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1/24. Rosai-Dorfman disease presenting as a pituitary tumour.

    A 45-year-old woman had pyrexia, headaches, collapse and hyponatraemia. Intracerebral abscess, bacterial meningitis and subarachnoid haemorrhage were excluded. She was given intravenous antibiotics and gradually recovered. One month later she was readmitted with diplopia, headache and vomiting. serum sodium was low (107 mmol/l) and a diagnosis of inappropriate ADH secretion was made. MRI scan showed a suprasellar tumour arising from the posterior pituitary gland. A skin rash gradually faded. serum cortisol, prolactin, gonadotrophins and thyroid hormone levels were low. A pituitary tumour was removed trans-sphenoidally, she had external pituitary radiotherapy, and replacement hydrocortisone and thyroxine. She was well for 12 months when she developed progressive weakness and numbness of both legs. Examination suggested spinal cord compression at the level of T2 where MRI scanning showed an intradural enhancing mass. This spinal tumour was removed and her neurological symptoms disappeared. Nine months after this she developed facial pain and nasal obstruction. CT scan showed tumour growth into the sphenoid sinus and nasal cavities. A right Cauldwell-Luc operation was done and residual tumour in the nasal passages was treated by fractionated external radiotherapy and prednisolone. Histological examination of the specimens from pituitary, spinal mass, and nasal sinuses showed Rosai-Dorfman disease, a rare entity characterized by histiocytic proliferation, emperipolesis (lymphophagocytosis) and lymphadenopathy. aged 48 she developed cranial diabetes insipidus. Although Rosai-Dorfman syndrome is rare, it is being reported with increasing frequency, and should be borne in mind as a possible cause of a pituitary tumour.
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keywords = diabetes
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2/24. Acute subdural hematoma and intracerebral hemorrhage. Rare complications of rhinocerebral mucormycosis.

    Rhinocerebral mucormycosis is a short-term and often rapidly lethal fungal disease. It is generally seen in uncontrolled cases of diabetes with ketoacidosis. This case exhibits many of the features of a typical fulminating rhinocerebral mucormycosis. However, the fatal complications of acute subdural hematoma and massive intracerebral hemorrhage due to rupture of aneurysm, as demonstrated by angiography, are unique clinical manifestations of patients with rhinocerebral mucormycosis.
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keywords = diabetes
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3/24. Nasal alar necrosis.

    OBJECTIVE: To describe an unrecognized clinical entity, nasal alar necrosis, and propose recommendations regarding the diagnosis, pathophysiology, and management of these cases. STUDY DESIGN: Retrospective review of four patients with this condition. methods: Histories, treatments, and outcomes were evaluated using office and hospital chart data. RESULTS: We noted that three patients had prior trauma or surgery, other than to the nose, in the head and neck region. All four patients had comorbidities such as diabetes, hypothyroidism, depression, or tobacco abuse. Three patients had sensory deficits over the distribution of the maxillary nerve, and three volunteered that they had a habit of picking the crusted wound. Two patients improved over several months with aggressive wound care. One patient refused treatment and another underwent successful reconstruction. CONCLUSIONS: After malignant and granulomatous diseases were ruled out, our evaluations suggested that the pathogenesis was multifactorial including several factors alone or in combination, such as, hypoesthesia, self-mutilation, and an inadequate blood supply. Deficits in vascularity and sensory innervation must be considered as potential obstacles in reconstruction. Psychological problems causing factitious wounding may complicate therapeutic interventions.
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keywords = diabetes
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4/24. mucormycosis in a transplant recipient.

    mucormycosis classically occurs in patients who have uncontrolled diabetes who develop rhinocerebral disease. A fatal case of rhinocerebral infection caused by rhizopus arrhizus in a 53-year-old man who had received a renal homograft three years previously is reported. Only five similar cases have been documented, all since 1970. Although direct smears of the purulent nasal exudate revealed the presence of numerous Gram-negative bacilli, later identified as haemophilus influenzae, the diagnosis of mucormycosis was made by demonstrating the typical broad, nonseptate branched hyphae in the necrotic tissue obtained by surgical debridement of the paranasal sinuses. culture of this material revealed growth of mold-like fungus which, upon direct microscopic examination, showed sporangiophores bearing spherical sporangia arising directly from a cluster of root-like structures of rhizoids. Despite the immediate institution of therapy with amphotericin b postoperatively, the patient died 48 hours later. Subsequently, the rhizopus isolated was shown to be resistant to both amphotericin b and 5-fluorocytosine. The present case and two others stress the importance of an aggressive diagnostic approach to patients suspected of having mucormycosis, because the usual microbiologic technics are frequently, inexplicably, unsuccessful, and possibly even misleading in this disease.
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keywords = diabetes
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5/24. Mucor endophthalmitis.

    PURPOSE: To report on a case of type 2 diabetes, with eyelid gangrene and endophthalmitis as a presenting manifestation of rhino-orbito-cerebral mucormycosis. RESULTS: CECT head showed proptosis, mucosal thickening in the ethmoid sinus and hypodense lesions in the frontal and occipital lobes. Vitreous tap showed right angle branched aseptate hyphae consistent with mucormycosis. CONCLUSIONS: A diabetic patient presenting with sudden loss of vision, eyelid gangrene and endophthalmitis, involvement by an angio-invasive fungus-like mucormycosis is an important consideration.
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keywords = diabetes
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6/24. Rhinocerebral zygomycosis in childhood acute lymphoblastic leukaemia.

    The hazards associated with invasive candidiasis and aspergillosis in oncology patients are well recognised. These conditions typically present late in treatment, often after prolonged or recurrent episodes of neutropenia. We report the occurrence of absidia corymbifera infection causing rhinocerebral zygomycosis in two children with acute lymphoblastic leukaemia, early in the induction phase of treatment and within a 3-month interval, in the same oncology unit. The initial presentation of facial pain was rapidly followed by the development of cranial nerve palsies, cavernous sinus thrombosis, diabetes insipidus, seizures and death within 9 days of symptom onset, despite aggressive management with high-dose liposomal amphotericin (Ambisome), surgical debridement and local instillation of amphotericin solution. These cases highlight the need for awareness of zygomycosis as a potentially lethal fungal infection that can present even with short duration exposure to the usual risk factors. Their occurrence within a limited time period raises questions as to the relative importance of environmental exposure. The failure of medical and surgical intervention to impact on the course illustrates the need to develop appropriate preventative strategies which may have to incorporate measures to reduce the environmental exposure of susceptible patients.
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keywords = diabetes
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7/24. Rhinocerebral mucormycosis in an 11-year-old boy.

    A patient with diabetic ketoacidosis admitted for treatment developed features characteristic of rhinocerebral mucormycosis, confirmed by histopathologic examination, is presented. The patient had been experiencing symptoms of diabetes for one month before presentation to the hospital in state of diabetic ketoacidosis. Within a few days of admission he developed mucormycosis of the nasal cavity and the orbit which extended to the brain very fast and resulted in death. High index of suspicion form the clinical presentation in an immunocompromised patient, especially, one with diabetic ketoacidosis, is suggested to effect early intervention.
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keywords = diabetes
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8/24. mucormycosis causing extensive destruction of maxilla.

    mucormycosis is a rare, often fatal opportunistic infection caused by a saprophytic fungus belonging to a class of Phycomycetes. It is characterized by its unrelenting progression towards vital organs with marked propensity towards arterial wall by direct extension producing vascular thrombosis leading to ischaemic necrosis. In normal circumstances the saprophytes are not pathogenic but represent opportunist-requiring impairments in patient's resistance to disease. If not treated the disease may prove fatal. We report a case of mucormycosis with extensive necrosis of the maxilla in a 57-year-old patient suffering from uncontrolled diabetes with ketoacidosis.
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keywords = diabetes
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9/24. Rhino-orbital-cerebral mucormycosis in type 1 diabetes mellitus.

    AIM: To describe the presentation and outcome of rhino-orbital-cerebral mucormycosis (ROCM) in adolescents with type 1 diabetes mellitus (T1DM). methods: The medical records of six patients of T1DM with ROCM admitted between October 2001 to January 2004 were analysed. RESULTS: The mean ( /- SD) age and duration of DM of these patients were 16.1 /-3.0 years and 26.3 /- 24.9 months respectively. Four patients had ROCM at presentation, while two developed it during their hospital stay when recovering from diabetic ketoacidosis. Proptosis (100%) and ptosis (100%) were the most common symptoms, and ophthalmoplegia (85%) and vision loss (85%) were the most common signs. maxillary sinus (85%) was the commonest paranasal sinus to be involved. All patients received amphotericin b and had appropriate surgery except one. Four patients survived. patients who had altered sensorium, facial necrosis, palatal perforation and cerebral involvement at presentation had poor outcome. CONCLUSION: High index of suspicion of ROCM in T1DM and combined approach with amphotericin b and appropriate surgery is rewarding.
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ranking = 23.747615592828
keywords = diabetes mellitus, diabetes, mellitus
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10/24. Rhino-orbitocerebral mucormycosis caused by Apophysomyces elegans.

    Rhino-orbitocerebral mucormycosis (ROCM) caused by more common zygomycetes (e.g., Mucor) is known to cause rapidly fatal infections in immunocompromised patients. Apophysomyces elegans is an emerging zygomycete that has been reported to cause invasive cutaneous and rhino-orbitocerebral infections in immunocompetent individuals. Limited data exist describing the syndrome of ROCM caused by A. elegans. We describe a recent case and performed a comprehensive literature review to delineate the clinical characteristics of ROCM caused by A. elegans. Our case is a 50-year-old man with diabetes mellitus who presented with facial pain and right eye proptosis. Endoscopic sinus sampling revealed A. elegans. He was treated with liposomal amphotericin b and multiple debridements, with no disease on 1.5-year follow-up examination. Seven cases were identified on literature review, including the present case. Most patients (86%) were male, with a mean age of 40 years. Most patients (71%) did not have predisposing medical conditions. Three patients had predisposing head trauma. All presented with facial and/or periorbital pain. All had magnetic resonance imaging or computed tomography of the head showing intraorbital and/or sinus inflammation. diagnosis was confirmed by histopathology and deep tissue culture in all cases. All patients required eye exenteration and extensive surgical debridement, in addition to intravenous amphotericin b. Six of the seven patients (86%) recovered. ROCM caused by A. elegans is rarely reported in the literature. Most such infections occurred in immunocompetent patients, often after facial trauma. survival in ROCM caused by A. elegans is favorable in reported cases, with prompt surgical debridement and antifungal therapy.
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ranking = 4.7495231185656
keywords = diabetes mellitus, diabetes, mellitus
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