Zaklad Patologii

Warsaw, Poland

Zaklad Patologii

Warsaw, Poland
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The aim: Diagnosis and efficacy of 5-amino-2-hydroxybenzoic acid (5-ASA) or Saccharomyces boulardii or magnesium in therapy of microscopic colitis with presence of foamy macrophages in children Material and methods: The basis for diagnosis and inclusion to the analysed group was presence of characteristic foamy macrophages in histological examination of hematoxylin end eosin-stained specimens collected from the large intestine, reviewed in microscope under x 200 or x 320 magnification. 144 children were included to the analysis. For 12 months 38 children were treated with mesalasine (5-amino-2-hydroxybenzoic acid), 35 children received Saccharomyces boulardii and 35 children were treated with magnesium aspartate. The control group consisted of 36 children who received no therapy. Results: No statistically significant improvement was found following the use of 5-amino-2-hydroxybenzoic acid in therapy of the disease. The use of Saccharomyces boulardii was associated with statistically significant improvement in clinical, endoscopic and histological condition. The treatment with magnesium caused a histological, statistically significant improvement, but failed to have any effect on the clinical and endoscopic presentation. In the group of children in whom none therapeutic intervention was provided, a statistically significant spontaneous clinical improvement was observed, but no statistically significant changes in endoscopic and microscopic condition were found. Conclusions: Efficacy of the therapy with probiotic Saccharomyces boulardii was confirmed in patients with microscopic colitis with presence of foamy macrophages. No fitness of 5-amino-2-hydroxybenzoic acid for therapy of the disease was demonstrated. Untreated children present a tendency for spontaneous clinical remission. A 12-month follow-up in that group showed no progression of endoscopic and microscopic lesions. © 2011 by Polskie Towarzystwo Pediatryczne Otrzymano.

Klimczak A.,Klinika Nowotworow Tkanek Miekkich | Pekul M.,Zaklad Patologii | Wiater K.,Klinika Nowotworow Tkanek Miekkich | Rutkowski P.,Klinika Nowotworow Tkanek Miekkich
Nowotwory | Year: 2011

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor composed mostly of perivascular epithelioid cells (PEC). The PEComa related tumors encompass angiomyolipoma, clear cell "sugar" tumor of the lung, lymphangioleiomyomatosis (AML) and clear cell myomelanocytic tumor (CCMT). Tumors of the PEComa family are rare and usually occur sporadically. LAM and AML are also frequently observed in patients with tuberous sclerosis simplex complex (TSC). Most PEComas are benign and do not recur after complete surgical resection. However, a subset of PEComas exhibit malignant behaviour. There is no known effective systemic therapy for PEComa. Recently published studies have shown that inhibition of the mTOR signaling pathway might constitute a promising treatment modality in patients with PEComa.

Lipka B.,Klinika Niemowleca | Milewska-Bobula B.,Klinika Niemowleca | Cielecka-Kuszyk J.,Zaklad Patologii | Pakula-Kosciesza I.,Pracownia Rezonansu Magnetycznego
Pediatria Polska | Year: 2011

The main cause of salivary glands swelling in children is acute inflammation (usually viral), sialolithiasis and tumors. The most common viral infection is parotitis epidemica caused by paramyxoviruses, then cytomegalovirus infection. Cytomegalovirus is the major cause of congenital viral infections, but isolated inflammatory salivary gland disease in the course of intrauterine cytomegalovirus infection is very rare. The article discusses the clinical course of cytomegalovirus infection in two patients with congenital enlargement of parotid gland, in which despite the use of antiviral treatment the local improvement was not achieved. © 2011 Polish Pediatric Society.

Taybert J.,Klinika Chorob Metabolicznych | Pronicki M.,Zaklad Patologii | Bednarska-Makaruk M.,Zaklad Genetyki | Sykut-Cegielska J.,Klinika Chorob Metabolicznych
Przeglad Pediatryczny | Year: 2011

Cholesterol ester storage disease (CESD) is a recessive disorder due to deficient activity of lysosomal acid lipase leading to lysosomal accumulation of cholesterol esters and triglycerides. Its prevalence in the Polish population is unknown. Clinical manifestation includes hepatomegaly (usually mild at onset) with hypercholesterolemia (hyperlipidemia) and hypertransminasemia. Liver insufficiency or cirrhosis may not appear until adulthood. Microscopic examination of liver biopsy shows relatively typical changes. Liver lipids chromatography reveals storage of cholesterol esters, which suggests diagnosis. Demonstration of acid lipase deficiency in leukocytes confirms the diagnosis. Hypercholesterolemia is often the predominant sign in cases with slight hepatic manifestation. Contrary to familial hypercholesterolemia (FH) of autosomal predominant inheritance (heterozygosity 1:500), in patients with CESD family history is negative (except when FH coexists). Differential diagnosis does not reveal hypoglycaemia typical for glycogen storage diseases or severe cholestasis. Up to now there is no specific treatment. Management includes dietary treatment of hypercholesterolemia, monitoring of liver function, and in individual cases statins are ordered. Enzymatic replacement therapy is in the course of trials. Genetic counseling should be provided. Risk of occurrence of CESD is different than in FH. To sum up, precise family history taking, palpable examination of abdomen and basic laboratory liver function tests are helpful for preliminary differential diagnosis in children with hypercholesterolemia.

Cukrowska B.,Zaklad Patologii | Ceregra A.,Klinika Pediatrii Instytutu Pomnika | Klewicka E.,Zaklad Mikrobiologii Technicznej Politechniki LO dzkiej | Slizewska K.,Zaklad Mikrobiologii Technicznej Politechniki LO dzkiej | And 2 more authors.
Przeglad Pediatryczny | Year: 2010

Introduction: Probiotics have been used both in the prevention and treatment of allergic diseases. Aim of the study: The evaluation of the activity of probiotic strains Lactobacillus (L.) casei ŁOCK 0900, L. casei ŁOCK 0908 and L. paracasei ŁOCK 0919 in children with food allergy demonstrating as atopic eczema (AE) in a 2-year observation. Material and methods: The randomized double blind placebo controlled clinical trial included 60 children in with cow's milk protein allergy under 2 years of age. Bacteria were applied per orally for 3 months in daily dose of 109 cells. AE symptoms were evaluated by SCORAD index before treatment, at the end of bacteria in-take, then 5 and at least 21 months later. Results: Probiotic application induced statistically significant clinical improvement (a decrease in SCORAD more than 50%), mainly in children with IgE-dependent AE. Two-year observation showed that irrespective of eczema type SCORAD index significantly decreased in comparison with 5 months after probiotic in-take only in group supplemented with probiotics. After 2 years 81% of children tolerated cow's milk proteins in probiotic group whereas in the control group this percentage was lower (68.5%). Conclusions: Application of probiotic strains L. casei and L. paracasei induces clinical improvment of AE in children and affects the developmnet of tolerance to cow's milk proteins in long term observation.

Koc Z.,Poradnia Specjalistyczna Chirurgii Onkologicznej | Latka D.,Oddzial Neurochirurgii | Sachanbinski T.,Oddzial Chirurgii Onkologicznej | Nasierowska-Guttmejer A.,Zaklad Patologii | Szawlowski A.W.,Centrum Onkologii Instytut Im. Marii Sklodowskiej Curie
Nowotwory | Year: 2012

Primary lepto-meningeal melanoma represents less than 0.1% of all tumours of the central nervous system. It occurs in two different forms: as a primary malignant lepto-meningeal tumour or as a primary lepto-meningeal melanomatosis. Current review of medical literature showed only 45 such case reports. Due to the lack of characteristic symptoms, each case was discovered by chance during surgery or at the time of autopsy. The histogenesis of primary melanoma of the CNS is still unknown issue. For the time being, many different theories have been proposed but have not become fully proven. In this paper the case of a 66-year old male patient with primary melanoma of lepto-meningeal space cone vertebral core was presented.

Background. Lobular neoplasia is a hyperplastic breast lesion - a borderline type of lesion with a high risk of subsequent development of invasive carcinoma. In case of radial scar diagnosis the risk of invasive carcinoma increases twice in comparison with healthy patients population while in the case of lobular neoplasia diagnosis within radial scar this risk increases 8 to 10 times. Basing on the presented case we analyse and review the literature regarding the clinical, radiological and pathological aspects of lobular neoplasia of the breast. Case report. A 67-yeas old patient was hospitalised in 1995 in order to consult the results of mammography, which revealed a "radial scar" lesion of the right breast. The patient was then referred to undergo wide local excision. Pathological examination showed dysplastic changes of the "radial scar"type with 1 cm multifocal lobular neoplasia. The patient was systematically followed clinically and radiologically during the decade 1996-2006 and all examination results were normal. In 2008, an ill-defined nodule appeared within the scar on mammography examination. This was categorized as BI-RADS 4C. The mammotomic biopsy performed under ultrasonography control revealed invasive lobular carcinoma. The tumor was totally locally excised and the sentinel node was histologically verified. The histopathological examination revealed a 1.6 cm focus of invasive lobular carcinoma and the sentinel node was negative. Conclusion. Patients with detected lobular neoplasia should be treated as a risk group of invasive breast cancer development (30-40% vs 10% in a healthy population during the entire life period). Systematic clinical and radiological follow-up should be mandatory.

Grajkowska W.,Zaklad Patologii
Aktualnosci Neurologiczne | Year: 2011

Medulloblastoma is the most common paediatric brain tumour. It is a malignant embryonal tumour of the cerebellum with predominantly neuronal differentiation and tendency to metastasises via CSF pathways. The WHO classification of CNS tumours (2007) distinguishes classic medulloblastoma and four variants: desmoplastic/nodular (D/N), medulloblastoma with extensive nodularity (MBEN), anaplastic medulloblastoma and large cell medulloblastoma. Classic medulloblastoma consists of sheets of small cells with prominent nucleus and scant cytoplasm. Neoplastic cells of classic medulloblastoma might be elongated with oval nuclei and display moderate nuclear pleomorphism. Some classic tumours contain Homer Wright rosettes or palisades. The main histopathological features of D/N medulloblastoma and MBEN are nodules of differentiated neurocytic cells and internodular desmoplasia. Anaplasia in medulloblastoma is defined as marked nuclear pleomorphism, cell molding, cell wrapping, high mitotic activity and apoptosis. The large cell medulloblastoma contains groups of large cells with round nuclei with a single nucleolus. Because of the rarity of the pure large cell medulloblastoma and mixture of large cell and anaplastic phenotypes in some medulloblastoma, it is suggested to combine the two variants into a single category of large cell/anaplastic medulloblastoma. MBENs and D/N medulloblastomas in infants have better prognosis than classic tumours. Large cell/anaplastic medulloblastomas are more aggressive tumours. © Aktualn Neurol 2011.

Jonska-Gmyrek J.,Zaklad Teleradioterapii | Bobkiewicz P.,Zaklad Patologii | Gmyrek L.,Kliniczny Oddzial Ginekologii | Zolciak-Siwinska A.,Zaklad Brachyterapii | And 2 more authors.
Ginekologia Polska | Year: 2013

Merkel cell carcinoma (MCC) is a rare malignant neoplasm, mostly affecting the skin (97% of cases). It is usually found in elderly people, in the sun-exposed areas of the skin. About 50-60% of MCC cases are located on the head and the neck, less often on the extremities and the torso, and extremely rarely in the genital area. Ultraviolet radiation may be the main factor responsible for the development of the tumors but viral etiology is also debated. Due to extremely rare incidence of MCC in the area of the vulva, proper management remains a challenging task. Aim: To present a case of an aggressive MCC of the vulva and a review of the literature. Material and methods: A previously healthy, 72-year-old patient presented at the Oncology Center of the Maria Sklodowska-Curie Institute, Warsaw, in June 2010. Four months previously the patient noticed a painless lump in the vestibular region of the vagina. She received anti-inflammatory treatment at her local gynecological clinic, with no success. In February the patient underwent removal of the vulvar tumor. Histopathological examination confirmed anaplastic carcinoma. Microscopic evaluation revealed the tumor diameter to be 15mm. Surgical margins were free of neoplastic infiltration. The patient did not receive adjuvant therapy due to the results from the histopathological protocol. The disease recurred after three months. Radical vulvectomy and bilateral inguinal femoral lymphadenectomy were performed in May 2010. Histopathological examination confirmed microcellular carcinoma with no metastases to the lymph nodes and complete resection of the tumor (R0). The disease recurred in the next two months: a 50-mm tumor was found in the right inguinal lymph nodes. The decision to verify all histopathological material obtained during all procedures performed so far was made. Immunohistochemical evaluation confirmed MCC. Adjuvant radiotherapy was recommended. The area of the vulva, pelvic and inguinal lymph nodes were irradiated. One month after therapy completion the patient complained of pain in the lumbar area. An ultrasound examination of the abdomen revealed a tumor (9 cm in diameter) in the para-aortic region but it was not histopathologically verified due to extremely poor overall condition of the patient. As the condition of the woman deteriorated systematically, the patient was referred to a hospice facility, where she died 9 months since the primary diagnosis. Conclusions: MCC of the vulva is a rare neoplasm with an aggressive course. Clinical and histopathological diagnostic difficulties and consequently lack of standardized management, result in low survival rates. © Polskie Towrzystwo Ginkoogiczne.

Wicher D.,Klinika Gastroenterologii | Jankowska I.,Klinika Gastroenterologii | Rubik J.,Klinika Nefrologii | Cielecka-Kuszyk J.,Zaklad Patologii | Pawlowska J.,Klinika Gastroenterologii
Pediatria Wspolczesna | Year: 2012

The primary hyperoxaluria type 1 (PH1) is an autosomal recessive, inherited disease caused by a defect in glyoxylate metabolism. PH1 is characterized by excessive production and urinary excretion of oxalate that lead to renal and systemic damage through the deposition of oxalate crystals into tissues. Although some patients may benefit from aggressive conservative management, the only curative treatment to date is a combined liver-kidney transplantation. © 2012 Cornetis.

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