1/83. Allergic reaction to the liposomal component of liposomal amphotericin b.A case of severe allergic reaction arising during treatment with Ambisome and unresponsive to antihistamine and steroid medication is reported. A 2.9-year-old female child with Hurler's syndrome received an allogeneic cord blood transplant from an unrelated donor. During the aplastic phase, liposomal amphotericin b (Ambisome) was administered as part of an empirical treatment for persistent fever. The patient developed an extensive maculopapular rash and severe itching that resolved only on discontinuation of the drug. The patient subsequently had interstitial pneumonia with ingravescent respiratory failure in spite of adequate antibiotic and antiviral treatment. Treatment with conventional amphotericin b was considered essential in this critically ill patient, and the conventional formulation was administered for 20 days without causing any reaction. Severe allergic reaction to Ambisome is a rare event but, taking into account that premedication or dose testing is not recommended for this formulation, careful monitoring of the patient being treated for the first time is warranted.- - - - - - - - - - ranking = 1keywords = pneumonia (Clic here for more details about this article) |
2/83. Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans.BACKGROUND: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. OBJECTIVE: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. methods: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. RESULTS: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. CONCLUSION: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family.- - - - - - - - - - ranking = 50.791993877365keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |
3/83. Diffuse panbronchiolitis, the first case reports in thailand.Diffuse panbronchiolitis (DPB) is an important cause of progressive obstructive lung or chronic suppurative lung disease in the far east (japan, china and korea). It is a distinctive sinobronchial syndrome with typical radiological and histologic features. We have identified three patients who have typical clinical manifestation and pathological confirmation. DPB should be suspected in patients who have clinical manifestations of chronic cough, productive sputum and shortness of breath. The chest radiograph often shows diffuse nodular shadows on a background of hyperinflated lungs. High resolution computed tomographic (HRCT) can guide the diagnosis and differentiate from other diseases. Finally, the most important issue of DPB is the treatment. Chronic treatment with low-dose erythromycin can improve the survival of patients.- - - - - - - - - - ranking = 4.6761253597163keywords = bronchiolitis (Clic here for more details about this article) |
4/83. Recurrent endocarditis caused by streptococcus pneumoniae.Pneumococcal endocarditis most often presents as an ulcerative endocarditis causing rapid destruction of the normal aortic valve, leading to aortic insufficiency and acute heart failure. alcoholism is the most frequent underlying medical condition. This case illustrates that pneumococcal endocarditis can reoccur and is able to attack healthy, as well as previously damaged, heart valves. It also illustrates that vaccination of certain groups should be considered. The importance of repeated heart stethoscopy in patients with pneumococcaemia is emphasized.- - - - - - - - - - ranking = 4keywords = pneumonia (Clic here for more details about this article) |
5/83. Persistent cerebellar deterioration in a patient with lobar pneumonia under lithium, carbamazepine, and trifluperidol treatment.We report on a patient with schizoaffective disorder who was on combination therapy of lithium, carbamazepine, and the neuroleptic trifluperidol. He experienced a lobar pneumonia and developed an acute and persistent cerebellar deterioration which was most likely due to lithium toxicity, while the serum lithium level was within the therapeutic range. The combination of lithium, carbamazepine, and neuroleptics is common, and is generally considered to be safe. The reported case suggests that this regimen might increase the risk of intoxication with potentially disabling side-effects.- - - - - - - - - - ranking = 5keywords = pneumonia (Clic here for more details about this article) |
6/83. Persistent cough in an adolescent.Jessica, a 14-year-old girl with a history of asthma, went to her pediatrician's office because of a persistent cough. She had been coughing for at least 3 months with occasional cough-free periods of less than a few days. The cough was nonproductive and was not accompanied by fever, rhinorrhea, or facial or chest pain. Jessica and her mother observed that the cough increased with exercise and typically was not present during sleep. She has used two metered-dose inhalers--albuterol and cromolyn--without any change in the cough pattern. For the past 5 years, Jessica has had mild asthma responsive to albuterol. She enjoys running on the cross-country team, soccer, and dancing. She is an average student and denies any change in academic performance. She has never been hospitalized or had an emergency department visit for asthma or pneumonia. There has been no recent travel or exposure to a person with a chronic productive cough, tobacco smoke, or a live-in pet. Jessica lives with her mother and younger sister in a 10-year-old, carpeted apartment without any evidence of mold or recent renovation. In the process of taking the history, the pediatrician noticed that Jessica coughed intermittently, with two or three coughs during each episode. At times, the cough was harsh; at other times, it was a quiet cough, as if she were clearing her throat. She was cooperative, without overt anxiety or respiratory distress. After a complete physical examination with normal findings, the pediatrician interviewed Jessica and her mother alone. Jessica's parents had been divorced for the past 6 years. She lived with her mother but visited her father, and his new family with two young children, every weekend. She spoke about this arrangement comfortably and said that she loved her father and mother but didn't like the tension she experienced at her father's home. "I don't like adults arguing when kids are around." When asked why she thought the cough persisted so long, she commented in a neutral tone, "I don't know. It's never been like this before." Jessica's pediatrician prescribed an inhaled steroid with the albuterol. When the cough did not respond after 1 week, he ordered a chest radiograph (normal) and a tuberculin skin test (purified protein derivative-negative), and he added montelukast (a leukotriene inhibitor) and monitored airway resistance with a peak flow meter. The cough persisted, and the peak flow recording showed normal airway resistance. At this time, Jessica's pediatrician suspected a conversion reaction and contemplated the next best therapeutic strategy.- - - - - - - - - - ranking = 1keywords = pneumonia (Clic here for more details about this article) |
7/83. diabetes mellitus associated with rapidly progressive glomerulonephritis with perinuclear antineutrophil cytoplasm antibodies.A 55-year-old woman who had been treated for diabetes mellitus for twenty-five years developed interstitial pneumonia and rapidly progressive glomerulonephritis (RPGN). The findings of light microscopy revealed fibrocellular crescent formation in all glomeruli and infiltration of lymphoid cells in interstitium. There were no deposits in the intracapillary area and mesangial area on both immunofluorescence and electron microscopy. Her interstitial pneumonia improved with pulse therapy of methylprednisolone and her hematuria disappeared with mix treatment of cyclophosphamide and double filtration plasmapheresis (DFPP). Her serum creatinine level improved from 2.2 mg/dl to 1.5 mg/dl. Interstitial pneumonia and hematuria did not recur at twelve months after the first hospitalization. This report presents a rare case with RPGN associated with diabetes mellitus who recovered with combination therapy of cyclophosphamide, steroid and DFPP.- - - - - - - - - - ranking = 3keywords = pneumonia (Clic here for more details about this article) |
8/83. Evolution of iga deficiency to IgG subclass deficiency and common variable immunodeficiency.FIRST REPORT: male child with repeated pulmonary infections from the age of 4 months. He was diagnosed as iga deficiency (undetectable IgA levels) at the age of 3 years, when he presented repeated bouts of pneumonia and tonsillitis. Several immunologic evaluations were made between the ages of 4 months and 8 years. At 8 years and 9 months, the diagnosis of iga deficiency was confirmed, and associated IgG2 and IgG4 deficiency (29.0 mg/dl y 0.01 mg/dl) with normal total IgG serum level was found. With the administration of intravenous gammaglobulin, the lung infections remitted and the subsequent clinical course has been uneventful up to now. SECOND REPORT: a boy with repeated infections since the age of 2 months. iga deficiency was diagnosed at 1 year 7 months (undetectable serum IgA levels). At age 51/2 years, his clinical course worsened and more serious infections appeared. A new immunologic study revealed iga deficiency associated with CD4 cell deficiency (432 cells/mm3) and normal CD3, CD19, and CD8 levels. Despite intensive antibiotic treatment and care, the child died. The findings suggest an association of iga deficiency and common variable immunodeficiency.- - - - - - - - - - ranking = 1keywords = pneumonia (Clic here for more details about this article) |
9/83. An autopsy case of atypical adrenoleukomyeloneuropathy in childhood.Adrenoleukomyeloneuropathy (ALMN) usually occurs in adulthood, it being extremely rare in childhood. We reported a quite atypical clinical case of ALMN as a variant of adrenoleukodystrophy (ALD). The onset was at 5 years 7 months and ataxia was the major symptom. His condition progressed rapidly to a vegetative state within 1 year. At the age of 11 years and 11 months he died of pneumonia and an autopsy was performed. We herein reported the neuropathological findings in this rare case. The autopsy revealed marked atrophy with diffuse demyelination and astrogliosis throughout the cerebrum, cerebellum and brainstem. Massive degeneration of the pyramidal tracts and loss of neurons were also seen in the spinal cord. The adrenal cortex showed marked atrophy with a striated cytoplasm in ballooned cells. These findings include pathological characteristics of both ALD and adrenomyeloneuropathy (AMN), suggesting ALMN. However, diffuse demyelination with gliosis in the cerebrum and cerebellum is quite atypical for ALMN. They might explain his atypical clinical course, especially the early onset of the disease with ataxia and rapid deterioration.- - - - - - - - - - ranking = 1keywords = pneumonia (Clic here for more details about this article) |
10/83. pemphigus vulgaris in association with silicosis.We report on a sixty-seven year old miner with pemphigus vulgaris characterised clinically by a three month history of relapsing oral lesions and blisters/erosions on the trunk, axillae and extremities, histologically by suprabasal cleavage due to acantholysis, immunologically by the epidermal intercellular net-like pattern due to deposits of IgG- and IgM-antibodies and complement c3 in the direct immunofluorescence as well as by serum antibodies to desmoglein 3 (130 KD) and plakoglobin (85 KD) by immunoblotting analysis. silicosis has already been known for 6 years. In addition, antinuclear antibodies, anti-ssDNA-antibodies and anti-topoisomerase antibodies were found. Clinical improvement and clearing of skin symptoms could be achieved by systemic steroids in combination with cyclophosphamide. However, the patient died of sepsis deriving from recalcitrant pneumonia. Although the association of silicosis with various autoimmune diseases such as systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis and dermatomyositis has been reported many times, our patient is, to the best of our knowledge, the second case with features of the two diseases: pemphigus vulgaris and silicosis.- - - - - - - - - - ranking = 1keywords = pneumonia (Clic here for more details about this article) |
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