1/5. Synchronous first manifestation of an idiopathic eosinophilic gastroenteritis and bronchial asthma.Eosinophilic gastroenteritis is a rare disease of the gastrointestinal tract in which the eosinophils seem to play an important role in the inflammation of the gut wall. We report on a case with a synchronous first manifestation of eosinophilic gastroenteritis and bronchial asthma, which also occurred synchronously in all further episodes. The diagnosis was first made at the end of the second episode during which the patient lost more than 13 kg in weight. Under steroid therapy, symptoms of both diseases disappeared quickly in the third episode. We assume that participation of the gastrointestinal tract in patients with bronchial asthma occurs more frequently than expected. In asthma patients with abdominal symptomatology, eosinophilic gastroenteritis should also be considered.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/5. Anesthetic management of a parturient with Ehlers Danlos syndrome type IV.PURPOSE: To describe the anesthetic management of a parturient with Ehlers Danlos syndrome (EDS) type IV. Clinical features: A 29-yr-old pregnant woman with EDS type IV was seen in the Obstetric anesthesia Pre-assessment Clinic at 30 weeks gestation. She had a history of vertebral artery dissection, resulting in a transient neurological deficit at 22 yr of age. She had a normal vaginal delivery with continuous epidural analgesia for the delivery of her first child at 27 yr of age, before the diagnosis of EDS was made. Recent fibroblast culture demonstrated the production of abnormal procollagen type III, which is pathognomonic for EDS type IV. The patient and obstetrician preferred a repeat vaginal birth with instrumental delivery in the second stage. analgesia for labour and delivery was provided with a continuous epidural infusion of ropivacaine and fentanyl. She delivered a healthy female infant with the use of outlet forceps, without complications. CONCLUSION: A pre-delivery, multidisciplinary, individualized management plan is required in patients with EDS, a rare disease with variable clinical features. In the case described, continuous epidural analgesia was effective and associated with excellent maternal and fetal outcomes.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/5. Relapsing polychondritis: reversible airway obstruction is not always asthma.Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation of cartilaginous and other proteoglycan-rich tissues. Respiratory tract involvement is a common cause of morbidity and mortality in RP. We describe a patient whose clinical features at onset of disease were typical of asthma. Later, the patient developed symptoms and signs characteristic of RP. tracheobronchomalacia necessitated airway support by stenting. The possibility that airway obstruction in the initial stages of RP is due to airway inflammation and that early, aggressive immunosuppressive treatment of RP may delay or prevent irreversible cartilaginous destruction and airway collapse are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/5. formaldehyde asthma--rare or overlooked?A total of 230 persons who had been exposed to formaldehyde and suffered from asthma-like respiratory symptoms were examined between January 1, 1977, and May 31, 1983. All the subjects had a bronchial provocation test with formaldehyde. On the basis of the medical and occupational history of the patients, the specific bronchial provocation test, and other test results, 12 cases were considered to be caused by specific sensitization to formaldehyde. All subjects had been exposed occupationally. An exposure period of between 1 mo and 19 yr preceded the onset of symptoms. Three persons displayed no bronchial hyperreactivity as assessed with a histamine or metacholine provocation test. Eleven of the 12 reactions were triggered by about 2.5 mg/m3 and one reaction by about 1.2 mg/m3 of formaldehyde. The late reaction in 1 patient was completely blocked by the inhalation of 100 micrograms of beclomethasone di-isoproprionate before the challenge with formaldehyde. Seventy-one of the 218 subjects who did not react when they were challenged with formaldehyde demonstrated bronchial hyperreactivity. We conclude that formaldehyde asthma, although apparently a rare disease, is under reported. Removal from exposure has a favorable effect on the symptoms. Low domestic exposures, however, may maintain the symptoms in individuals already sensitized.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/5. Eosinophilic pancreatitis mimicking pancreatic neoplasia: EUS and ERCP findings--is nonsurgical diagnosis possible?Eosinophilic pancreatitis is a rare disease with, to date, only 10 reported cases. We report two patients whose presentation was suggestive of a pancreatic tumor. An 18-year-old man presented with a short history of obstructive jaundice, epigastric pain, and weight loss. Endoscopic ultrasonography revealed a 12-mm round, hypoechoic lesion in the head of the pancreas, suggestive of a pancreatic endocrine tumor. A duo-denopancreatectomy was performed, and the patient made an uneventful recovery. A 64-year-old man was referred with weight loss and obstructive jaundice due to a stricture of the common bile duct. Retrograde pancreatography demonstrated a very narrow, regular main pancreatic duct and early parenchymography. Endoscopic retrograde cholangiopancreatography revealed a tight, regular stenosis of the intrahepatic portion of the common bile duct. A gastrojejunal anastomosis and biliary-digestive bypass were performed without postoperative complications. In both cases, the diagnosis of eosinophilic pancreatitis was revealed after surgical resection. The features of eosinophilic pancreatitis, including eosinophilic infiltration of the digestive wall, a history of atopy, and the radiological characteristics are described. If these are of sufficient discriminatory value, unnecessary surgery may be avoided.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |