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 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Elderly individuals are susceptible to autoimmune bullous dermatoses (in particular, pemphigoid, epidermolysis bullosa acquisita and paraneoplastic pemphigus). Bullous dermatoses are associated with high morbidity and mortality. Bullous dermatoses result from autoimmune responses to one or more components of the basement membrane or desmosomes. Pemphigoid results from autoimmunity to hemidesmosomal proteins present in the basement membrane of stratified squamous epithelia. Patients present with tense blisters in flexural areas of the skin. Mild or moderate bullous pemphigoid may be treated with potent topical corticosteroids while extensive disease usually requires systemic corticosteroids or systemic immunosuppressive agents such as azathioprine. Mucosal pemphigoid affects one or more mucous membranes that are lined by stratified squamous epithelia. The two most commonly involved sites are the eye and the oral cavity. Lesions frequently result in scar formation, which may cause blindness. Patients with severe disease or ocular involvement require aggressive therapy with corticosteroids and cyclophosphamide. Epidermolysis bullosa acquisita results from autoimmunity to type VII collagen in the anchoring fibrils of the basement membrane area. Lesions may either arise on an inflammatory base or be non-inflammatory and result primarily from trauma. The inflammatory type of the disease is more responsive to therapy than the non-inflammatory type. Treatment options include corticosteroids, dapsone, cyclosporin, plasmapheresis and immunoglobulin G. Paraneoplastic pemphigus results from autoimmunity to multiple antigens within the desmosomes. The disorder is associated with neoplasms, especially leukaemia and lymphoma. Patients present with severe stomatitis and polymorphous skin eruption. The mucosal and cutaneous involvement may respond to successful treatment of the underlying neoplasm or may require immunosuppressive therapy.
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PMID:Autoimmune bullous dermatoses in the elderly: diagnosis and management. 1283 Dec 91

In dogs gastrinomas are rare endocrine neoplasms that have always been reported to arise from the pancreas. We report here what we believe to be the first case of a duodenal gastrinoma in a dog. A nine-year-old, male, Pekinese dog was presented with a three-day history of anorexia, vomiting and mucous diarrhoea. Clinical examination and laboratory findings suggested the presence of a severe hepatobiliary disorder. Abdominal ultrasonography showed a diffuse increase in echogenicity of the liver, with severe gallbladder dilation and marked dilation of the cystic duct, common bile duct and extrahepatic bile ducts. Based on these findings, an extrahepatic biliary tract obstruction (EBTO) of unknown cause was suspected. At laparotomy, the gallbladder and the extrahepatic bile ducts appeared severely dilated. The gallbladder was tense and could not be compressed suggesting an outflow obstruction. The duodenum at the level of the common duct orifice appeared slightly thickened and severely hardened for a length of 1 cm. Biopsies from the duodenum and liver were obtained and a cholecystoduodenostomy was performed. The duodenal biopsy revealed severe fibrosis of the submucosa and a infiltrate of small pockets and cords of round to polygonal cells with granular cytoplasm. Based on this appearance the differential diagnoses included neuroendocrine tumours and poorly differentiated carcinoma. Despite surgery and supportive therapy the dog continued to be anorexic and to vomit 3-6 times daily. After euthanasia and necropsy, histopathology showed the presence of a neuroendocrine neoplasia involving the duodenal wall with focal invasion of the adjacent pancreas and small liver metastases. On immunohistochemistry, the cytoplasm of approximately 90% of neoplastic cells intensely expressed neuron specific enolase and gastrin. These findings were consistent with a diagnosis of gastrinoma.
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PMID:Common bile duct obstruction due to a duodenal gastrinoma in a dog. 1599

Mortality due to the abdominal compartment syndrome is extremely high (38-71%). It may be defined as adverse physiologic consequences that occur as a result of an acute increase in the intraabdominal pressure. The most common causes of abdominal compartment syndrome are retroperitoneal haemorrhage, visceral oedema, pancreatitis, bowel distension, venous mesenterial obstruction, tense ascites, peritonitis, tumor. The mostly affected organ systems include cardiovascular, pulmonary, renal, central nervous and splanchnic. The diagnosis depends on the recognition of the clinical syndrome followed by an objective measurement of intraabdominal pressure, preferably that of the urinary bladder. The treatment consist of adequate fluid resuscitation and surgical decompression when necessary.
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PMID:[The abdominal compartment syndrome (ACS) in general surgery]. 1693 89

A 13-day-old female infant was admitted with hydrocephalus that had been diagnosed on prenatal ultrasound at 33 weeks' gestation. She was delivered by Caesarean section at 34 weeks with an Apgar score of 10. On admission, she weighed 2,103 g. The head circumference was 32.3 cm, and the fontanelle was tense. T(1)- and T(2)-weighted MR images revealed an isointense mass occupying the fourth ventricle with multiple cysts in the vermis. The mass was not enhanced after gadolinium administration. CT showed no definite calcification in the lesion. Preoperatively, vermian tumors, including medulloblastoma, ependymoma, astrocytoma, and hamartomas, were considered in the differential diagnosis. Hamartoma was strongly suspected due to the lack of enhancement on MRI. After a suboccipital midline craniotomy, subtotal resection of a soft grayish tumor with areas of hematoma was carried out. The pathological diagnosis was medulloblastoma. Despite chemotherapy, CSF dissemination resulted in death at 11 months. We report this case of congenital medulloblastoma with atypical MRI findings and discuss the clinical characteristics of this lesion.
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PMID:Congenital medulloblastoma with atypical MRI appearance. 1823 Sep 34

We report a 4-year-old boy presenting with a tense massive ascites and large hydrocele. History and physical examination were unremarkable. Routine laboratory studies were normal. Abdominal ultrasonography revealed massive ascites. Contrast CT was suggestive of a large cyst covering the entire peritoneal cavity. At laparotomy, a large cystic tumor was found extending into the scrotum through the left inguinal ring. Histopathologic examination diagnosed the tumor as a cystic lymphangiomatous hemartoma. Although abdominal lymphangiomas are seen in children, but presenting as massive ascites with hydrocele is very rare.
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PMID:Cystic lymphangiomatous hamartoma masquerading as massive ascites. 1947 59

Elderly individuals are susceptible to autoimmune bullous dermatoses (ABDs), which may be associated with high morbidity and mortality. ABDs result from an autoimmune response to components of the basement membrane zone at the dermal-epidermal junction or desmosomes. Bullous pemphigoid results from autoimmunity to hemidesmosomal proteins present in the basement membrane of stratified squamous epithelia. Patients present with tense blisters in flexural areas of the skin. Mild disease may be treated with potent topical corticosteroids, while extensive disease usually requires systemic corticosteroids or systemic immunosuppressive agents such as azathioprine. Mucosal pemphigoid affects one or more mucous membranes that are lined by stratified squamous epithelia. The two most commonly involved sites are the eye and the oral cavity. Lesions frequently result in scar formation that may cause blindness. Patients with severe disease or ocular involvement require aggressive therapy with corticosteroids and cyclophosphamide. Epidermolysis bullosa acquisita results from autoimmunity to type VII collagen in the anchoring fibrils of the basement membrane. Lesions may either arise on an inflammatory base or be non-inflammatory and result primarily from trauma. Treatment options include corticosteroids, dapsone, ciclosporin, methotrexate and plasmapheresis/immunoapheresis. Paraneoplastic pemphigus results from autoimmunity to multiple desmosomal antigens. The disorder is associated with neoplasms, especially leukaemia, lymphoma and thymoma. Patients present with stomatitis and polymorphous skin eruption. The disease may respond to successful treatment of the underlying neoplasm or may require immunosuppressive therapy.
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PMID:Autoimmune bullous dermatoses in the elderly: an update on pathophysiology, diagnosis and management. 2003 Apr 29

Desmoplastic small round-cell tumor is an uncommon, highly aggressive tumor with a predilection for pediatric age groups and young adults. It is very unusual in the elderly population. Although Agent Orange has been associated with soft-tissue sarcoma, an association with desmoplastic small round-cell tumor has not been reported. A 52-year-old male presented with abdominal distention, dyspnea, and a 9 kg weight loss. Prior history was significant for hepatitis C and diabetes. He was a Vietnam veteran and he admitted being exposed to Agent Orange. On physical examination, the abdomen was distended and tense. Computed tomography scan of the chest, abdomen and pelvis demonstrated extensive mediastinal and retroperitoneal adenopathy, diffuse omental masses and extensive pleural, intra-abdominal and pelvic ascites. Omental core needle biopsy was consistent with desmoplastic small round-cell tumor based on morphology and immunohistochemistry. He responded poorly to chemotherapy with high-dose cyclophosphamide, doxorubicin and vincristine and died 5 months after presentation secondary to neutropenic sepsis despite G-CSF support and antibiotics.
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PMID:Desmoplastic small round-cell tumor: an adult with previous exposure to agent orange. 2038 35

Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into "definitely congenital" (present or producing symptoms at birth), "probably congenital" (present or producing symptoms within the first week of life), and "possibly congenital" (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors, where aggressive surgical treatment leads to disease-free survival.
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PMID:Congenital tumors of the central nervous system. 2042 59

A 10-year-old spayed female Boxer-mix was presented with a history of several weeks of soft stools, straining to defecate, inappetance, and lethargy and several days of hematochezia, melena, and dyschezia. Physical examination findings included mild tachycardia and tense cranial abdomen. CBC results indicated moderate mature neutrophilia. Ultrasonographic examination of the abdomen revealed a large mass with complex echogenicity in the cranial abdomen, likely associated with the intestines. Cytologic examination of a fine-needle aspirate revealed a population of round, stellate, and spindle-shaped cells arranged individually and in aggregates with occasional cells embedded in an eosinophilic extracellular matrix. The cytologic interpretation was malignant mesenchymal neoplasm with osteosarcoma being the primary differential. Surgical exploration of the abdomen revealed a 10-cm-diameter mass located at the intestinal mesenteric root. The mass occluded blood flow to portions of the gastrointestinal tract. The dog was euthanized due to the nonresectable nature of the tumor. Histopathologic examination revealed an expansile poorly demarcated mesenchymal neoplasm composed predominantly of spindloid and pyriform cells, occasionally embedded in a matrix compatible with osteoid. The diagnosis was extraskeletal osteosarcoma of the intestinal mesenteric root, only rarely reported in dogs.
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PMID:Primary mesenteric root osteosarcoma in a dog. 2064 59

The authors present the case of a 3-month-old boy with a third ventricular tumor consistent with a choroid plexus papilloma. This child presented with macrocephaly, irritability, inability to roll over, and vomiting. He was found to have an enlarged head circumference, a full and tense fontanel, splayed sutures, and forced downward gaze. Imaging revealed severe ventriculomegaly and a brightly enhancing third ventricular lesion consistent with papilloma. Treatment planning included placement of a ventriculoperitoneal shunt to treat hydrocephalus and to allow the child to grow prior to resection. Due to the vascular nature of these tumors and the age of this child, the tumor was embolized with a plan for eventual resection; however, embolization resulted in involution and total regression of the tumor. There is no residual disease at last follow-up of 16 months. In this specific scenario of a choroid plexus papilloma in an infant, when operative intervention may be technically difficult and associated with significant morbidity, embolization with close observation may be a valid treatment option. If used, the patient would need to be closely followed for evidence of residual or recurrent disease, which would require operative intervention.
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PMID:Treatment of third ventricular choroid plexus papilloma in an infant with embolization alone. 2112 34


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