Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital stenosis of individual pulmonary veins is uncommon. Of the 49 cases reported, four were seen at the IWK Hospital for Children and are reported here. Plain radiographs show a shift of the heart toward the side of major involvement, Kerley B lines, fluid in the fissures, and interstitial edema of the affected lobes. These findings, while subtle, should nevertheless suggest stenosis. Technetium-99m macro-aggregate lung perfusion scans show absence of or diminished perfusion of the affected lobes. The diagnosis can usually be confirmed with angiography. Congenital stenosis of individual pulmonary veins should be considered in children with repeated pulmonary infections, dyspnea, failure to thrive, hemoptysis, or unexplained pulmonary hypertension.
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PMID:Stenosis of individual pulmonary veins: radiologic findings. 376 52

We present the pulmonary findings in 36 autopsies of children affected by the acquired immunodeficiency syndrome (AIDS). Twenty-three patients were male and 13 were female, ranging in age between 3 days and 13 years. Twenty children had human immunodeficiency virus (HIV)-positive parents or parents who were at high risk of exposure (intravenous drug abusers and prostitutes), five had a history of transfusion, and one had a history of renal transplantation and blood transfusion. Clinically, the patients presented with recurrent infections, failure to thrive, hepatosplenomegaly, fever, cough, and/or hemoptysis. Histologically, specific infectious processes were the most common finding (75% of cases), with Pneumocystis carinii pneumonia being the most prevalent type of infection, followed by bacterial pneumonia. Neoplastic conditions and lymphoid interstitial pneumonia were less frequent (approximately 10% of cases). In addition, in approximately 10% of the cases the pulmonary findings were non-specific (ie, pulmonary edema and atelectasis) and probably unrelated to HIV infection. Our findings suggest that specific infectious conditions constitute the most common type of pulmonary pathology in children with AIDS. However, because there is a small percentage of children with nonspecific findings, a transbronchial biopsy is important for proper evaluation before institution of therapy.
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PMID:The spectrum of pathological changes in the lung in children with the acquired immunodeficiency syndrome: an autopsy study of 36 cases. 808 62

Heiner syndrome (HS) is a food hypersensitivity pulmonary disease that affects primarily infants, and is mostly caused by cow's milk (CM). Only a few reports have been published, which may be due to its misdiagnosis. We review here a series of eight cases. When first diagnosed they were 4-29 months of age. They were fed CM from birth and their chronic respiratory symptoms began at age 1-9 months. The symptoms were in the form of cough in seven, wheezing in three, hemoptysis in two, nasal congestion in three, dyspnea in one, recurrent otitis media (OM) in three, recurrent fever in four, anorexia, vomiting, colic or diarrhea in five, hematochezia in one, and failure to thrive (FTT) in two. All had radiologic evidence of pulmonary infiltrates. High titers of precipitating antibodies to CM proteins were demonstrated in six of six and milk-specific immunoglobulin E (IgE) was positive in one of two. Pulmonary hemosiderosis (PH) was confirmed in one patient who showed iron-laden macrophages (ILM) in the bronchoalveolar lavage (BAL), gastric washing, and open lung biopsy. Additional findings, in a descending frequency, were eosinophilia, anemia, and elevated level of total IgM, IgE or IgA. Milk elimination resulted in remarkable improvement in symptoms within days and clearing of the pulmonary infiltrate within weeks. Parents consented to milk challenge in only three cases, all of whom developed recurrence of symptoms. After 2 yr of milk avoidance in one patient, milk challenge was tolerated for 2 months, and then the patient developed symptoms, serum milk precipitins, pulmonary infiltrate, and ILM. The HS should be suspected in young children with chronic pulmonary disease of obscure cause. The diagnosis is supported with a positive milk precipitin test and improvement on a trial of milk elimination. Severe cases may be complicated with PH, which should be suspected in the presence of anemia or hemoptysis and be confirmed with the demonstration of ILM.
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PMID:Milk-induced pulmonary disease in infants (Heiner syndrome). 1617 5

Can all congenital cystic lung lesions be treated conservatively, without the need for surgery? Our purpose here is to present the morbidity associated with symptomatic cystic lung lesions which have failed to respond to medical treatment. In the past 8 years, 22 consecutive cystic thoracic lesions were retrospectively assessed for clinical presentation, diagnostic modalities, operative findings, technical tribulations, and outcome. The endpoint was complete cessation of recurrent pneumonia and dysphagia. Age at presentation was 7.7 +/- 2.2 years, with 4 +/- 2 episodes per year of lower respiratory tract infection, which had been treated for the past 2.6 +/- 0.3 years. Cough and dyspnea (100%) were the common symptoms, with episodes of cyanosis occurring in 58%. Other significant clinical presentations were dysphagia (55%), failure to thrive (55%), chest pain (46%), haemoptysis (18%), and pleuritic pain (18%). Definitive growth was seen in 91% of the excised specimens. Preoperative morbidity resulted from intractable pneumonia, dysphagia, and failure to thrive. Surgical excision was curative. All 22 children after resection are thriving, with an absence of pneumonia and dysphagia, with normal ventilation/perfusion scans, at 48 +/- 6 months of follow-up. In conclusion, surgical excision of a symptomatic cystic lung lesion that has not responded to medical treatment is recommended.
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PMID:Congenital cystic lung lesions: is surgical resection essential? 1620 Jun 19

A 28-year-old girl suffering from lysinuric protein intolerance was referred on account of severe chronic respiratory failure. Since childhood she had failure to thrive with chronic hyperammonaemia and interstitial lung disease but normal respiratory function. At the age of 21, she experienced haemoptysis and worsening of the respiratory disease. CT scan of the chest showed bilateral, symmetrical ground glass opacities and subpleural cysts. At the age of 25, nocturnal non-invasive ventilation was initiated. Lysinuric protein intolerance is an exceptional genetic cause of interstitial lung disease.
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PMID:[Unusual pulmonary presentation of lysinuric protein intolerance]. 2086 80

Pulmonary manifestations of hyperthyroidism not only include pulmonary hypertension and hydrostatic pulmonary oedema, but also treatment/drug-associated pulmonary diseases have to be considered as an exclusion diagnosis. A 27-year-old woman with hypoxaemic respiratory failure under an arterial-venous extra-corporeal membrane oxygenator (AV-ECMO) was admitted to the intensive care unit (ICU). The patient had progressive dyspnoea with haemoptysis, palpitations and failure to thrive. The patient had Graves' disease treated previously with propylthiouracil (PTU). Diffuse alveolar haemorrhage is a non-specific syndrome characterised by evidence of diffuse alveolar damage, exclusion of infectious aetiology and progressively bloodier bronchoalveolar lavage (and/or 20% hemosiderin laden macrophages on cytological examination). PTU associated perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) vasculitis appears to be more common in younger female patients presenting with leukocytoclastic vasculitis, myalgias and arthralgias. The latter compared to non-drug associated ANCA vasculitis which are more common in older males with visceral involvement. PTU-induced ANCA vasculitis prognosis appears to be better compared to primary ANCA syndromes.
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PMID:A 27-year-old woman presenting with refractory hypoxaemic respiratory failure, haemoptysis and thyrotoxicosis: a rare manifestation of propylthiouracil therapy. 2515 Feb 34

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease characterized by the triad of hemoptysis, pulmonary infiltrates on chest radiograph, and anemia. Its diagnosis should be considered in any child presenting with moderate to severe anemia and failure to thrive of unclear etiology. Consideration of the differential diagnosis in such a child should include the review of both extravascular and intravascular causes of hemolysis. Systemic treatment of IPH with glucocorticoids has been shown to decrease morbidity, mortality, and disease progression to pulmonary fibrosis. Thus, diagnostic delays can impact prognosis. Here, we present a case of a 15-month-old boy with IPH who presented with anemia, jaundice, and failure to thrive, as well as a history of hemoptysis that was not initially elicited.
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PMID:Idiopathic Pulmonary Hemosiderosis Presenting as Anemia, Failure to Thrive, and Jaundice in a Toddler. 2641 32

Pulmonary vein stenosis is a rare and poorly understood condition causing obstruction of the large pulmonary veins and of blood flow from the lungs to the left atrium. This results in elevated pulmonary venous pressure and pulmonary edema, pulmonary hypertension, potentially cardiac failure, and death. Clinical signs of the disease include failure to thrive, increasingly severe dyspnea, hemoptysis, respiratory difficulty, recurrent respiratory tract infections/pneumonia, cyanosis, and subcostal retractions. On chest radiograph, the most frequent finding is increased interstitial, ground-glass and/or reticular opacity. Transthoracic echocardiography with pulsed Doppler delineates the stenosis, magnetic resonance imaging and multislice computerized tomography are used for further evaluation. Interventional cardiac catherization, surgical techniques, and medical therapies have been used with varying success as treatment options.
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PMID:The many faces and outcomes of pulmonary vein stenosis in early childhood. 3250 38