Sex differences in renal damage induced in the mouse by Amanita virosa

The sensitivity of male and female mice to Amanita virosa was compared. Dried, homogenized mushroom was given orally by stomach tubing at doses of 100, 200, 400 and 800 mg dried mushroom/kg body weight. Both in males and in females, the kidneys were the only organs showing macroscopical changes. The dose of 100 mg/kg caused renal damage in females, whereas in males the first signs of kidney damage were seen at the dose of 400 mg/kg. The renal lesions observed in the males were located in the cortex, while in the females they were limited to the outer stripe of the outer medullary zone. Testectomy diminished the nephrotoxicity of A. virosa in male mice and caused changes in the localization of renal lesions.     

Fibrosing cholestatic hepatitis: a report of three cases

Fibrosing cholestatic hepatitis is an aggressive and usually fatal form of viral hepatitis in immunosuppressed patients. We report three cases of fibrosing cholestatic hepatitis in various clinical situations. Case 1 was a 50-year-old man who underwent a liver transplant for hepatitis B virus (HBV)-associated liver cirrhosis. Two and a half years after the transplant, he complained of fever and jaundice, and liver enzymes were slightly elevated. Serum HBsAg was positive. Case 2 was a 30-year-old man in an immunosuppressed state after chemotherapy for acute lymphoblastic leukemia. He was a HBV carrier. Liver enzymes and total bilirubin were markedly elevated. Case 3 was a 50-year-old man who underwent renal transplantation as a known HBV carrier. One year after the transplant, jaundice developed abruptly, but liver enzymes were not significantly elevated. Microscopically lobules were markedly disarrayed, showing ballooning degeneration of hepatocytes, prominent pericellular fibrosis, and marked canalicular or intracytoplasmic cholestasis. Portal inflammation was mild, but interphase activity was definite and cholangiolar proliferation was prominent. Hepatocytes were diffusely positive for HBsAg and HBcAg in various patterns. Patients died of liver failure within 1 to 3 months after liver biopsy in spite of anti-viral treatment.     

致死性侏儒-附三例报告

目的研究致死性侏儒的临床、影像学、致病基因和病理学表现。方法复习1438例尸检中诊断为致死性侏儒的3例作回顾性研究。结果致死性侏儒胎儿长骨极短，可有不同程度的弯曲，四肢软组织堆积，胸腔狭小、肋骨短，心胸比例大于正常，腹部膨隆，头颅大、前额突，塌鼻。结论致死性侏儒是一种最常见的致死性短肢畸形，由纤维母细胞生长因子受体3（FGFR3）基因突变所致，具有明确的临床、影像学和病理学特征。     

Juvenile dermatomyositis: A report of three cases

Juvenile dermatomyositis (JDM), an autoimmune idiopathic myositis, is characterized by rash and proximal muscle weakness. Immunohistopathology typically shows perivascular inflammatory infiltrate with predominance of CD4+ T lymphocytes, perifascicular atrophy, and upregulation of major histocompatibility complex class I. JDM has been attributed to a humoral-driven muscle microangiopathy probably implicating the type I interferon pathway. Tubulo-reticular inclusions present in endothelial cell of muscle are biomarkers of interferon exposure, and so may be an indirect data of this myopathy especially in the absence of rash and inflammatory infiltrate. We report on three patients in which electron microscopy solves the differential diagnosis among infantile myositis showing peculiar inclusions.     

Brunner gland cyst: report of three cases

Brunner gland cyst is a benign polypoid or nodular lesion of the duodenum that may not be familiar to gastroenterologists and pathologists because of its rarity. There have been only 9 cases documented in the world literature under various names. In this report, we describe the clinicopathologic features of three such cases to raise the awareness of its existence.     

Alpha-fetoprotein-producing lung carcinoma: report of three cases

Three cases of alpha-fetoprotein (AFP)-producing lung carcinoma were studied histologically and immunohistochemically. Samples were obtained from two men and one woman who ranged in age from 64 to 71 years. Serum AFP levels for the three samples were 9826, 74.4 and 24.3 ng/mL. One case was classified as stage IIIA and two as stage IIIB. Two cases were diagnosed as large cell neuroendocrine carcinoma, and AFP expression was detected immunohistochemically. One of these samples showed differentiation to a hepatoid carcinoma, while the other was combined with a squamous cell carcinoma. The remaining case was a squamous cell carcinoma, and AFP was detected in only some of the tumor cells. All patients died within 2 years. The Ki-67 labeling indices of the AFP-producing pulmonary carcinomas (30.2 +/- 4.6%) were significantly higher than those of AFP-negative pulmonary carcinomas (P < 0.05). The high proliferative activity, advanced stage at presentation, vascular endothelial growth factor expression and vascular invasion observed in these tumors may explain the poor prognosis of AFP-producing lung carcinomas.     

Anophthalmia-Waardenburg syndrome: A report of three cases

We report on 2 Turkish families with children who had bilateral anophthalmia, upper and lower limb abnormalities, mental retardation and consanguineous parents. We have evaluated the 2 cases in the first family and the only case in the second as anophthalmia-Waardenburg syndrome. This is an extremely rare autosomal recessive syndrome. © 1996 Wiley-Liss, Inc.     

Malignant eccrine poroma: report of three cases

The characteristic clinical and histological features of three cases of malignant eccrine poroma are discussed, in addition to the metastatic disease that had occurred in two cases. These cases were compared with previously reported cases of malignant eccrine poroma that had metastasised, and it is suggested that a strict classification of malignant eccrine sweat gland tumours should be made.     

CFC syndrome: report on three additional cases

We describe three patients, originating from three different Italian localities, affected by the cardio-facio-cutaneous (CFC) syndrome. In addition to a varying degree of mental retardation, these patients present characteristics consisting of a peculiar face with bitemporal frontal constriction and other anomalies involving the eyes, nose, ears, hair, skin, and heart that are consistent with this diagnosis.     

Ependymoma of the ovary: report of three cases

Three apparently pure ependymomas of the ovary occurred in young women 25 to 35 years of age who presented with abdominal swelling or pain. At operation one tumor was stage IC, and two were stage III. On microscopic examination the tumors contained cells with fibrillary cytoplasmic processes, often arranged around blood vessels to form perivascular pseudorosettes. Immunohistochemical staining for glial fibrillary acidic protein confirmed the nature of the tumor in each case. The patient with the stage IC tumor was alive and well five years postoperatively. One patient with stage III disease died of tumor six years postoperatively, and insufficient time elapsed after treatment of the second to allow meaningful follow-up data. These cases illustrate the rare occurrence of ovarian tumors resembling differentiated tumors of the central nervous system. Recognition and distinction of these tumors from common epithelial tumors, which they may resemble, have important prognostic and therapeutic implications.     

Restrictive dermopathy: a report of three cases.

We report three infants with a rare syndrome of restrictive dermopathy, in which rigidity of the skin at birth is associated with characteristic facial anomalies, generalised arthrogryposis, bony abnormalities, and lung hypoplasia. The skin has a distinctive pathology with compaction of the dermal collagen and fibrosis of the subcutaneous tissue. The inheritance is likely to be autosomal recessive and the condition appears to be fatal in the early neonatal period.     

Giant cell fibroblastoma: a report of three cases

Three cases of giant cell fibroblastoma are presented. All three patients were boys younger than four years of age. The neoplasms involved the subcutaneous tissue of the back, perineum, and shoulder, respectively. The tumors were characterized histologically by a mixture of spindle cells and multinucleated giant cells in a myxoid or collagenous background and by the presence of irregular, sinusoidal spaces. All three patients had local recurrence, but all were tumor-free at latest follow-up.     

W syndrome: report of three cases and review

Only three cases of W syndrome have been reported. These patients have a typical "pugilistic" face, incomplete oral cleft, absent upper incisors, mental retardation, spasticity, seizures, and acne scars. Two of them had additional skeletal anomalies. Here we report on three male patients with findings compatible with the W syndrome. We emphasize the importance of some constant findings and describe additional signs. Familial history supports X-linked dominant heredity, as postulated previously.     

Filippi syndrome: report of three additional cases.

Filippi syndrome is an autosomal recessive condition characterized by variable soft tissue syndactyly of the fingers and toes, microcephaly, pre- and postnatal growth retardation, mildly abnormal craniofacial appearance, and mental retardation. We report on three unrelated individuals with Filippi syndrome. All have microcephaly, minor facial anomalies, variable syndactyly of digits, growth impairment, and developmental delay. One patient also has polydactyly, which has not been reported previously in the Filippi syndrome.     

Cytological features of lipoblastoma: a report of three cases

Lipoblastoma is a rare benign neoplasm occurring mostly in children under the age of three. Accurate preoperative diagnosis is mandatory for planning the treatment. The main aim of this study is to establish the cytological features of lipoblastoma and to answer the question: “Is cytological diagnosis of lipoblastoma reliable?” Preoperative fine‐needle aspiration biopsy (FNAB) and tissue sections of three children treated for lipoblastoma at the Division of Pediatrics, University Medical Center, Ljubljana, Slovenia, in the period from 1997 to 2004 were reexamined. The Giemsa‐ and Papanicolaou‐stained FNAB smears were moderately or poorly cellular and contained lipocytes, lipoblasts, and spindle cells in various proportions. The tumor cells were in clusters and tissue fragments or as single cells. Thin branching capillaries were observed in most of the clusters and tissue fragments. In the background, abundant myxoid extracellular material and naked oval nuclei were present. In the first case, the cytological diagnosis was benign soft‐tissue tumor, in the second the diagnosis was not conclusive and the last case was correctly diagnosed as lipoblastoma. In the differential diagnosis of the second case, both lipoblastoma and liposarcoma were considered cytologically as well as histologically. At 7‐yr follow‐up, there is no evidence of the disease. Thus, we conclude that lipoblastoma with typical cytological features could be accurately diagnosed by FNAB. However, tumors containing numerous lipoblasts could pose a diagnostic problem. Diagn. Cytopathol. 2005;33:195–200. © 2005 Wiley‐Liss, Inc.     

Sebaceous glands within the thymus: report of three cases

Sebaceous glands in the thymus were discovered in three patients--two with myasthenia gravis and the other, who was operated on because of aortic valvular disease--with an unsuspected thymoma. Sebaceous glands not associated with hair follicles occur in diverse sites, the majority of which are in the head and neck or anogenital regions. Although most of these sites arise from the ectoderm, sebaceous glands have been reported in a thyroglossal duct, the larynx, and the esophagus, which are entodermal structures. Thus, precedents for ectodermally derived sebaceous glands in endodermal sites exist, but sebaceous glands have not previously been reported in the thymus. The authors speculate that their occurrence in the thymus may be related to the reported ectodermal contribution to the developing thymus by the epibranchial placode or cervical sinus.     

Lymphangiomyomatosis: A report of three cases treated with tamoxifen

Summary Three cases are reported of lymphangiomyomatosis with pulmonary and abdominal manifestations. Two had a chylous pleura effusion, while the third presented a retroperitoneal manifestation, which was completely resectable. Antiestrogen therapy with tamoxifen was administered in all three cases. Two patients died of pulmonary progression after 4 months of therapy. The third is still alive, with stable disease for more than 6 years, and has been receiving tamoxifen for 66 months. These observations indicate that antiestrogen treatment may be as effective as oophorectomy when started at an early stage of the disease.