Sporadic Lymphangioleiomyomatosis Disease: A Case Report.

Pulmonary Lymphangioleiomyomatosis (LAM) is a rare disease of the lung and lymphatic system that primarily affects women of childbearing age. LAM is a progressive disease with a terrible prognosis, which worsens over time and is extremely difficult to treat. In this study, we discuss the case of a 31-year-old woman with LAM who was initially misdiagnosed with leiomyoma and the way that led to a true diagnosis and effective treatment. Following a precise diagnosis based on comprehensive clinical data and particular immunohistochemical tests, sirolimus treatment was initiated, and the patient entirely responded to the treatment. This case report demonstrated that LAM is an uncommon condition that is challenging to diagnose, which causes its treatment to be delayed.

Acute Physiology and Chronic Health Evaluation II score for the assessment of mortality prediction in the intensive care unit: a single-centre study from Iran.

BACKGROUND: The Acute Physiology and Chronic Health Evaluation (APACHE) II is still commonly used as an index of illness severity in patients admitted to intensive care unit (ICU) and has been validated for many research and clinical audit purposes. AIMS AND OBJECTIVES: To investigate the diagnostic value of the APACHE II score for predicting mortality rate of critically ill patients. DESIGN: This was a single-centre, retrospective study of 200 Iranian patients admitted in the medical-surgical adult ICU from June 2012 to May 2013. METHODS: Demographic data, pre-existing comorbidities and variables required for calculating the APACHE II score were recorded. Receiver operating characteristic (ROC) curves were constructed, and the area under the ROC curves was calculated to assess the predictive value of the APACHE II score. RESULTS: Of the 200 patients with a mean age of 55·27 ± 21·59 years enrolled in the study, 112 (54%) were admitted in the medical ICU and 88 (46%) in the surgical ICU. Finally, 116 patients (58%) died, and 84 patients (42%) survived. The overall actual and predicted ICU mortality were 58% and 25·16%, respectively. The mean APACHE II score was 16·31 in total patients, 17·78 in medical ICU and 14·45 in surgical ICU patients (P = 0·003). Overall, the APACHE II score had the highest prognostic value for predicting the mortality rate of critically ill patients with an area under the cure of 0·88, and with a cut-off value of 15, the APACHE II score predicted mortality of patients with a sensitivity of 85·3%, a specificity of 77·4%, a positive predictive value of 83·9% and a negative predictive value of 73·9%. CONCLUSION: This study shows that an APACHE II score of 15 provides the best diagnostic accuracy to predict mortality of critically ill patients. Our observed mortality rate was greater than the predicted death rate, in comparison to the other prestigious centres in the world. Therefore, it appears that we must improve our intensive care to reduce mortality. RELEVANCE TO CLINICAL PRACTICE: There is a need to create a suitable scoring system to predict the mortality rate of critically ill patients in accordance with the advanced technological equipment and experienced physicians and nurses in that ICU.

The prevalence of chronic obstructive pulmonary disease in hookah smokers.

Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality worldwide. Hookah smoking is growing worldwide and particularly in Iran. The aim of this study was to determine the prevalence of obstructive pulmonary dysfunction in hookah smokers. We conducted a population-based study in Bushehr Province, Iran. A total of 245 subjects aged 35 years or older who were taking hookah for at least 15 years and 245 healthy controls were enrolled in the study and spirometry was done. Statistical analyses were performed using SPSS for windows software version 19. The prevalence of COPD among the exposed group of hookah smoke was 10.2%, with the rate being significantly higher in the patients with older age ( p < 0.001), duration of hookah smoking ( p < 0.001), men ( p = 0.026), ≥3 hookahs/day ( p = 0.006), history of cough for ≥2 years ( p = 0.002), in patients with a history of sputum for ≥2 years ( p = 0.031), and in patients with a history of dyspnea for ≥2 years ( p = 0.001). The results of the logistic regression analysis demonstrated that older age, male gender, smoking, and occupational exposure were independent predictive factors for COPD. The results of our study suggest that hookah smoking significantly increases the risk of COPD. Given the importance of COPD in the global burden of diseases, it is necessary to carry out further studies on the relationship between hookah use and COPD.

Acetylcysteine in the treatment of subacute sinusitis: A double-blind placebo-controlled clinical trial.

Sinusitis is a common disease with harmful effects on the health and finances of patients and the economy of the community. It is easily treated in most of its acute stages but is associated with some management difficulties as it goes toward chronicity. Therefore, we tried to improve the treatment of subacute sinusitis by using acetylcysteine, which is a safe mucolytic and antioxidant agent. Thirty-nine adult patients with subacute sinusitis proved by computed tomography (CT) were enrolled in a double-blind, placebo-controlled trial. They received oral amoxicillin-clavulanic acid and normal saline nasal drops for 10 days and oral pseudoephedrine for 7 days. In addition, the patients received acetylcysteine (600 mg orally, once daily) in the intervention group or placebo in the control group for 10 days. A paranasal CT scan was taken at baseline and 30 days after patients finished the treatment and was evaluated quantitatively by Lund-Mackay (LM) score. Symptoms and some aspects of quality of life also were assessed at baseline and 14 days after initiation and 30 days after termination of the treatment via the Sino-Nasal Outcome Test questionnaire. The groups showed no significant difference in LM score after treatment. A positive correlation was observed between the LM and SNOT-20 scores. We concluded that adding oral acetylcysteine to amoxicillin-clavulanic acid, pseudoephedrine, and intranasal normal saline has no benefit for the treatment of subacute sinusitis.

99mTc-IgG-Lung Scintigraphy in the Assessment of Pulmonary Involvement in Interstitial Lung Disease and Its Comparison With Pulmonary Function Tests and High-Resolution Computed Tomography: A Preliminary Study.

BACKGROUND: The discrimination of inactive inflammatory processes from the active form of the disease is of great importance in the management of interstitial lung disease (ILD). OBJECTIVES: The aim of this study was to determine the efficacy of 99mTc-IgG scan for the detection of severity of disease compared to high-resolution computed tomography (HRCT) and pulmonary function test (PFT). PATIENTS AND METHODS: Eight known cases of ILD including four cases of Mustard gas (MG) intoxication and four patients with ILD of unknown cause were included in this study. A population of six patients without lung disease was considered as the control group. The patients underwent PFT and high-resolution computed tomography, followed by 99mTc-IgG scan. They were followed up for one year. 99mTc-IgG scan assessment of IgG uptake was accomplished both qualitatively (subjectively) and semiquantitatively. RESULTS: All eight ILD patients demonstrated a strong increase in 99mTc-IgG uptake in the lungs, compared to the control patients. The 99mTc-IgG scan scores were higher in the patient group (0.64[95% confidence interval(CI)=0.61-0.69])) than the control group (0.35 (0.35[95% CI=0.28-0.40]), (P<0.05)). In patients, a statistically significant positive correlation was detected between 99mTc-IgG scan and HRCT scores (Spearman's correlation coefficient = 0.92, P < 0.008). The 99mTc-Human Immunoglobulin (HIG) scores were not significantly correlated with PFT findings (including FVC, FEV1, FEV1/FVC), O2 saturation and age ( P values > 0.05). There were no significant correlations between 99mTc-IgG score and HRCT patterns including ground glass opacity, reticular fibrosis and honeycombing (P value > 0.05). CONCLUSION: The present results confirmed that 99mTc-IgG scan could be applied to detect the severity of pulmonary involvement, which was well correlated with HRCT findings. This data also showed that the 99mTc-IgG scan might be used as a complement to HRCT in the functional evaluation of the clinical status in ILD; however, further studies are recommended.

99mTc-MIBI Lung Scintigraphy in the Assessment of Pulmonary Involvement in Interstitial Lung Disease and Its Comparison With Pulmonary Function Tests and High-Resolution Computed Tomography: A Preliminary Study.

The differentiation of active inflammatory processes from an inactive form of the disease is of great value in the management of interstitial lung disease (ILD). The aim of this investigation was to assess the efficacy of 99mTc-methoxy-isobutyl-isonitrile (99mTc-MIBI) scans in distinguishing the severity of the disease compared to radiological and clinical parameters.In total, 19 known cases of ILD were included in this study and were followed up for 1 year. Five patients without lung disease were considered as the control group. The patients underwent pulmonary function tests (PFTs) and high-resolution computed tomography scans, followed by 99mTc-MIBI scanning. The 99mTc-MIBI scans were analyzed either qualitatively (subjectively) or semiquantitatively.All 19 ILD patients demonstrated a strong increase in 99mTc-MIBI uptake in the lungs compared to the control group. The 99mTc-MIBI scan scores were higher in the patient group in both the early phase (0.24[0.19-0.31] vs 0.11[0.10-0.15], P < 0.05) and the delayed phase (0.15[0.09-0.27] vs 0.04[0.01-0.09], P < 0.05) compared with the control group. A positive correlation was detected between the 99mTc-MIBI scan and the high-resolution computed tomography (HRCT) scores (Spearman's correlation coefficient = 0.65, P < 0.02) in the early phase but not in the delayed phase in patients (P > 0.14). The 99mTc-MIBI scan scores were not significantly correlated with the PFT findings (P > 0.05). In total, 5 patients died and 14 patients were still alive over the 1-year follow-up period. There was also a significant difference between the uptake intensity of 99mTc-MIBI and the outcome in the early phase (dead: 0.32[0.29-0.43] vs alive: 0.21[0.18-0.24], P < 0.05) and delayed phase (dead: 0.27[0.22-0.28] vs alive: 0.10[0.07-0.19], P < 0.05).The washout rate was ~40 min starting from 20 min up to 60 min and this rate was significantly different in our 2 study groups (ILD: 46.61[15.61-50.39] vs NL: 70.91[27.09-116.36], P = 0.04).The present study demonstrated that 99mTc-MIBI lung scans might distinguish the severity of pulmonary involvement in early views, which were well correlated with HRCT findings. These results also revealed that 99mTc-MIBI lung scans might be used as a complement to other diagnostic and clinical examinations in terms of functional information in ILD; however, further investigations are strongly required.

Eosinophilic ascites and duodenal obstruction in a patient with liver cirrhosis.

Eosinophilic gastroenteritis (EG) is a rare disease characterized by eosinophilic infiltration of portions of the gastrointestinal tract. Eosinophilic ascites is probably the most unusual and rare presentation of EG and is generally associated with the serosal form of EG. Hereby, we report a case of eosinophilic ascites with duodenal obstruction in a patient with liver cirrhosis. A 50-year-old woman was admitted to our hospital because of abdominal pain, nausea, bloating, and constipation. She had a history of laparotomy because of duodenal obstruction 2 years ago. Based on clinical, radiological, endoscopic, and pathological findings, and given the excluding the other causes of peripheral eosinophilia, the diagnosis of eosinophilic gastroenteritis along with liver cirrhosis and spontaneous bacterial peritonitis was established. Based on the findings of the present case, it is highly recommended that, in the patients presented with liver cirrhosis associated with peripheral blood or ascitic fluid eosinophilia, performing gastrointestinal endoscopy and biopsy can probably reveal this rare disorder of EG.

The Relationship between Nocturnal Hypoxemia and Left Ventricular Ejection Fraction in Congestive Heart Failure Patients.

Congestive heart failure (CHF) is a major cause of mortality and morbidity. Among patients with heart failure, sleep disordered breathing (SDB) is a common problem. Current evidence suggests that SDB, particularly central SDB, is more prevalent in patients with CHF than in the general population, but it is underdiagnosed as SDB symptoms that are less prevalent in CHF. The main aims of this study were to determine the relationship between nocturnal hypoxemia and left ventricular ejection fraction in patients with chronic heart failure. By means of echocardiography, 108 patients with left ventricular ejection fraction ≤45% were divided into mild, moderate, and severe CHF. Hypoxemia was recorded overnight in the hospital and was measured by portable pulse oximetry. In the 108 patients with CHF, 44 (40.7%) were severe, 17 (15.7%) moderate, and 47 (43.6%) mild CHF. 95 (88%) of patients with CHF had abnormal patterns of nocturnal hypoxemia suggestive of Cheyne-Stokes respiration. Ejection fraction correlated negatively with dip frequency. There was no correlation between nocturnal hypoxemia with BMI and snoring. This study confirms strong associations between sleep apnea and heart disease in patients with CHF. Overnight oximetry is a useful screening test for Cheyne-Stokes respiration in patients with known heart failure.

99mTc-hexakis methoxy isobutyl isonitrile MIBI scintigraphy and bronchoalveolar fluid lactic dehydrogenase in pulmonary tuberculosis.

In cases of chronic pulmonary tuberculosis (PTB) with negative sputum smears, particularly when they are symptomatic, physicians encounter problems in differentiating the active from the inactive stage of the disease. This study was undertaken to determine the usefulness of technetium-99m hexakis methoxy isobutyl isonitrile ((99m)Tc-MIBI) pulmonary scintigraphy and lactic dehydrogenase (LDH) in bronchoalveolar lavage fluid (BALF), in differentiating active from inactive PTB. According to the methods we used, BALF LDH level was measured in 12 patients with documented active PTB (Group 1) before of treatment and in 7 patients with treated PTB (Group 2) after treatment. Lung scan with (99m)Tc-MIBI was performed in 7/12 patients of Group 1 (Group 1a) and in all of Group 2. Five patients of Group 1 refused the lung scan (Group 1b). Five adults who had a normal myocardial perfusion scan were considered as normal lung cases (Group 3). Our results showed that the mean LDH level in BALF was not statistically higher in Group 1 (252.42+/-189.06 mIU/ml) than in Group 2 (106.28+/-139.99 mIU/ml). Very low values, less than 24 mIu/ml, excluded active PTB. Of the 7 patients of Group 1a, 6 had a positive lung scan (85.7%). Of the 7 patients of Group 2, 6 had negative lung scan (85.7%). Both tests had a positive correlation in differentiating active and inactive PTB. In conclusion, although none of the tests were specific for PTB, low BALF LDH of less than 24 mIUL and negative (99m)Tc-MIBI pulmonary scintigraphy, seemed to indicate inactive PTB. If our findings are confirmed by others with more related cases, these tests can be shown useful in the follow up of treated PTB patients.