Interlobar Septa Research Articles

Alveolar Capillary Dysplasia with Misaligned Pulmonary Veins diagnosed at autopsy in association with novel FOXF1 variant

Abstract Introduction/Objective Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins (ACD/MPV) is a rare disorder characterized by abnormal development of the pulmonary vasculature resulting in intractable pulmonary hypertension in neonates. Affected newborns present with progressive hypoxemia that is nearly always fatal. ACD/MPV is typically seen in the setting of other congenital anomalies, including gastrointestinal, genitourinary, and cardiac systems. Although the pathogenesis of ACD/MPV is poorly understood, inactivating mutations in the FOXF1 gene have been implicated. We present a case of ACD/MPV with associated gastrointestinal/cardiac anomalies and a novel heterozygous mutation of FOXF1. Methods/Case Report A complete autopsy was perfomed. Rapid whole exome sequencing of whole blood was done via reference laboratory. Results (if a Case Study enter NA) A 36-week gestational age male was born via cesarean delivery following a pregnancy course complicated by polyhydramnios. The neonate developed respiratory failure requiring intubation and Extracorporeal Membranous Oxygenation (ECMO). Further clinical evaluation revealed a patent foremen ovale and ductus arteriosus with right to left shunting. After 18 days on ECMO, he was transferred to comfort care where he later died. The body was that of a small for gestational age, phenotypic male neonate without gross dysmorphia noted on external examination. Internal examination revealed duodenal atresia, a patent ductus arteriosus and foramen ovale, and heavy, edematous lungs. Histologic examination of the lungs demonstrated prominent congested veins abutting small arteries with medial hypertrophy within shared adventitial sheaths. The interlobar septa contained dilated lymphatic spaces. Airspaces showed a dense neutrophilic exudate. Rapid whole exome sequencing revealed heterozygous mutations in FOXF1 (c.182T>C, p.Ile61Thr), NOTCH1 (c.7495A>C, p.Ser2499Arg) and a hemizygous mutation in SSR4 (c.20G>T, p.Gly7Val). The FOXF1 mutation was predicted to be damaging and has not yet been reported in the literature; however, its clinical significance was interpreted as uncertain. The significance of the NOTCH1 and SSR4 mutations were considered unknown. Given these findings, a diagnosis of ACD/MPV was given as the cause of death. Conclusion This case illustrates the classic clinical, gross and histologic findings described in ACD/MPV. Additionally, a novel heterozygous mutation of FOXF1 was identified and predicted to be damaging, supporting the role of FOXF1 mutations in the pathogenesis of ACD/MPV.

Morpho-histological Study of the Nictitans Gland and Cartilage in Dogs and Cats in Sulaimani Province

This comparative study aims to assess the differences in the third eyelid gland and cartilage morphology and histology between dogs (Canis lupus familiaris) and cats (Felis catus). Twenty nictitans membranes were collected from 20 adult dogs and 20 adult cats. The nictitans membranes were dissected to examine the structure, shape, and positions of the nictitans gland and cartilage. Histologically, the samples were fixed in 10% neutral buffer formalin and then prepared for H&E stains. Generally, in both species, the cartilage consists of a long, slender body, and pigmented large dorsal and small ventral wings. The cartilage had a curving shape that resembled the eyeball's curvature and had two surfaces; palpebral and corneal or bulbar surface. At the basal end of the corneal surface, there are several lymph nodes aggregates. In the dogs, the nictitans cartilage was longer and slenderer than in the cats. The body of the cartilage was wrapped by a spherical and light pink nictitans gland in the dogs while the cats had oval nictitans gland. Microscopically, the nictitans gland in the dogs was composed of a compound tubuloacinar gland whereas; in cats, it was composed of a compound acinar gland. There was no difference histologically in the intralobular and the interlobar septa between species, the isogenous group of chondrocytes was higher in cats Vs. the dogs that showed few in number. In conclusion, the nictitans gland and cartilage were varied in their anatomical and histological structure. This should help researchers make preparations to prevent negative impacts and could help them investigate changes made more pronounced by disease conditions.

Pulmonary alveolar microlithiasis: A case report from Morocco with review of literature

Background: Pulmonary alveolar microlithiasis (PAM) is a rare infiltrative chronic lung disease characterized by deposition of spherical calcium phosphate microliths called calcospherites within the alveoli with predominance in lower and mid zones. Etiology and pathogenesis is not fully understood. Case presentation: We report a case of 58 year old female patient presenting with progressive shortness of breath and chronic bronchitis over a period of 4 years. Physical examination revealed she had signs of chronic respiratory failure. Chest radiograph showed dense micronodular opacities giving classical sandstorm appearance. High resolution computed tomography showed diffuse microcalcifications, bilateral ground glass attenuation and septal thickening with calcification along the interlobar septa and subpleural regions. Conclusion: According to our research this is the 15th case of PAM that has been declared in Morocco. Given the rarity of this entity, the purpose of this study was to increase knowledge of this disease among pulmonologists, in order to provide timely diagnosis.

ROLE OF IMAGING TECHNOLOGY AND CURRENT STATUS OF CORONAVIRUS DISEASE 2019 (COVID-19): A CRITICAL REVIEW

Lately, there was an outbreak of Coronavirus disease (COVID-19) in Wuhan, a city of province Hubei, China. The infectious and transferrable virus originated in bats, seafood and animals. COVID-19 belongs to Coronviradae family. Severe Acute Respiratory Syndrome (SARS) and Middle East Respiratory Syndrome (MERS) also belong to this family and their outbreak occurred in China and Saudi Arabia back in 2003 and 2012. The number of deaths due to COVID-19 is much greater than that of SARS and MERS. The clinical symptoms of infected individuals from virus are almost the same but diagnostically they are different from one another. Reverse transcription polymerase chain reaction (RT-PCR) laboratory technique is a standard test for evaluation and confirmation of COVID-19 disease. In early stages, imaging modalities can be used for screening purposes. X-Ray is used to identify the gross changes within the chest region while Computed Tomography Scanning (CT Scan) chest is used for detailed modification which occurs due to coronavirus. CT scan evaluates the different characteristics in the individuals infected from COVID-19 such as presence of bilateral ground glass opacities, presence of diffuse consolidation, presence of nodules with halo sign, enlarge thoracic lymph nodes, presence of thicken inter-lobar septa, presence of bronchiectasis and pleural effusion. Each lung lobe was assigned different score on the basis of lobe area involvement. A lobe having no involvement is of score 0, minimal involvement lobe score is 1, mild involvement lobe score is 2, moderate involvement lobe score is 3, and severe involvement lobe score is 4. The lobes are assessed for different degree of involvement and then are divided as none (0%), minimal (1% to 25%), mild (26% to 50%), moderate (51% to 75%) and severe 76% to above level. These all techniques are very effective and responsible for the exact location of the area involved and also show the extension of pathology that can only be possible on CT scan. CT scan not only evaluates the disease but it also provides the assessment of the disease severity. Keywords: Computed Tomography scan; Coronavirus; Real time polymerase chain reaction; SARS; SARS-CoV-2; MERS

A case of early diagnosis of pulmonary capillary hemangiomatosis in a worker with exposure to silica

BackgroundPulmonary capillary hemangiomatosis (PCH) is a progressive and refractory vascular disease in the lung. Pulmonary hypertension is frequently combined with PCH when capillary proliferation invades to nearby pulmonary vascular systems. It is difficult to differentiate PCH from other diseases such as pulmonary venoocclusive disease and pulmonary arterial hypertension that cause pulmonary hypertension as they frequently overlap.Case presentationA 29-year-old female who had worked at a bathtub factory presented with progressive exertional dyspnea for the past 2 years. Computed tomography revealed centrilobular, diffusely spreading ground-glass opacities sparing subpleural parenchyma with some cystic lesions and air-trapping in both lungs, suggesting a peculiar pattern of interstitial lung disease with airway involvement. There was not any evidence of right heart failure or pulmonary hypertension on echocardiogram, as well as radiography. Microscopic examination of the lung by thoracoscopic resection showed atypical proliferation of capillary channels within alveolar walls and interlobar septa, without invasion of large vessels.ConclusionWe experienced a pathologically diagnosed PCH in a young female complaining progressive dyspnea with prior exposure to occupational silica or organic solvent without elevated right ventricular systolic pressure (RVSP) who showed atypical pattern of radiologic findings.

Pulmonary Alveolar Microlithiasis: A Case Report with Emphasis on Imaging Findings

Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by the presence of small calculi in the alveolar space. The authors report a case of a 21-year-old man with a 2-year history of shortness of breath on exertion and dry cough. Physical examination was altered only for crackles at auscultation. Pulmonary function revealed a mild restrictive ventilatory defect and the chest radiograph demonstrated paracardiac confluence of dense micronodular infiltrate. High-resolution CT scan revealed diffuse ground glass attenuation and septal thickening, more pronounced in lower pulmonary regions, with calcifications along the interlobar septa and subpleural regions. A transbronchial lung biopsy confirmed the diagnosis of PAM.

Further studies on the kidney of the hook-lipped African rhinoceros, Diceros bicornis.

A healthy, pregnant Diceros bicornis (No. 29455), with histologically normal but relatively large kidneys containing a correspondingly large number of nephrons, died suddenly from an injury. Renal lobation was studied partly from serial transverse cuts across the kidney. The fibromuscular pelvic conduits, which are a craniocaudal bifurcation of the ureter, are associated with prominent longitudinally disposed paraconduital veins which anastomose with the interlobar veins. The arcuate veins open widely into the paraconduital veins. The latter drain into the major tributaries of the renal vein at the renal sinus. The interlobar arteries enter the parenchyma through the interlobar septa. These arteries release internal perforator branches, through the septa, which pass to the corticomedullary border, branch along that border as arcuate arteries, and release cortical branches centrifugally. All these branches give off twigs to the glomeruli. Relative renal mass of mammals is inversely proportional to their adult body mass. This is indicated by a regression line which includes rhinoceroses. D. bicornis No. 29455, accordingly, has exceptionally large kidneys. The mesonephros of the 75 mm fetus of D. bicornis has mature glomeruli and tubules. The metanephros has pelvic conduits, paraconduital veins, but, as yet, no medullary loops.

Renal morphology of the hook‐lipped african rhinoceros, Diceros bicornis, linnaeus

The kidney of Diceros bicornis has about 60 lobes, all appearing peripherally. These are separated by interlobar septa, except for small septal defects through which tubules pass. Renal capsule and interlobar septa are fibromuscular and contain small blood vessels. The kidney is about 65% cortex. It contains about 12.5 x 10(6) glomeruli, which form about 7% of the cortical mass and 4.6% of the renal mass. Diameter of a glomerular capsule is about 244 microns, there being no difference in size across the cortex in these adults. The ureter bifurcates into a cephalic and a caudal, fibromuscular, urothelial-lined conduit, into which open about 23 urothelial-lined infundibula. The common large collecting duct, or tubus maximus, of every lobe opens at the apex of its infundibulum. Two tubi may join into one infundibulum. The tubi and their terminal collecting ducts (of Bellini) are part of the inner medulla. Musculature of conduits and infundibula is largely longitudinal. The calyx may be represented by a circular muscle bundle near the apex of every infundibulum. The large intralobar veins are partly adherent to their infundibulum and calyx and receive arcuate veins via valved orifices. Most branches of the renal artery enter via the interlobar septa. Within a septum they branch again and also supply numerous perforators, which thence enter the cortex. Remaining branches of the renal artery enter cortex directly from without. A fibromuscular scaffolding lies deep to arcuate veins where they contact medulla. Where these veins contact cortical tubules; however, their walls become merely endothelium, like the walls of the interlobular veins.

Linear and Similar X-Ray Hieroglyphs

Since the discovery of the x-rays in 1895 a great deal of individual and concerted endeavors have brought about interpretation of linear shadows of x-rays of the chest which represent normal entities or pathologic manifestations. One of the best known eponyms relative to abnormal pulmonary x-ray shadows is represented in Kerley A and B lines (Kerley, P, in Shanks, S C and Kerley, P: A Textbook of X-Ray Diagnosis (2-nd ed), London, Lewis, H K, 1951). The A lines which extend from the peripheral to the central lung fields are visualized as the result of accumulation of transudate in the pulmonary interstitial spaces and of distention of lymphatics in the interlobar septa in patients with chronc pulmonary venous hypertension. Too, they may be seen in instances when interlobular lymphatics are filled with tumor cells. Kerley B lines, localized in the peripheral basal lung areas, portray thickened edematous interlobular septa together with their lymphatic channels and veins. Also, their visualization has been attributed to dense hemosiderin particles, mineral dust particles, tumor cells, sarcoid changes, and fibrosis. Another well-deserved eponym is attached to the term Fleischner’s plate-like atelectasis (Am J Roentgenol 46:610, 1941; in Rabin, C B: Roentgenology of the Chest, Springfield, Thomas C C, 1958). X-ray shadows of this category are usually 1-3 cm above the diaphragm. They may be unilateral or bilateral, single or multiple, from hair-like appearance to a diameter of 5 mm. Usually they are evanescent unless fibrotic transformation replaces atelectasis. Fleischner’s plaques may be brought about by ponopnea in connection with chest trauma, rib fracture, costochondral separation, inflammatory diseases of the pleura, chest wall, diaphragm, acute abdominal disease and neoplasia of abdominal organs. Other possible causal factors include bronchial occlusion by exudate, compression or spasm, pulmonary embolism, encapsulated pleural effusion, injury to the spinal cord, poliomyelitis, neuromuscular anomalies, such as amyotonia congenita, severe shock, and rarely myocardial infarction. Fleischner’s pertinent comment is worth remembering. “Plate atelectasis may accompany a distinct infarct, it may precede the formation or recognition of infarcts, or it may appear where no infarct is formed at all. Thus plate atelectasis may be an early or the only roentgen sign suggesting pulmonary embolism.” In some instances x-ray evidence of infarction may not be detectable until 12 hours after the event. Even then pathologic changes may be incomplete and associated with transient hemorrhage and edema. Subsequently necrosis and fibrosis may develop with a linear scar. One must be aware of short or extended arcuate lines which may signify eventration of the diaphragm or diaphragmatic hernia. Occasionally in the basal areas of the chest tenting of the diaphragm may be observed. Evanescent tenting may result from transient basal atelectasis of limited extent. Polyarcuate (scaloping or double-contour) outline of the diaphragm may be seen at the anterior and middle parts of this structure in patients with bronchial asthma, bronchitis, emphysema, and pulmonary fibrosis. It results from sequential rather than simultaneous contraction of the component muscle bundles of the diaphragm. Scimitar syndrome is observed in x-ray films as a crescent-like paracardiac shadow cast by an aberrant pulmonary vein which drains into the vena cava. At the site of the minor interlobar fissure the pleura may be visualized as a discrete line or closely set parallel short lines. Occasionally pleura separating the lingula or some of the lung segments may be observed. In pneumomediastinum the pleura is delineated by a thin cephalo-caudal line. Miniature pear-shaped shadow of the azygos vein may be observed close to the right border of the upper mediastinum in about 0.4 percent of chest x-rays.

The localization of pulmonary collapse-consolidation

Summary A method of using the interlobar septa, and the ‘outlines’ of the mediastinum, diaphragm, and chest wall as a means of localizing segmental pulmonary consolidation is described. The interference with the normal radiographic contrast of overlying bone structures caused by lower lobe consolidation is used as a means of diagnosis and localization.

Atelectatic bronchiectasis of the right middle lobe

Summary (1) Fourteen cases of atelectatic bronchiectasis of the middle lobe are described. This condition is considered to be relatively common but easily capable of being overlooked or wrongly diagnosed. (2) A description is given of the method of x-ray examination in the lordosis position. It is considered that this method provides a very simple and valuable method of investigating pathological conditions of the right middle lobe and the interlobar septa, that it deserves to be better known and more widely used.

A Study of the Roentgenologic Appearance of the Lobes of the Lung and the Interlobar Fissures

ROENTGENOGRAMS consist of superimposed shadows of a three-dimension object projected on a flat plane. The shadows obtained on the film are of two dimensions and do not give a true picture of the shape of the process or its location. To bring out the third dimension, stereo-roentgenograms are often taken. The use of postero-anterior and lateral projections gives us two films in planes at right-angles to each other, which is of great aid in studying the position and shape of the parts under consideration. The necessity of knowing the exact position and shape of a disease process is especially important in the chest. Processes occurring in the interlobar septa offer particular difficulty in interpretation on a single postero-anterior projection. Because of the oblique position of the septa, the horizontal ray projects the shadow of the septa on the roentgenogram as a broad surface, giving the impression of a diffuse area of lung involvement. Stereo-roentgenograms do not bring out the true nature or location of this process. A lateral view, in the same plane as the interlobar fissure, gives us a totally different conception as to the limits of the process. No longer should roentgenologists be satisfied to make a diagnosis of shadows of increased density occupying the upper or lower part of the chest. With the demand for information made by the chest surgeon, roentgenologists are called upon to be more accurate in locating the disease process, as well as to be more specific regarding its nature. Considerable literature has appeared concerning the roentgenologic appearance of certain disease processes, but there is no satisfactory study available as a guide to the exact appearance on the roentgenogram of the lobes of the lung and the interlobar septa. This study was undertaken to provide such a set of diagrams, showing the appearance of the lobes of the lung and interlobar septa in the postero-anterior and lateral views. The shadows obtained were subsequently compared to clinical cases of unquestioned diagnoses. We do not mean to imply that all disease processes affecting these divisions of the lung would occupy the areas designated in the diagrams: previous disease conditions of the lung may have resulted in retraction, distortion, or displacement of the region of the lung under consideration. The entire interlober septum may not be, and usually is not, involved by the disease process. Likewise, only a portion of one or more lobes may be affected. Notwithstanding such factors which may play a part in the distortion of these shadows, the general configuration of the affected parts of the lung closely simulate the diagrams experimentally produced. In a previous article one of us described the roentgenologic appearance of the potential interlobar spaces. The present study is a continuation of the subject, illustrating the affected spaces by clinical cases.