bronchiolar bud of the tracheobronchial diverticulum.
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PMID:The ciliated hepatic foregut cyst, an unusual bronchiolar foregut malformation: a histological, histochemical, and immunohistochemical study of 7 cases. 1068 41

Despite being relatively common in the rectum, foamy histiocytes have received scant attention as to the antecedent lesion that causes them to form or their histologic characterization on the types of muco-substances they accumulate. One-hundred consecutive tissue sections of the rectum from an equal number of patients were reviewed for the presence of foamy histiocytes, evaluated for their associated histologic features, and examined histochemically for five types of mucin. Immunohistochemical and electron microscopic studies were performed. Forty (40%) of the rectal biopsy tissues contained foamy histiocytes. Patients presented with diarrhea, hematochezia, intestinal habit change, constipation, hemorrhoids, and abdominal pain. Endoscopically, 19 patients were thought to have rectal nodules or polyps. Histologically, 25 of the patients had regenerative changes in the adjacent mucosa and 14 had hyperplastic changes. In 36 patients (90%), the foamy histiocytes were located superficially in the lamina propria. Associated changes indicated that they are found in areas that are subject to an injury that is in a healing phase. These changes included mild fibrosis and chronic inflammation of lamina propria with mild architectural distortion. Thirty-five (88%) cases showed staining for D-PAS, Alcian blue stain pH 2.5, and the cocktail Alcian blue stain/PAS. Mucicarmine was positive in 25 (63%) cases. The Alcian blue stain pH 1.0 was positive in 19 (59%) of 32 cases. Ultrastructural studies showed electron-dense globules. Two cases were histologically identical to the other 38 but they did not stain for any mucin. Ultrastructural features disclosed clear vacuoles and thus represent a xanthelasma of the rectum. The foamy cells in all cases were confirmed to be histiocytes by immunohistochemistry and electron microscopy. Although muciphages and xanthelasma of the rectum may mimic polyps endoscopically, they are not related to any specific symptom or clinical finding, despite the fact that they probably represent remnants of a previous injury. Muciphages contain neutral, weakly acidic or strongly acidic mucin. The main type of acidic mucin is sialomucin with a smaller component of sulfated mucin.
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PMID:Histochemical and immunohistochemical characterization of foamy histiocytes (muciphages and xanthelasma) of the rectum. 1089 24

Whipple's disease is a systemic bacterial infection and the common though not invariable manifestations are diarrhoea, weight loss, abdominal pain, and arthralgia. Arthritis or arthralgia may be the only presenting symptom, predating other manifestations by years. Virtually all organs in the body may be affected, with protean clinical manifestations. Various immunological abnormalities, some of which may be epiphenomena, are described. The causative organism is Tropheryma whippelii. The disease is uncommon though lethal if not treated. Recent data suggest the disease occurs in an older age group than previously described. The characteristic histopathological features are found most often in the small intestine. These are variable villous atrophy and distension of the normal villous architecture by an infiltrate of foamy macrophages with a coarsely granular cytoplasm, which stain a brilliant magenta colour with PAS. These pathognomonic PAS positive macrophages may also be present in the peripheral and mesenteric lymph nodes and various other organs. The histological differential diagnoses include histoplasmosis and Mycobacterium avium-intercellulare complex. The clinical diagnosis of Whipple's disease may be elusive, especially if gastrointestinal symptoms are not present. A unique sign of CNS involvement, if present, is oculofacial-skeletal myorhythmia or oculomasticatory myorhythmia, both diagnostic of Whipple's disease. A small bowel biopsy is often diagnostic, though in about 30% of patients no abnormality is present. In patients with only CNS involvement, a stereotactic brain biopsy can be done under local anaesthetic. A recent important diagnostic test is polymerase chain reaction of the 16S ribosomal RNA of Tropheryma whippelii. Whipple's disease is potentially fatal but responds dramatically to antibiotic treatment. In this review the current recommended treatments are presented. The response to treatment should be monitored closely, as relapses are common. CNS involvement requires more vigorous treatment because there is a high rate of recurrence after apparently successful treatment.
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PMID:Whipple's disease. 1108 66

A 27 years old female presented with diffuse abdominal pain, malaise, weight loss and a palpable mass in the left abdomen. Abdominal CAT scan showed a 18 cm heterogeneous tumoral mass that originated in the kidney. The patient was operated on and it was not possible to completely remove the mass. In the postoperative period the patient received chemotherapy with vincristine, cyclophosphamide and adryamicin but the disease progressed and the patient died 16 months later. The pathological study of the surgical specimen disclosed a tumor with monotonous proliferation of small round cells with a basophilic nucleus and scanty cytoplasm with PAS positive granules. Immunohistochemistry was positive for specific neuronal enolase and CD99. Involvement of the urinary system with primary neuroectodermal tumors is very infrequent.
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PMID:[Primitive neuroectodermal tumor of the kidney. A case report]. 1135 72

We present the first case in Bulgaria of cytologically verified undifferentiated (embryonal) sarcoma of the liver. A 10-year-old girl was admitted to the hospital with abdominal pain, extreme hepatomegaly, one-month fever, anemia and highly elevated erythrocyte sedimentation rate (95 mm). A large formation was found in the right liver lobe on ultrasound examination, which was polycystic on CT scan. Neither bone marrow abnormalities nor elevation of serum alpha-fetoprotein was found. In the scanty specimen obtained by fine-needle aspiration biopsy under ultrasound control a characteristic prevalence of polygonal cells within mesenchyme tissue was found. The cells had eccentric hyperchromic nuclei which were pushed to the periphery by large clusters of PAS-positive, diastase-resistant granules. Vimentin and alpha 1-antitrypsyn were expressed immunohistochemically. The cells are pathognomonic of undifferentiated (embryonal) sarcoma of the liver. After preoperative chemotherapy with ifosfamide, vincristine and actinomycin D (CEVAIE, CWS--96) resection of the tumor including IV, V, VI and VII liver segments was performed. A 30 x 30-cm soft gray-whitish tumor with fine capsule was found. The cytological diagnosis was histologically verified. The patient had a mechanical subileus episode after the 6th course of chemotherapy. One year after the surgery she is in good condition. The histogenesis of this rare tumor is discussed regarding its unique cytology, immunohistochemical constellation and ultrastructure. A literature review is presented. The case indicates that the unique cell appearance in embryonal sarcoma of the liver provides a correct diagnosis even in a scanty specimen.
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PMID:A case of undifferentiated (embryonal) sarcoma of the liver: fine needle aspiration cytology diagnosis by one cell type. 1193 Aug 35

Whipple's disease is a rare systemic disease of infectious etiology. Its clinical presentation is highly variable. The most common symptoms are diarrhea, weight loss, abdominal pain and joint manifestations. Non-digestive manifestations frequently precede digestive symptoms by several years. For all these reasons, diagnosis is difficult. Definitive diagnosis is established by the finding of PAS-positive macrophages in the lamina propria of the thin intestine. It is important to start appropriate antibiotic therapy early, as this improves the prognosis. We present the case of a male patient in whom the first manifestations of the disease were constipation and upper gastrointestinal hemorrhage, two forms of clinical presentation that have rarely been described in the literature on Whipple's disease.
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PMID:[Constipation and digestive bleeding as an atypical presentation of Whipple's disease]. 1198 1

Whipple disease is an uncommon chronic bacterial infection due to Tropheryma whipplei. Clinical manifestations are protean (joint pain, fever, weight loss, abdominal pain, lymphadenopathies), and the diagnosis is often delayed. Although previously considered a late manifestation of Whipple disease, neurological involvement is now frequently the initial clinical manifestation and represents the greatest risk for long-term disability. All patients should be treated and monitored as if they had central nervous system disease even if they are asymptomatic. Neurological manifestations include dementia (56 percent), abnormalities of eye movements (33p. cent), involuntary movements (28 percent), seizures, hypothalamic dysfunction, myelopathy, ataxia and psychiatric manifestations. Uveitis, retinitis, optic neuritis and papilloedema may be found. 80 percent of the reported cases of neuro-Whipple had associated systemic symptoms or signs but many patients are presenting without concurrent intestinal manifestation. Thus, the disease may remain undiagnosed or misdiagnosed, as rheumatoid arthritis or sarcoidosis. Traditionally, the diagnostic procedure of choice is biopsy of the duodenal mucosa by demonstrating PAS-positive foamy macrophages. However, not all cases have small bowel infiltration and tissue obtained from sites clinically affected may be helpful. CT and MR images of the central nervous system are normal or not specific: atrophic changes, mass lesions, focal abnormalities and hydrocephalus. The application of a PCR assay against Tropheryma whipplei has transformed the diagnosis. Positive results have been obtained from several tissues and from CSF and PCR is more sensitive than other techniques. All patients must be treated with antibiotics which cross the blood-brain barrier. Most agree that initial treatment with a combination of parenteral penicillin and streptomycin for at least 14 days is appropriate, thereafter cotrimoxazole orally 3 times a day for at least one and probably for two years. Third generation cephalosporins, rifampicin and chloramphenicol have been used successfully. PCR is recognized to be a useful tool for monitoring progress but it is sometimes difficult to reverse established neurological defects.
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PMID:[Neurological manifestations of Whipple disease]. 1240 8

Whipple's disease is a rare, without antibiotic treatment deadly systemic infectious disease caused by the ubiquitary Gram positive bacterium Tropheryma whipplei. The agent can be demonstrated by light and electron microscopy and by PCR in tissues and body fluids affected. Men are affected more often than women. Most patients lose weight, have diarrhea, abdominal pain and arthralgias. In 10-40% of cases involvement of the CNS, often asymptomatic, is observed. PAS-positive macrophages in the lamina propria of duodenal biopsies are typical but not pathognomonic of Whipple's disease. Nowadays, the infectious agent should also be demonstrated by PCR. Treatment, mostly as a sequential therapy, is with antibiotics that cross the blood-brain barrier and should last for at least 1 year in order to avoid relapses. Before ending therapy the absence of Tropheryma whipplei in duodenal tissue and cerebrospinal fluid must be confirmed.
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PMID:[Whipple disease--a rare systemic disease]. 1242 73

The paper reports the clinical case of a 58 -year-old male patient admitted for diarrhea (6-7 stools/day, diffuse abdominal pain, borborygma, weight loss (20 kgs in two years), asthenia and fatigue. Physical examination evidenced a poor nutritional state (body mass index 19 kg/m2). The abdomen was slightly distended. Biological tests evidenced moderate/severe anemia, hypoproteinemia and hypoalbuminemia. Endoscopic examination evidenced oedematous duodenal mucosa with white-yellowish deposits. Histology (HE stain) revealed the presence of foamy cells and the PAS-staining of the duodenal mucosa evidenced PAS-positive macrophages and numerous intracellular bacilli. Penicillin therapy 2 x 1 million U/day for 14 days, followed by tetracycline 4 x 250 mg/day improved the clinical picture, the patient had only one stool per day and gained weight. After 7 months of treatment the general condition was good and the patient had gained 17 kgs, the duodenal mucosa was normal. HE staining did not evidence foamy cells and no PAS-positive macrophages could be found.
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PMID:Whipple's disease. Case report. 1253 5


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