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Pulmonary alveolar proteinosis (PAP) is a rare pulmonary pathology characterized by the accumulation of surfactant within type II alveolar epithelial cells. Whole lung lavage is the standard treatment for pulmonary alveolar proteinosis involving a large volume of fluid is infused into one lung and subsequently retrieved while the other lung is remains ventilated. Fast-tracking a patient undergoing whole lung lavage requires vigilant monitoring of arterial blood gases, fluid status, and respiratory mechanics. We report a case of a patient who underwent whole lung lavage for PAP, where early extubation was performed, avoiding the complications associated with prolonged mechanical ventilation. [ABSTRACT FROM AUTHOR]

Pulmonary Alveolar Proteinosis (PAP) is an unusual diffuse lung disease characterised by the alveolar accumulation of phospholipoprotein material, with a peak incidence in the 3rd to 4th decades of life and a male predominance. However, it has also been described in children. The recorded prevalence is 0.1 per 100,000 individuals. The major clinicopathogenetic subtypes include the autoimmune (idiopathic) form, which is associated with Granulocyte-macrophage Colony-stimulating Factor (GM-CSF) autoantibodies, the secondary form and the congenital form (associated with surfactant gene mutations). Common presenting features include dyspnoea, cough, low-grade fever, inspiratory crackles and digital clubbing. Pulmonary function tests typically show restrictive ventilatory defects. Herein, the authors present a case of a three-month-old male infant who presented with cough, dyspnoea and failure to thrive over the past 10 days. X-rays revealed a bilateral reticular pattern with non homogeneous opacities. Computed Tomography (CT) of the chest showed features consistent with interstitial lung disease. High-resolution Computed Tomography (HRCT) demonstrated a crazy-paving appearance, suggestive of interstitial lung disease or a surfactant-related genetic disorder. Bronchial Alveolar Lavage (BAL) yielded milky fluid. Characteristic microscopic findings on lung biopsy included the filling of terminal bronchioles and alveolar spaces with deep pink granular Periodic Acid Schiff (PAS)-positive eosinophilic material, while the alveolar architecture remained preserved. Patients with minimal symptoms are managed conservatively, whereas those with hypoxaemia require a more aggressive approach. Whole Lung Lavage (WLL) is the safest and most effective form of treatment. To date, there have been 240 case reports and 410 case series documented in the literature. This is the first case of PAP reported in the study institution. The authors present this case report here due to its rarity and diagnostic significance.

Pulmonary alveolar proteinosis (PAP) is an uncommon disease characterized by the excessive accumulation of lipoprotein material in the alveoli. No uniform treatment existed. However, whole lung lavage (WLL) remains the main treatment. We report the case of a 33-year-old non-smoking man, with no reported history of medical problems, who complained of dyspnea on exertion and cough for over 5 years. A CT scan revealed diffuse pulmonary interstitial infiltrates. Bronchoalveolar lavage was milky. WLL was planned, and the procedure was repeated 70 times and a total of 3,5 liters of saline serum was instilled and 2,8 liters of milky liquid was removed. The operation procedure was interrupted because of acute pulmonary edema. The patient was transferred to the intensive care unit for acute respiratory distress syndrome. He received 3 days of noninvasive ventilation and regression of supplemental oxygen. Faced with the seriousness of the complication it was decided not to perform a third wash and to monitor the patient functionally and radiologically. Currently, after one year of the lung wash the patient is stable functionally and radiologically. We presented a rare complication of a WLL in a patient with PAP. Physicians should evaluate the patient carefully before suggesting WLL, which is an invasive procedure requiring general anesthesia for a pathology that may evolve spontaneously favorably.

The article describes a rare respiratory disease – alveolar proteinosis (AP). The etiopathogenesis, clinic al picture, differential diagnostic signs of the disease are considered, allowing to make a correct diagnosis, to exclude other pathological conditions that have a similar X-ray pattern. A clinical case of a patient with AP is presented, and the difficulties encountered in diagnosing this pathology are described. The correct diagnosis was made one year after the detection of the disease as a result of monitoring the general condition, the dynamics of X-ray examinations, thoracoscopy with biopsy performed twice, morphological assessment of biopsy specimens using immunohistochemical methods. During two years of observing the patient, there was no negative dynamics on computed tomography images, the condition remained stable. Basic treatment for concomitant diseases was prescribed, bronchoalveolar lavage was not performed. Therapists, general practitioners and narrow specialists need to be aware of orphan diseases and include them in the differential diagnostic list.

Niemann–Pick disease type C (NPC) is an autosomal recessive lipid storage disorder. There are two types, NPC1, which is the predominant form (95%), and the rare NPC2, which represents less than 5% of the reported cases. Niemann–Pick disease type C2 usually presents with respiratory symptoms, cholestasis, neurological impairment, and hepatosplenomegaly. Case report: Here, we report a 3-year-old boy who presented to our hospital with exacerbation of chronic lung disease requiring invasive ventilatory support. He was previously diagnosed with interstitial lung disease. His parents used to instill olive oil in his nose (a few drops in each nostril daily for several months) to treat frequent nasal bleeding. A detailed history revealed prolonged neonatal jaundice for four months, with hepatosplenomegaly. In his second year, generalized hypotonia and delayed psychomotor development were observed. Upon presentation to our institute, chest CT showed evidence of intraparenchymal fat; therefore, lipoid pneumonia and lipid storage disease were suspected. The bronchoalveolar lavage results suggested pulmonary alveolar proteinosis (PAP). Whole-exome sequencing (WES) revealed a class one homozygous pathogenic variant in the NPC2 gene. Our patient faced a range of difficulties, including prolonged mechanical ventilation and diagnostic and therapeutic challenges. Conclusion: Niemann–Pick disease type C2 is a progressive and lethal condition that requires a high index of suspicion to pinpoint the diagnosis. Gene study remains the method of choice to confirm the diagnosis. There are limited choices of therapeutic interventions; therefore, genetic counseling and the prevention of recurrence should be the ultimate goal for affected families. [ABSTRACT FROM AUTHOR]

Case Presentation: A 34-year-old man presented to our institution with lightheadedness and dyspnea on exertion. His medical history included chronic pancreatitis, juvenile rheumatoid arthritis (JRA), gastroesophageal reflux disease, hypertension, lumbar degenerative disc disease, seizure disorder, anterior uveitis, and multiple vertebral fractures. In addition, he was a cigarette smoker with a 10-pack-year smoking history. [ABSTRACT FROM AUTHOR]

Background: Lysinuric protein intolerance (LPI) is a metabolic disorder that leads to dysfunctional intestinal absorption and kidney clearance of cationic amino acids. Chronic kidney disease develops in many LPI patients and leads to end-stage kidney disease in at least 10% of patients. Since data on kidney transplants in LPI patients are limited, we analysed the outcomes of LPI patients after transplantation in Finland., Methods: This retrospective cohort study includes all Finnish LPI patients who have received a kidney transplant. The data were collected from the Finnish Transplant Registry and electronic medical records from 2005 through May 2023 or patient death. The plasma amino acid profile was analysed before and after transplantation., Results: Eight LPI patients (75% female, mean age at transplant 41.9 years) received a kidney allograft and two of the patients received a second transplant. Nine transplants were from deceased donors and one was from a living donor. Acute rejection occurred after four transplantations (two T-cell mediated and two antibody mediated). One patient died 6 months after transplantation due to alveolar proteinosis. Apart from lower citrulline and higher lysine concentrations, plasma amino acid levels showed no changes after transplantation. The 1-, 5- and 10-year graft survivals were 80%, 68.6% and 51.4%, and patient survivals were 88%, 86% and 50%, respectively., Conclusions: Kidney transplantation is feasible in patients with LPI, although the acute rejection rate seems high and severe complications such as pulmonary alveolar proteinosis may occur. Transplantation led to changes in plasma citrulline and lysine concentrations., Competing Interests: I.H. has received research funding from Hansa Biopharma and MSD and has ongoing consultancy agreements with AstraZeneca, Hansa Biopharma, MSD and Sandoz. K.A. receives lecture fees from Hansa Biopharma and Astellas. F.O. has consultancy agreements with Vifor, Takeda, GSK, MSD and Stada and receives lecture fees from AstraZeneca and Astellas. M.L. receives lecture fees from Astellas and is a board member of Scandiatransplant. S.K. receives support for attending meetings and/or travel from Novo Nordisk, Amicus and Kyowa Kirin., (© The Author(s) 2024. Published by Oxford University Press on behalf of the ERA.)

Niemann–Pick disease type C (NPC) is an autosomal recessive lipid storage disorder. There are two types, NPC1, which is the predominant form (95%), and the rare NPC2, which represents less than 5% of the reported cases. Niemann–Pick disease type C2 usually presents with respiratory symptoms, cholestasis, neurological impairment, and hepatosplenomegaly. Case report: Here, we report a 3-year-old boy who presented to our hospital with exacerbation of chronic lung disease requiring invasive ventilatory support. He was previously diagnosed with interstitial lung disease. His parents used to instill olive oil in his nose (a few drops in each nostril daily for several months) to treat frequent nasal bleeding. A detailed history revealed prolonged neonatal jaundice for four months, with hepatosplenomegaly. In his second year, generalized hypotonia and delayed psychomotor development were observed. Upon presentation to our institute, chest CT showed evidence of intraparenchymal fat; therefore, lipoid pneumonia and lipid storage disease were suspected. The bronchoalveolar lavage results suggested pulmonary alveolar proteinosis (PAP). Whole-exome sequencing (WES) revealed a class one homozygous pathogenic variant in the NPC2 gene. Our patient faced a range of difficulties, including prolonged mechanical ventilation and diagnostic and therapeutic challenges. Conclusion: Niemann–Pick disease type C2 is a progressive and lethal condition that requires a high index of suspicion to pinpoint the diagnosis. Gene study remains the method of choice to confirm the diagnosis. There are limited choices of therapeutic interventions; therefore, genetic counseling and the prevention of recurrence should be the ultimate goal for affected families.

We present the case of a 71-year-old woman with severe bilateral primary alveolar proteinosis admitted for bilateral whole lung lavage (WLL) with a double-lumen endotracheal tube. She had a cardiac arrest of respiratory origin during the procedure and recovered after one minute of advanced resuscitation. A second LLP was scheduled under respiratory support with veno-venous extracorporeal membrane oxygenation (VV-ECMO). During this second WLL the patient was completely VV-ECMO-dependent, and the procedure was successfully completed. She was gradually weaned over the next 48 h. The patient was finally discharged after clinical improvement and home oxygen therapy was discontinued. WLL is the treatment of choice for severe cases of alveolar proteinosis. In rare cases the intervention may be poorly tolerated due to the degree of lung involvement. This case illustrates how VV-ECMO support is an option that may benefit this subgroup of at-risk patients., (Copyright © 2023 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.)

Lung ultrasound (LUS) has been proven to yield valuable information for lung and pleural pathology. It is well validated for assessing extravascular lung water. It can also be used to monitor stages of controlled lung de-aeration in whole lung lavage (WLL) which is the treatment for Pulmonary Alveolar Protienosis (PAP),characterized by abnormal surfactant in the alveoli affecting gas exchange .LUS can help decide the point of termination of lung flooding. A 55 year old lady with biopsy proven pulmonary alveolar proteinosis presented with respiratory failure. WLL was planned. LUS was used to study the stages of lung flooding as previously described for ARDS model.6 areas screened based on six areas that are normally examined like upper zone, mid zone and lower zone showed alveolar interstitial pattern. One lung ventilation (OLV) was done and isolation of lavage lung was confirmed which was seen as lung collapse (lung pulse) on LUS. Saline infusion resulted in increase in B lines followed by tissue like pattern with fluid bronchogram on LUS(alveolar flooding) in all the areas. During the lavage of the second lung, appearance of alveolar flooding pattern resulted in termination of saline infusion. The use of LUS in monitoring WLL reduced amount of saline used for lavage, pick up complications like pleural effusion and spillage.

We report a clinical case of a 39-year old male, without any known previous medical condition but with occupational exposure to paints and dust cement, who presented an autoimmune pulmonary alveolar proteinosis (PAP) triggered by exposure to toxic inhalation at his workplace. PAP is a rare lung disease characterized by intra-alveolar abnormal accumulation of surfactant. The presence of a crazy-paving pattern in high-resolution computed tomography scan brings the suspicion of PAP although histopathology results of bronchoalveolar lavage are always required for its final diagnosis. The autoimmune form of PAP due to toxic inhalation, such as the one here described, is rare and it is usually difficult to establish a causal relationship.

Alveolar proteinosis is a rare pulmonary disease characterized by intra-alveolar accumulation of surfactant composed of lipoproteinaceous material, related to a lack of surfactant resorption by alveolar macrophages. Crazy paving pattern is characteristic, but not specific. The multinodular forms of this affection remain exceptional.

Occupational lung disease secondary to inhalation of silica particles is variable and potentially life‐threatening. As the artificial stone industry has grown over the last two decades, the development of silicosis has been seen to accelerate and behave differently to chronic silicosis. In this case report, we present two patients who underwent lung transplantation for silicosis at the Alfred Hospital, both with predominantly artificial stone masonry exposure. We have identified the presence of both fibrotic/nodular silicosis and conspicuous alveolar proteinosis within the same lung parenchyma of both patients. We then demonstrate the radiological and histopathological correlates of disease; the first time this has been shown clearly in the literature.

Pulmonary alveolar proteinosis (PAP) – an unusual diffuse lung disease characterized by alveolar accumulation of phospholipoprotein material, with a peak incidence in third to fourth decade and male predominance is also described in children. Recorded prevalence is 0.1/100,000 individuals. Major clinicopathogenetic subtypes include autoimmune (idiopathic) associated with granulocyte–macrophage colony-stimulating factor autoantibodies, secondary form, and the congenital form (associated with surfactant gene mutations). Common presenting features include dyspnea, cough, low-grade fever, inspiratory crackles, and digital clubbing. Pulmonary function shows a restrictive ventilatory defect. X-rays show bilateral patchy to extensive consolidations, and bronchial lavage yields a milky fluid. Characteristic microscopic findings on lung biopsy include filling of terminal bronchioles and alveolar spaces by deep pink granular PAS-positive material. Whole lung lavage is the safest and most effective form of treatment. We present brief profiles of two young children identified as having PAP, along with follow-up data on one of them.

Alveolar proteinosis is a rare lung disease characterized by the accumulation of protein-lipid complexes in the alveoli due to impaired surfactant utilization by alveolar macrophages. The frequency is from 2 to 4 cases per 1 million adult population. We present an observation of pulmonary alveolar proteinosis in a patient with a history of coronavirus pneumonia.

Genetic autoinflammatory diseases are now a recognized and rapidly expanding group. The lung involvement historically associated with autoinflammatory diseases is inflammatory seritis, primarily seen in familial Mediterranean fever and other interleukin-1 mediated diseases. Over the last ten years, pulmonary involvement has been the core presentation of two autoinflammatory diseases associated with constitutive type I interferon activation, i.e. SAVI and COPA syndrome. Most patients with these diseases usually develop early progression to pulmonary fibrosis, which is responsible for high rates of morbidity and mortality. Other rare autoinflammatory diseases are associated with alveolar proteinosis, particularly when related to MARS mutations. Additionally, in adults, VEXAS is frequently associated with pulmonary involvement, albeit without prognosis effect. A molecular approach to autoinflammatory diseases enables not only the definition of biomarkers for diagnosis, but also the identification of targeted treatments. Examples include JAK inhibitors in SAVI and COPA syndrome, even though this therapy does not prevent progression to pulmonary fibrosis. Another illustrative example is the efficacy of methionine supplementation in alveolar proteinosis linked to MARS mutations. Overall, in autoinflammatory diseases the lung is now emerging as a possible affected organ. Continuing discovery of new autoinflammatory diseases is likely to uncover further pathologies involving the lung. Such advances are expected to lead to the development of novel therapeutic perspectives., (Copyright © 2023. Published by Elsevier Masson SAS.)

Lung ultrasound (LUS) has been proven to yield valuable information for lung and pleural pathology. It is well validated for assessing extravascular lung water. It can also be used to monitor stages of controlled lung de-aeration in whole lung lavage (WLL) which is the treatment for Pulmonary Alveolar Protienosis (PAP), characterized by abnormal surfactant in the alveoli affecting gas exchange .LUS can help decide the point of termination of lung flooding. A 55 year old lady with biopsy proven pulmonary alveolar proteinosis presented with respiratory failure. WLL was planned. LUS was used to study the stages of lung flooding as previously described for ARDS model.6 areas screened based on six areas that are normally examined like upper zone, mid zone and lower zone showed alveolar interstitial pattern. One lung ventilation (OLV) was done and isolation of lavage lung was confirmed which was seen as lung collapse (lung pulse) on LUS. Saline infusion resulted in increase in B lines followed by tissue like pattern with fluid bronchogram on LUS(alveolar flooding) in all the areas. During the lavage of the second lung, appearance of alveolar flooding pattern resulted in termination of saline infusion. The use of LUS in monitoring WLL reduced amount of saline used for lavage, pick up complications like pleural effusion and spillage. [ABSTRACT FROM AUTHOR]

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the deposition of extracellular lipoproteinaceous material within the air spaces. Although the diagnosis is mainly based on histopathological findings, sometimes, the diagnostic yield of transbronchial and even open lung biopsy can be unsatisfactory. The advantage with bronchoalveolar lavage (BAL) cytology is that apart from being safer for the patient, it can sample a much wider area and help in giving an early diagnosis and treatment to the patient. Herein, we present a case of PAP diagnosed on BAL fluid cytology in an elderly female. [ABSTRACT FROM AUTHOR]

Pulmonary hyalinosis is an idiopathic, typically incidental lesion of old dogs, characterized by multifocal aggregates of epithelioid and multinucleate macrophages that surround periodic acid–Schiff (PAS)-positive hyaline material in airways. Lung lesions resembling pulmonary hyalinosis were observed in 6 captive adult sugar gliders (Petaurus breviceps; 5 females and 1 male) in a retrospective review of 18 autopsied animals. Clinical signs for 3 of the sugar gliders included lethargy, tachypnea, and dyspnea. At autopsy, 5 of 6 animals had comorbid lesions that were the primary cause of death. Gross pulmonary lesions were characterized by mildly firm, discolored, vaguely nodular areas of parenchyma. Histologic examination of the lung revealed granulomatous inflammation with intracellular and extracellular amphophilic hyaline bodies within alveoli and airways. Hyaline bodies were positive for PAS and oil red O staining, blue via crystal violet staining, and displayed birefringence under polarized light, similar to findings in dogs with pulmonary hyalinosis. [ABSTRACT FROM AUTHOR]

A lavagem pulmonar é uma técnica desenvolvida na década de 60 do século xx com o intuito de remoção física de material proteináceo dos brônquios, em doentes com proteinose alveolar, levando a uma melhoria clínica e funcional. A técnica foi evoluindo e o que era inicialmente realizado com anestesia local a um segmento do pulmão é actualmente realizado sob anestesia geral, sequencialmente, a ambos os pulmões. A presente revisão descreve a técnica de lavagem pulmonar total, a sua principal indicação, a proteinose alveolar e, a propósito, relatamos alguns dados relativos à experiência do nosso serviço, onde a realizam com regularidade desde 2002.Whole lung lavage is a technique that was developed in the 1960s with the purpose of removing lipoproteinaceous material that accumulates in the bronchi of patients with alveolar proteinosis, leading to clinical and functional improvement. There has been an evolution in the technique; initially it was performed under local anesthesia to each segment of the lung and currently it is performed under general anesthesia sequentially to both lungs. This review describes the whole lung lavage, its major indication, alveolar proteinosis and some data on the experience of our hospital.

Background: Niemann-Pick disease type C (NPC) is an inherited metabolic disorder; due to defect in cellular cholesterol trafficking. It is clinically a heterogeneous disease with variable age of onset with multiple organ systems being involved. NPC1 gene is involved in 95% cases where as remaining ~5% cases are linked with NPC2 gene. Case presentation: Case-1, a 14-months-old female presented with recurrent respiratory distress, failure to thrive and hepatosplenomegaly. Lung biopsy was suggestive of alveolar proteinosis and liver biopsy confirmed foamy macrophages. Molecular analysis revealed homozygous mutation c.141C > A in exon 2 of NPC2 gene. Case-2, a 3-year-old male presented with dyspnoea and hepatomegaly noticed at 1 year of age. HRCT-scan of thoracic region showed consolidation with mediastinal lymphadenopathy. Broncho-alveolar lavage revealed moderate amount of foamy macrophages and bone marrow examination detected foam cells. Homozygous T > C transition in intron 1 of the NPC2 gene was identified. Conclusion: Our study demonstrates that NPC2 can present in early years of life with pulmonary complications like alveolar proteinosis and hepatosplenomegaly or hepatomegaly due to mutation in NPC2 gene. An early suspicion will help clinicians to clinch its diagnosis, management and genetic counselling. [ABSTRACT FROM AUTHOR]

Pulmonary alveolar proteinosis (PAP) is an orphan disease characterized by the accumulation of excess of surfactant within alveoli and bronchioles. The primary form of PAP (P-PAP; also referred to as idiopathic or autoimmune) is the most common form. It is mediated through a circulating neutralizing antibody against granulocyte-macrophage colony-stimulating factor. Secondary PAP (S-PAP) can be induced by a host of inciting agents and is far more liable to progress to terminal respiratory failure. We describe a rare case of S-PAP occurring in a renal transplant recipient due to mycophenolate and cyclosporine combination-therapy, which resolved spontaneously following withdrawal of these drugs.

Abstract A 51-year-old man with Behçet's disease complained of fever, dry cough and dyspnea during exertion. Chest CT showed ground glass opacities with interstitial septal thickening in both lungs. Bronchoalveolar lavage (BAL) revealed amorphous and lipoproteinaceous material that was periodic acid-Schiff (PAS) stain positive. Transbronchial biopsy specimen demonstrated PAS positive alveolar eosinophilic material consistent with pulmonary alveolar proteinosis. Serum anti-granulocyte-macrophage colony stimulating factor (GM-CSF) antibody was negative. Recent studies have reported anti-GMCSF not present in the the serum of patients with secondary pulmonary alveolar proteinosis (PAP) but they have not reported so in patients with idiopathic PAP. We report a case of alveolar proteinosis in the setting of Behçet's disease with spontaneous remission.

Background Very rapidly progressive 'acute silicosis' was observed prior to the 1930 International Labour Office Conference on silicosis, but its clinical significance and pathologic relationship to classic silica caused pneumoconiosis were not settled. Methods Textual analysis of the 1930 Conference proceedings identified data relevant to rapidly progressive silicosis. Standard bibliographic searches identified relevant biomedical literature dating from before and after the Conference. Results The 1930 Johannesburg Conference contained descriptions of acute silicosis, especially in the abrasive powders industry, but acute silica-related lung disease did not conform to a three-stage disease model in which tuberculosis supra-infection caused advanced disease, a model accepted at the Conference. Over following decades, additional reports appeared of rapidly progressive silicosis, unrelated to tuberculosis. Pulmonary alveolar proteinosis was identified only in 1958. Conclusions Adoption by the 1930 Johannesburg Conference of a classification scheme into which acute rapidly progressive disease unrelated to tuberculosis fitted poorly may have impeded the understanding of acute silicosis and its importance. Am. J. Ind. Med. 58:S39-S47, 2015. © 2015 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Background and objectives: Pulmonary alveolar proteinosis (PAP) is characterized by alveolar accumulation of surfactant lipoproteins. Proteasomes are involved in the nonlysosomal protein degradation. We hypothesize that enzymatically active proteasome is increased in the alveolar space of PAP. Patients and methods: 31 PAP patients (29 with primary, 2 with secondary form), 14 disease controls (10 with COPD and 4 with emphysema) and 18 healthy controls were studied. 20S Proteasome was measured by ELISA in bronchoalveolar lavage fluid (BALF) and in serum. Enzyme activity of extracellular proteasome (pkat/mg) was measured through fluorogenic substrate cleavage. Results: Proteasome concentration in BAL was higher in PAP patients than in disease controls or healthy subjects (566 ± 420 vs 53 ± 27 vs 60 ± 42 ng/ml, respectively, p < 0.0001 for both). Serum proteasome levels were higher in PAP patients than in healthy controls (825 ± 712 vs 405 ± 176 ng/ml, p = 0.018). PAP patients with active disease had higher serum levels than those who achieved remission (1317 ± 1176 vs 439 ± 422 ng/ml, p = 0.008). Proteasomal enzyme activity was increased in BAL of PAP patients (p < 0.05). Conclusions: The 20S proteasome is increased and active in BAL of patients with PAP. Extracellular proteasome may contribute to the alveolar degradation of accumulated proteins in PAP. [ABSTRACT FROM AUTHOR]

The article presents the case study of a 38-year-old patient with progressive dry cough and dyspnoea on exertion that has been present for six months. It describes the patient's condition who was a smoker and used to work as a metal processor, locksmith, and sandblaster. The results of the patient's laboratory examinations are explored which led to the diagnosis of accelerated silicosis due to inhalation of silica. The patient died while undergoing lung transplantation.

Pulmonary alveolar proteinosis is a rare cause of recurrent chest infections in children. It is characterized by intra alveolar accumulation of lipid and proteinaceous material that is periodic acid Schiff positive. Clinically is associated with respiratory difficulty and derangement of gas exchange. We report the case of a 13 years old boy who presented with recurrent chest infections for the last four years. He was treated in another hospital with antibiotics also for pulmonary tuberculosis due to the respiratory symptoms. On presentation he had dyspnea with signs of respiratory insufficiency. X-ray chest showed bilateral pulmonary involvement with diffuse infiltration of the lung parenchyma. CT scan of lungs showed diffuse alveolar shadowing. Echocardiography showed mild right ventricular hypertrophy. Thoracoscopic biopsy confirmed the diagnosis of pulmonary alveolar proteinosis. The child was treated with four sessions of whole lung saline lavage and showed significant improvements of symptoms. He was well at 6 months follow up. [ABSTRACT FROM AUTHOR]

Background and objective The gold standard for the histological diagnosis of interstitial lung diseases ( ILD) is an open lung biopsy ( OLB). Tissue samples obtained by forceps transbronchial lung biopsies ( TBB) are usually too small. We aim to evaluate the efficacy and safety of cryo- TBB for the diagnosis of ILD and to explore its role as substitute for OLB. Methods Seventy-five patients (mean age 56.2 years) with clinical and radiological features suggestive of ILD underwent cryo- TBB under moderate sedation. The diagnostic contribution on the work-up of suspected ILD was assessed. Results No major complications occurred during cryo- TBB procedures. The mean cross-sectional area of the biopsy specimen obtained was 9 mm2 with an average of 70% alveolated tissue. The most common pathological diagnoses were idiopathic nonspecific interstitial pneumonitis ( n = 22), cryptogenic organizing pneumonia ( n = 11) and usual interstitial pneumonitis ( n = 7). There were three patients of pulmonary Langerhans cell histiocytosis and one patient of pulmonary lymphangioleiomyomatosis. A definite and probable clinicopathological consensus diagnosis was possible in 70% and 28% of patients, respectively. In only 2% of patients' diagnosis could not be established. Conclusions Cryo- TBB is a safe and effective minimally invasive modality for the diagnosis of ILD. No OLB is needed in the majority of patients. [ABSTRACT FROM AUTHOR]