Δευτέρα 24 Απριλίου 2017

Forsaken Foregut: Case Report of Simultaneous Black Esophagus and Ischemic Cholangiopathy

Black esophagus or acute esophageal necrosis rarely occurs after severe hemodynamic compromise or low-flow states. Other contributing factors may include corrosive injury from gastric contents and diminished mucosal repair mechanisms. Ischemic cholangitis, another rare clinical entity, is also usually the result of a significant vascular and/or hypotensive insult to the biliary tree. We describe the first case of combined acute esophageal necrosis and ischemic cholangiopathy in a 62-year-old male who completely recovered from the esophageal injury but developed progressive liver failure from ischemic cholangiopathy.

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Radiobiological evaluation of simultaneously dose-escalated versus non-escalated intensity-modulated radiation therapy for patients with upper thoracic esophageal cancer

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Malignant middle cerebral artery infarction

Description

A 55-year-old man with hypertension was referred from a rural hospital. Two days before, he had developed a cardioembolic ischaemic stroke, which resulted in left-sided hemiparesis and hemianaesthesia. On the day of referral, he developed repeated vomiting and severe headache, followed by progressive obtundation and coma. There was papilloedema. Both planter reflexes were extensors. A non-contrast CT brain scan was done (figure 1). The initial CT brain scan was not available for comparison. The patient was outside the international guidelines on timing of decompressive hemicraniectomy (DCH) for ischaemic stroke and was extremely unwell. After a few hours, he died from 'malignant middle cerebral artery (MCA) infarction'.

Figure 1

Axial non-contrast CT brain scan of the patient 2 days after developing right-sided middle cerebral artery embolic occlusion. Note that almost the entire right cerebral hemisphere is swollen and has compressed the left hemisphere. The midline structures...



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Migration of clips to the colon after laparoscopic partial nephrectomy

A 78-year-old man was treated with laparoscopic partial nephrectomy for a 4 cm tumour in the lower pole of the left kidney. The patient was discharged 2 days postoperatively. Preoperatively there was no sign of metastasis; the pathology indicated complete removal of the tumour, a renal cell carcinoma. 53 days postoperatively the patient had acute onset of rectal bleeding. A colonoscopy revealed necrosis and three clips on the wall of the colon corresponding to where the descending colon passes over the left kidney. The patient was treated conservatively. Clip migration has been reported previously, but to our knowledge we present the first case of clip migration to the colon after laparoscopic partial nephrectomy. Suggestions to avoid the presented complication are made.



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Diabetes mellitus and acquired haemophilia: new association?

Diabetes mellitus encompasses a group of highly prevalent carbohydrate metabolic disorders with an increasing incidence. Some subtypes are thought to be associated with other immune-mediated diseases. Acquired haemophilia on the other hand is a quite rare autoimmune disease that is thought to be secondary to the emergence of inhibiting anticoagulation factor VIII antibodies (inhibitors) in patients with previously normal haemostatic function. More recently, numerous different diseases have been associated with acquired haemophilia namely immune-mediated diseases, drugs and solid and haematologic neoplasms. The authors report on a case of a patient with new onset acquired haemophilia arising in the setting of diabetic ketoacidosis.



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A tenebrous tale: malignant melanoma of the oesophagus

A 57-year-old man presented with a 4-month history of worsening symptoms of oesophageal obstruction. The physical examination was unremarkable. An oesophagogastroduodenoscopy revealed an exophytic tumour in the distal oesophagus. A biopsy demonstrated malignant melanoma. Staging of the melanoma showed disseminated lymph node and bony-spine metastases. He had no prior history of cutaneous or ocular melanoma. Following full multidisciplinary team input, he was palliated with a metal mesh stent and immunotherapy. He died 3 months later following community management.



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De Novo PTEN Mutation in a Young Boy with Cutaneous Vasculitis

Phosphatase and tensin homolog (PTEN) is the protein encoded by the PTEN gene (10q23.3). PTEN mutations are related to a variety of rare diseases referred to collectively as PTEN hamartoma tumor syndromes (PHTS), which include Cowden Syndrome, Bannayan-Riley-Ruvalcaba syndrome, Proteus Syndrome, and Proteus-like syndrome. These diseases are associated with an increased risk of malignancy and for this reason an accurate and early diagnosis is essential in order to institute cancer surveillance. PTEN is a regulator of growth and homeostasis in immune system cells, although there are limited data describing immune dysregulation caused by PTEN mutations. We describe a case of PHTS syndrome caused by a de novo mutation in PTEN detected using a targeted next generation sequencing (NGS) gene panel which was instigated for workup of cutaneous vasculitis. We highlight the diagnostic utility of this approach and that mutations in PTEN may be associated with immune-dysregulatory features such as vasculitis in young children.

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