Michael C. Zacharisen

Asthma is a risk factor for acute chest syndrome and cerebral vascular accidents in children with sickle cell disease

BackgroundAsthma and sickle cell disease are common conditions that both may result in pulmonary complications. We hypothesized that children with sickle cell disease with concomitant asthma have an increased incidence of vaso-occlusive crises that are complicated by episodes of acute chest syndrome.MethodsA 5-year retrospective chart analysis was performed investigating 48 children ages 3–18 years with asthma and sickle cell disease and 48 children with sickle cell disease alone. Children were matched for age, gender, and type of sickle cell defect. Hospital admissions were recorded for acute chest syndrome, cerebral vascular accident, vaso-occlusive pain crises, and blood transfusions (total, exchange and chronic). Mann-Whitney test and Chi square analysis were used to assess differences between the groups.ResultsChildren with sickle cell disease and asthma had significantly more episodes of acute chest syndrome (p = 0.03) and cerebral vascular accidents (p = 0.05) compared to children with sickle cell disease without asthma. As expected, these children received more total blood transfusions (p = 0.01) and chronic transfusions (p = 0.04). Admissions for vasoocclusive pain crises and exchange transfusions were not statistically different between cases and controls. SS disease is more severe than SC disease.ConclusionsChildren with concomitant asthma and sickle cell disease have increased episodes of acute chest syndrome, cerebral vascular accidents and the need for blood transfusions. Whether aggressive asthma therapy can reduce these complications in this subset of children is unknown and requires further studies.

The spectrum of respiratory disease associated with exposure to metal working fluids

Occupational respiratory diseases have been reported following exposure to metal working fluids. We report a spectrum of respiratory illnesses occurring in an outbreak in 30 workers of an automobile parts engine manufacturing plant. Workers presented with respiratory complaints and, after clinical and laboratory evaluations, were classified as those having hypersensitivity pneumonitis, occupational asthma, or industrial bronchitis, or those without occupational lung disease. Hypersensitivity pneumonitis affected seven workers, with six exhibiting serum precipitins to Acinetobacter Iwoffii. Occupational asthma and industrial bronchitis affected 12 and six workers, respectively. Oil-mist exposures were below current recommendations. Gram-negative bacteria, but no fungi, Thermophiles, or Legionella, were identified. Although specific agents responsible for each individual case could not be identified, probably both specific sensitizing agents and non-specific irritants from metal working fluids, additives, or contaminants contributed to this spectrum of occupational respiratory illness.

The long-term outcome in acute, subacute, and chronic forms of pigeon breeder's disease hypersensitivity pneumonitis

BACKGROUND Pigeon breeders disease (PBD) is an avian-induced hypersensitivity pneumonitis. The limited data that exist on the long-term outcome for the disease are conflicting. OBJECTIVE We sought to determine the long-term outcome of patients with PBD to evaluate the course of the disease. METHODS Review a case series of 18 pigeon breeders that were followed from 4 to 26 years after initial diagnosis. RESULTS Fourteen breeders reduced their bird contact significantly; four kept their birds. In eight patients with the acute form of the disease, pulmonary function either did not change from normal or improved to normal. Of five patients with subacute disease, four had persistent symptoms. All had mildly abnormal spirometry with either restrictive or obstructive changes which persisted. Five patients with chronic disease exhibited daily dyspnea. They had severe abnormalities of lung function at diagnosis. Of these patients, three improved and two deteriorated. Chest radiographs were of little value in predicting outcome. CONCLUSIONS Patients with the acute form of PBD have the best prognosis, compared with those with subacute and chronic forms. Although some improvement could be seen in most individuals, recovery may still not be complete. Those with the chronic form are at particular risk for morbidity.

Recurrent respiratory papillomatosis in children: masquerader of common respiratory diseases.

BACKGROUND. Recurrent respiratory papillomatosis in children is an uncommon but potentially life-threatening benign tumor of the respiratory tract with laryngeal predilection. The diagnosis of recurrent respiratory papillomatosis may be challenging unless there is a high index of suspicion and awareness of the variable presentations. METHODS. We reviewed the medical charts of children with recurrent respiratory papillomatosis treated at a tertiary children’s hospital. The presentation of recurrent respiratory papillomatosis is illustrated by a series of case reports. We provide a paradigm to assist in the early diagnosis of children with recurrent respiratory papillomatosis. RESULTS. Five patients, aged 2 to 6 years, were erroneously diagnosed with recurrent croup, asthma, laryngeal hemangioma, and tracheomalacia after presenting with variable degrees of chronic dyspnea, cough, stridor, dysphonia, weak cry, and syncope. Once the diagnosis of recurrent respiratory papillomatosis was made, recurring surgical ablation of papillomata was initiated. CONCLUSIONS. Any child presenting with a voice disturbance with or without stridor is recommended to have diagnostic flexible fiber-optic laryngoscopy. Recurrent respiratory papillomatosis should be considered in children when other common pediatric airway diseases either do not follow the natural history or do not respond to treatment of the common disorder.

Anaphylaxis to rabbit : a case report

BACKGROUND While rabbits are common as pets, severe allergic reactions to domestic rabbits in homes are unusual. Typically, allergic manifestations are mild to moderate allergic rhinitis, conjunctivitis, pruritus and/or asthma in laboratory animal caretakers with frequent exposure. METHODS We report an atopic child with a severe allergic reaction following inhalant exposure to a rabbit. We performed percutaneous skin tests and determined serum-specific IgE to commercial preparation of rabbit epithelium and extracts of rabbit fur and serum. RESULTS Percutaneous skin test was positive to rabbit epithelium. The patient had elevated serum-specific IgE to rabbit epithelium and fur but not to rabbit serum.

New-onset rheumatoid arthritis after anthrax vaccination.

BACKGROUND Anthrax vaccine was licensed in 1970 and is used to protect individuals exposed to biological warfare and those who may come in contact with Bacillus anthracis in infected animals or in laboratory settings. The current adsorbed anthrax vaccine is regarded as effective and safe. Adverse effects reported include fever, chills, myalgia, arthralgia, and nausea. Four cases of rheumatoid arthritis (RA) temporally related to anthrax vaccine have been reported. As the number of administered doses increases, a better understanding of its adverse events profile will be forthcoming. OBJECTIVE To describe another patient with RA temporally related to anthrax vaccination. METHODS A 42-year-old man developed bilateral knee stiffness and pain in all the proximal interphalangeal joints 5 days after receiving the first dose of anthrax vaccine. He reported chills, fever, and joint and neck pain, with a tender nodule at the injection site after dose 2. Hours after receiving dose 3 he experienced fever, chills, nausea, vomiting, and neck, hand, and shoulder pain. The vaccination series was terminated after the third dose. RESULTS Physical examination revealed moderate swelling and tenderness of his bilateral proximal interphalangeal joints. His complete blood cell count was normal; rheumatoid factor level, 198 IU/mL; erythrocyte sedimentation rate, 53 mm/h; antinuclear antibodies, negative; C-reactive protein level, 2.7 mg/L; and anti-cyclic citrullinated peptide antibody level, 168 EU. Radiographs revealed mild degenerative changes in his hands and knees bilaterally. CONCLUSIONS This case represents a fifth patient with RA temporally related to anthrax vaccine.

Asthma deaths outside the hospital in an urban community from 2004 to 2008

BACKGROUND Several studies have been performed reviewing medical examiners autopsy reports of asthma deaths. None, to our knowledge, have focused on the characteristics of asthma deaths in the urban community alone. OBJECTIVE To characterize factors related to asthma deaths occurring outside the hospital setting in an urban community. METHODS We reviewed the medical records of 22 patients who died outside the hospital of asthma and underwent autopsy performed by the Milwaukee County medical examiner from 2004 to 2008. RESULTS The mean age of the patients was 32 years (range, 12-71 years), 11 patients were male, and 14 patients (64%) were African American. Seventeen patients (77%) died during the night or shortly on awakening. Twelve patients (55%) died in June, July, or August. A history of illicit drug, alcohol, or tobacco use was discovered in 13 patients (59%). Toxicologic test results for drugs of abuse were positive in 4 patients (18%). Twenty patients were using or overusing a short-acting β-agonist, 1 patient was taking omalizumab, and none were taking long-acting β-agonists alone. Two patients were taking no medications. Asthma severity and medication adherence were not consistently reported. Lung pathologic testing revealed eosinophils in 18 patients and a lack of neutrophils in every case. CONCLUSION In this small and limited series of asthma deaths occurring in an urban setting outside the hospital, individuals were more likely to be African Americans, with deaths occurring more frequently at night, during the summer months, and in those with substance abuse and not taking anti-inflammatory asthma medications.

Occupational asthma secondary to enzymes used in cheese production

Occupational asthma (OA) accounts for 5-10% of all asthma in adults. Although OA secondary to enzymes has been reported, it is rare in the context of food preparation. In the cheese production industry, multiple powdered enzymes are used to soften and flavor cheese. Work-related asthma secondary to enzymes used in this manner has not been previously reported. We present two cases of OA after exposure to airborne enzyme powders used in cheese production. Both patients were adult women without histories of asthma who worked in a facility that used fungal and pancreatic-based enzymes to soften and flavor cheese. Both developed asthma symptoms within 1 year of employment and experienced relief of symptoms away from work. One patient had occupational rhinitis. Each underwent allergy skin testing, chest radiograph, pulmonary function testing, and methacholine challenge. Both patients had markedly positive skin tests to multiple enzyme antigens used at work. Spirometry, lung volumes, and chest radiographs were normal for both patients when they were asymptomatic and had implemented avoidance measures. Methacholine challenge was positive in one patient (PC(20) = 0.13 mg/mL). Both workers took appropriate respiratory protection measures during powder exposure and their symptoms improved. Enzyme powder used in cheese production is a trigger for OA.

Anaphylaxis to beans

The leguminoaceae family includes many genera: Arachis (peanuts), Glycine max (soybeans), Pisum (peas), Cicer (garbanzo beans or chick peas), and Phaseolus (kidney, garden, lima, butter, snap, sugar, string, navy, pinto, and white beans). Although reactions to peanuts and soybeans are common, reactions to beans have rarely been reported.1, 2 We report a case of anaphylaxis resulting from the ingestion of beans (Phaseolus) in homemade chili.

Fatal hypersensitivity pneumonitis.

BACKGROUND Hypersensitivity pneumonitis (HP) is an uncommon, non-IgE-mediated interstitial lung disease caused by the inhalation of a variety of organic dusts, most commonly from exposure at work or in the pursuit of hobbies. Typically, after the disease is recognized, the causative allergen or environment is identified and treatment initiated through avoidance measures and corticosteroids. Progression of the disease is then usually halted and even reversed. Fatal cases of HP are unusual. OBJECTIVE To report a case of progressive and deadly HP in a 40-year-old printer who developed subacute bird fanciers disease with its clinical characteristics and positive precipitins to pigeon proteins. METHODS Chest x-ray examinations and tests of lung function were performed in the patient. Two months after initial consultation, when the diagnosis was still elusive, an open lung biopsy was performed and the patient was treated with prednisone for 3 months. A subsequent chest x-ray examination was performed 4 months after the biopsy. RESULTS With avoidance of birds and treatment with corticosteroids, the patients symptoms resolved and lung function normalized. He was subsequently diagnosed as having asthma followed by bronchitis and 2 episodes of pneumonia. He did not fully recover from these but developed progressive dyspnea. After linking his symptoms to work by history, he underwent lung biopsy with findings consistent with chronic HP. Serum antibody titers were positive for Aspergillus but not pigeon proteins. Based on exposure to water-based coolants, he was suspected of having chronic occupational HP, although this could not be confirmed. Despite aggressive treatment, he developed a progressive course that was ultimately fatal. CONCLUSIONS This report details the progressive disease course in an individual who presented initially with subacute HP. Unfortunately, even after appropriate diagnosis and management, the course of the disease can be fatal.