Pulmonary Alveolar Proteinosis in Children: Diagnosis and Treatment Outcomes.

Background: Pulmonary Alveolar Proteinosis (PAP) is an uncommon pulmonary disease characterized by the accumulation of surfactant composed of proteins and lipids due to disruption of surfactant clearance by alveolar macrophages. The current standard treatment is lung lavage. There are no specific criteria for lavage, but in case of observing these signs it is recommended to perform lavage for the patient: progressive respiratory failure, no labored breathing at rest, and drop in oxygen level during activity (>5%). Materials and Methods: In this study, patients with PAP admitted to Pediatric ward of Masih Daneshvari Hospital were studied. The required data were collected including the patient's demographic data, clinical signs and radiographic data, the number of admissions, the age of diagnosis, detection and treatment methods, number of lavage, current condition of the patient, and in case of death, the cause of death. Results: In this study, 17 patients with PAP who were admitted during the past 15 years were examined; among which 7 patients were boys (41.2%) and 10 were girls (58.8%). The mean age of population was 11.79±7.21 years. Transbronchial Lung Biopsy (TBLB) (47.1%) and open lung biopsy (52.9%) were used for diagnosis of patients. Lung lavage was used to treat patients, 15 of whom were treated by this method. Five of the patients died because of their serious conditions. Conclusion: Therapy method in the present study was lavage for both lungs, and it was performed for all patients except for two patients due to their anatomical complications. This method is still considered as the gold standard for PAP. Considering the findings from previous studies and the present study, it seems that Whole Lung Lavage (WLL) was fruitful for patients who had the indication for using this therapy and it played a significant role in improving the prognosis of patients. Besides, it is recommended to do follow-up regularly in order to have more therapeutic efficacy and increased patient longevity.

Pulmonary complications of predominantly antibody immunodeficiencies in a tertiary lung center.

BACKGROUND AND AIMS: Respiratory infections are expressed very soon in the life in humoral immunodeficiencies and often lead to chronic irreversible complications such as bronchiectasis and chronic airflow limitation. This study was conducted to evaluate the pulmonary complications of predominantly antibody immunodeficiencies to show the benefits of timely diagnosis and appropriate therapy. PATIENTS AND METHODS: The information of 48 patients involved with a type of predominantly antibody immunodeficiencies, including sex, type of primary immunodeficiency, age at the onset of symptoms, age at diagnosis, recurrent infections, respiratory symptoms, and pulmonary radiological and functional abnormalities were recorded and analyzed. RESULTS: In 48 patients evaluated, the mean age at diagnosis was 25.63 years. The mean diagnostic delay was estimated to be 13.62 years. The most recurring clinical manifestations, sinusitis (69.6%), otitis (43.5%), and recurrent pneumonia were the cause of frequent admissions in 68.8% of these patients. Bronchiectasis was frequently found (58.3%) in these patients mostly involving the middle and lower lobes (48.8% and 41.5%, respectively). CONCLUSIONS: Respiratory complications, infectious or non-infectious, determine the prognosis of the disease in patients with predominantly antibody immunodeficiencies. Timely diagnosis and appropriate management may improve life expectancy and the quality of life in these patients.

The effect of supplemental zinc on the height and weight percentiles of children.

BACKGROUND: Considering the high incidence of low weight and short stature among Iranian children as well as the high prevalence of both mild and moderate types of zinc deficiency, this study was conducted among patients who were referred to the Pediatric Clinic at Massih Daneshvari Hospital during 1997 - 1999. The aim of this study was to determine the effect of zinc supplementation on the children's height and weight percentiles. METHODS: Age, sex, and height and weight percentiles before and after administration of zinc were determined and recorded. The zinc supplement used in this project was in the form of a zinc sulfate solution in 0.1% and 0.5% concentrations. The dose was the upper limit of a tolerable dose, which was given in three divided doses per day. The serum zinc level was measured with the atomic absorption spectrophotometry method. The effect of age, sex, and initial serum zinc level on the percentile changes were determined. RESULTS: This study was conducted on 42 children with equal distribution of gender (21 male and 21 female patients). The mean age was 4.9 +/- 4.1 years. Means of height and weight were 101.8 +/- 23 cm and 16.5 +/- 8.1 kg, respectively. The initial serum zinc level was 85.1 +/- 31.6 microg/dL. The duration of zinc administration was 3.2 +/- 2.6 months. The subjects were followed for a minimum of one month to a maximum of 10 months. The percentage of children with weight and height percentiles below the 50th percentile was reduced from 69% to 54% (P < 0.05) and from 50% to 33% (P < 0.01), respectively. Also, it was noted that children with low serum zinc levels demonstrated greater percentile changes. CONCLUSION: It was concluded that supplemental zinc increases height and weight percentiles.

The anesthetic management of an intrabronchial escape of a hydatid's ruptured laminated membrane during rigid bronchoscopy.

We describe the anesthetic management of a case in whom a previously undiagnosed pulmonary hydatid cyst manifested as a large amount of intrabronchial fragmented pieces of laminated membrane suddenly and unexpectedly during diagnostic rigid bronchoscopy.