Pulmonary alveolar proteinosis

We report eight pediatric cases of pulmonary alveolar proteinosis (PAP) that illustrate the polymorphic nature of this disease: two cases with severe neonatal onset, three cases with progressive respiratory distress in patients under 1 year old, and three cases in older children with mild symptoms. Consanguineous parents or affected siblings were identified or suspected in four families. Three patients suffered from associated immune or blood disorders (severe combined immune deficiency, myelodysplasia). The respective roles of a macrophagic dysfunction and of an anomaly of the surfactant are discussed according to the various clinical presentations of pediatric PAP. We performed eight unilateral pulmonary lavages under endoscopy and selective ventilation for two patients under 7 kg in weight. These interventions led to progressive discontinuation of oxygen therapy in one case, and temporarily stabilized the disease for the second. Subsequent recurrence in this second patient was treated by massive lavage under extracorporeal oxygenation. A third infant was successfully transplanted with no recurrence within 3 years. Ambroxol was administered in one case. The three oldest children of our series remained asymptomatic, whereas three of the younger patients died. In the light of this experience, we propose that the treatment administered should be determined according to the age of the patient, the degree of respiratory deficiency, and the nature of any associated pathology.

Pulmonary alveolar proteinosis: experience with eight pediatric cases and a review

Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm that usually develops in the pleura and peritoneum. The head and neck region is involved in only 6% of the cases. Involvement of the parotid gland is a rare phenomenon, with only 24 cases reported in the literature. The aim of this study is to report an additional case of SFT affecting the parotid gland, and to review the literature on previously reported cases. The patient was a 42-year-old male with a 4-cm, fibro-elastic, movable, painless nodule in the inferior lobe of the parotid gland. The lesion was surgically excised and, following histopathological and immunohistochemical analysis, a diagnosis of SFT was rendered. The patient has been followed-up for ten months, with no signs of recurrence. Clinical, histopathological, immunohis
tochemical and treatment aspects of the tumor are discussed.

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Solitary fibrous tumor of the parotid gland: Case report.

The demand for accurate and reproducible phenotyping of a disease trait increases with the rising number of biobanks and genome wide association studies. Detailed analysis of histology is a powerful way of phenotyping human tissues. Nonetheless, purely visual assessment of histological slides is time-consuming and liable to sampling variation and optical illusions and thereby observer variation, and external validation may be cumbersome. Therefore, within our own biobank, computerized quantification of digitized histological slides is often preferred as a more precise and reproducible, and sometimes more sensitive approach. Relatively few free toolkits are, however, available for fully digitized microscopic slides, usually known as whole slides images. In order to comply with this need, we developed the slideToolkit as a fast method to handle large quantities of low contrast whole slides images using advanced cell detecting algorithms. The slideToolkit has been developed for modern personal computers and high-performance clusters (HPCs) and is available as an open-source project on github.com. We here illustrate the power of slideToolkit by a repeated measurement of 303 digital slides containing CD3 stained (DAB) abdominal aortic aneurysm tissue from a tissue biobank. Our workflow consists of four consecutive steps. In the first step (acquisition), whole slide images are collected and converted to TIFF files. In the second step (preparation), files are organized. The third step (tiles), creates multiple manageable tiles to count. In the fourth step (analysis), tissue is analyzed and results are stored in a data set. Using this method, two consecutive measurements of 303 slides showed an intraclass correlation of 0.99. In conclusion, slideToolkit provides a free, powerful and versatile collection of tools for automated feature analysis of whole slide images to create reproducible and meaningful phenotypic data sets.

/pdf/slidetoolkit-an-assistive-toolset-for-the-histological-a5p2b1y0zr.pdf

SlideToolkit: An Assistive Toolset for the Histological Quantification of Whole Slide Images

Histopathology is an art of analyzing and interpreting the shapes, sizes and architectural patterns of cells and tissues within a given specific clinical background and a science by which the image is placed in the context of knowledge of pathobiology, to arrive at an accurate diagnosis. To function effectively and safely, all the procedures and activities of histopathology laboratory should be evaluated and monitored accurately. In histopathology laboratory, the concept of quality control is applicable to pre-analytical, analytical and post-analytical activities. Ensuring safety of working personnel as well as environment is also highly important. Safety issues that may come up in a histopathology lab are primarily those related to potentially hazardous chemicals, biohazardous materials, accidents linked to the equipment and instrumentation employed and general risks from electrical and fire hazards. This article discusses quality management system which can ensure quality performance in histopathology laboratory. The hazards in pathology laboratories and practical safety measures aimed at controlling the dangers are also discussed with the objective of promoting safety consciousness and the practice of laboratory safety.

Quality and safety aspects in histopathology laboratory.

The concept of quality control in histopathology is relatively young and less well understood. Like in other disciplines of laboratory medicine, the concept of quality and its control is applicable to pre analytical, analytical and post analytical activities. Assessment of both precision and accuracy performances is possible by appropriate internal and external quality control and assessment schemes. This article is a review of all processes that achieve quality reporting in histopathology. There is a special focus on external quality assessment - a scheme that lacks organization on a national level in our country. Statistical data derived from a small scale external quality assurance program is also analyzed along with recommendations to organize an effective national scheme with the participation of authorized zonal centers.

Quality control in the histopathology laboratory: An overview with stress on the need for a structured national external quality assessment scheme

Declared as a pandemic by WHO on March 11, 2020, COVID-19 has brought about a dramatic change in the working of different laboratories across the country. Diagnostic laboratories testing different types of samples play a vital role in the treatment management. Irrespective of their size, each laboratory has to follow strict biosafety guidelines. Different sections of the laboratory receive samples that are variably infectious. Each sample needs to undergo a proper and well-designed processing system so that the personnel involved are not infected and also their close contacts. It takes a huge effort so as to limit the risk of exposure of the working staff during the collection, processing, reporting or dispatching of biohazard samples. Guidelines help in preventing the laboratory staff and healthcare workers from contracting the disease which has a known human to human route of transmission and high rate of mortality. A well-knit approach is the need of the hour to combat this fast spreading disease. We anticipate that the guidelines described in this article will be useful for continuing safe work practices by all the laboratories in the country.

Guidelines for various laboratory sections in view of COVID-19: Recommendations from the Indian Association of Pathologists and Microbiologists.

The last two decades have seen considerable progress in the use of digital technology in histopathology. Digital photography of microscopic slides and the use of static images gave way to robotic microscopes. These technologies had their own limitations that precluded their widespread use. Creation of whole slide scanners that can produce digitized whole slide images (WSI) and the "comparable to conventional microscope" experience opened multiple avenues for their utilization not only in specific applications such as expert consults, quality assessment programs, education and archiving, but also for routine day-to-day reporting. Industry pressures driven by consumer requirements have led to great development in image quality, speed of scanning, size of stored files, and capital cost of scanners. User-friendly software and analytical algorithms have further enhanced user experience. Challenges that need to be either accepted or overcome would include capital expense not significantly yielding a return on investment, and management of storage space. This review attempts to take the reader through the evolution of WSI scanners and to share the author's experience with WSI for routine histopathology reporting, education, and external quality assessment along with a review of available literature.

Whole slide imaging: The futurescape of histopathology

Solitary fibrous tumor presenting as a mass in the parotid gland.

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.

Jayaram N Iyengar

Papers

Quality control in the histopathology laboratory: An overview with stress on the need for a structured national external quality assessment scheme

Guidelines for various laboratory sections in view of COVID-19: Recommendations from the Indian Association of Pathologists and Microbiologists.

Whole slide imaging: The futurescape of histopathology

Solitary fibrous tumor presenting as a mass in the parotid gland.

Pulmonary alveolar proteinosis in children: An unusual presentation with significant clinical impact.