Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators.

INTRODUCTION: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non-eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs. METHODS: This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the 'Vertex treatment-Finder' on the Vertex® website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators. RESULTS: Among the study population (N = 1527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z-score, CF-associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, p = .010), lower median height z-scores (-0.87 vs. -0.55, p < .001), lower median body mass index z-scores (-0.65 vs. -0.50, p < .001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0-2] vs. 0 [IQR: 0-7], p = 0.001), and more non-invasive ventilation support (2.6% vs. 0.9%, p = 0.008) than patients in group 1. CONCLUSION: The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life-changing drugs to improve clinical outcomes.

Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment. METHODS: This retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment. RESULTS: In 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased. CONCLUSIONS: Patients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide.

Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data.

BACKGROUND: We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV1) decline and LT candidates without rapid FEV1 decline in the last year to identify a preventable cause in patients with such rapid FEV1 decline. METHODS: All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV1 below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV1 decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. RESULTS: Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV1 in 2017 in Group 1 and between FEV1 values in 2017 and 2018 in Group 2. CONCLUSIONS: There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral.

The evaluation of disease awareness, caregiver burden, and workday loss in caregivers of COPD patients.

BACKGROUND: Our aim is to determine the caregiver burden of chronic obstructive lung disease (COPD) patient's caregivers, and to determine whether there is a workday loss. METHODS: 252 COPD patients and their caregivers were included. Disease information of the patients were recorded and a questionnaire was applied. Socio-demographic characteristics of the caregivers were recorded and a questionnaire consisting of 24 questions including COPD disease, treatment and loss of working days, and the Zarit Scale were used. RESULTS: 128(50.8%) of the patients according to GOLD were group-D, 97(38.5%) of the patient's relatives were working, 62(24.7%) were not able to go to work for 1-14 days, and 125(57.1%) spent outside the home from 1-14 nights, because those accompanied to patients. In univariate analysis were detected modified medical research council (mMRC) (p < 0.001), CAT (p < 0.001), the number of comorbidities of patients (p = 0.027), forced expiratory volume in 1 FEV1cc (p = 0.009), FEV1% (p < 0.001), the presence of long term oxygen therapy (LTOT), and the number of comorbidities of the patient's relatives (p = 0.06) increased the care load. In multiple linear regression analysis, age (p = 0.03), COPD assessment test (CAT) score (p = 0.001), FEV1% (<0.068) and the number of comorbidities of patients (p = 0.01) and the number of comorbidities of caregivers (p = 0.003) increased the caregiving burden. DISCUSSION: In COPD increases caregiving burden. This burden is greater in symptomatic patients and when comorbidities are present. Psychosocial and legal regulations should be investigated and solutions should be produced for the caregivers of COPD patients.

The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care.

BACKGROUND: Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. METHODS: Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. RESULTS: There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). CONCLUSIONS: The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.

Evaluation of the effect of obstructive sleep apnea syndrome on retinal microvascularity by optical coherence tomography angiography.

PURPOSE: Using optical coherence tomography angiography (OCTA), we sought to determine whether there are retinal microvascular changes in patients with obstructive sleep apnea syndrome (OSAS). METHOD: The study included 56 patients diagnosed with OSAS by polysomnography [21 patients with mild OSAS (group 1), 14 with moderate OSAS (group 2) and 21 with severe OSAS (group 3)], and 26 healthy individuals as a control group (group 4). The vascular densities of the superficial capillary plexus and deep capillary plexus of the retinal segmentations, together with the foveal avascular zone (FAZ) width, were measured for all participants, using OCTA. RESULT: Compared with the control group, vascular densitiy in whole image of superficial capillary plexus were found to be significantly lower in group 2. In addition, vascular densitiy in fovea region of superficial capillary plexus was significantly lower in group 1 than the control group. In deep capillary plexus, vascular densitiy of whole image, superior hemi and nasal regions were found to be significantly lower in group 2 and group 3 compared with the control group. Parafoveal region in group 3 had significantly lower vascular densities than the controls. A significantly larger FAZ was also found in group 1 and group 3 in the deep capillary plexus, compared with the controls. CONCLUSION: We detected smaller vascular densities in both the superficial capillary plexus and deep capillary plexus and a larger deep capillary plexus FAZ in OSAS patients. Therefore, OCTA may be useful as a non-invasive method to understand the systemic effects of OSAS.

Evaluation of children and adolescent with cystic fibrosis by pancreatic elastography.

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessively inherited disease. Clinical findings vary by age of the patient, the organ systems involved, and the severity of the CFTR gene mutation. Pancreatic and liver involvement is prominent and exocrine pancreatic insufficiency is observed in the majority of patients. Point shear wave elastography (pSWE) is a non-invasive method that can quantitatively determine tissue elasticity and stiffness. In this study, the morphological evaluation of the pancreas was performed using the pSWE technique in pediatric patients diagnosed with CF. The effectiveness of this method for the early detection of pancreatic insufficiency was investigated. METHODS: Fifty-five patients with CF (24 girls, 31 boys) and 60 healthy children (29 girls, 31 boys) without any chronic diseases and who were suitable for the pSWE examination were included in the study. RESULTS: The mean value of pSWE was 1.12 ± 0.16 in the healthy group and 0.97 ± 0.16 in the patients with cystic fibrosis. There was a statistically significant difference between the two groups (P < 0.001). Significant negative correlations were found between pSWE and age (r = -0.319; P = 0.018), height (r = -0.293; P = 0.03), serum glucose (r = -0.346; P = 0.01), HbA1C (r = -0.592; P = 0.02), and duration of the disease (r = -0.806; P < 0.001). CONCLUSIONS: Investigating pancreatic elasticity and detecting pancreatic insufficiency using pSWE (a simple, inexpensive, and non-invasive method) in the early period before overt laboratory and clinical symptoms of EPI appear can contribute positively to long-term results in young patients with CF.

Serum bicarbonate level improves specificity of Berlin Sleep Questionnaire for obstructive sleep apnea.

Several questionnaires have been developed to assist the diagnostic process in obstructive sleep apnea syndrome (OSAS). Berlin Sleep Questionnaire (BSQ) represents a validated screening tool for OSAS. Totally 450 patients admitted to the Sleep Center at Dicle University Medical Faculty were included prospectively. A risk analysis was performed for presence of OSAS using the BSQ. Arterial blood gas measurements were performed including bicarbonate (HCO3) level. The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of BSQ for presence of OSAS and severe OSAS were determined. In patients with arterial HCO3 >24.94 mEq/L; sensitivity, specificity, PPV and NPV, of the BSQ were 93.04, 57.1, 98.3, and 23.5%, respectively. The addition of arterial HCO3 value increased the sensitivity of the BSQ in detecting OSAS patients. Although the cost of sleep studies is high for false positives from the BSQ plus arterial HCO3 level, this cost should be compared with the loss of work efficiency and severe healthcare costs of undiagnosed cases in the future. Therefore, finding possible OSAS cases in primary care health centers is important and adding serum HCO3 value to BSQ questionnaire may contribute to this topic.

Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis.

BACKGROUND: Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population. METHODS: The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS. RESULTS: Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS. CONCLUSION: PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.

Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.

BACKGROUND: A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs. METHODS: Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy. RESULTS: Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups). CONCLUSIONS: The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.

Cystic fibrosis in Turkey: First data from the national registry.

BACKGROUND: Cystic fibrosis (CF) care has been implemented in Turkey for a long time; however, there had been no patient registry. For this purpose, the Turkish National CF Registry was established. We present the first results of registry using data collected in 2017. METHODS: The data were collected using a data-entry software system, which was accessed from the internet. Demographic and annually recorded data consisted of 15 and 79 variables, respectively. RESULTS: There were 1170 patients registered from 23 centers; the estimated coverage rate was 30%. The median age at diagnosis was 1.7 years (median current age: 7.3 years); 51 (4.6%) patients were aged over 18 years. Among 293 patients who were under 3 years of age, 240 patients (81.9%) were diagnosed through newborn screening. Meconium ileus was detected in 65 (5.5%) patients. Genotyping was performed in 978 (87.4%) patients and 246 (25.2%) patients' mutations were unidentified. The most common mutation was deltaF508 with an allelic frequency of 28%, followed by N1303K (4.9%). The median FEV1% predicted was 86. Chronic colonization with Pseudomonas aeruginosa was seen in 245 patients. The most common complication was pseudo-Bartter syndrome in 120 patients. The median age of death was 13.5 years in a total of 15 patients. CONCLUSIONS: Low coverage rate, lack of genotyping, unidentified mutations, and missing data of lung functions are some of our greatest challenges. Including data of all centers and reducing missing data will provide more accurate data and help to improve the CF care in Turkey in the future.

Sleep disturbances in patients with lung cancer in Turkey.

INTRODUCTION: Sleep quality is known to be associated with the distressing symptoms of cancer. The purpose of this study was to analyze the impact of cancer symptoms on insomnia and the prevalence of sleep-related problems reported by the patients with lung cancer in Turkey. MATERIALS AND METHODS: Assesment of Palliative Care in Lung Cancer in Turkey (ASPECT) study, a prospective multicenter study conducted in Turkey with the participation of 26 centers and included all patients with lung cancer, was re-evaluated in terms of sleep problems, insomnia and possible association with the cancer symptoms. Demographic characteristics of patients and information about disease were recorded for each patient by physicians via face-to-face interviews, and using hospital records. Patients who have difficulty initiating or maintaining sleep (DIMS) is associated with daytime sleepiness/fatigue were diagnosed as having insomnia. Daytime sleepiness, fatigue and lung cancer symptoms were recorded and graded using the Edmonton Symptom Assessment Scale. RESULT: Among 1245 cases, 48.4% reported DIMS, 60.8% reported daytime sleepiness and 82.1% reported fatigue. The prevalence of insomnia was 44.7%. Female gender, patients with stage 3-4 disease, patients with metastases, with comorbidities, and with weight loss > 5 kg had higher rates of insomnia. Also, patients with insomnia had significantly higher rates of pain, nausea, dyspnea, and anxiety. Multivariate logistic regression analysis showed that patients with moderate to severe pain and dyspnea and severe anxiety had 2-3 times higher rates of insomnia. CONCLUSIONS: In conclusion, our results showed a clear association between sleep disturbances and cancer symptoms. Because of that, adequate symptom control is essential to maintain sleep quality in patients with lung cancer.

Alterations of the neutrophil-lymphocyte ratio during the period of stable and acute exacerbation of chronic obstructive pulmonary disease patients.

OBJECTIVES: We aimed to investigate the importance of neutrophil-lymphocyte ratio (NLR) in patients with chronic obstructive pulmonary disease (COPD) for identifying the severity of inflammation and recognition of acute exacerbation. METHODS: We retrospectively enrolled 100 patients with a diagnosis of COPD exacerbation who were admitted to our clinic. Complete blood count (CBC), measurement of C reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were determined within 2 h of hospital admission. Three months after an acute exacerbation, these measurements were obtained from the same patients during the stable period of COPD. The control group included 80 healthy subjects. NLR was calculated from CBC. RESULTS: NLR and other inflammatory markers, such as WBC, CRP and ESR were found to be significantly elevated in exacerbated COPD compared to stable COPD and control participants. There was a significant correlation of NLR with CRP (r = 0.415, P < 0.001), WBC (r = 0.304, P = 0.002) and ESR (r = 0.275, P = 0.035). For an NLR cutoff of 3.29, sensitivity for detecting exacerbation of COPD was 80.8% and specificity was 77.7% (AUC 0.894, P = 0.001). Some patients presenting with acute exacerbation of COPD and CRP, WBC or ESR levels lower than the optimal cut-off value had high NLR values. CONCLUSIONS: Elevated NLR can be used as a marker similar to CRP, WBC and ESR, in the determination of increased inflammation in acutely exacerbated COPD. NLR could be beneficial for the early detection of potential acute exacerbations in patients with COPD who have normal levels of traditional markers.

Effect of human development index parameters on tuberculosis incidence in Turkish provinces.

INTRODUCTION: A country's development level is measured with a quantitative parameter called the human development index (HDI). The present study researched the effects of HDI parameters (such as healthcare standards, income, and education level) on the incidence of tuberculosis. METHODOLOGY: HDI data of 36 provinces of Turkey and the tuberculosis surveillance data were obtained from the Ministry of Development and the Ministry of Health, respectively. The associations between the incidence of tuberculosis and other HDI parameters were analyzed. RESULTS: Higher population density (n/km2) (CI = 0.05 to 0.40) and higher relapse rate of tuberculosis (CI = 0.36 to 1.48) were identified to be independent predicting factors that increased the incidence of tuberculosis, whereas higher gross national product (CI = -0.06 to 0.00), the population that holds a green Medicare card (CI=-0.58 to -0.04), increased general practitioners per 100,000 people (CI=-0.66 to -0.01), female population (CI = -0.70 to -0.06), married population (CI = -1.34 to -0.03), were found to be significant negative predicting factors that were relevant to the incidence (protective against tuberculosis). CONCLUSIONS: Tuberculosis is a disease that is affected by multiple factors, including the components of HDI. Improvement of income level, facilitation of access to health services via health insurance, urbanization with lower population density strategy, and provision of enough general practitioners may be useful in reducing the incidence of TB' in provinces of developing countries such as Turkey.

The Protective Effects of Caffeic Acid Phenethyl Ester on Acetylsalicylic Acid-induced Lung Injury in Rats.

AIM: We aimed to investigate the protective effect of caffeic acid phenethyl ester (CAPE) on acetylsalicylic acid (ASA)-induced lung damage in rats in the present study. METHODS: A total of 40 rats were randomly divided into five groups, with eight rats in each group-group 1: control, not receiving any medication; group 2: ASA (50 mg/kg/day); group 3: ASA (50 mg/kg/day) plus CAPE (20 µg/kg/day); group 4: ASA (100 mg/kg/day); and group 5: ASA (100 mg/kg/day) plus CAPE (20 µg/kg/day). ASA and CAPE were given via orogastric gavage for 5 days. The total oxidant status (TOS), total antioxidant capacity (TAC), oxidant stress index (OSI), and paraoxonase-1 (PON-1) activity of the blood samples and lung tissues were determined. Histopathological examinations of the lung tissues were performed by using light microscopic methods. RESULTS: CAPE treatment significantly increased antioxidant PON-1 level both in the lung tissue and plasma (p < .05). Plasma antioxidant marker (TAC, PON-1) levels significantly increased and oxidant marker (TOS, OSI) levels significantly decreased in CAPE-treated rats (groups 3,5) compared to ASA given no-CAPE groups (group 2,4) (p < .05). Treatment with CAPE improved pulmonary interstitial inflammation and eosinophil accumulation due to ASA histopathologically. CONCLUSION: Eosinophil-rich inflammation and oxidative stress play important roles in ASA-induced lung toxicity, and CAPE may protect against ASA-induced lung toxicity by reduction of oxidative damage and inflammation in rats.

The Relationship of Fluid Overload as Assessed by Bioelectrical Impedance Analysis with Pulmonary Arterial Hypertension in Hemodialysis Patients.

BACKGROUND Pulmonary arterial hypertension (PAH) is common disease among hemodialysis (HD) patients and is associated with increased morbidity and mortality. However, its pathogenesis has not been completely elucidated. We aimed to evaluate the frequency of PAH in HD patients, as well as the relationship between fluid status and PAH. MATERIAL AND METHODS We enrolled 77 HD patients in this study. Multifrequency bioimpedance analysis (BIA) was used to assess fluid status. BIA was performed before and 30 min after the midweek of HD. Overhydration (OH)/extracellular water (ECW)% ratio was used as an indicator of fluid status. Fluid overload was defined as OH/ECW ≥7%. Echocardiographic examinations were performed before and after the HD. Pulmonary arterial hypertension was defined as systolic pulmonary artery pressure at rest (sPAP) higher than 35 mmHg. RESULTS PAH was found in 33.7% of the HD patients. OH/ECW and the frequency of fluid overload were significantly higher in HD patients with PAH than those without PAH, whereas serum albumin and hemoglobin levels were significantly lower. sPAP level was significantly higher in HD patients with fluid overload than in those without fluid overload after hemodialysis session. Furthermore, sPAP, OH/ECW levels, and the frequency of PAH were significantly reduced after HD. We also found a significant positive correlation between sPAP and OH/ECW. Multivariate logistic regression analysis demonstrated fluid overload to be an independent predictor of PAH after HD. CONCLUSIONS PAH is prevalent among HD patients. This study demonstrated a strong relationship between fluid overload and PAH in HD patients.

Serum Apelin 13 Levels in Patients With Pulmonary Embolism.

INTRODUCTION AND AIM: Expression and peptide immunoreactivity of apelin messenger RNA have been described in a variety of tissues, including gastrointestinal tract, adipose tissue, brain, kidney, liver, cardiovascular system, and lungs. This study aimed to investigate the possible involvement of the endogenous apelin in the pathophysiological events that occur in patients with pulmonary embolism (PE). MATERIALS AND METHODS: In total, 53 patients with PE and 35 healthy volunteers were included the study. This cross-sectional study was conducted at a tertiary care university hospital and among patients diagnosed as having PE. The control group consisted of healthy volunteers who applied to hospital for a routine checkup examination. Serum apelin 13 levels were measured in both the groups and their results were compared. RESULTS: The median ages were 57 and 53 years, and female-male ratios were 30/23 and 20/15, in the PE and control groups, respectively. The mean serum apelin 13 levels were found to be significantly higher in the PE group (76.94 ± 10.70 ng/mL) than in the control group (50.01 ± 7.13 ng/mL; P < .001). CONCLUSION: This study demonstrated that apelin 13 levels are elevated in patients with PE. These results suggest that apelin may be a novel biomarker and a potential therapeutic target in patients with acute PE in the future.

Should Low-Molecular-Weight Heparin be Preferred Over Unfractionated Heparin After Thrombolysis for Severity Pulmonary Embolism?

PURPOSE: The role of low-molecular-weight heparin (LMWH) in managing nonmassive pulmonary embolism (PE) is well known. In unstable cases, especially after thrombolytic therapy for massive PE, unfractionated heparin (UFH) is preferred for PE management. This study aimed to investigate the effectiveness and safety of LMWH after thrombolytic therapy. METHODS: A prospective, observational multicenter trial was performed in 249 patients with acute PE who required thrombolysis. Massive and submassive PEs were categorized into 2 groups depending on whether they were treated with LMWH or UFH after thrombolytic treatment. The primary end point was all-cause mortality during the first 30 days; the secondary end point included all-cause mortality, nonfatal symptomatic recurrent PEs, or nonfatal major bleeding. RESULTS: The mean age at diagnosis was 60.7 ± 15.5 years. The PE severity was massive in 186 (74.7%) patients and submassive in 63 (25.3%). The incidence of all-cause 30-day death was 8.2% and 17.3% in patients with LMWH and UFH, respectively (P = .031). Major hemorrhage occurred in 4% (n = 5) and 7.9% (n = 10) of patients and minor hemorrhage occurred in 9% (n = 11) and 13.4% (n = 17) of the cases treated with LMWH and UFH, respectively. CONCLUSION: These results suggest that LMWH treatment can be used safely in patients with PE after thrombolytic therapy.

Primary antiphospholipid syndrome associated with pneumonia: a case report of a 16-year-old male patient.

Antiphospholipid syndrome (APS) is an autoimmune disease characterised by arterial and/or venous thrombosis and/or recurrent pregnancy loss in the presence of antiphospholipid (APL) antibodies. It is evaluated as APS when it develops associated with other systemic autoimmune diseases or primary APS if there is no concomitant disorder. In this study, we present a case of a 16-year-old male patient with primary APS. The patient was admitted with presumptive diagnosis of pneumonia, but multiple pulmonary thromboembolism (PTE) was observed on computerized tomography (CT) pulmonary angiography. APL antibodies positivity and thrombocytopenia developed in our patient. The patient was evaluated as primary APS since another etiology that could explain PTE was not found. Primary APS is a rare disease in children along with adolescents, compared with APS associated with other systemic autoimmune diseases. We present here a young male patient with primary APS and PTE to contribute to the literature. The patient initially had pneumonia but later developed PTE and thrombocytopenia.

Tuberculosis congènita: presentación de un caso infrecuente / Congenital tuberculosis: presentation of a rare case

La tuberculosis congènita es una enfermedad rara, con alta tasa de mortalidad. Es considerada el resultado de la transmisión vertical de la infección desde la placenta al feto, a través de la aspiración de líquido amniótico o por vía transplacentaria a través de las venas umbilicales. El diagnóstico de la enfermedad suele ser difícil porque los signos clínicos son inespecíficos. Se presenta el caso de un lactante varón de 48 días de vida, que fue internado por pérdida de peso, fiebre, tos, hemoptisis y dificultad respiratoria durante los últimos 20 días. Había recibido antibióticos de amplio espectro durante ese lapso, sin presentar mejoría. La radiografía de tórax mostró una consolidación con una lesión cavitaria en los campos medio y superior izquierdo. Se detectó Mycobacterium tuberculosis por reacción en cadena de la polimerasa en una muestra tomada por lavado broncoalveolar y, con ese hallazgo, se diagnosticó tuberculosis congènita. Se comenzó, entonces, el tratamiento con tuberculostáticos. El paciente falleció al 13er día de tratamiento. En lactantes con pérdida de peso, fiebre, tos, hemoptisis y dificultad respiratoria, debiera considerarse la posibilidad de tuberculosis congénita.

Congenital tuberculosis is a rare disease with a high mortality rate. Congenital tuberculosis is considered the result of mother-to-child transmission from the placenta to the fetus, through the ingestion of the amniotic fluid, or via transplacental transmission through the umbilical vein. Given the non-specific clinical signs of tuberculosis, it is usually difficult to diagnose it. The case of a 48-day-old male infant hospitalized due to weight loss, fever, cough, hemoptysis, and respiratory distress for the past 20 days, is presented. In this period, he had received broad spectrum antibiotics but with no improvement. A chest x-ray showed the presence of consolidation and a cavitary lesion in the upper and middle left lung fields. Mycobacterium tuberculosis was detected by polymerase chain reaction in a bronchoalveolar lavage specimen. Congenital tuberculosis was diagnosed based on this finding; hence, a tuberculostatic regimen was started accordingly. The patient died 13 days after treatment initiation. Congenital tuberculosis should be considered in infants with weight loss, fever, cough, hemoptysis and respiratory distress.