Calculi ; Humans ; Lung Diseases ; Male ; Middle Aged ; Pulmonary Alveoli ; pathology

OBJECTIVETo report a newborn infant who died of alveolar capillary dysplasia (ACD). The literature on about 20 cases of ACD was reviewed.

METHODSA retrospective review of records of infants from Medline with a diagnosis of ACD was carried out.

RESULTSThe case was a newborn female infant who developed respiratory distress 5 hours after an uncomplicated delivery. She died at the fourth day after birth despite full ventilatory support. The lung autopsy provided a diagnosis of ACD. In the 21 infants, 7 were male and 14 were female; 19 infants were born full-term and 2 were born pre-term. The birth weight of 19 infants and Apgar score of 15 infants were normal; 16 infants developed progressing tachypnea and cyanosis within 24 hours of age, 5 developed cyanosis at 1 day to 19 days. Echocardiography demonstrated a right to left shunt in the hearts of all the 21 infants, and pulmonary hypertension in 20 infants. Twenty infants were treated with conventional mechanical ventilation, 7 infants with high-frequency oscillatory ventilation and 12 infants with extracorporeal membrane oxygenation (ECMO). Fourteen infants were also treated with inhaled nitric oxide therapy and 4 with exogenous surfactant. Diagnostic open lung biopsy was performed in 6 infants. The chest radiography showed normal findings in 3 infants, pneumothoraces in 9 infants, reticular markings, granular, patchy or diffuse opacity in lungs of 7 infants, and decreased pulmonary vascular markings in two infants. All the 21 infants died; 8 of them died within 10 days of age, 7 within 30 days of age, and one died at the age of 4 months who was the longest survivor. Fourteen infants were associated with congenital malformations, such as cardiovascular, gastrointestinal, and genitourinary systems, including one infant associated with chromosomal abnormalities, two infants of familial genetic predisposition.

CONCLUSIONSAt present, ACD is still a disease with poor prognosis, significant medical expenses and no specific treatment. When respiratory failure or persistent pulmonary hypertension (PPHN) is persistent after routine treatment in an infant, ACD should be highly suspected and conventional open-lung biopsy should be preformed to confirm the diagnosis.

Female ; Humans ; Infant, Newborn ; Male ; Persistent Fetal Circulation Syndrome ; diagnosis ; pathology ; Pulmonary Alveoli ; abnormalities ; pathology

OBJECTIVETo explore the possible mechanisms of lung necrosis by examining the effects of Streptoccus pneumoniae (S.p) on the ultrastructure of alveolar epithelial cells type Ⅱ(AEC-Ⅱ) in the lung tissues of mice and children.

METHODSThe suspended solutions of S.p strains cultured from the blood of a child with pneumococcal necrotizing pneumonia (PNP) (0.3 mL, CFU: 1×108/L) were instilled into the trachea of pathogen-free mice to prepare PNP model. The same amount of normal saline was given for the control group (10 mice). The samples (1 mm3) from the lower lobe of right lung of the mice were obtained 92 hrs later and fixed in 2.5% glutaraldehyde. Normal and abnormal lung tissues (1 mm3) were obtained while operation for the left lower lobe pulmonary cavity excision in the child with PNP. The specimens were fixed in 2.5% glutaraldehyde and stored at 4℃. A transmission electron microscope was employed for the examination of the ultrastructure of AEC-Ⅱ in the lung tissues.

RESULTSQuantitative reduction and exfoliation of microvilli in S.p-infected AEC-Ⅱ were observed in both mice and this child compared with the control. Enlarged size, enhanced evacuation and reduced density of the lamellar bodies were also presented. The number of mitochondria was obviously reduced. The nucleolus chromatin concentrated and showed an inhomogeneous distribution.

CONCLUSIONSS.p infection results in comparable damage to the ultrastructure of AEC-Ⅱ in mice and children that may represent one of the primary causes responsible for S.p-induced lung tissue necrosis.

Animals ; Child ; Epithelial Cells ; ultrastructure ; Female ; Humans ; Mice ; Pneumonia, Pneumococcal ; pathology ; Pulmonary Alveoli ; ultrastructure

Acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) is characterized by non-cardiogenic, acute and progressive respiratory failure mediated by a variety of injurious stimuli. ALI can progress to ARDS if an effective management is not taken. The mortality rate remains high due to the complex pathogenesis and ineffective management of ARDS. At present, effective treatment methods for ALI are not available and thus it is important to study the pathogenesis and early diagnosis of ALI. This article reviews some of the biomarkers associated with ALI, with a focus on early diagnosis and future studies.
Acute Lung Injury ; diagnosis ; pathology ; Biomarkers ; Cytokines ; physiology ; Early Diagnosis ; Endothelial Cells ; pathology ; Humans ; Lung ; pathology ; Pulmonary Alveoli ; pathology

BACKGROUNDHigh positive end-expiratory pressure (PEEP) and low tidal volume (VT) ventilation is thought to be a protective ventilation strategy. It is hypothesized that the stabilization of collapsible alveoli during expiration contributes to lung protection. However, this hypothesis came from analysis of indirect indices like the analysis of the pressure-volume curve of the lung. The purpose of this study was to investigate isolated healthy and injured rat lungs by means of alveolar microscopy, in which combination of PEEP and VT is beneficial with respect to alveolar stability (I-E%).

METHODSAlveolar stability was investigated in isolated, non-perfused mechanically ventilated rat lungs. Injured lungs were compared with normal lungs. For both groups three PEEP settings (5, 10, 20 cmH2O) were combined with three VT settings (6, 10, 15 ml/kg) resulting in nine PEEP-VT combinations per group. Analysis was performed by alveolar microscopy.

RESULTSIn normal lungs alveolar stability persisted in all PEEP-VT combinations (I-E% (3.2 +/- 11.0)%). There was no significant difference using different settings (P > 0.01). In contrast, alveoli in injured lungs were extremely instable at PEEP levels of 5 cmH2O (mean I-E% 100%) and 10 cmH2O (mean I-E% (30.7 +/- 16.8)%); only at a PEEP of 20 cmH2O were alveoli stabilized (mean I-E% of (0.2 +/- 9.3)%).

CONCLUSIONSIn isolated healthy lungs alveolar stability is almost unaffected by different settings of PEEP and VT. In isolated injured lungs only a high PEEP level of 20 cmH2O resulted in stabilized alveoli whereas lower PEEP levels are associated with alveolar instability.

Animals ; Female ; Lung ; pathology ; Lung Injury ; pathology ; Microscopy ; Pulmonary Alveoli ; pathology ; Rats ; Rats, Wistar ; Tidal Volume ; physiology

Pulmonary alveolar microlithiasis (PAM) is a rare disease with unknown etiology and pathogenesis. It is characterized by diffuse, innumerable, and minute calculi, called microlithiasis in the alveoli. More than half of reported cases are asymptomatic at the time of diagnosis. We describe the first case of PAM in Korea. A 19-yr-old man without respiratory symptoms presented with interstitial thickening on the chest radiograph. His chest high resolution CT scan showed diffusely scattered, ill defined tiny micronodules and interstitial thickening. Open lung biopsy confirmed the diagnosis of PAM. He was followed up for 6 months without treatment, and no progression was noticed.
Humans ; Lithiasis/*diagnosis/pathology/radiography ; Lung Diseases/*diagnosis/pathology/radiography ; Male ; Pulmonary Alveoli/*pathology/radiography ; Republic of Korea ; Young Adult

OBJECTIVETo describe the pathologic features and diagnostic algorithm of pulmonary alveolar proteinosis (PAP).

METHODSThirty-nine biopsy and postmortem cases of PAP were studied by light microscopy and histochemical staining using periodic acid-Schiff (with digestion) (PAS-D), mucicarmine (with digestion) (mucicarmine-D) and alcian blue.

RESULTSHistologically, the affected lung tissue displayed the following characteristic features: (1) alveoli and some of the small bronchioles were filled with eosinophilic and fine granular proteinaceous material with needle-like clefts; (2) proteinaceous material was seen admixed with various numbers of degenerated and sometimes exfoliated pneumocytes; (3) pneumocytes were hyperplastic; (4) alveolar capillaries and alveolar septa had become hyperemic, but pulmonary interstitial inflammation was not obvious; (5) no significant inflammation was identified in the bronchial wall; (6) compensating emphysema was noted in the surrounding lung parenchyma. Fragments of eosinophilic, finely granular proteinaceous material with needle-like clefts were also found in the bronchoalveolar lavage fluid under light microscopy. The proteinaceous material was stained red by PAS-D. The staining for mucicarmine-D was negative, while alcian blue staining was either weakly positive (faint blue staining) or negative. Pathologic examination of lung biopsies and bronchoalveolar lavage fluid thus remaines the gold standard for diagnosis of PAP.

CONCLUSIONSIdentification of homogeneous, eosinophilic, finely granular and PAS-D-positive proteinaceous material with needle-like clefts in alveolar spaces or bronchoalveolar lavage fluid is of diagnostic importance in PAP. Bronchoalveolar lavage, being a relatively safe and non-invasive procedure, can be a useful adjunct in arriving at the final conclusion.

Adult ; Bronchoalveolar Lavage ; Bronchoalveolar Lavage Fluid ; cytology ; Female ; Humans ; Lung ; pathology ; Male ; Middle Aged ; Periodic Acid-Schiff Reaction ; Pulmonary Alveolar Proteinosis ; pathology ; therapy ; Pulmonary Alveoli ; pathology

BACKGROUNDp120 catenin (p120ctn) is an adheren junction protein that regulates barrier function, but its role has not been explored in alveolar edema induced by ventilation. We measured stretch-induced cell gap formation in MLE 12 cells due to the loss of p120. We hypothesized that alveolar permeability was increased by high lung inflation associated with alveolar epithelia cell tight junctions being destroyed, which resulted from the loss of p120.

METHODSCultured MLE12 cells were subjected to being stretched or un-stretched (control) and some cells were pretreated with pp2 (c-src inhibitor). After the end of stretching for 0, 1, 2, and 4 hours, the cells were lysed, and p120 expression and c-src activation was determined by Western blotting analysis. In vivo, SD rats were taken to different tidal volumes (Vt 7 ml/kg or 40 ml/kg, PEEP = 0, respiratory rate 30-40 betas/min) for 0, 1, 2, and 4 hour and some were pretreated with pp2, and alveolar edema was calculated.

RESULTSIt was found that p120 expression was reduced and c-src activation increased in a time-dependent and strain-dependent manner due to cyclic-stretch of the alveolar epithelial cells. These changes could be reversed by inhibition of c-src. We obtained similar changes in rats when they were subjected to large tidal volumes and the alveolar edema increased more than in rats in the low Vt group. Pretreated the rats with inhibition of c-src had less pulmonary edema induced by the high tidal volume ventilation.

CONCLUSIONSCyclic stretch MLE 12 cells induced the loss of p120 and may be the same reason by high tidal volume ventilation in rats can aggravate alveolar edema. Maintenance of p120 expression may be a novel therapeutic strategy for the prevention and treatment of ventilation induced lung injury (VILI).

Animals ; Blotting, Western ; Catenins ; physiology ; Cells, Cultured ; Mice ; Pulmonary Alveoli ; pathology ; Pulmonary Edema ; pathology ; Rats ; Rats, Sprague-Dawley ; Tidal Volume ; Ventilator-Induced Lung Injury ; pathology

Animals ; Inflammation ; chemically induced ; Lung ; drug effects ; Oligodeoxyribonucleotides ; pharmacology ; Pulmonary Alveoli ; drug effects ; pathology ; Silicon Dioxide ; adverse effects

To study the difference of changes on apoptosis, necrosis and respiratory burst of the polymorphonuclear neutrophils (PMN) in endotoxemia rat model. LPS (O(55)B(5), 5 mg/kg) was injected into abdominal cavity of 20 random normal Wistar rat. 2, 4, 8 and 12 hours after injection, the changes of apoptosis, necrosis and respiratory burst of the rats between PMN from the peripheral blood and from the bronchoalveolar lavage fluid were observed using the flow cytometer. At the same time, 5 uninjected rats were taken as control. The results demonstrated that the quantity proportions of apoptosis of PMN between the peripheral blood PMN and the bronchoalveolar lavage fluid PMN in rat's endotoxemia were similar. However, comparison with the uninjected LPS rat, the necrosis of peripheral blood PMN obviously increased and the respiratory burst capacity was clearly inhibited. Contrarily, the necrosis of bronchoalveolar lavage fluid PMN obviously decreased and the respiratory burst obviously increased in the injecting LPS rat. It was concluded that the necrosis and apoptosis displayed differently between the pulmonary and peripheral blood PMNs in endotoxemia. Under state of inflammation, the surviving PMN in tissue increased and kept the activated state due to tissue injury.
Animals ; Apoptosis ; Bronchoalveolar Lavage Fluid ; cytology ; Endotoxemia ; blood ; Necrosis ; Neutrophils ; physiology ; Pulmonary Alveoli ; pathology ; Rats ; Rats, Wistar ; Respiratory Burst