Traumatic asphyxia with a 'masque ecchymotique' in a 14-year-old adolescent.

Traumatic asphyxia, which is manifested by facial edema, cyanosis, subconjunctival hemorrhage, and petechiae on the upper chest and abdomen, is a very rare clinical syndrome in children. In adults, the incidence of traumatic asphyxia was reported as 1 case/18,500 accidents, but the actual incidence is not known for pediatric population. Traumatic asphyxia is a mechanical cause of hypoxia resulting from sudden compression of the thoracic-abdominal region and the valsalva maneuver is necessary for the development of this syn-drome. Here, we describe a case of traumatic asphyxia with an ecchymotic mask in a 14-year-old boy who was referred to our pediatric emergency department.

Roles of Vitamin-K-dependent Factors Protein S and GAS6 With TAM Receptors and HMGB1 in Pediatric COVID-19 Disease.

OBJECTIVE: This study was designed to evaluate serum high-mobility group box 1 (HMGB1), protein S (PS), growth arrest-specific gene 6 (GAS6), and TAM receptor (TYRO3, AXL, and MERTK) levels in children with COVID-19 disease. METHODS: A prospective case-control study was conducted in our pediatric emergency department and 57 patients with SARS-CoV-2 polymerase chain reaction (PCR) positivity, 6 patients with multisystem inflammatory syndrome in children (MIS-C), and 17 healthy children were included. Demographic data, clinical findings, laboratory and radiologic data, the need for hospitalization, and prognosis were recorded. Serum HMGB1, PS, GAS6, and TAM receptor levels were studied by enzyme-linked immunosorbent assay method. RESULTS: While SARS-CoV-2 PCR-positive patients and healthy controls were similar in terms of gender and age, GAS6 and MERTK levels were significantly lower in SARS-CoV-2 PCR-positive patients compared with healthy controls. Among SARS-CoV-2 PCR-positive patients, no difference was found in terms of serum markers in those with and without gastrointestinal or respiratory system symptoms. However, in patients with respiratory distress at admission, PS and TYRO3 levels were significantly lower. AXL levels were lower in patients diagnosed with MIS-C compared with healthy controls. Activated partial thromboplastin time was negatively correlated with HMGB1, PS, GAS6, and AXL levels. CONCLUSION: Our results suggest that such measurements may be informative and warranted in children with COVID-19 who show evidence of coagulopathy and respiratory distress. Further studies are needed to clarify the roles of these markers in diagnosis, to predict clinical severity, and to evaluate their roles in treatment approaches for COVID-19 disease.

Distribution of Viral Respiratory Pathogens During the COVID-19 Pandemic: A Single-Center Pediatric Study from Turkey.

OBJECTIVE: To evaluate the distribution and characteristics of respiratory viral pathogens and to assess the epidemiological data, clinical features, and prognoses of infected children in a pediatric emergency department during the COVID-19 pandemic. MATERIALS AND METHODS: Between September 1, 2020, and April 30, 2021, patients aged between 0 and 18 years arrived at the pediatric emergency department and were tested by nasopharyngeal/tracheal specimen polymerase chain reaction for both SARS-CoV-2 and other viral respiratory pathogens. Demographics, symptoms, laboratory and radiologic investigations, respiratory viruses detected by PCR, presence of co-infection and co-infecting viruses, need for respiratory support, hospitalization, length of hospital stay, and prognosis were recorded. RESULTS: There were 327 patients for whom PCR tests were performed and 118 (36.0%) of them had positive results for SARS-CoV-2 and/or other respiratory viruses. Rhinovirus was the most commonly detected pathogen with 74 (62.7%) cases, followed by enterovirus with 38 (32.2%) and adenovirus with 20 (16.9%) cases. There was no detection of influenza virus or respiratory syncytial. SARS-CoV-2 PCR results were positive in 14 (11.9%) cases and there was only 1 coinfection of SARS-CoV-2 occurring together with rhinovirus. For 43 (36.4%) patients, there was co-infection, and among co-infections, the most common was that of rhinovirus and enterovirus, seen in 37 (86.0%) cases. CONCLUSION: A decrease was observed in the positivity rate of respiratory viral pathogens, while no cases of influenza virus or respiratory syncytial virus were observed in our study. Circulating viruses may change due to multifactorial approaches during the COVID-19 pandemic.

Uncommon presentation of Meckel's diverticulum in a child with decompensated hypovolemic shock.

Meckel's diverticulum (MD) is the most common congenital anomaly of the gastrointestinal tract and may cause serious complications such as intestinal obstruction, gastrointestinal hemorrhage, or inflammation with/without perforation, which can present with non-spe-cific symptoms and signs. We report on the case of a 2.5-year-old boy admitted to our emergency department in poor condition, with compatible signs of decompensated hypovolemic shock. This case finally resulted in intestinal volvulus and internal hernia, a very rare combination of two complications of MD, as determined in the operating room.

Determinants of mortality and intensive care requirement in pediatric thoracoabdominal injuries.

BACKGROUND: Thoracoabdominal injuries (TAI) are an important cause of trauma-related morbidity in children. Early and correct intervention is essential to reduce mortality. We aimed to determine factors associated with mortality and the need for intensive care in TAI. METHODS: The children admitted to the pediatric emergency department of a tertiary care hospital with TAI in a 6-year-period were enrolled. Demographic data; mechanism of injuries; clinical, laboratory and imaging findings; length of hospital and intensive care unit (ICU) stay; invasive procedures and medical treatments; surgical interventions; and survival outcomes were recorded. RESULTS: The median age of the 136 children was 9 (IQR: 5-14) years and 72.8% were male. The vast majority of injuries were caused by blunt trauma (92.7%). Pulmonary contusion, pneumothorax, splenic, and liver injuries were the most common diagnoses. Motor vehicle accidents were seen in more than half of the cases (52.2%). The median length of hospital stay was 5 (IQR: 2-8) days; 21 patients were hospitalized in the ICU (15.4%). The need for intensive care was higher in patients with lower Glasgow Coma Scale (GCS) scores and lower Pediatric Trauma Scores (PTSs), in the presence of multiple injuries, pulmonary contusion, and pneumothorax (p<0.001). Mortality was seen in nine patients, eight of whom had multiple injuries. The mortality rate was higher in patients with pulmonary contusion and pneumothorax (p=0.002 and p=0.003, respectively). The PTS and GCS were found to be lower in patients who died in hospital (p<0.001). Prolongation of coagulation parameters and hyperglycemia was more common in the non-survivor group (p=0.005 and p=0.004, respectively). CONCLUSION: Although thoracoabdominal trauma is not common in childhood, it is an important part of trauma-associated mortality. Multiple injuries, pulmonary contusion, pneumothorax, lower GCS, and PTSs can be a sign of serious injuries to which physicians must be alert.

Which Device Is Favorable for Intubation Attempts of Pediatric Residents on Four Different Pediatric Airway Simulations?

OBJECTIVES: Endotracheal intubation is an essential skill for the pediatric airway management. Although direct laryngoscopy (DL) is the standard method, several videolaryngoscopes (VLs) have been recently enhanced as an alternative especially for difficult intubations. We aimed to compare McGrath MAC (McG) and Storz C-MAC PM (ST) VLs with DL in terms of intubation success rate, time to intubation (TTI), and duration of obtaining glottis view of intubation attempts performed by pediatric residents on different pediatric airway manikins. METHODS: The pediatric residents with no experience in videolaryngoscopy were included. After a brief demonstration, intubation attempts with 3 randomly handled different devices with 6 different blades were performed on a child manikin, an infant manikin, a Pierre-Robin sequence infant manikin, and a child manikin with cervical immobilization sequentially. RESULTS: Fifty pediatric residents were enrolled. The attempts of DL on child manikin and attempts of all devices on child with cervical immobilization simulation were completely successful. For the attempts on both infant manikin and Pierre-Robin sequence infant manikin, the success rate of McG was significantly lower than ST and DL (P = 0.011 and P = 0.001). In the child manikin, McG and ST had prolonged TTI compared with DL (P = 0.016 and P = 0.001). For the child with cervical immobilization simulation, TTI of DL was significantly shorter than McG and ST (P = 0.011 and P = 0.001). Time to intubation of McG was significantly longer than DL and ST for the attempts on both 2 infant manikins. The rate of Cormack-Lehane grade I glottis view was similar for the attempts on both 2 child airway simulations. For infant manikin and Pierre-Robin sequence infant simulation, the rate of Cormack-Lehane grade I of ST was higher than attempts of DL and McG. CONCLUSIONS: The attempts of pediatric residents on infant normal airway and Pierre-Robin sequence infant airway simulations resulted with enhanced glottis view by the utilization of Storz C-MAC PM, but both 2 VLs did not provide the improvement of intubation success rate and TTI compared with DL on these 4 different pediatric manikins. Further clinical studies of different VLs in different clinical courses are required for a reliable utilization in children.

High-mobility Group Box 1 Protein in Pediatric Trauma Patients With Acute Traumatic Coagulopathy or Disseminated Intravascular Coagulation.

OBJECTIVES: Trauma can induce the release of high-mobility group box 1 (HMGB1), which plays an important role in the activation of coagulation. In this study, we aimed to evaluate the role of HMGB1 in the early diagnosis of acute traumatic coagulopathy (ATC), disseminated intravascular coagulation, and clinical course. MATERIALS AND METHODS: One hundred pediatric trauma patients and 50 healthy controls were enrolled. Demographic data, physical examination results, trauma scores, International Society on Thrombosis and Hemostasis score, laboratory values, transfusion requirements, and needs for mechanical ventilation were recorded. Blood samples for HMGB1 were assessed by an enzyme-linked immunosorbent assay. RESULTS: Thirty-five patients had ATC and 3 patients had overt disseminated intravascular coagulation. In trauma patients, HMGB1 levels were statistically higher than those in the control group (P<0.001). There was a positive correlation between HMGB1 levels and D-dimer levels (r=0.589, P<0.001). ATC patients had higher plasma HMGB1 levels than those without ATC (P=0.008). High HMGB1 levels were associated with the duration of mechanical ventilation, need for intensive care unit observation, length of hospital stay, and mortality. CONCLUSION: This study showed the early increase of HMGB1 in pediatric trauma cases and demonstrated the significant association of high HMGB1 levels with the development of ATC, disseminated intravascular coagulation, trauma severity, clinical outcome, and mortality.

Is Ischemia-Modified Albumin a Reliable Marker in Accurate Diagnosis of Appendicitis in Children?

BACKGROUND: Acute appendicitis is one of the most common abdominal emergencies. Despite all improvements in diagnostic techniques, there are still ongoing problems as proper diagnosis, misdiagnosis and perforated appendicitis. The aim of this study is to demonstrate the clinical value of IMA in patients with appendicitis and to determine the accurate diagnosis of appendicitis in clinically suspected patients. METHODS: Pediatric patients with acute abdominal pain who had the Pediatric Appendicitis Score (PAS) ≥ 7 (n = 109) and a control group of 35 patients were included in this prospective case-control study. Patients were divided into two groups: patients with appendicitis (pathologically confirmed) (n = 78) and no appendicitis (n = 31). No appendicitis included observation patients and negative appendectomy. Serum samples were collected for routine laboratory parameters and IMA before surgery. RESULTS: Patients with appendicitis had significantly higher IMA levels than no appendicitis and control groups (p = 0.001 and p < 0.001; respectively). Moreover, patients with negative appendectomy had significantly lower IMA levels than patients with appendicitis (p = 0.009). IMA and PAS were used together, and in the ROC analysis, we obtained 0.81 AUC for PAS and 0.89 AUC for PAS and IMA. CONCLUSION: The current study indicated that IMA is a reliable marker for accurate diagnosis of appendicitis. The combination of IMA with PAS score has been shown to facilitate the diagnosis of appendicitis.

Viral respiratory infections diagnosed by multiplex polymerase chain reaction in pediatric patients.

Syndromic diagnosis by multiplex nucleic acid amplification tests is the most practical approach to respiratory tract infections since the symptoms are rarely agent-specific. The aim of this study was to investigate the respiratory viruses in children admitted to a university hospital with acute respiratory tract infection during the last 8 years by a multiplex polymerase chain reaction (PCR) assay. A total of 3162 respiratory samples collected from children between April 2011 and April 2018 tested by a multiplex real-time PCR assay. Two different commercial assays were used during the study period, "AusDiagnostics/Respiratory Pathogens 12 (AusDiagnostics)" used between April 2011 and December 2015, which changed to "Fast Track Diagnostics/Respiratory Pathogens 21 (Fast Track Diagnostics)" after January 2016 to cover more viruses. Nucleic acid extraction was done by EZ1 Advanced XL platform (QIAGEN). Respiratory pathogens detected in 1857 of the 3162 (58.7%) samples. The most prevalent viruses during the 8-year period were rhinovirus/enterovirus (RV/EV; 36.2%), respiratory syncytial virus (RSV; 19%), and influenza virus A/B (14.7%). Rhinovirus was the main contributor to the RV/EV group as shown by the assay used during the 2016-2018 period. RV/EV and adenoviruses detected throughout the year. Influenza virus was most frequently detected during January to March when both RSV and metapneumovirus were also in circulation. The coinfection percentage was 10.2%. Rhinovirus was the most common virus in coinfections while RSV plus rhinovirus/enterovirus were the most frequent combination. RSV and metapneumovirus showed a similar seasonal distribution to the influenza virus, which made it necessary to use a virological diagnostic assay during the influenza season.

Fecal calprotectin: can be used to distinguish between bacterial and viral gastroenteritis in children?

OBJECTIVE: Fecal calprotectin is used as a good indicator of intestinal mucosal inflammation. The aim of this study is to evaluate the diagnostic value of fecal calprotectin (f-CP) for the etiology of acute gastroenteritis in children. MATERIALS AND METHODS: All patients presenting with acute diarrhea (<18 years) who had 3 or more soft or watery stools per day were enrolled in this study. Stool microscopic examination and cultures for bacteria and parasites were performed. Polymerase chain reaction test was also applied to stool samples for viruses (Rotavirus, Adenovirus, Norwalk, and Astrovirus). The level of f-CP was carried out by using enzyme-linked immunosorbent assay test. RESULTS: Eighty-four patients with diarrhea were enrolled. The f-CP level was higher in patients with microscopic examination positive (n=17) (median with interquartile range, 1610.0 [908.8-2100] mg/L) than in patients with microscopic examination negative (n=67) (123.8 [25.0-406.3] mg/L) (P<.001). Concentrations of f-CP in patients with stool culture positive (1870.0 [822.5-2100] mg/L) were significantly elevated compared with the concentrations of the patient with virus detected in stool (95.0 [21.3-240.9] mg/L) (P<.001). In the diagnosis for bacterial acute gastroenteritis, the area under the receiver operating characteristic curve for f-CP was 0.867 (95% confidence interval, 0.763-0.971), sensitivity was 88.9%, and specificity was 76.0% if the threshold was taken as 710 mg/L. CONCLUSION: We conclude that f-CP, which is useful, valuable, noninvasive, easily and rapidly measured laboratory test along with simple microscopic examination of stool, can be used as an indicator of intestinal inflammation and to distinguish the bacterial gastroenteritis from the viral gastroenteritis.

Evaluation of neutrophil gelatinase-associated lipocalin in pediatric patients with acute rotavirus gastroenteritis and dehydration.

BACKGROUND: Dehydration caused by acute rotavirus gastroenteritis is a frequent finding in pediatric patients. The most important treatment modality in these patients is recognising and treating dehydration, electrolyte imbalance and acute kidney injury. Neutrophil gelatinase-associated lipocalin (NGAL) is used widely as a biomarker for the diagnosis of acute or chronic renal injury in numerous clinical studies. It is recognized as an early marker of acute renal failure before the elevation of routine biochemical tests such as creatinine. The aim of this study is to investigate the plasma and urine NGAL concentrations in mildly or moderately dehydrated patients with acute rotavirus gastroenteritis. MATERIAL AND METHODS: A total of 30 patients (13 girls, mean age 62.5 ± 46.2 months) with diarrhea and mild/moderate dehydration and 35 healthy controls (17 girls, mean age 81.1 ± 41.8 months) were enrolled in the study. Plasma and urine NGAL levels of the two groups were compared. RESULTS: The mean age, gender and serum creatinine levels of the patients and healthy controls were similar. The mean plasma and urine NGAL levels of the patients were significantly higher than controls (plasma: 118.6 ± 81.2 vs. 66.5 ± 11.3, p = 0.001 and urine: 17.7 ± 17.5 vs. 10.6 ± 7.9, p = 0.035, respectively). CONCLUSION: Mildly or moderately dehydrated children have higher plasma and urine NGAL levels compared to control subjects. Plasma and/or urine NGAL levels can be used for the early prediction of renal impairment in children with mild or moderate dehydration.

The contribution of genotypes at the MICA gene triplet repeat polymorphisms and MEFV mutations to amyloidosis and course of the disease in the patients with familial Mediterranean fever.

OBJECTIVE: To evaluate the effects of MEFV genotypes and the major histocompatibility complex class I chain-related gene A (MICA) triplet repeat polymorphism on the severity and clinical features of familial Mediterranean fever (FMF) and amyloidosis in a group of Turkish FMF patients. METHODS: We evaluated 105 adult FMF patients (with or without amyloidosis, 33 and 72, respectively) along with 107 healthy controls who were neither related to the patients nor had a family history of FMF or Behcet's disease. After recording the demographic and clinical data, the predominant mutations in the MEFV gene locus (M694V, M680I, V726A, M694I, and E148Q) were investigated by direct sequencing. MICA transmembrane polymorphisms in exon 5 were studied by vertical gel electrophoresis and fragment analysis of the amplicons obtained from MICA locus with appropriate primers. RESULTS: Earlier age at onset, increased frequency of attacks, arthritis attacks, erysipelas-like erythema, increased severity scores and amyloidosis were significantly more common in M694V homozygous patients compared to the patients not M694V homozygous (P = 0.005, OR 4.55; P = 0.001, OR 7.60; P = 0.003, OR 4.57; P = 0.002, OR 7.58; P = 0.004, OR 5.15 and P = 0.018, OR 3.33, respectively). We did not detect any modifying effects of MICA alleles as an independently risk factor on the amyloidosis development. However, when we examined the effects of MICA alleles on the course of the disease and development of amyloidosis in the M694V homozygous patients, A5 allele had a protective effect against the development of amyloidosis (P = 0.038, OR(adj) 0.26 with A5 and P = 0.009, OR(adj) 4.42 without A5). CONCLUSION: Though the effects of the MEFV genotypes seem clear, there are definitely other modifying factors or genes on the development of amyloidosis and on the course of the disease. For example, some MICA alleles have a protective effect on the prognostic factors in FMF.

The importance of alpha-fodrin antibodies in the diagnosis of Sjögren's syndrome.

OBJECTIVE: We wanted to determine the prevalence of IgA and IgG antibodies against alpha-fodrin in the patients with primary and secondary Sjögren's syndrome (SS) and also to compare with anti-Ro and anti-La antibodies in the diagnosis of SS. METHODS: We tested the prevalence of anti-alpha-fodrin IgA, IgG, anti-Ro, anti-La antibodies, anti-nuclear antibodies (ANA) and rheumatoid factor (RF) in naive patients with primary (n = 20) and secondary SS (n = 20) (Rheumatoid Arthritis [RA]+SS, n = 10; Systemic Lupus Erythematosus [SLE] + SS, n = 10), RA (n = 10), SLE (n = 10) and in healthy controls (n = 20). Salivary gland biopsies were performed in the patients with primary and secondary SS. RESULTS: In primary SS, anti-alpha-fodrin IgA, IgG, anti-Ro and anti-La antibodies were detected as 20, 10, 55 and 20% respectively. In RA + SS, anti-alpha-fodrin IgA was detected to be 10% and IgG was negative; however, anti-Ro antibodies and anti-La antibodies were found to be 40% and 20% respectively. In SLE + SS, anti-alpha-fodrin IgA was found to be 20% and IgG was found to be 10%, but anti-Ro and anti-La antibodies were found to be 90% and 20% respectively. Alpha-fodrin antibodies were not detected in RA, SLE and healthy controls. CONCLUSION: The detection of anti-alpha-fodrin antibodies by used ELISA does not give much contribution to the diagnosis of SS, and anti-Ro and anti-La are still useful serological markers in the diagnosis of SS.

Lack of association of tumor necrosis factor-alpha gene polymorphisms with disease susceptibility and severity in Behçet's disease.

Although it has been reported that the MHC class I molecule, HLA-B51, is a risk factor for Behçet's disease (BD), contribution of the tumor necrosis factor (TNF) genes, which are located in the vicinity of the HLA-B locus, to the genetic susceptibility for BD has yet to be elucidated. The purpose of this study was to analyze the effect of TNF-alpha promoter polymorphisms at positions -308, -238 and -376 on the susceptibility, severity and clinical features of BD. The TNF-alpha gene sequences from 107 patients with BD and 102 healthy subjects were amplified by the polymerase chain reaction. Sequence analysis of the TNF-alpha gene locus, which contains promoter polymorphisms at positions -376, -308, and -238, was performed with a DNA sequencing kit on automated sequencer. The patients were classified according to disease severity and clinical features. Serum TNF-alpha level in the study groups was measured by sandwich enzyme immunoassay. In patients with BD the frequencies of TNF-alpha -308 (19.4% vs 18.4%), -238 (3.7% vs 5.9%), and -376 (0.9% vs 2.9%) gene polymorphisms were not found to be significantly different from those in healthy subjects. The TNF-alpha gene polymorphisms did not show any association with disease severity or clinical features. Serum TNF-alpha level was significantly higher in patients with BD than in healthy controls (3.10 +/- 1.45 pg/ml vs 2.43 +/- 1.94 pg/ml, P < 0.01). Serum TNF-alpha level was not found to be significantly associated with disease severity, activity, clinical findings and TNF-alpha genotypes. The results of this study suggest that the TNF-alpha gene polymorphisms are unlikely to play an important role in the pathogenesis and severity of BD.

Life-threatening mediastinal-retroperitoneal hemorrhage in a child with moderate hemophilia A and high inhibitor titer: successful management with recombinant activated factor VII.

The authors describe an 11-year-old boy with hemophilia A and high titer inhibitor who developed a life-threatening mediastinal-retroperitoneal hemorrhage. Chest CT showed a large hematoma beginning in the retrotracheal area, filling the mediastinum, compressing the carina, and extending retroperitoneally up to the kidneys. As the surgical approach has a high mortality rate, the authors chose a more conservative approach initially and obtained excellent bleeding control with recombinant activated factor VII without the need for surgical intervention. As reported in other patients, the authors also showed a decrease in the factor III inhibitor while this patient was successfully treated with bypassing agents.

Diabetes insipidus presentation before renal and pulmonary features in a patient with Wegener's granulomatosis.

We report a case of a 47-year-old woman with Wegener's granulomatosis complicated by central diabetes insipidus. The patient had initially seronegative polyarthritis which mostly responded well to methotrexate and steroid therapy. Eight months later the patient suffered from polyuria and polydipsia. There were no abnormalities of the anterior pituitary hormones. MR images showed only loss of brightness of the posterior pituitary. Extensive evaluation of the patient revealed the presence of ANCA, in c-ANCA pattern and also PR3 positivity. Three months later findings of glomerulonephritis, as suggested by an active urine sediment and gradual proteinuria, and, finally, asymptomatic pulmonary nodules completed the clinical picture of Wegener's disease within 1 year. Renal biopsy showed crescent formation in two glomeruli, consistent with ANCA-related glomerulonephritis which showed pauci-immün depositions by direct immunofluorescence. Diabetes insipidus symptoms mostly regressed; renal and pulmonary findings completely disappeared with glucocorticoid and pulse cyclophosphamide treatment. These findings show that diabetes insipidus may rarely develop early in the disease process and ANCA positivity was directly indicative of Wegener's granulomatosis before the classic clinical signs of the disease.

The levels of serum-soluble Fas in patients with rheumatoid arthritis and systemic sclerosis.

Different defects in Fas/APO-1 interaction with its ligand or in signaling of apoptosis may contribute to autoimmune disease. The aim of this study was to examine whether elevated serum-soluble Fas (sFas) levels are associated with rheumatoid arthritis (RA) or systemic sclerosis (SSc). sFas level was assayed using a sandwich ELISA in serum from 37 patients with RA, 30 patients with SSc and 20 healthy controls. The RA patients were classified according to disease activity, anatomical joint damage, and the presence of pulmonary involvement. Presence of pulmonary fibrosis, CO diffusion capacity (DLCO) and skin score were determined in patients with SSc. Serum sFas levels were not significantly different between study groups. Serum sFas level in the active RA patients was significantly higher than in the patients with inactive disease (p < 0.05). The untreated active RA patients had significantly higher sFas level than healthy controls (p < 0.05). In RA patients, sFas level was significantly correlated with rheumatoid factor titer (p = 0.01), C-reactive protein (p < 0.05), and erythrocyte sedimentation rate (p < 0.05). The RA patients with severe joint damage had significantly higher sFas level than those with mild joint damage (p < 0.05). The untreated SSc patients had significantly higher sFas levels than the treated SSc patients and healthy controls (p < 0.01). Serum sFas level was not correlated with presence of pulmonary fibrosis, DLCO or skin score. The soluble Fas molecule may provide a useful additional marker for assessment of disease activity and severity in patients with RA.

Intracranial hemorrhage and hematuria in a neonate associated with heavy maternal smoking.

Maternal smoking during pregnancy is associated with greater rates of premature deliveries, low birth weight, perinatal morbidity and mortality, and impaired intellectual development. It also causes a three-fold greater risk for intracranial hemorrhage in neonates. To our knowledge no neonatal case with intracranial hemorrhage and hematuria related to heavy maternal smoking has been published to date. In this case report we present a neonate with intracranial hemorrhage and hematuria, which were associated with heavy maternal smoking, to emphasize the importance of heavy maternal smoking as a causal factor in neonatal bleeding.

Neutrophil apoptosis in patients with beta-thalassemia major.

Increased susceptibility to infection is reported in patients with beta-thalassemia major due to toxic effect of iron on neutrophil functions and reticuloendothelial system dysfunction. This study investigated the association between the neutrophil apoptosis and frequency of infection episodes, desferrioxamine treatment, and serum ferritin levels in patients with beta-thalassemia major. A total of 35 children diagnosed with beta-thalassemia major were enrolled. Group 1 consisted patients who were receiving desferrioxamine (DFO) and group 2 consisted of patients who did not start to receive DFO. A total of 15 healthy children were enrolled to serve as a control group. Frequency of infection episodes within a year was noted from hospital records. In all patients, the same method based on flow cytometry (annexin V labeled with FITC) was used to assess neutrophil apoptosis. Neutrophil count and percentage of apoptotic neutrophils did not differ significantly between the groups. When frequency of infection episodes among groups was evaluated, frequency of infection episodes of the patients who were receiving DFO was significantly higher than in the other groups. When correlation between neutrophil apoptosis and frequency of infection episodes, serum ferritin levels, and neutrophil count of the patients was analyzed according to groups, no significant correlation was found. The results indicate that high serum ferritin level and DFO use in patients with beta-thalassemia major do not enhance neutrophil apotosis in vivo and enhanced neutrophil apoptosis cannot be a possible cause for increased susceptibility to infections in these patients.