Polycystic liver disease presenting with an exudative pleural effusion: a case report

Introduction

Polycystic liver disease is asymptomatic in 95% of patients. In the remaining 5% it causes symptoms due to the local mass effect of the polycystic liver. We describe the case of a patient who presented with symptoms of a pleural effusion and was also found to have polycystic liver disease. The effusion recurred despite repeated efforts at drainage and only resolved following surgical debridement of the cystic liver.

Case presentation

A 50-year-old Caucasian woman presented with a two-week history of increasing dyspnoea. An examination revealed a large right pleural effusion and gross hepatomegaly. An ultrasound confirmed a large polycystic liver and diagnostic thoracocentesis revealed an exudate, which was sterile to culture. The pleural effusion proved refractory to drainage and our patient underwent surgery to deroof the main hepatic cysts in an attempt to reduce the pressure on her right diaphragm. The histology was compatible with that of polycystic liver disease. No evidence of malignancy was found. After surgery, our patient had no recurrence of her effusion and, to date, has remained asymptomatic from her polycystic liver disease.

Conclusion

The case in this report illustrates that an exudative pleural effusion is a rare complication of polycystic liver disease. We feel that the mechanical effects of a large polycystic liver, and subsequent disruption of sub-diaphragmatic capillaries, resulted in a persistent exudative pleural effusion. Thus, surgical debulking of the hepatic cysts is required to manage these effusions.     

Cytology of pleural effusion associated with disseminated infection caused by varicella-zoster virus in an immunocompromised patient. A case report

BACKGROUND: Pleural effusion caused by varicella-zoster virus (VZV) is rare. We report a case of a woman with acute lymphocytic leukemia (ALL) who developed a pleural effusion caused by VZV infection. CASE: A 55-year-old woman with ALL treated with consolidation therapy developed skin vesicles and a pleural effusion. Pleural fluid smears contained numerous mesothelial cells, which had ground-glass nuclei or eosinophilic nuclear inclusions. Some multinucleated giant cells were also seen. Electron microscopic examination revealed intranuclear virus particles, about 150 nm in diameter, in some mesothelial cells. Tissue samples from the skin, lungs, pleura, liver, pancreas, kidneys and gastrointestinal tract, obtained at autopsy, contained many virus-infected cells. They were positive for VZV glyco-protein 1 by immunohistochemistry. CONCLUSION: VZV infection should be considered in the differential diagnosis of an unexplained exudative pleural effusion, especially in immunocompromised hosts.     

Chylothorax as a possible diagnostic pitfall: a report of 2 cases with cytologic findings

BACKGROUND: Chyothorax is an uncommon medical condition. To the best of our knowledge, there have been no detailed English-language report dealing with its cytopathologic findings and diagnostic pitfalls CASES: A 12-year-old boy, hemodialysis dependent, with congenital nephrotic syndrome due to focal segmental glomerular sclerosis and a failed renal transplant, developed shortness of breath. Physical and radiologic examinations revealed a left pleural effusion. A 7-year-old boy developed shortness of breath, with a subsequent finding of a left pleural effusion. Multiple osteolytic skeletal lesions were found in this patient. Both patients underwent thoracocentesis. Cytologically, both fluids contained many relatively uniform, large lymphoid cells with high nuclear/cytoplasmic (N/C) ratio, condensed chromatin and occasional nucleoli, resembling blasts. Some nuclei were convoluted. Mitotic figures were present. Foamy macrophages were present in both cases. The differential diagnosis of these populations of cells included a lymphoproliferative disorder. However, the mature T-lymphocytic nature of the cells was confirmed by immunohistochemistry performed on cell block preparations, confirming the clinical impression of chylothorax in both cases. The first patient had chylothorax as a result of trauma due to therapeutic interventions (subclavian vein cannulation), in the second patient the chylothorax was a part of Gorham-Stout syndrome. CONCLUSION: The large T-lymphocytes that are the major cellular component of chylothorax may arouse suspicion of a lymphoproliferative disorder. Attention to the clinical history and immunophenotyping confirm the benign nature of the pleural space fluid. Also, abundant foamy macrophages can be considered a low-power clue to this diagnosis.     

Prenatal diagnosis of triploidy by transabdominal aspiration of chorionic villi

A case of triploidy identified in second trimester fetal diagnosis is presented. Cytogenetic study was undertaken in chorionic willi obtained by transabdominal placentocentesis. The diagnosis was confirmed by cytogenetic analysis of cultured amniotic fluid cells. The observation was revealed within a programme of combined ultrasound and cytogenetic prenatal monitoring, in association with maternal age. The fetus aborted at 23 weeks of pregnancy was abnormal, including congenital malformations and hypoplasia of internal organs and placenta.     

A case of nonimmune hydrops fetalis with a rare cardiac anomaly in a rhesus monkey

A case of nonimmune hydrops fetalis in a rhesus monkey was identified by ultrasound. The 68-day fetus exhibited generalized edema, pleural effusion, and mild ascites. Intrauterine fetal demise occurred between 75 and 80 days gestation. Necropsy revealed marked anasarca and a rare cardiac anomaly characterized by aortic and left atrioventricular valve atresia, hypoplasia of the ascending aorta and arch, and absence of the left ventricle.     

Merkel cell carcinoma in a malignant pleural effusion: case report

BACKGROUND: Merkel cell (neuroendocrine) carcinoma is a small round blue cell malignant neoplasm that primarily presents in the skin. The diagnosis of Merkel cell carcinoma in a pleural fluid is challenging because of the morphological similarity to many other malignant neoplasms. Immunohistochemical stains can be essential to establish the diagnosis of Merkel cell carcinoma. CASE PRESENTATION: A 77 year-old woman presented with a mass in her right buttock thought clinically to be a boil or sebaceous cyst. Upon histopathologic review including immunohistochemical analysis, a diagnosis of Merkel cell carcinoma was rendered. Wide-excision and sentinel lymph node biopsy revealed negative margins and no evidence of metastasis. Ten months later she complained of bone pain and a bone scan revealed multiple lesions. An abdominal CT scan revealed a T4 vertebral mass and local radiotherapy was administered. Two months later the patient presented with shortness of breath. A chest radiograph showed an effusion and thoracentesis was performed. The fluid was confirmed to contain metastatic Merkel cell carcinoma by cytology and immunohistochemical analysis. CONCLUSIONS: Merkel cell carcinoma is an aggressive neoplasm that can, despite careful surgical management, occasionally present as a malignant pleural effusion in a relatively short time period. Immunohistochemical analysis can aid in confirming this rare outcome.     

Cytologic diagnosis of a metastatic oligodendroglioma in a pleural effusion. A case report

BACKGROUND: Extraneural metastasis of oligodendroglioma is extremely rare and is diagnosed primarily by biopsy or autopsy and very occasionally by fine needle cytologic examination. We report a case of metastatic oligodendroglioma diagnosed by cytologic examination of a pleural effusion. Such a diagnosis has not been reported before. CASE: A 64-year-old woman developed anemia and bilateral pleural effusion 7 years after an operation for an oligodendroglioma over the left frontal lobe. Cytologic examination of the pleural effusion showed aggregates of atypical polygonal cells containing round, hyperchromatic nuclei and scanty, granular cytoplasm in Liu's and Papanicolaou stain and cell blocks. Immunohistochemical staining of the tumor cells revealed a positive reaction for antibodies to glial fibrillary acidic protein, S-100 and Olig2. Pleural biopsy confirmed the cytologic diagnosis of pleural effusion. A pathologic fracture of the right humeral and femoral bones was noted 1 month later, and the specimen also showed infiltrating oligodendroglioma cells in bone tissue. CONCLUSION: To the best of our knowledge, this is the first metastatic oligodendroglioma diagnosed by pleural cytology. Fine needle cytology can provide a reliable and rapid way to detect an extracranial metastatic oligodendroglioma in different organs.     

Dynamic and Volumetric Variables Reliably Predict Fluid Responsiveness in a Porcine Model with Pleural Effusion

Background

The ability of stroke volume variation (SVV), pulse pressure variation (PPV) and global end-diastolic volume (GEDV) for prediction of fluid responsiveness in presence of pleural effusion is unknown. The aim of the present study was to challenge the ability of SVV, PPV and GEDV to predict fluid responsiveness in a porcine model with pleural effusions.

Methods

Pigs were studied at baseline and after fluid loading with 8 ml kg⁻¹ 6% hydroxyethyl starch. After withdrawal of 8 ml kg⁻¹ blood and induction of pleural effusion up to 50 ml kg⁻¹ on either side, measurements at baseline and after fluid loading were repeated. Cardiac output, stroke volume, central venous pressure (CVP) and pulmonary occlusion pressure (PAOP) were obtained by pulmonary thermodilution, whereas GEDV was determined by transpulmonary thermodilution. SVV and PPV were monitored continuously by pulse contour analysis.

Results

Pleural effusion was associated with significant changes in lung compliance, peak airway pressure and stroke volume in both responders and non-responders. At baseline, SVV, PPV and GEDV reliably predicted fluid responsiveness (area under the curve 0.85 (p<0.001), 0.88 (p<0.001), 0.77 (p = 0.007). After induction of pleural effusion the ability of SVV, PPV and GEDV to predict fluid responsiveness was well preserved and also PAOP was predictive. Threshold values for SVV and PPV increased in presence of pleural effusion.

Conclusions

In this porcine model, bilateral pleural effusion did not affect the ability of SVV, PPV and GEDV to predict fluid responsiveness.     

Expression of laminin subunits in human fetal skeletal muscle

Summary The laminin variant of adult skeletal muscle fibres and Schwann cells is known as merosin, and is composed of M-B1-B2 chains. Blood vessels and immature fibres express the A chain in association with B1 or S, and B2. The importance of merosin has recently been shown by its absence in one form of congenital muscular dystrophy and in the mutantdy/dy mouse, and by its partial deficiency in Fukuyama congenital muscular dystrophy. We have examined the immunocytochemical localization of the M, A, B1 and B2 laminin chains in human fetal muscle from 7 to 40 weeks' gestation to ascertain their developmental expression. The B1 and B2 chains were detected on muscle fibres at 7 weeks, but only traces of the A or M chain were seen. By 21 weeks maximal levels of all four subunits were observed on all fibres. This suggests that the basement membrane is still being assembled until this stage of development. Expression of the A chain on muscle fibres was not reduced until 34 weeks and low levels persisted at birth. The concomitant expression of the M and A chains at early stages may indicate a laminin variant, in addition to merosin, that is highly expressed in fetal muscle. Merosin was seen in intramuscular nerves at 11 weeks. B1 and B2 subunits were detected in blood vessels from 7 weeks' gestation and the A chain from 11 weeks. The capillary network, however, is not fully established in fetal muscle. Merosin is therefore detected early during human fetal muscle development, and this should be taken into account when assessing aborted fetuses at risk for congenital muscular dystrophy.     

Interleukin -1beta (IL-1 beta), interleukin 6 (IL-6) and tumor necrosis factor (TNF) in plasma and pleural fluid of pneumonia, lung cancer and tuberculous pleuritis

Interleukins IL-1beta, IL-6 and TNF are increased in plasma of patients with severe infections and septic shock. Our objective was the evaluation of IL-1beta, IL-6 and TNF in plasma and exudates of pleural fluid and their contribution to the diagnosis. We studied 44 patients, 27 men and 17 women with mean age 66.81 +/- 11.75 years; 16 with pneumonia and parapneumonic effusion, 14 with primary lung cancer and pleural effusion and 14 with tuberculous pleuritis. We measured IL-1beta, IL-6 and TNF in serum and pleural fluid with ELISA. In patients with pneumonia and parapneumonic effusion the mean value of IL-1beta IL-6 and TNF in plasma was 9.05, 19.24 and 21.34 pg/ml and in pleural fluid 10.34, 32.19 and 25.30 pg/ml. In patients with lung cancer the mean values of IL-1beta, IL-6 and TNF were 5.33, 11.74 and 11.51 pg/ml and 6.70, 13.13, 20.89 pg/ml, respectively. In those with tuberculous pleuritis the respective mean values were 10.33, 49.94, 21.27 pg/ml and 14, 56.59, 23.58 pg/ml. In conclusion, IL-1beta and IL-6 were found increased in plasma and tuberculous pleural fluid, indicating an inflammatory status.     

血清和胸水中CA125在结核性和癌性胸水中的表达及临床意义

目的:探讨血清和胸水中CA125在结核性和癌性胸水中的表达及鉴别诊断意义。方法:抽选我院确诊的结核性胸水病人85例(结核组)和癌性胸水病人71例(癌症组),检测两组患者血清和胸水中CA125表达,并以胸水/血清中CA125比值10(p-CA125/s-CA12510)为临界值,观察其对癌性胸水的鉴别特异度、灵敏度及准确性。结果:癌症组胸水中CA125表达及p-CA125/s-CA125比值均显著高于结核组(P0.05);但血清中两组CA125表达比较差异无显著性(P0.05);两组胸水中,以35U/ml为临界值,两组患者阳性率92.9%(79/85)、100%(71/71)比较差异无显著性(X2=7.0718,P=0.0078)。癌症组中p-CA125/sCA125比值10的比率(84.5%VS 17.6%)明显高于结核组(X2=66.6244,P=0.0000);并以其为诊断癌性胸水的临界值,鉴别诊断特异度、灵敏度及准确性分别为82.3%、84.5%、83.3%。结论:血清和胸水中CA125表达对于鉴别结核性或者是癌性胸水的临床意义不大,但是p-CA125/s-CA125比值对于鉴别结核性和癌性胸水具有一定临床价值。     

Ontogeny of tracheal fluid, pulmonary surfactant, and plasma corticoids in the fetal lamb.

We examined fetal plasma corticoids and flow rate, electrolyte composition, and surfactant content of tracheal fluid in chronic experiments with eight fetal lambs. From 120 to 148 days of gestation the rate of fluid production was 4.5 ml/kg per h, and there was no change in mean fluid sodium (147.8 meq/1), chloride (153.1 meq/1), calcium (2.2 mg/100 ml), and pH (6.23). Tracheal fluid potassium increased from 4.3 meq/1 at 120-130 days to 8.9 meq/1 at term, while plasma sodium, chloride, calcium, pH, and potassium were constant at 146.1 meq/1, 110.0 meq/1, 12.1 mg/100 ml, 7.39, and 4.0 meq/1, respectively. Plasma corticoids were less than 1.5 mug/100 ml total (0.3 mug/100 ml free) until 130 days, when they increased rapidly to 10.5 total (3.2 free) at 148 days. Surfactant was first detected in tracheal fluid between 124 and 133 days and its secretion increased rapidly after 135 days to a value of 125 mug/kg per h at 148 days. A sudden increase in fetal plasma corticoids does not seem to be the stimulus for appearance of surfactant in the lamb, although these hormones may induce the rapid accumulation of surfactant prior to delivery.     

IL-6，IL-8 的检测在鉴别良恶性胸水中的诊断价值分析

目的:分析检测血清、胸水中白细胞介素6(IL-6)、白细胞介素8(IL-8)水平对良恶性胸水的鉴别诊断价值。方法:采用酶联免疫吸附试验(ELISA)检测32例良性胸水患者和40例恶性胸水患者血清及胸水中IL-6、IL-8水平,以及30例健康体检者血清中IL-6、IL-8水平。结果:良性胸水组及恶性胸水组血清IL-6、IL-8水平均显著高于正常对照组,差异具有统计学意义(P<0.05);与恶性胸水组比较,良性胸水组胸水及血清中IL-6、IL-8水平升高更为显著(P<0.05),良性胸水组IL-6、IL-8的胸水/血清比值也显著高于恶性胸水组,差异具有统计学意义(P<0.05)。结论:胸水及血清中IL-6、IL-8的检测对良、恶性胸水患者具有鉴别诊断意义。     

Prenatal diagnosis of de novo unbalanced translocation 8p;21q using subtelomeric probes

We report an unbalanced translocation involving chromosomes 8 and 21 in a fetus showing ultrasonographic abnormalities in the second trimester of pregnancy. A 41-year-old pregnant woman, gravida 1 para 0, was referred to the Genetics Clinic at the 16th week of gestation because of advanced maternal age and fetal pelvicaliectasis on ultrasonographic examination. Pregnancy had occurred following ICSI treatment. After genetic counseling amniocentesis was performed. Fetal karyotype analysis revealed a 46,XY,8p+ karyotype. Ultrasonographic examination was repeated at the 20th week of gestation and showed intrauterine growth retardation, ventriculomegaly, cerebellar structural abnormality and pelvicaliectasis. Chromosomes of both parents were normal. Molecular cytogenetic studies (FISH) using chromosome-specific subtelomere probes showed a terminal deletion of 8p and trisomy of the 21q subtelomeric region. Further analysis with Down Syndrome specific region probes revealed two signals. The couple decided to terminate the pregnancy. This is the first prenatally diagnosed case of unbalanced t(8p;21q) of de novo origin.     

Effect of thoracic duct drainage on hydrostatic pulmonary edema and pleural effusion in sheep

Positive end-expiratory pressure (PEEP) increases central venous pressure, which in turn impedes return of systemic and pulmonary lymph, thereby favoring formation of pulmonary edema with increased microvascular pressure. In these experiments we examined the effect of thoracic duct drainage on pulmonary edema and hydrothorax associated with PEEP and increased left atrial pressure in unanesthetized sheep. The sheep were connected via a tracheostomy to a ventilator that supplied 20 Torr PEEP. By inflation of a previously inserted intracardiac balloon, left atrial pressure was increased to 35 mmHg for 3 h. Pulmonary arterial, systemic arterial, and central venous pressure as well as thoracic duct lymph flow rate were continuously monitored, and the findings were compared with those in sheep without thoracic duct cannulation (controls). At the end of the experiment we determined the severity of pulmonary edema and the volume of pleural effusion. With PEEP and left atrial balloon insufflation, central venous and pulmonary arterial pressure were increased approximately threefold (P less than 0.05). In sheep with a thoracic duct fistula, pulmonary edema was less (extra-vascular fluid-to-blood-free dry weight ratio 4.8 +/- 1.0 vs. 6.1 +/- 1.0; P less than 0.05), and the volume of pleural effusion was reduced (2.0 +/- 2.9 vs. 11.3 +/- 9.6 ml; P less than 0.05). Our data signify that, in the presence of increased pulmonary microvascular pressure and PEEP, thoracic duct drainage reduces pulmonary edema and hydrothorax.     

Congenital high airway obstruction syndrome

Congenital high airway obstruction syndrome (CHAOS) is a very rare fetal malformation caused by obstruction of fetal airway because of laryngeal or tracheal atresia, subglottic stenosis, laryngeal cyst or laryngeal web. The prenatal diagnosis is inferred from secondary changes such as enlarged, hyperechogenic lungs, ascites and/or hydrops, flattened or everted diaphragms, dilated distal airways and mediastinal compression. There are only few cases of long-term survival described in literature. We present the case of fetus with such secondary changes diagnosed during routine ultrasound evaluation in 20 weeks' gestation. There were no other abnormalities and the kariotype was normal. In 26 weeks' gestation fetal hydrops appeared and subsequent polyhydramnios occurred in 28 weeks' gestation. The patient was planned for EXIT procedure during labor in experienced in CHAOS cases center. In 29 weeks' gestation the premature rupture of membranes and regular uterine contractions occurred and we've performed cesarean section. A multidisciplinary team of neonatologists, laryngologists and pediatric surgeons made their efforts to save the newborn, but there was complete laryngeal atresia and tracheal agenesia and immediate tracheostomy was impossible. The most important about CHAOS are early diagnosis, detailed fetal assessment and an adequate postnatal intervention for establishing fetal airways.     

Cytopathological spectrum of unusual malignant pleural effusions at a tertiary care centre in north India

BACKGROUND: Cytological examination of pleural fluid is one of the most informative laboratory procedures in the diagnosis of pleural effusions. Although tuberculosis is the commonest cause of pleural effusions in developing countries, tumours, including grade ones, can present with effusions. OBJECTIVE: The aim of the present study was to evaluate the uncommon causes of malignant pleural effusion. METHODS: A 2-year retrospective analysis of pleural fluid cytological specimens submitted to the Department of Cytopathology, PGIMER, Chandigarh between January 2003 and December 2004 was performed to retrieve unusual metastases. Out of a total of 898 samples reviewed, 710 were negative for malignancy and 24 cases were suspicious for malignancy. The remaining 164 cases were positive for malignancy, out of which 38 cases revealed malignancies other than adenocarcinoma. RESULTS: The 38 unusual malignancies metastasizing to the pleural cavity included 29 haematological malignancies (non-Hodgkin's lymphoma, acute lymphoid leukaemia, multiple myeloma and chronic myeloid leukaemia) and nine non-haematological malignancies (Ewing's sarcoma, neuroblastoma, Wilms' tumour, squamous cell carcinoma, small-cell carcinoma and malignant fibrous histiocytoma). CONCLUSION: Although metastatic adenocarcinoma was the commonest aetiology of malignant pleural effusions, a significant number of unusual causes of malignant pleural effusion were also encountered.     

Proteomic profiling of human pleural effusion using two-dimensional nano liquid chromatography tandem mass spectrometry

Pleural effusion, an accumulation of pleural fluid, contains proteins originated from plasma filtrate and, especially when tissues are damaged, parenchyma interstitial spaces of lungs and/or other organs. This study details protein profiles in human pleural effusion from 43 lung adenocarcinoma patients by a two-dimensional nano-high performance liquid chromatography electrospray ionization tandem mass spectrometry (2D nano-HPLC-ESI-MS/MS) system. The experimental results revealed the identification of 1415 unique proteins from human pleural effusion. Among these 124 proteins identified with higher confidence levels, some proteins have not been reported in plasma and may represent proteins specifically present in pleural effusion. These proteins are valuable for mass identification of differentially expressed proteins involved in proteomics database and screening biomarker to further study in human lung adenocarcinoma. The significance of the use of proteomics analysis of human pleural fluid for the search of new lung cancer marker proteins, and for their simultaneous display and analysis in patients suffering from lung disorders has been examined.     

Real-time ultrasonography for determining pregnancy status and viable fetal numbers in ewes

The reproductive tracts of 26 estrus synchronized, bred ewes were scanned with a portable 5.0 MHz real-time ultrasound unit within 1 to 6 d postbreeding. Intrarectal scanning was performed on alternate days until Days 28 to 30 and twice weekly until Days 50 of gestation. Transabdominal uterine scans were conducted twice weekly from Days 25 to 65 and continued weekly until parturition. A total of 24 ewes (92%) became pregnant. A nonpregnant ewe was recognized 100% of the time by both methods of ultrasonic screening. Correct identification of a gravid ewe as pregnant was 100% from Days 51 to 150 of gestation using transabdominal real-time ultrasonography. There was a significant association (P < 0.005) between the number of lambs born and the number of fetuses observed using transabdominal real-time ultrasonography after Day 25 of gestation. Accurate differentiation of fetal numbers by transabdominal scanning was 100.0% for ewes carrying one lamb and 97.3% for ewes carrying two lambs at Days 51 to 75 of gestation. Fetal attrition was documented in one ewe at Day 49 of gestation. Hydrops allantois was diagnosed in another ewe at 110 d of gestation. A total of 37 lambs were born to 23 ewes in the project flock. No congenital abnormalities were noted in any of the lambs. Transabdominal real-time ultrasonography is a safe, rapid, accurate and practical method for assessing pregnancy status, fetal number and fetal viability in sheep.