Cutaneous lymphatic amyloid deposits in "Hungarian-type" familial transthyretin amyloidosis: a case report.

Multiple transthyretin (TTR) mutations have recently been identified and implicated in the development of familial systemic amyloidoses, but early diagnosis of these disorders is still largely unresolved. We investigated the presence and tissue distribution of TTR-derived amyloid in skin biopsies of a 59-year-old woman carrying the "Hungarian-type" mutation of TTR (Asp18Gly). Clinical symptoms involved severe central nervous system dysfunction without signs of polyneuropathy, also referred to as the "central form" of TTR-related systemic amyloidosis.

Development of the myocardium of the atrioventricular canal and the vestibular spine in the human heart.

To establish the morphogenetic mechanisms underlying formation and separation of the atrioventricular connections, we studied the remodeling of the myocardium of the atrioventricular canal and the extracardiac mesenchymal tissue of the vestibular spine in human embryonic hearts from 4.5 to 10 weeks of development. Septation of the atrioventricular junction is brought about by downgrowth of the primary atrial septum, fusion of the endocardial cushions, and forward expansion of the vestibular spine between atrial septum and cushions.

Atrial development in the human heart: an immunohistochemical study with emphasis on the role of mesenchymal tissues.

The development of the atrial chambers in the human heart was investigated immunohistochemically using a set of previously described antibodies. This set included the monoclonal antibody 249-9G9, which enabled us to discriminate the endocardial cushion-derived mesenchymal tissues from those derived from extracardiac splanchnic mesoderm, and a monoclonal antibody recognizing the B isoform of creatine kinase, which allowed us to distinguish the right atrial myocardium from the left. The expression patterns obtained with these antibodies, combined with additional histological information derived from the serial sections, permitted us to describe in detail the morphogenetic events involved in the development of the primary atrial septum (septum primum) and the pulmonary vein in human embryos from Carnegie stage 14 onward.

Immunohistochemical and Ultrastructural Analysis of a Mammary Cystic Hypersecretory Carcinoma.

Cystic hypersecretory carcinoma (CHC) is a rare variant of intraductal carcinoma. A CHC in a 50-year-old woman was excised and processed for light and electron microscopy and immunohistochemistry. The tumor had a marked cystic appearance.

Histological investigations of the nasal mucosa in human fetuses.

The adult nasal mucosa has been exposed to various external agents and selected physiological conditions. Changes in intranasal airflow influenced the morphological appearance of the mucosa. Studies of agents on the fetal mucosa and its development may contribute to better understanding of the morphology of the nasal mucosa.

[Diagnostic problems of Wilson disease].

A family (three siblings) of Wilson's-disease is described. The authors review the pathogenesis, diagnostics, pathology and treatment of Wilson's-disease. The diagnostic difficulties are emphasised.

Cell surface glycoconjugates and the extracellular matrix of the developing mouse embryo epicardium.

Cell surface glycoconjugates and the extracellular matrix (ECM) of the proepicardium and the developing epicardium were studied in early mouse embryos by light and electron microscopy with histochaemical and immunocytochaemical techniques. The extracardially located proepicardium consists of polarized mesothelial cells forming the proepicardial vesicles. These vesicles contain a fine proteoglycan network and an acellular ECM rich in hyaluronic acid.

Dystrophin expression in the developing conduction system of the human heart.

Duchenne muscular dystrophy (DMD) is frequently associated with myocardial involvement. Dystrophin, the DMD protein, is found at the plasmamembrane of striated muscle fibers. Although dystrophin is missing in most or all muscle fibers of DMD patients, cardiac muscle is not as severely affected as skeletal muscle.

Formation of the tricuspid valve in the human heart.

Background: Some of the problems concerning the origin of the inlet component of the definitive right ventricle were resolved in a previous study in which we showed it to be derived exclusively from the embryonic right ventricle. Questions remain, however, concerning the relative contributions of endocardial cushion tissue and myocardium to the definitive valvar apparatus guarding the right atrioventricular orifice and the origin of the valvar leaflets.

Methods And Results: The formation of the tricuspid valve was studied by scanning electron microscopic and immunohistochemical techniques.

Bacillary angiomatosis in a patient with lymphocytic leukaemia.

A 78-year-old man, who suffered from chronic lymphocytic leukaemia and diabetes mellitus, but was human immunodeficiency virus (HIV)-negative, developed disseminated angiomatous papules following a cat scratch. Bacillary angiomatosis was diagnosed by light and electron microscopic demonstration of the causative bacteria in the vascular lesions. The lesions resolved completely when he was treated with erythromycin.

[Bacillary angiomatosis].

In a 78 years old patient with chronic lymphoid leukemia, diabetes mellitus a cat scratch induced disseminated angiomatous papules were observed. In the lesions great number of bacilluses were observed with light -and electron microscope. As a result of antibiotic treatment the lesions regressed without trace.

Early development of quail heart epicardium and associated vascular and glandular structures.

As in the other vertebrates the epicardium of the quail embryo develops from proepicardial tissue located between the sinus horns and the liver primordium. The cuboidal cells of the coelomic lining above the proepicardium are transformed into mesothelial cells which in cooperation with the underlying mesenchymal cells elaborate a large quantity of extracellular matrix, so producing the villous outgrowths of the proepicardium. The mesenchymal cells of this area are attached to each other with typical desmosomes and have anti-alpha cytokeratin-stained tonofilament bundles.

Immunohistochemical delineation of the conduction system. II: The atrioventricular node and Purkinje fibers.

Using an antibody that reacts specifically with the myocytes of the conduction system of the bovine heart, we have studied the atrioventricular node and the spatial distribution of the Purkinje fibers in the bovine heart. This study was complemented by studying the distribution of the gap junction protein connexin43 in these areas in the bovine heart and in the human heart. The large Purkinje fibers in the bovine heart are arranged in a two-dimensional network underneath the endocardium.

Immunohistochemical delineation of the conduction system. I: The sinoatrial node.

We have raised a mouse monoclonal antibody that reacts specifically with the myocytes of the sinoatrial node of the bovine heart. By use of this antibody (445-6E10) and antibodies against the gap junction protein connexin43, the periphery of the sinoatrial node and the distribution of gap junctions in the nodal region were studied. The reaction patterns of 445-6E10 and anti-connexin43 are exactly complementary; ie, connexin43 was not detected in the nodal myocytes but was clearly present in the atrial myocytes.

New findings concerning ventricular septation in the human heart. Implications for maldevelopment.

Background: The mechanics involved in development of the inlet component of the morphologically right ventricle are, as yet, undecided. Some argue that this component is derived from the descending limb of the ventricular loop, and that the inlet and apical trabecular components of the muscular ventricular septum have separate developmental origins. Others state that the entirety of the right ventricle grows from the ascending limb of the loop, and that the muscular septum, apart from its outer component, has a unitary origin.

Spatial distribution of "tissue-specific" antigens in the developing human heart and skeletal muscle. III. An immunohistochemical analysis of the distribution of the neural tissue antigen G1N2 in the embryonic heart; implications for the development of the atrioventricular conduction system.

A monoclonal antibody raised against an extract from the Ganglion Nodosum of the chick and designated G1N2 proves to bind specifically to a subpopulation of cardiomyocytes in the embryonic human heart. In the youngest stage examined (Carnegie stage 14, i.e.

Spatial distribution of "tissue-specific" antigens in the developing human heart and skeletal muscle. II. An immunohistochemical analysis of myosin heavy chain isoform expression patterns in the embryonic heart.

The spatial distribution of alpha- and beta-myosin heavy chain isoforms (MHCs) was investigated immunohistochemically in the embryonic human heart between the 4th and the 8th week of development. The development of the overall MHC isoform expression pattern can be outlined as follows: (1) In all stages examined, beta-MHC is the predominant isoform in the ventricles and outflow tract (OFT), while alpha-MHC is the main isoform in the atria. In addition, alpha-MHC is also expressed in the ventricles at stage 14 and in the OFT from stage 14 to stage 19.

Spatial distribution of "tissue-specific" antigens in the developing human heart and skeletal muscle. I. An immunohistochemical analysis of creatine kinase isoenzyme expression patterns.

Using monoclonal antibodies against the M and B subunit isoforms of creatine kinase (CK) we have investigated their distribution in developing human skeletal and cardiac muscle immunohistochemically. It is demonstrated that in skeletal muscle, a switch from CK-B to CK-M takes place around the week 8 of development, whereas in the developing heart, CK-M is the predominant isoform from the earliest stage examined onward (i.e.

[Unity of diagnostic pathology, continuing education and research at our institute].

Authors give a brief view on the activity in Institute of Pathology and Histopathology of Post-graduate University of Medicine. In its frame, close relation of diagnostic, post-graduate education and research activity is illustrated by examples of different fields (intestinal, cardiac, vascular system, respiratory tract, organ of locomotion, urological diseases, electronmicroscopic tumor diagnostics, endocrinology). Importance of modern morphological methods and their place both in practical and scientific activity are dealt with.

[Development of the avian and mammalian heart prior to the onset of blood circulation, studied in chick and mouse embryos].

Heart development, its morphological and functional development prior the start of blood circulation were compared in chicken and mouse embryos. At beginning of neurula stage, when also somites appear, the cardiogenic mesoderm forms into tissue of epithelial type and cardiac plate is developed in wall of pleuroperitoneal cavity. In conjugated cardiac plate, primordial cardiac cavity fundaments appear in the about 30-hour old chicken embryo with 6-7 somites.

Formation of the primitive myo- and endocardial tubes in the chicken embryo.

The morphogenesis of the mesenchymal cardiogenic plate, the formation of the bilateral heart primordia leading to the primitive heart tube, and also the genesis of the endocardial tube, have been studied in 1 to 13 somite chick embryos. The morphological data were compared with those obtained in electrophysiological studies of the development of the cardiac action potentials (Fujii et al., 1981a).

[Ultrastructure of Ito cells in fibrotic liver diseases].

Authors have studied the ultrastructure of Ito-cells in percutane needle-biopsy samples of hepatic tissue from 36 patients. In 3 samples no alteration was found, in four only moderate steatosis occurred without fibrosis. In four samples portal, in ten centrilobular and in five periportal fibrosis was detected, while in five samples cirrhosis was pointed out.

Early bronchopulmonary lesions in rat lung after normobaric 100% oxygen exposure and their evolution. A light and electron microscopic study.

In order to clarify the early phenomena involved in the lung reaction to hyperoxia, twenty adult male rats were exposed to 100% oxygen at 1 ATA. Morphological pulmonary lesions were detectable after only 24 h hyperoxia, and included vasoconstriction and perivascular oedema, bronchiolar constriction, and pericyte reaction. The lesions were irregularly scattered within the lung parenchyma and occurred preferentially in areas centred on bronchiolo-vascular stems.

Ultrastructure of the Ito cells in liver disease associated with fibrosis.

The ultrastructure of Ito cells was examined in percutaneous needle liver biopsies of 36 patients. In three cases the liver was normal. Four specimens showed mild or moderate fatty degeneration.