ceroid/hemorrhage

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[Formation of the ceroid pigment in hemorrhagic necrosis of fatty tissue].

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From intraplaque haemorrhages to plaque vulnerability: biological consequences of intraplaque haemorrhages.

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Intraplaque hemorrhages are mainly related to inward neoangiogenesis, initiated from the adventitia by lipid-dependent outwardly convected signals, and by the immaturity of these neovessels, allowing leaks and hemorrhages. Repeated intraplaque hemorrhages play a major role in the evolution of

Ceroid-containing histiocytic granuloma of the endometrium.

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In an endometrial curettage specimen, taken from a 67-year-old woman with post-menopausal bleeding, the endometrium was replaced by aggregates of swollen histiocytes filled with yellowish-brown pigment. Histochemical and ultrastructural examination of the specimen revealed that the pigment was of

Identification of proteins in the ceroid-like autofluorescent aggregates from liver lysosomes of Beige, a mouse model for human Chediak-Higashi syndrome.

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Chediak-Higashi syndrome is characterized by oculocutaneous albinism, a bleeding tendency and severe recurrent infections. Age-dependent formations of autofluorescent ceroid-like substances have been noted in a variety of tissues. In this study, we isolated an autofluorescent ceroid-like aggregate

Hyperandrogenism in girls with juvenile neuronal ceroid lipofuscinosis.

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Acne and hirsutism are common findings in girls with juvenile neuronal ceroid lipofuscinosis (JNCL). A study on their hormonal status was conducted to investigate the mechanisms underlying these symptoms. Sixteen girls with JNCL entered the study. Ten of the girls had periodic menstruation, while

Ceroid granulomas in the female genital system.

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Three cases of ceroid granulomas of the female genital system are presented, involving the cervix in two and lesions in the ovaries and bowel serosa in the other. Ceroid granulomas are unusual and interesting lesions formed when suitable substrates accumulate within macrophages to such an extent

[Albinism, thrombopathy, ceroid storage disease--Hermansky-Pudlak syndrome. Overview and description with immunodeficiency].

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The Hermansky-Pudlak Syndrome (HPS) is a rare autosomal recessively inherited triad of oculocutaneous albinism hemorrhagic diathesis and accumulation of ceroid in tissues. This article comprising the published reports of 232 patients is extended by the personal observation of a 7-year-old boy with

Hermansky-Pudlak syndrome; a case report with analysis of auto-fluorescent ceroid-like pigments.

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The Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disease characterized by a triad of oculocutaneous albinism, accumulation of auto-fluorescent ceroid-like pigments within macrophages, and bleeding tendency due to a storage pool disease of the platelets. We report an autopsy case (39y,

Correction of the bleeding time in patients with storage pool deficiency by infusion of cryoprecipitate.

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Five patients with Hermansky-Pudlak syndrome: storage pool deficiency, albinism and ceroid containing bone marrow macrophages and three patients with uncomplicated storage pool deficiency were treated with cryoprecipitate from 16 donors. Within 2 h of transfusion, bleeding times decreased towards a

Gene therapy for late infantile neuronal ceroid lipofuscinosis: neurosurgical considerations.

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OBJECTIVE The authors conducted a phase I study of late infantile neuronal ceroid lipofuscinosis using an adenoassociated virus serotype 2 (AAV2) vector containing the deficient CLN2 gene (AAV2(CU)hCLN2). The operative technique, radiographic changes, and surgical complications are

Pathology of human plaque vulnerability: mechanisms and consequences of intraplaque haemorrhages.

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Atherothrombotic diseases are still major causes of inability and mortality and fighting atherothrombosis remains a public health priority. The involvement of repeated intraplaque haemorrhages (IPH) in the evolution of atherothrombotic lesions towards complications was proposed as early as 1936.

Phagocytosis and macrophage activation associated with hemorrhagic microvessels in human atherosclerosis.

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OBJECTIVE Previously, we demonstrated that activated inducible NO synthase (iNOS)-expressing foam cells in human carotid plaques often produce autofluorescent (per)oxidized lipids (ceroid). Here, we investigate whether intraplaque microvessels can provide foam cells with lipids and trigger

Pseudomelanosis duodeni.

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Pseudomelanosis duodeni, speckled black pigmentation of the duodenal mucosa, presents a striking appearance at endoscopy. Among the 14 reported cases there is a predominance of black women greater than 40 years old, but it can occur in any race and age group. There is no known association with

DNA diagnosis and management of Hermansky-Pudlak syndrome in pregnancy.

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Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder characterized by oculocutaneous albinism (OCA), platelet storage pool deficiency, and ceroid lipofuscin deposition. Sequelae including pulmonary fibrosis, colitis, and hemorrhagic diathesis can impact obstetric management. An

Effects of normal and aspirin platelets on defective secondary aggregation in the Hermansky-Pudlak syndrome. A test for storage pool deficient platelets.

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Platelets from patients with the Hermansky-Pudlak syndrome (albinism, accumulation of ceroid-like pigment in bone marrow macrophages and mild hemorrhagic symptoms) and aspirin-treated normal platelets fail to develop irreversible secondary waves of aggregation when exposed to aggregating agents on

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