臨床症例報告ジャーナル

Toxic epidermal necrolysis is a rare but severe and often fatal adverse cutaneous drug reaction. Early diagnosis and prompt discontinuation of the culprit drug is of the utmost importance, making drug causality assessment the mainstay when managing this condition. We present the first reported case of toxic epidermal necrolysis induced by tiapride, in a 43 year-old male Caucasian with a history of liver cirrhosis and HIV infection. This case report should raise awareness on the medical community to a potentially fatal reaction to a frequently prescribed drug in alcoholic patients. It also underlines the importance of further research regarding HIV infection, immune deregulation and adverse cutaneous drug reactions.

This issue covers 3 interesting case reports. In the first case the authors highlight the issue of sedation during anaesthesia in a case of Mucopolysaccharidosis-IV. Patients with Mucopolysaccharidosis-IV may have craniofacial deformities and short neck, along with stiffening of the temporo-mandibular joints, anteriorly positioned larynx and an unstable atlanto-axial joint.

Transcatheter placement of patent foramen ovale occluders is an alternative to open heart surgery for repair of PFO’s. We present a case of a 33 years old male with a previously unknown nickel allergy who developed a severe allergic response after placement of a nitinol based cribriform amplatzer device, for closure of symptomatic PFO. Our patient presented with neurological symptoms consisting of visual field defects and right sided weakness. MRI showed a left hemispheric parieto-occipital. An echocardiogram showed a large PFO. He was discharged eventually with a plan for long term anticoagulation. However he presented again with similar symptoms in a few days. Due to recurrence of symptoms his neurologist recommended PFO closure. This was successfully closed percutaneously using a 25 mm amplatzer cribriform device and no residual shunt was seen. The patient returned two days later with shortness of breath, chest pain and a pruritic rash. All further laboratory testing was negative. Suspecting nickel allergy, testing was performed with a 25 mm cribriform device taped to the patients left arm. He was then referred to cardiothoracic surgery for explantation of the amplatzer device which resulted in resolution of his symptoms. Nitinol is the material that constitutes amplatzer occluders. Though considered safe for PFO closure, there are reports of allergic responses to it. Here we have an early onset case of an allergic response to the device with a novel way to test for allergies using the device itself, with an emphasis on questioning patients regarding allergies and testing them as well.

We report a case of a patient who developed neuromyelitis optica one year after discontinuation of her immunosuppressive agent used to treat her ulcerative colitis. We highlight the association between the two diseases that their similarities may not be limited to their autoimmune etiology, but also due to abnormal water channels, or aquaporins, and briefly discuss the possible role of aquaporins in ulcerative colitis pathogenesis

\r\n

We report a case of 23-year old male from Tangail who developed intermittent fever and blackening of skin 7 months back. He was locally diagnosed as a case of kala-azar and was treated with Cap Miltefosine 50 mg 12 hourly for 28 days. But within 4 months after completing the treatment he again developed the symptoms. Referred from the local hospital he presented to us with fever, weight loss and discoloration of skin. He was anemic and had splenomegaly. Laboratory investigations showed pancytopenia, elevated Erythrocyte Sedimentation Rate (ESR). Leishman-Donovan body was found inbone marrow biopsy. In our hospital he was treated with injection Liposomal Amphotericin B 15 mg/kg in 6 divided doses. His anemia was corrected, spleen size became normal and bone marrow aspiration showed no Leishman-Donovan body on discharge and he remained symptoms free in the outpatient department.

We describe the sedation of a 22 year old man with mucopolysaccharidosis VI scheduled for a myringotomy procedure. His medical history was significant for difficult airway, severe aortic insufficiency and adenoid hypertrophy. We briefly review the anesthesia literature related to this case.