Cases reported "Disease Progression"

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1/121. A retired shipyard worker with rapidly progressive pulmonary interstitial fibrosis.

    We present a case of progressive interstitial fibrosis in a retired shipyard worker who was exposed to asbestos during the postwar era of the late 1940s and 1950s, when asbestos exposures in the workplace were not regulated. Forty years later, at 63 years of age, the patient presented with restrictive lung disease. The patient was diagnosed with asbestos-related pleural disease and parenchymal asbestosis. He remained stable for the next 7 years, but then he began to manifest rapid clinical progression, which raised the possibility of an unusual variant of asbestosis, a concomitant interstitial process, or an unrelated disease. lung biopsy was not undertaken because of the patient's low pulmonary reserve and limited treatment options. An empiric trial of oral steroids was initiated, but his pulmonary status continued to deteriorate and he died of pulmonary failure at 72 years of age. Many diseases result in pulmonary interstitial fibrosis. Ideally, open lung biopsy should be performed, but this procedure inevitably causes complications in many patients with end-stage restrictive lung disease. Furthermore, while the presence of asbestos bodies in tissue sections is a sensitive and specific marker of asbestos exposure, neither this finding nor any other charge is a marker indicative of asbestosis or the severity of asbestosis. With the enactment of the Asbestos Standard in the united states, asbestos exposures have been decreasing in this country. However, industries that produce asbestos products and wastes continue to expand in developing countries. Prevention of asbestos-related lung disease should be a global endeavor, and asbestos exposures should be regulated in both developed and developing countries.
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ranking = 1
keywords = progressive interstitial, interstitial
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2/121. 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.
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ranking = 0.0066527377343277
keywords = interstitial pneumonia, interstitial, pneumonia
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3/121. 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.
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ranking = 0.0025522978513703
keywords = pneumonia
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4/121. 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.
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ranking = 0.0031903723142128
keywords = pneumonia
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5/121. 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.
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ranking = 0.00063807446284256
keywords = pneumonia
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6/121. Rapidly progressive fibrosing interstitial nephritis associated with Chinese herbal drugs.

    Rapidly progressive fibrosing interstitial nephritis after a slimming regimen containing aristolochic acid has been identified as Chinese herbs nephropathy (CHNP). From 1995 to 1998, we observed 12 Chinese people from different areas of taiwan who underwent renal biopsy for unexplained renal failure. Medical history gave no clue to the causes of impaired renal function except for the ingestion of traditional Chinese herbs. Although these patients ingested herbal drugs from various sources for different purposes, their renal biopsy samples showed amazingly similar histological findings, with extensive hypocellular interstitial fibrosis and atrophy and loss of tubules in all cases. Glomeruli were apparently intact. They also had similar clinical features, such as normal or mildly elevated blood pressure, early and severe anemia, low-grade proteinuria, glycosuria, and insignificant urinary sediments. Renal function deteriorated rapidly in most patients despite discontinuation of the herbal medicines. Seven patients underwent dialysis, and the remainder experienced slowly progressive renal failure. Bladder carcinoma was found in one patient. Morphologically and clinically, the nephropathy in our patients was similar to CHNP, reported in belgium. Because of the complexity and unknown types of herbs used in different clinical situations, unidentified phytotoxins other than aristolochic acid might be responsible for this unique disease entity. We conclude that the relation of this nephropathy to the consumption of Chinese herbs is striking. Using uncontrolled herbal remedies carries a high risk for developing interstitial renal fibrosis and urothelial malignancy.
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ranking = 0.0087201722082442
keywords = interstitial
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7/121. 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.
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ranking = 0.013943549931498
keywords = interstitial pneumonia, interstitial, pneumonia
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8/121. 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.
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ranking = 0.00063807446284256
keywords = pneumonia
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9/121. Interstitial deletion of the short arm of chromosome 12 during clonal evolution in myelodysplastic syndrome with t(5;12)(q13;p13) involving the ETV6 gene.

    We report here a 65-year-old man with a myelodysplastic syndrome (MDS), refractory anemia with excess of blasts. He had received chemotherapy with tegafur for renal carcinoma. Chromosome analysis of bone marrow cells revealed complex karyotypes; del(5)(q13) was observed in all 20 metaphase spreads, and two related aberrations, add(12)(p11) and add(12)(p13), were detected in 13 and 7 cells, respectively. fluorescence in situ hybridization (FISH) analysis with chromosome-specific DNAs revealed that these alterations originated from a reciprocal translocation (5;12)(q13;p13). Therefore, del(5)(q13), add(12)(p11), and add(12)(p13) were revised as der(5)t(5;12)(q13;p13), der(12)del(12)(p11p13)t(5;12)(q13;p13), and der(12)t(5;12)(q13;p13), respectively. fluorescence in situ hybridization with a series of cosmid probes spanning the ETV6 gene showed that the 12p13 breakpoint on the der(12)t(5;12)(q13;p13) was located in intron 1, but the exon 1 signal was deleted. Our results suggest that a fusion gene was generated between the 5'-end of an unidentified partner at 5q13 and the 3'-end of ETV6 by t(5;12)(q13;p13), and that the interstitial deletion (12)(p11p13) occurred following t(5;12) during clonal evolution. del(12)(p11p13), including the rearranged ETV6 gene, may be implicated in the progression of MDS.
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ranking = 0.001245738886892
keywords = interstitial
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10/121. Clinicopathological study of atypical motor neuron disease with vertical gaze palsy and ballism.

    The case of a 38-year-old patient with rapidly progressing motor neuron disease, complicated by major dysfunction of the extrapyramidal system and of vertical gaze is described. Neuropathological examination revealed a degenerative process that severely affected the lower motor neurons, as well as the neurons of the pars compacta of the substantia nigra, the nucleus of Darkschewitsch, the nucleus interstitialis of Cajal, the colliculi superiores, and the pallidum. The long tracts were unaffected at all levels of the brain stem and spinal cord. There was no convincing evidence for the presence of a multiple system atrophy or progressive supranuclear palsy; the results rather revealed a pattern of vulnerability characteristic of a variant of motor neuron disease.
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ranking = 0.001245738886892
keywords = interstitial
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