Biomedical subjects

A Chlumská

Publications and source records attributed to A Chlumská.

At least 19 recordsLinked to original sources

PubMed2006

Sessile serrated adenomas of the large bowel. Clinicopathologic and immunohistochemical study including comparison with common hyperplastic polyps and adenomas.

Sessile serrated adenoma (SSA) is a newly characterized type of the large bowel adenoma. It arises in hyperplastic polyp (HP) and represents a precursor lesion of colorectal carcinoma with microsatellite instability. SSAs differ from common HPs by abnormal proliferation of the crypt epithelium and by nuclear atypia. We examined 15 SSAs from 15 patients. The age range was 25-80 years (average 60 years). Six patients were females and 9 were males. For comparison, we examined 10 conventional tubular adenomas and 10 common HPs with vesicular cells. The sites of SSAs were as follows: 8 in rectum, 4 in rectosigmoid colon, 1 in transverse colon, 1 next to mucinous carcinoma of ascending colon, 1 in anastomosis after resection of the transverse colon adenocarcinoma. The diameter of the lesions ranged from 5 to 12 mm. Histologically, SSAs showed asymmetrical proliferation of the epithelium, irregular shape of the crypts with their branching and some crypt dilatations especially in the basal parts of the crypts. Cellular atypia (dysplasia) was usually low. In 5 cases the nuclei were focally stratified and localized in the lower part of the cells. High-grade dysplasia was found only in SSA adjacent to mucinous adenocarcinoma. Immunohistochemically, SSAs showed secretion of gastrointestinal mucin expressing MUC2 and MUC5A. Both MUC2 and MUC5A were also positive in mucinous carcinoma. In previous studies these expressions were considered specific for serrated type of carcinogenesis. However, our study found positivity of MUC2 and MUC5A also in conventional adenomas. Expression of p53 in SSAs was minimal. SSAs have malignant potential comparable with conventional adenomas and for this reason they must be distinguished from HPs.

Adenoma↗

PubMed2005

Autoimmune gastritis. A clinicopathologic study of 25 cases.

The histopathological diagnosis of autoimmune gastritis (AG) in its early stages can be a diagnostic challenge. Even some advanced cases with complete atrophy of the corpus mucosa may be difficult to recognize. To establish the diagnosis of autoimmune gastritis, several histological features should be assessed and combined with immunostains for enterochromaffin cell-like (ECL) cells and G-cells. The main histological criteria include a mononuclear infiltrate within the lamina propria, foci of destruction of oxyntic glands, intestinal metaplasia (IM), pyloric metaplasia, and parietal cell pseudohypertrophy. These criteria were evaluated in our series of 25 patients with achlorhydria and/or megaloblastic anemia. Some of our patients presented with nonspecific gastrointestinal symptoms. The age ranged between 46 and 79 years; one male patient was only 31 years old. Histologically, the corpus mucosa displayed in all cases chronic inflammation with focal complete IM and advanced pyloric metaplasia. In 4 patients, oxyntic glands were destructed in some sites. There was a pancreatic metaplasia of acinar type in 2 patients and a minimal focal pseudohypertrophy of parietal cells in the 31-year-old man. A tubular adenoma with a low-grade dysplasia was found in one female patient. Immunohistochemically, chromogranin-A highlighted linear or nodular hyperplasia of ECL cells in 19 patients, and adenomatoid ECL hyperplasia in one case (80%). In the remaining cases hyperplasia of ECL cells could not be recognized from their normal count. In 13 cases (52%) a few ECL cells were seen also in IM. Regarding associated pathology, in one woman with nodular ECL cell hyperplasia, a gastric carcinoid was removed endoscopically. The reaction with gastrin antibody revealed in 11 cases (44%) a small number of G cells in IM in the corpus mucosa. In 18 patients, antral mucosa was examined as well. In 8 patients, the mucosa was normal; in 10 cases, a mild chronic inactive gastritis was diagnosed, and in 15 patients G-cell hyperplasia was found. In accordance with other studies, we show that the diagnosis of AG may be established microscopically in endoscopic specimens of the gastric body mucosa when histologic features and immunohistochemical detection of ECL and G cell hyperplasia are combined.

Aged↗

PubMed2005

Gastrointestinal stromal tumor (GIST) with glandular component. A report of an unusual tumor resembling adenosarcoma.

A case of gastrointestinal stromal tumor (GIST) with an unusual glandular component is reported. The tumor was found in the gastric fundus of a 93-year-old woman. Histologically, the lesion showed a biphasic adenosarcoma-like structure. Typical low-grade spindle cell patterns of GIST were intermingled with numerous and partly cystic glands. The glandular epithelium had pyloric/foveolar-like appearance, with foci of intestinal metaplasia and low-grade dysplasia. The stromal component was immunoreactive for CD117 (c-kit) and CD34, and negative for myoid and neuroid markers. The ultrastructural examination found nondescript and undifferentiated spindle cells. The gastric mucosa and submucosa near the tumor contained a small area with features of gastritis cystica profunda, with glands similar to those present inside the tumor. Therefore, a collision of GIST and gastritis cystica profunda is suggested in the histogenesis of the lesion.

Adenosarcoma↗

PubMed2004

The histological findings in the gastroesophageal junction of fetuses.

The development of the esophageal and gastric mucosa in the gastroesophageal junction was studied in 61 fetuses of 13-41 weeks of the gestational age. During the 13th-15th week, the esophageal multilayered epithelium was covered by a continuous layer of columnar mucous ciliated cells which were present only focally till the 25th week and disappeared later. Before the 15th week, the gastric mucosa was formed by pits only. The glands started as proliferating tubules in the basal parts of the pits in the 15th week. Further, they differentiated into oxyntic glands. The mucosa of the corpus was fully developed in the 27th week. The cardiac mucosa was absent in all the 10 fetuses examined between the 27th and 41st week of gestation. This supports the view that the gastric cardiac mucosa is not a physiological structure but that it results from glandular metaplasia of the distal esophageal mucosa due to gastroesophageal reflux.

Esophagogastric Junction↗

PubMed2004

[Ulcerative colitis--contemporary morphological criteria].

Regular bioptical examinations of patients with ulcerative colitis (UC) performed in recent years show that the inflammatory changes of the mucosa of the large intestine are not necessarily diffuse, and that their extent may vary in the course of the disease. To establish the diagnosis of UC and to assess the treatment efficacy it is important to examine histologically multiple mucosal specimens from different levels of the large intestine. In our series of 27 patients with ulcerative colitis (18 men and 9 women at the age of 17 to 76 years), active or active and inactive pancolitis was diagnosed in 25 cases (93%). In 11 of these, the whole of the large intestine was affected. Two patients showed diffuse pancolitis without caecal involvement, in 5 cases there was inactive inflammation in the rectum or in the sigmoid colon. Seven patients had active colitis of the rectum and sigmoid. In another 2 patients (7%), the inflammation was limited to several segments of the large intestine only (the descending colon, and the descending and transverse colon). On bioptical examination of 6 patients repeated after 2-29 months (mean 14 months), there were changes in the distribution and appearance of the inflammation. Thus our findings correspond with the results of previous studies: UC does not always affect the mucosa of the large intestine diffusely. Further, the extent and distribution of inflammatory changes vary in the course of the disease.

Adolescent↗

PubMed2002

Intestinal necrosis following Calcium Resonium-sorbitol administration in a premature uraemic infant.

Sodium polystyrene sulphonate (Resonium A) in sorbitol given as an enema or orally to treat hyperkalaemia has been described to induce intestinal necrosis in uraemic patients. We report a case of a premature infant with acute renal insufficiency who developed focal transmural necrosis and perforation of the small intestine after 10 days of administration of calcium polystyrene sulphonatum (Calcium Resonium) in sorbitol by enema and by nasogastric tube. On histological examination of the resected part of the small intestine, numerous strongly basophilic angular crystals of resonium were found in the lumen, in the necrotic wall, as well as in the organized exudate on the peritoneal surface. The crystals showed a strong direct Schiff positivity without preoxidation. They were also stained using PAS, Giemsa, Ziehl-Neelsen, Schmorl, and Gram method. In contrast, the crystals were Congo red and Alcian blue (pH 2.5) negative and non-birefringent. The direct Schiff positivity without preoxidation is virtually pathognomonic for resin crystals in routinely processed tissues. The same crystals were observed in the lumen of the small intestine and in peritoneal adhesions at autopsy. Thus our case provides additional evidence that Resonium A/Calcium Resonium in sorbitol administered as an enema or orally can lead to intestinal necrosis in uraemic patients.

Diuretics, Osmotic↗

PubMed2002

Composite dermatofibrosarcoma protuberans-giant cell fibroblastoma recurring as Bednár tumor-giant cell fibroblastoma with mucoid lakes and with amputation neuroma.

We report an unusual case of composite giant cell fibroblastoma-dermatofibrosarcoma protuberans (DFSP) that, in its second recurrence, contained a pattern of Bednár tumor (BT) and giant cell fibroblastoma (GCF). The recurrent tumor showed extreme myxoid change with creation of mucoid lakes, which mimicked a pattern of myxoid liposarcoma. One area in the recurrent lesion contained amputation neuroma overgrown with neoplastic spindle cells, which simulated a nerve sheath neoplasm. This case demonstrates common histogenesis of GCF, DFSP and BT, and it shows how broad morphological spectrum can be produced by a composite tumor, especially when the tumor includes unconventional growth pattern or additional non-neoplastic lesion.

Dermatofibrosarcoma↗

PubMed2001

[Epithelioid hemangioendothelioma of the liver (case report)].

Epitheliolid hemangioendothelioma of the liver is a very rare vascular tumor. We report the case of this tumor found during the autopsy of a 74-year-old man. The liver was infiltrated by multiple greyish nodes (diam. 3-30 mm), partially calcified. Microscopically, the tumor was composed of dense sclerotic and fibrous stroma with focal calcifications. In this stroma, groups and cords of irregular cells of two main types were dispersed. The first type was represented by big cells with large clear eosinophilic cytoplasm. The second cell type was characterised by medium-sized elongated, sometimes bizarre-shaped cells. Tumorous cells did not produce mucin. Immunohistochemically, the tumorous cells were positive for FVIIIR--Ag, actin, vimentin, and desmin and also showed disperse weak positivity for S100 protein. Staining for epithelial membrane antigene (EMA), keratins, and CD68 was negative. Neoplastic cells were positive for Ulex europaeus lectin. Electronmicroscopically, the tumorous cells had irregular nuclei, lobular or cleft shape with one or two nucleoli. Cytoplasm contained a high amount of intermediate filaments forming large aggregates. The cells were connected by desmosomes and partially surrounded by dense basal lamina. The diagnosis of epithelioid hemangioendothelioma is based on a histological picture, supported by immunohistochemical positivity for FVIIIR: Ag and Ulex europaeus and by additional ultrastructural study.

Aged↗

PubMed2001

Chlorpromazine-induced cholestatic liver disease with ductopenia.

We describe a 30-year-old pregnant woman in whom cholestatic liver disease developed 16 resp. 18 days after the medication of chlorprothixeni hydrochloridum and chlorpromazine treatment in the 33rd week of pregnancy. Clinically, the course was characterized by severe jaundice lasting 10 months, fever, pruritus, high serum alkaline phosphatase level, transient aminotransferase elevation, and hypercholesterolemia. The pregnancy was terminated in the 35th week by cesarean section with the birth of a premature female newborn without any signs of liver damage. The histological examination of the mother's liver revealed ductopenia, defined by the absence of interlobular bile ducts in at least 50% of the small portal tracts, and long-standing cholestasis with pseudoxanthomatous transformation of hepatocytes and ductular epithelia, and small lobular xanthomas. The jaundice resolved very slowly after ursodeoxycholic acid therapy. The liver function tests 26 months after the onset of jaundice showed only a slight elevation of alkaline phosphatase and aminotransferases. In the control liver biopsy, non-active periportal and septal fibrosis without signs of cholestasis was seen. To our knowledge this is the sixth report to document chlorpromazine-induced ductopenia in pregnancy and the first to describe a newborn without any liver damage.

Adult↗

PubMed2001

Neonatal (perinatal) hemochromatosis.

Autopsy findings are described of six cases (from four families) of neonatal hemochromatosis, a defect featured by severe prenatal iron storage in the liver and in a number of visceral organs similarly as in the hereditary adult-type hemochromatosis. Genetically, the two disorders are different, however. All the cases showed a characteristic liver damage with heavy iron deposits in the liver cells, lobular disarray and intralobular fibrosis, tendency towards multinuclear hepatocyte formation, ultimately resulting in pigmented cirrhosis and liver failure. In five instances the cirrhosis present was atrophic, in one case it was hepatomegalic. Death occurred prenatally in one case (31st week), perinatally in three, and two cases died as young infants. The mechanism of the liver disorder, mainly that of iron accumulation, has remained unresolved. Extrahepatally, hemosiderosis affected various epithelia (in particular, thyreocytes, renal distal tubular epithelia and those of pancreatic acini) and myocardial cells which, however, did not show any damage. In two instances the placenta was conspicuously hyperplastic, in one case it showed prominent hydropic transformation of its villi. In one case there was simultaneous cytomegaly. Entities which must be considered in the differential diagnosis of neonatal hemochromatosis because of considerable hepatic and extrahepatic iron accumulation have been discussed.

Adult↗

PubMed2000

Subclinical course of cholesteryl ester storage disease in an adult with hypercholesterolemia, accelerated atherosclerosis, and liver cancer.

Few cases of asymptomatic cholesteryl ester storage disease (CESD) due to low enzymatic activity of human lysosomal acid lipase/cholesteryl ester hydrolase (hLAL) have been reported thus far in adults Here, we describe a 51-year-old man with a long clinical history of mixed hyperlipoproteinemia and severe premature atherosclerosis, but with no signs of hepatomegaly, liver dysfunction, or splenomegaly. The disease was discovered by chance in a biopsy performed because of suspected liver cancer (proven to be a cholangiocarcinoma). Residual hLAL activity in peripheral leukocytes was determined to be 6% of control values. DNA sequence and restriction fragment length polymorphism analysis demonstrated that the patient was a compound heterozygote for the prevalent CESD exon 8 splice site mutation (G934A) and the deletion of a C (nucleotide 673, 674, or 675) in exon 6 of the hLAL gene, resulting in premature termination of protein translation at residue 195. The patient died of liver failure as a consequence of extensive tumor infiltration at age 52. Lipid analysis revealed moderate cholesteryl ester storage in the liver and in the suprarenal cortex, and massive accumulation in the testicular histiocytes and Leydig cells, resulting in a pronounced secondary atrophy of the seminiferous tubules. Our case study demonstrates that hepatomegaly is an inconstant feature, even in CESD patients compound heterozygous for a Wolman mutation which results in complete loss of hLAL enzymic activity. It also highlights the need to be aware of this condition as it may be underdiagnosed.

Adult↗

PubMed1999

[Lysosomal acid lipase deficiency. Overview of Czech patients].

Lysosomal lipase deficiency is a hereditary autosomal recessive enzymopathy leading to lysosomal storage of triacylglycerols (TAG) and cholesterol esters (CE). In particular cells with a permanently high receptor-mediated LDL endocytosis are affected (liver, kidneys). There are two basic phenotypes. The fatal infantile phenotype (Wolman's disease) with generalized storage of both types of apolar lipids. This form was diagnosed in this country only once. The opposite is the protracted, oligosymptomatic form encountered in all age groups. It is characterized by the storage of CE (which gave this entity the name of cholesteryl storage disease--CESD). Its main sign is affection of the liver (hepatomegaly, hepatopathy), which in some instances may lead to organ failure, directly or after cirrhotic transformation. Furthermore there is permanent hypercholesterolaemia (high LDL cholesterol) due to increased VLDL synthesis by hepatocytes, low HDL cholesterol and variably raised TAG. This constellation of blood lipids is a risk factor for the development of atherosclerosis. In the course of 25 years in the Czech Republic 13 cases of CESD were diagnosed in 11 families. Ten of these cases were characterized by clinically manifest hepatopathy with hepatomegaly, detected incidentally during medical examinations (at the age of 2-14 years). In three adult patients with permanent hypercholesterolaemia the storage process was subclinical and the diagnosis was established quite incidentally by examination of non-specific secondary and tertiary manifestations of the disease. The diagnosis was established in all cases of CESD at the tissue level (liver biopsy), at the biochemical (acid lipase deficiency) and molecular genetic level (mutation in enzyme locus). In all instances mutation of G934A was found leading to reduction and loss of the eighth exon. This mutation was present in five patients in a homozygous state. Six mutations were heterozygous. In one instance for technical reasons only one allele was analyzed. In three instances a point "missense" mutation was found: T323A (Trp74Arg), T4(75)A (Asp124Glu), A210T (Asp36Gl), in one instance a "nonsense" mutation: C233T (Arg44-stop) and twice a deletion mutation delta C673-5 and delta G1068-8 leading to impairment of the reading frame and to premature stop of the codon.

Adult↗

PubMed1999

Liver preneoplastic changes in mice treated with the herbicide fomesafen.

1. Effect of the diphenyl ether herbicide fomesafen on liver preneoplastic changes and porphyrin biosynthesis was examined in male C57BL/6J mice (0.23% in the diet for 14 months) and ICR mice (0.3% in the diet for 50 weeks). Fomesafen treatment resulted in preneoplastic changes (liver nodules and foci of altered hepatocytes) in both strains, uroporphyria developed only in ICR mice. 2. Iron pretreatment (600 mg/kg as a single dose) accelerated the development of fomesafen-induced preneoplastic changes in both mouse strains. The number of foci containing altered hepatocytes, as well as the number and size of liver nodules, were increased in iron-pretreated animals. 3. A single injection of iron induced marked uroporphyria in C57BL/6J mice after 14 months (liver porphyrin content 102 nmol/g). This uroporphyria was further potentiated by fomesafen administration (208 nmol/g). 4. In ICR mice, liver histology was apparently normal after a 3 month recovery from fomesafen treatment (0.32% for 9 months). Liver porphyrin content (260 nmol/g) started to decrease immediately after fomesafen withdrawal, but was still significantly elevated after 3 months (5 nmol/g), as compared to controls (1 nmol/g). 5. It is concluded that the toxicological evaluation of fomesafen should focus on liver porphyrin biosynthesis.

Animals↗

PubMed1998

[Morphologic findings in liver tissue in mice after long-term administration of the herbicide fomesafan].

Administration of herbicide fomesafen and of fomesafen combined with one dose of iron to 44 mice during 3 to 14 months caused hyperplastic and preneoplastic changes in the liver tissue which had been described in experimental carcinogenesis* small groups of altered hepatocytes storing glycogen or lipids and foci of small basophilic liver cells occurred as early as after 3 months. Altered hepatocytes were found more frequently in mice getting fomesafen and iron. Later nodular hyperplasia of liver cells developed with nodes 3-20 mm in diameter which mostly consisted of altered hepatocytes with plenty of glycogen. After 12 and 14 month-lasting administration of fomesafen and fomesafen with iron, the hepatocellular carcinoma was proved in 5 mice. In 4 mice, the preneoplastic changes in liver tissue were accompanied by micronodular hyperplasia of liver cells which did not participate on the development of big nodes and hepatocellular carcinoma.

Animals↗

PubMed1998

Cytoplasmic liver cell inclusions--a typical feature of porphyria cutanea tarda--are absent in porphyria-related hepatic neoplasias.

Crystalline cytoplasmic needle-shaped inclusions in hepatocytes are considered to represent a specific morphological feature of porphyria cutanea tarda (PCT) and experimental PCT-like porphyrias. The cytoplasmic inclusions, however, are absent in hyperplastic hepatic nodules and hepatocellular carcinomas arising in the course of these conditions. It is assumed that porphyrins and related substances accumulated in hyperplastic and neoplastic hepatic lesions differ from those found in non-neoplastic liver tissue: the highly carboxylated porphyrins are stored in both sites, the crystal-forming substance only in non-proliferating liver tissue.

Animals↗

PubMed1998

[Needle-like inclusions in liver cells are specific diagnostic signs of porphyria cutanea tarda].

Needle-shaped cytoplasmic liver cell inclusions are considered to represent a feature typical of porphyria cutanea tarda (PCT). Histological sections of 849 liver biopsies were stained with ferricyanide reduction reaction for demonstration of the inclusions. They were detected in 18 cases, and in all of them the diagnosis PCT was clinically and/or biochemically confirmed. In our group of patients, PCT was associated with alcoholic liver disease in three cases, with chronic hepatitis C in three cases and with hepatocellular carcinoma in two cases. In the liver with hepatocellular carcinoma, the inclusions were present only in non-neoplastic liver tissue, not in the tumourous tissue. No inclusions were found in the liver tissue of patients without clinical signs of PCT.

Adolescent↗

PubMed1997

Liver cell cytoplasmic inclusions in experimental porphyrias: their demonstration with the ferric ferricyanide reduction reaction.

In the present paper we describe needle-shaped and granular cytoplasmic inclusions in the liver cells of mice and rats with experimental porphyria biochemically resembling human porphyria cutanea tarda. The inclusions were inconspicuous in routine histological slides. The ferric ferricyanide reduction reaction, however, enabled us to demonstrate their shape and location within the hepatic lobule. Needle-shaped inclusions are considered to represent a structure specifically seen in experimental porphyrias resembling porphyria cutanea tarda. These structures are similar to the inclusions seen in human porphyria cutanea tarda.

Animals↗

PubMed1997

[The ferricyanide reduction reaction as a staining method in liver biopsy].

In paraffin sections from human liver tissue, ferric ferricyanide reduction reaction enables to visualize various reducing substances. Firstly it is cholestasis due to reduction capacity of bilirubin. Another reducing substance is lipofuscin pigment. Finally, the reaction represents the only way to demonstrate the needle-shaped cytoplasmic inclusions in routine practice considered to be specific for porphyria cutanea tarda. The method is simple and inexpensive and it may be recommended as a suitable supplementary staining procedure for liver biopsies.

Biopsy↗