Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 23-year-old woman was supposed to undergo thoracoscopic surgery for the 10th pneumothorax that accompanied histiocytosis X. The past history included Lylle's disease, asthma, myocarditis, drug-induced leucocytopenia and bronchitis obliterans. The preoperative arterial blood gas analysis under receiving O2 at rate of 2 l.min-1 via a nasal cannula revealed normal values. General anesthesia and intubation with a double-lumen endotracheal tube would have been preferable, but regional anesthesia was chosen because of her medical history and positive results of the skin tests for vecuronium, pancronium, diazepam and midazolam. During the first 10 min of thoracoscopic procedure, her respiration became rapid and shallow and she was restless and comatose. The operation was cancelled. Arterial blood gas analysis under receiving O2 at rate of 4 l.min-1 via a face mask revealed: pH 7.025, PaO2 113.8 mmHg, PaO2 244.8 mmHg, HCO3- 29.7 mEq.l-1, BE-5.6, and O2 saturation 99.1%. Manual artificial ventilation with a mask and bag was initiated. Her spontaneous respiration and consciousness recovered in next 30 min. The postoperative course was uneventful. Tachypnea, caused from anxiety, dyspnea and stimulation of irritant receptors in the airway, were considered to be responsible for the event. The duration of inspiration became shorter as tachypnea developed, that made the tidal volume to decrease and hypercapnea ensued.
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PMID:[Hypercapnea during thoracoscopic surgery under regional anesthesia]. 925 15

We describe the outcome of a 20-month-old female and a 6-year-old male, both of whom had acutely developed severe respiratory distress with tachypnea, cyanosis and, in Patient 2, thoracic pain. Chest X-ray and CT scan showed interstitial pulmonary involvement and a bullous process with bilateral pneumothoraces for both children. Pulmonary biopsy confirmed the diagnosis of Langerhans cell histiocytosis (LCH). Laboratory testing and skeletal radiography did not reveal any other involvement of LCH. The patients received chemotherapy (prednisone, vinblastine, 6-mercaptopurine). They had recurrent episodes of pneumothorax during follow-up and placement of chest tubes was the treatment chosen. They were asymptomatic, with regression of bullae and disappearance of pneumothorax at 58 and 63 months of follow-up, respectively. Pulmonary function tests done during follow-up were normal in both patients. Despite severe pulmonary involvement, conservative surgical treatment and moderate chemotherapy produced good results in these two rare cases.
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PMID:Isolated pulmonary Langerhans cell histiocytosis presenting with recurrent pneumothorax. 1620 6

Langerhan's cell histiocytosis (LCH) is a rare disorder in which granulomatous deposits occur at multiple sites within the body. The aetiology is unknown. Is more frequent in children. Presenting symptoms are polyuria and polydipsia, due to diabetes insipidus, other symptoms are skin rash, dyspnea and tachypnea. Diagnosis is reached by biopsy of lesions, in which Langerhan's cell are found. Prognosis is variable, depending the site affected; therefore, treatment must be individually. The hyperprolactinaemia in LCH is very rare and its related with anterior pituitary deficiency. There are not many cases described, all of them during the course of the disease, not as the onset. We describe a 22 year-old woman with Langerhan's cell histiocytosis which initial presentation was fever and hyperprolactinaemia.
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PMID:[Fever and hyperprolactinaemia as the onset of Langerhans cell histiocytosis]. 1645 88