[Alpha 1-antitrypsin deficiency--a hereditary storage disease of the hepatic endoplasmic reticulum].
Explore the source record for details and available documents.
Endoplasmic Reticulum↗
Biomedical subjects
K Husek
Publications and source records attributed to K Husek.
[Malignant transformation in multiple eccrine spiradenoma].
Development of a poorly differentiated eccrine carcinoma was observed in a 6-year-old woman. She had been operated on many times during 20 years for some tens of classical as well as less usual forms of eccrine spiradenomas, e.g. giant vascular spiradenomas. They were mostly localized in the skin of back, thorax and neck. The patient died of an extensive skin involvement and spine and liver secondaries 12 months after occurrence of the carcinoma. The structure of carcinoma was trabecular and partly papillary, tumour cells had bulky nuclei and striking nucleoli. There was a juxtaposition of spiradenomas with carcinoma and direct transformation of spiradenoma into carcinoma was observed. Immunohistological positivity of carcinoma concerned S-100 protein, slightly CEA, focally cytokeratin 7 and 18; cytokeratin 14 was negative. Ultrastructure of tumour cells showed irregular intercellular lumina with some microvilli, but structures characteristical for eccrine glandular of ductal differentiation were lacking.
Adenoma, Sweat Gland↗
[Liver diseases in children with alpha 1-antitrypsin deficiency in infancy].
The authors evaluate the health status of children with alpha-1-antitrypsin deficiency, focused on liver disease in infant age. The children were selected by neonatal screening. Of 21 children one had severe neonatal hepatitis with progression to cirrhosis, 2 children had clinically apparent jaundice to the age of two months, 6 children had elevated total bilirubin and transaminase levels without clinical signs of the disease, 12 of the remaining children had no clinical and laboratory signs of liver disease. In the discussion the authors compare the results with data published abroad.
Female↗
[Vaginal adenosis and problems in its treatment].
The authors investigated four patients with suspected vaginal adenosis without prenatal exposure to DES. Adenosis was confirmed by histological examination in two instances. In one case the adenosis was formed by glands of the endometrial type. In the second case it was formed by glands of the endocervical type with atypical features. On check-up biopsy an adenocarcinoma was revealed (non-light cell) resembling morphologically a cervical adenocarcinoma. The authors discuss the embryogenesis, histogenesis, epidemiology and differential diagnosis of adenosis. At the same time the authors reflect on problems of its therapy. Finally the authors summarize some practical recommendations for diagnostic and therapeutic purposes.
Adenocarcinoma↗
[Secondary adenocarcinoma of the ovary and problems in diagnosis].
In a group of 10 secondary ovarian adenocarcinomas primary tumours were verified by biopsy in colorectal region (8 cases), once in gall bladder and once in appendix. The age of patients varied between 36 up to 60 years. The lesion was unilateral in 8 cases, bilateral in two. Six colorectal adenocarcinoma secondaries reminded a bit of endometroid carcinoma. Two colorectal and a gall bladder carcinoma secondaries resembled ovarian mucinous cystadenocarcinoma. Signet ring cells were not present. Appendical mucinous cystadenocarcinoma produced a metastasis that was alike borderline ovarian mucinous cystadenoma. Intracellular mucin reaction was negative or focally positive in former 6 cases, a diffuse positivity was found in 4 cases.
Adenocarcinoma↗
[Aggressive angiomyxoma of the female pelvis and perineum, M-8841/1].
Aggressive angiomyxoma of pelvis and perineum was identified in two women (of 31 and 53). Its site was in the right fossa ischiorectalis and vulva. There was not observed any relaps in 60 and/or 9 months after surgery. Amorphous tumour mass was soft with finger-like projections. Spindle and starshaped tumour cells were spread in a loose edematous myxoid vascularized stroma; they had a benign character lacking of mitoses and nuclear atypia. Myxoid stroma could be stained rather faintly with Alcian blue at pH 1. Dilated capillaries, veins and arterioles were a substantial component of the tumour. Nerve and muscle fibres and their fragments were included here and there in the tumour. Tumour cells had a fibroblast ultrastructure which was supported by immunohistology. Discussion comprised differential diagnosis of myxoma, myxoid liposarcoma and myxoid type malignant fibrous histiocytoma among others.
Adult↗
[Anaplastic osteoclastic carcinoma of the gallbladder].
Description of an unusual carcinoma of the gall bladder in a 55 year-old woman. Bioptical finding based on light microscopy, electron microscopy and immunohistology is compared with autopsy. Histogenesis of osteoclast-like structures in the carcinoma is discussed.
Adenocarcinoma↗