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Pulmonary disease associated with L-tryptophan-induced eosinophilic myalgia syndrome. Clinical and pathologic features.

Tablets and capsules containing the essential amino acid L-tryptophan are currently being investigated as a cause of the newly recognized eosinophilia/myalgia syndrome. In the five histologically documented cases reported herein, L-tryptophan ingestion was associated with prominent pulmonary complications. All patients were women ranging from 34 to 65 years, and all presented with respiratory symptoms that began after one to nine months of L-tryptophan therapy. Peripheral eosinophilia was present in four patients as were bilateral interstitial infiltrates on chest roentgenograms. One patient had a normal chest roentgenogram. Lung biopsies were done in all patients and biopsy specimens showed a vasculitis and perivasculitis associated with a mild chronic interstitial pneumonitis and eosinophilia. Three patients had clinical and/or histologic evidence of pulmonary hypertension, and one had a follicular bronchiolitis. Four patients recovered promptly with steroid therapy and discontinuation of L-tryptophan therapy, and one patient has had minimal symptomatic improvement.

Adult↗

Reduced diffusing capacity as an isolated finding in asbestos- and silica-exposed workers.

From a cohort of 286 patients referred to an Occupational Medicine Clinic because of exposure to asbestos and/or silica, we identified 53 patients with a reduced diffusing capacity (Dco) (less than 75 percent predicted) as their only abnormality. Specifically, their clinical evaluation, chest roentgenograms, and remaining pulmonary function test results were all normal. These patients were divided into non-smokers (n = 13) and smokers (n = 40). The significance of the isolated reduction in diffusing capacity in these patients (n = 53) was explored with graded exercise testing (n = 19) and bronchoalveolar lavage (BAL) (n = 50). The results obtained from the patients with reduced diffusion were compared with those obtained from comparable smoking (n = 35) and nonsmoking patients (n = 37) in the original cohort who had normal chest roentgenograms and normal results of pulmonary function studies, including normal Dco values (greater than or equal to 75 percent of predicted value). Patients with low diffusion demonstrated a tendency for elevated alveolar to arterial O2 differences both at rest and during exercise, and a significant reduction in exercise capacity (VO2 max) was observed in the smoking patients with reduced diffusion when compared with their smoking counterparts with normal diffusion. All other exercise testing indexes were normal in the study groups and there was no correlation between the percent predicted Dco value and any of the exercise variables. In contrast, BAL revealed significant differences between patient groups. Both the smoking and nonsmoking patient groups with low Dco values had greater numbers of total BAL cells, alveolar macrophages, neutrophils, lymphocytes, and eosinophils in their BAL fluid than did their comparable controls with normal diffusion values. These differences were statistically significant (p less than .05) for total BAL cells and total macrophages in the nonsmoking patients and for total BAL cells, total macrophages, and total lymphocytes in the smoking patients expressed as either the total cell number per BAL or total cells per milliliter of BAL. In contrast to the observed exercise testing results, there was significant and inverse correlation between Dco values and each BAL cell type for all four groups combined as well as nonsmokers alone. The Dco values from smokers were significantly and inversely correlated with total BAL cells and total macrophages. These results suggest that the finding of a reduced Dco may be related to an active inflammatory process in the lung caused by occupational dust exposure.(ABSTRACT TRUNCATED AT 400 WORDS)

Air Pollutants, Occupational↗

Pulmonary functional impairment associated with pleural asbestos disease. Circumscribed and diffuse thickening.

To define the pulmonary functional impairment associated with pleural asbestos signs (PAS), we compared 738 men with only circumscribed (plaques) or diffuse pleural thickening on chest roentgenograms but no irregular opacities by ILO pneumoconiosis criteria (1980) with 738 age-matched asbestos-exposed men without any roentgenographic signs and with 228 men unexposed to asbestos. All men were white. Spirometry and total thoracic gas volumes (TGV) were measured and expressed as percentage of predicted of white Michigan men who have been modeled for spirometric values thereby adjusting for height, age, and in current and ex-smokers for duration of smoking. Asbestos-exposed men who never smoked had reduced FEF75-85 (p less than 0.01) and increased TGV (p less than .0001) as compared with unexposed men. The 155 men with PAS who had never smoked had reduced flows (p less than .0001), FVC (p less than 0.0056), and TGV (p less than .0001) when compared with 155 age-matched asbestos-exposed men. The 325 asbestos-exposed current smokers with normal chest roentgenograms compared with unexposed smokers had reduced expiratory airflows (p less than 0.0001), reduced FEV1 (p less than 0.004), and increased TGV (p less than 0.0001). The 325 current smokers with PAS had additional air trapping that further reduced vital capacity. Thus, PAS were associated with significant pulmonary dysfunction in men who never smoked, and current and ex-smokers had additional dysfunction even after adjustment for duration of smoking.

Asbestosis↗

Acute lobar atelectasis. A comparison of two chest physiotherapy regimens.

Fourteen cases of acute lobar atelectasis were alternately allocated to one of two chest physiotherapy regimens for treatment. Treatment in group 1 comprised positioning, vibrations, hyperinflation, and suction, and in group 2, treatment consisted of hyperinflation and suction alone. Treatment in either group was given hourly for six hours. Patients in group 1 had a significantly higher mean percentage resolution of their atelectasis (mean value, 60.1 percent), as seen on chest roentgenogram, after one treatment intervention than patients in group 2 (mean value, 7.6 percent; p less than .006). After the intensive six-hour treatment period, the difference between the groups was marginally statistically significant, still favoring group 1 over group 2 (p less than .055). Follow-up roentgenograms at 24 and 48 hours revealed no significant difference between the treatment groups (p greater than .10 and greater than .20, respectively). These results suggest that, at least initially in the course of acute lobar atelectasis, positioning and vibrations add to the efficacy of a treatment of hyperinflation and suction alone.

Acute Disease↗

[Human adiaspiromycosis. A report of a case treated with ketoconazole].

A case of human disseminated pulmonary adiaspiromycosis is reported. The patient, from Goianésia, GO, was admitted to the Brasilia University Hospital, in November 1992, with wet cough, dyspnea and weight loss--manifestations that had appeared two months before. Prior to admission, he had been treated for a suspected miliary tuberculosis, because a chest roentgenogram had shown a diffuse reticulonodular infiltrate in both lungs. This therapy brought no improvement to the patient status. An open chest biopsy was then performed, and the microscopic examination of the lung tissue revealed the fungal nature of the disease. Ketoconazole, 400mg/day, was started and the patient discharged from the hospital. He was seen again two months later: the respiratory manifestations had disappeared and a new chest roentgenogram showed complete resolution of the pulmonary lesions. The usefulness of ketoconazole is, however, questioned, since, as there is no multiplication of the fungus in the host organism--adiaspiromycosis is believed to be, usually, a self-healing disease--the efficacy of this imidazole derivative against the agent in animal tissues remains to be confirmed.

Adult↗

[Lung volume reduction surgery (LVRS): criteria for selecting patient in Antonio Pedro Hospital, Federal Fluminense University, Niter i city, Rio de Janeiro, Brazil].

BACKGROUND: Chronic Obstructive Pulmonary Disease (CPOD) is an important problem in Brazil and other countries. The clinical treatment of ambulatory patients is now reasonably standardized. Lung volume reduction surgery (LVRS) is an alternative of surgical treatment of emphysema (aside from lung transplantation). OBJECTIVES: The aim of this study was to analyse patient selection criteria for LVRS. METHODS: This study was carried out using a sample of 31 patients referred to and given assistance to at the Smoking and Chronic Obstructive Pulmonary Disease Outpatient Ward Antonio Pedro Hospital, who were evaluated to check whether or not they could be eligible for LVRS. Aiming at evaluating the major criteria for eligibility for LVRS, the patients underwent a protocol including standardized anamnesis and physical examination, spirometry with maximum inspiratory and expiratory flow volume curves associated with post-bronchodilatator test, room air arterial blood gases, a six-minute walking test, plain chest roentgenograms and left lateral chest roentgenograms, high-resolution chest computed tomographic scans and lung perfusion scans. Statistical analysis was conducted with description of data proportions. RESULTS: Contraindications to surgery included chronic bronchitis (29% or nine patients), forced expiratory volume in one second (FEV 1) more than 80 percent predicted (13% or four patients), negative consent for surgery (10% or three patients), fibrosis pattern, severe depression and bronchiectasis (two patients each one), solitary pulmonary nodule, severe obesity, severe bullous emphysema, pulmonary hipertension, pulmonary resection history, current cigarette smoking, diffuse emphysema with alpha-1-antitrypsin deficiency and severe cardiac disease (one patient each). Indication. One selected patient with severe symptoms of emphysema met criteria for LVRS (bilateral superior lobe heterogeneous pattern of emphysema on chest computed tomography and lung perfusion scan). CONCLUSIONS: Chronic bronchitis was the major isolated factor to contraindicate LVRS. Most patients who fit the criteria for the surgery performance refused to undergo the LVRS for fear of the surgery and after pulmonary rehabilitation. The most important criteria for LVRS is bilateral superior lobe heterogeneous pattern of emphysema.

Adult↗

Maxillary and mandibular displacement in hemifacial microsomia: a longitudinal Roentgen stereometric study of 21 patients with the aid of metallic implants.

OBJECTIVE: To record maxillary and mandibular displacement with articular growth and in response to bimaxillary surgical repositioning in patients with hemifacial microsomia (HFM) and to observe ipsilateral corpus/ramus growth in severely affected children. DESIGN: Prospective roentgen stereometric analysis (mean age 7 years 10 months to 18 years 0 months) and retrospective profile and panoramic roentgenograms. Mean total observation period was 9 years 1 month. SETTING: Department of Plastic and Reconstructive Surgery, Malmö University Hospital (Malmö, Sweden). PATIENTS: Twenty-one patients consecutively diagnosed from 1976 through 1988 with HMF, five of whom had bimaxillary surgery. INTERVENTIONS: Surgery was performed at the Department of Plastic and Reconstructive Surgery. Implants were inserted at the initial reconstructive surgical procedure under general anesthesia. Roentgen examinations were performed in connection with continued clinical evaluations and treatment. MAIN OUTCOME MEASURES: Stereo roentgenograms were digitized at the Department of Orthopedic Surgery, Malmö University Hospital (Malmö, Sweden). RESULTS: Displacement of the jaws with articular growth and in response to bimaxillary surgical repositioning varied interindividually with no apparent common pattern. Relapse displacement occurred several years after bimaxillary surgery. Mandibular growth changes were found in the corpus/ramus area and alveolar process on the affected side. CONCLUSIONS: A marked interindividual variability of maxillary and mandibular displacement indicates that the relevance of statistical analysis of HFM growth data may be questioned. We would suggest that precise and accurate longitudinal recordings of growth and response to surgery in individual HFM patients be more appropriate.

Adolescent↗

Congenitally missing teeth in the Israeli cleft population.

OBJECTIVE: The purpose of the study was to determine the prevalence of congenitally missing teeth in a group of Israeli children with various types of clefts. DESIGN: Prevalence of congenitally missing teeth was determined for 179 children with cleft lip, cleft lip and alveolar ridge, cleft lip and palate, and cleft palate. Subgroupings were assessed according to patient sex, origin, cleft type, tooth type, and the side of hypodontia. The diagnosis of congenitally missing teeth (CMT) was based on initial and follow-up panoramic roentgenograms. In case of doubt, occlusal or periapical roentgenograms as well as clinical dental photographs were also used. SETTING: Data collection was conducted at the Rambam Medical Center, Orthodontic and Craniofacial Unit, Haifa, Israel. RESULTS: In the total cleft group, 67.6% of the patients presented with hypodontia, totaling 246 missing teeth. A statistically significant difference was found in the distribution of patients with CMT of Jewish and minority origin according to sex. Cleft lip and palate was the most frequently affected group in which 195 teeth were missing. The most frequently missing tooth among the cleft population was the maxillary incisor. The order of frequency of the other missing teeth was the same as in the normal population. Hypodontia and malformations of permanent teeth were most common on the cleft side. CONCLUSION: The frequency of CMT in the Israeli cleft population studied was higher than in the intact population. This confirms findings in other populations worldwide.

Adolescent↗

Quintuplets with clefts: follow-up at ten years of age.

A set of quintuplets, three of which were born with various degrees of oral clefting, were followed up to the age of 10 years. Study models, as well as panoramic and intraoral roentgenograms were collected at ages 5 and 10 years. Cephalometric roentgenograms were added at the age of 10 years. Analysis of the growth charts for height and weight indicated normal somatic development, save for a persistent, albeit diminishing, lag for the two most severely affected siblings. The latter also demonstrated a more vertical craniofacial growth pattern. The dental arch dimensions were within normal range, except for the maxillary and mandibular widths in the two more affected siblings, who also exhibited a delay of one or two stages of dental development in an intersibling comparison. These findings indicate that in the more severely affected siblings, there was only partial growth catch-up at the age of 10 years.

Cephalometry↗

Facial growth in a cleft palate patient treated with the Herbst appliance: a long-term profile roentgenographic and roentgen stereometric analysis of profile changes and displacement of the jaws.

OBJECTIVE: To monitor facial development in a patient with cleft palate who was treated with the Herbst appliance. Monitoring was in terms of changes in the skeletal profile and growth in the circummaxillary sutures and temporomandibular joints (TMJs). DESIGN: Prospective profile roentgenography (between the ages of 6 and 20 years) and roentgen stereometric analysis (between the ages of 8 and 19 years). SETTING: Center for Craniofacial Anomalies and Department of Plastic and Reconstructive Surgery, Malmö University Hospital, Malmö, Sweden. PATIENT: Boy with cleft of the soft and posterior part of the hard palate and marked facial convexity. INTERVENTIONS: Surgical repair of the soft palate at age 9 months, velopharyngeal flap at age 8 years, and insertion of implants under general anesthesia and treatment with the Herbst appliance at age 11 years. Roentgen examinations were performed in connection with continued clinical evaluations and treatment. MAIN OUTCOME MEASURES: Profile roentgenograms were traced and measured by one of the authors using conventional point-based analysis. Stereo roentgenograms were digitized by the Department of Orthopedic Surgery, Malmö University Hospital. RESULTS: The direction of profile changes was partly discordant with the direction of articular growth in the circummaxillary sutures and TMJs. The successful treatment result was accomplished by a temporary influence on sagittal growth direction in the circummaxillary sutures and on rotational growth direction in the TMJs, combined with a favorable natural remodeling and articular growth pattern. CONCLUSIONS: The mode of growth by which treatment aims were reached was partly unexpected, i.e., discordant with the generally accepted principal concept that treatment with the Herbst appliance positions the mandible forward.

Adolescent↗

Osteosarcoma arising from giant cell tumor of bone ten years after primary surgery: a case report and review of the literature.

Giant cell tumor of the bone (GCT) is a relatively uncommon tumor. It is characterized by the presence of multinucleated giant cells. GCT is a primary benign tumor but may evolve into a malignant tumor, usually after irradiation. We report a rare case of osteosarcoma arising ten years after the primary surgery for GCT without radiation. A 45-year-old woman presented with severe right knee pain after suffering contusion. Roentgenogram revealed a bone tumor in the lateral femoral condyle of the right knee. Histopathological examination demonstrated the features of GCT, and treatment consisted of curettage and bone grafting. Four months after the operation, multiple lung metastases of GCT occurred, which were treated by partial lobectomy. Ten years after the primary treatment, severe knee pain recurred. Roentgenogram and magnetic resonance imaging (MRI) demonstrated a destructive lesion in the lateral condyle of the right knee. Histopathological examination demonstrated a lacy pattern of osteoids and abnormal mitoses in the aggregated atypical mononuclear cells, indicating osteosarcoma. Despite above-knee amputation and chemotherapy, scapular and lung metastases developed and the patient died five months after above knee amputation. One may question whether the primary GCT contained some malignant cells. However, given the aggressiveness of the malignant tumor, this is unlikely. The recurrence of pain and aggravation of bone destruction many years after the primary treatment suggest malignant transformation of GCT.

Bone Neoplasms↗

The postdialytic plasma cyclic guanosine 3':5'-monophosphate level as a measure of fluid overload in chronic hemodialysis.

The postdialytic plasma level of cGMP, a marker for the release of atrial natriuretic peptide (ANP) in humans, is closely related to hypervolemia in chronic hemodialysis patients. In order to test the practicability of routine postdialysis cGMP determination for the detection of fluid overload, ANP and cGMP levels in the total hemodialysis population of 81 patients were measured with blood samples drawn immediately after hemodialysis. Twenty-three patients had a cGMP level of more than 20 pmol/mL. In 13 of these, pulmonary congestion was present on the chest roentgenogram. Two of these patients refused a gradual reduction of their dry body weight. In the remaining 21 patients, the weight reduction was associated with a decrease in cGMP levels in all cases and with a decrease in ANP levels in all but two cases. Fourteen of the 21 patients reached a cGMP level below 20 pmol/mL after weight reduction, and at that time, none of these showed signs of pulmonary congestion on chest x-ray. All seven patients, whose cGMP levels remained above 20 pmol/mL despite the reduction, had documented heart disease with impairment of left ventricular function. These results suggest that the plasma cGMP level after hemodialysis is more apt for the determination of dry body weight than is ANP or a chest roentgenogram.

Adult↗

Study of the healing process after transplantation of pasteurized bone grafts in rabbits.

Different bone allografts (pasteurized, autoclaved, and frozen) were compared based on their osteoinductive properties. Our primary purpose was to examine the biologic qualities of pasteurized allografts, as pasteurization inactivates most viruses transmitted by transplantation. Frozen, pasteurized, and autoclaved allografts were packed into a standard defect of rabbit ulna. The animals were sacrificed at 2 and 4 weeks after surgery. The parts of bones with experimental defects were explored en bloc, and a roentgenogram was carried out. Ulna bone samples were then embedded in methyl-methacrylate. Roentgenograms showed that after 2 weeks, calluses were well-formed, but irregular in shape in all 3 types of allografts. After 4 weeks, the calluses were regular in shape in all but the autoclaved grafts. After 2 weeks, the healing processes had begun in the frozen and pasteurized grafts, with the reaching approximately the same stage, while in the autoclaved grafts these processes were not seen and the bone particles were surrounded by connective tissue without any changes. After 4 weeks, osteoinductive processes were very strong, with the first signs of complete bone remodeling at the bone edges of the defect in pasteurized and frozen allografts. The osteoinductive values of these 2 types were very high and similar. Autoclaved allografts, on the other hand, had very low osteoinductive values, as they were still at the very beginning of the healing process. Histomorphometric analysis revealed a significant difference in both newly formed osteoid thickness and osteoblast number per microm of bone surface in all experimental groups (P < 0.005). Values of osteoid thickness and osteoblast number were significantly higher in both frozen and pasteurized grafts when compared with the autoclaved ones (P < 0.005). Osteogenic properties of pasteurized bone allografts were preserved, and the allografts have been gradually replaced with newly formed bone. As such, pasteurized bone grafts from a bone bank have approximately the same biologic validity as frozen grafts, while autoclaved grafts impair bone healing.

Animals↗

Spinal deformity in patients who have fibrodysplasia ossificans progressiva.

We reviewed roentgenograms and clinical records in order to characterize the spinal deformity in forty patients who had an established diagnosis of fibrodysplasia ossificans progressiva. Twenty-six (65 per cent) of the patients had scoliosis, which, according to the clinical records and the recollection of the patients, had been present during childhood. Twenty-three (88 per cent) of the twenty-six curves were unbalanced c-shaped curves, while the remaining three (12 per cent) were balanced s-shaped curves. Twenty-one (91 per cent) of the twenty-three c-shaped curves involved the thoraco-lumbar or lumbar spine. The c-shaped curves ranged in magnitude from 15 to more than 80 degrees. Curves became rigid by early adulthood and many resulted in severe pelvic obliquity with impaired sitting or standing balance. An osseous bridge developed between the posterolateral aspect of the iliac crest and the posterolateral aspect of the rib cage in twenty-two (55 per cent) of the forty patients. Nineteen (86 per cent) of these twenty-two patients had scoliosis; there was a significant association between the development of scoliosis and the presence of the osseous bridge (p < 0.005). Ossification of the paravertebral muscles and fascia during the first decade of life limited the development of a normal thoracic kyphosis in ten (42 per cent) of twenty-four patients for whom lateral roentgenograms of the spine were available. A spinal orthosis was used to treat the scoliosis in two patients, but this method resulted in breakdown of the skin and failed to halt progression of the curve.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Effectiveness of treatment with a brace in girls who have adolescent idiopathic scoliosis. A prospective, controlled study based on data from the Brace Study of the Scoliosis Research Society.

In a prospective study by the Scoliosis Research Society, 286 girls who had adolescent idiopathic scoliosis, a thoracic or thoracolumbar curve of 25 to 35 degrees, and a mean age of twelve years and seven months (range, ten to fifteen years) were followed to determine the effect of treatment with observation only (129 patients), an underarm plastic brace (111 patients), and nighttime surface electrical stimulation (forty-six patients). Thirty-nine patients were lost to follow-up, leaving 247 (86 per cent) who were followed until maturity or who were dropped from the study because of failure of the assigned treatment. The end point of failure of treatment was defined as an increase in the curve of at least 6 degrees, from the time of the first roentgenogram, on two consecutive roentgenograms. As determined with use of this end point, treatment with a brace failed in seventeen of the 111 patients; observation only, in fifty-eight of the 129 patients; and electrical stimulation, in twenty-two of the forty-six patients. According to survivorship analysis, treatment with a brace was associated with a success rate of 74 per cent (95 per cent confidence interval, 52 to 84) at four years; observation only, with a success rate of 34 per cent (95 per cent confidence interval, 16 to 49); and electrical stimulation, with a success rate of 33 per cent (95 per cent confidence interval, 12 to 60).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

A successful therapy with plasma exchange for interstitial pneumonia of progressive systemic sclerosis.

A 47-year-old woman who was diagnosed as progressive systemic sclerosis (PSS) had acute and severe interstitial pneumonia. Based on the results of her chest roentgenogram, computed tomography, transbronchial lung biopsy (TBLB) and bronchoalveolar lavage (BAL), her interstitial pneumonia was considered to be atypical of PSS. Although she was treated with corticosteroid, methylprednisolone pulse therapy and immunosuppressive drug, the effect of these drugs was insufficient as treatment for the interstitial pneumonia. Therefore, plasma exchange was attempted. After plasma exchange was carried out for three days, her symptoms improved as well as the laboratory data and chest roentgenogram without any severe side effects. We recommend plasma exchange for interstitial pneumonia of PSS as an effective treatment.

Adrenal Cortex Hormones↗

Two cases of lung carcinoma with osteoplastic bone metastasis.

Two cases of lung carcinoma with osteoplastic bone metastasis are reported. The bone roentgenogram of a 66-year-old woman with pleuritis carcinomatosa revealed multiple sclerotic changes in the right forearm, lumbar spine, and pelvis. Biopsy at internal fixation of the left humerus revealed metastatic adenocarcinoma with osteoplastic change. In a 77-year-old man with small cell carcinoma of the lung, after chemotherapy, osteoplastic bone changes were detected in roentgenograms. An ivory vertebra formation was seen in L1, L2 and L4, and bone scintigram showed, additionally, uptake in the ilium.

Adenocarcinoma↗

Intrapulmonary lymph nodes.

We report two cases of intrapulmonary lymph nodes detected by a chest roentgenogram or CT scan. The first patient was a 61-year-old fisherman referred complaining of cough and hemosputum. Chest roentgenogram showed a subpleural small nodular shadow at the superior segment of the right lower lobe. Thoracotomy showed a small anthracotic lymph node. The other case was a 68-year-old female patient admitted for further examination of a subpleural small nodular shadow at the latero-basal segment of the left lower lobe detected on a chest CT scan. Thoracoscopic surgery revealed that the black nodule was an anthracotic lymph node. The appearance of an intrapulmonary lymph node on radiological examination is rare, however, it should be considered in the differential diagnosis of a solitary or multiple peripheral pulmonary nodules in adults. A small nodular shadow should be resected if malignancy is suspected though not proven. Subpleural intrapulmonary lymph node warrants thoracoscopic surgery.

Aged↗