Patent ductus arteriosus management in very-low-birth-weight prematurity: a place for an early operation?

OBJECTIVES: The goal was to evaluate neonatal outcomes based on treatment strategies and time points for haemodynamically significant patent ductus arteriosus (hsPDA) in very-low-birth-weight preterm infants, with a particular focus on surgical closure. METHODS: This retrospective study included very-low-birth-weight infants born between 2014 and 2021 who received active treatment for hsPDA. Neonatal outcomes were compared between (i) primary surgical closure versus primary ibuprofen; (ii) early (<14th post-natal day) versus late primary surgical closure (≥14th post-natal day); and (iii) primary versus secondary surgical closure after ibuprofen failure. Further analysis using 1:1 propensity score matching was performed. Logistic regression was conducted to analyse the risk factors for post-ligation cardiac syndrome (PLCS) and/or acute kidney injury (AKI). RESULTS: A total of 145 infants with hsPDA underwent active treatment for closure. The in-hospital death rate and the incidence of severe bronchopulmonary dysplasia (BPD) were similar between the primary surgical closure group and the primary ibuprofen group in a 1:1 matched analysis. Severe BPD was significantly higher in the late surgical closure group than in the early primary surgical closure group with 1:1 propensity score matching (72.7% vs 40.9%, P=0.033). The secondary surgical closure group showed the mildest clinical condition; however, the probability of PLCS/AKI was highest (38.6%) compared to the early (15.2%) or the late primary surgical group (28.1%, P<0.001), especially in extremely premature infants (gestational age < 28 weeks). CONCLUSIONS: Surgical patent ductus arteriosus closure is not inferior to pharmacologic treatment. Considering the harmful effect of a prolonged patent ductus arteriosus shunt exposure, a timely decision and timely efforts should be made to minimize the risk of severe BPD and PLCS/AKI after surgical closure.

A Single-Center, Randomized, Double-Blind, and Placebo-Controlled Comparative Human Study to Verify the Functionality and Safety of the Lespedeza cuneata G. Don Extract for the Improvement of Aging Male Syndrome.

OBJECTIVE: Aging male syndrome is a clinical biochemical syndrome characterized by typical aging symptoms and serum testosterone deficiency. Although it is accompanied by various health problems, directly affects life satisfaction, and requires proper management, no clear prevention or treatment other than hormone replacement therapy is currently available for this syndrome. Here, we aimed to determine the efficacy and safety of the Lespedeza cuneata extract in the management of the aging male syndrome. METHODS: Males aged 43-70 years who provided consent for participation and had a total Aging Males' Symptom questionnaire score ≥ 37 and testosterone level ≤ 500 ng/dL were enrolled in this study. This study was conducted in a randomized, double-blind manner. Participants were randomly assigned to either the experimental or control groups and orally administered the assigned product twice a day. Efficacy was evaluated by measuring changes in Aging Males' Symptom score, Androgen Deficiency in the Aging Male questionnaire score, International Index of Erectile Function score, International Prostatic Symptom Score, blood test results, and body mass index at 8 weeks. RESULTS: After 8 weeks, the experimental group had significantly improved symptom scores compared to the control group on the Aging Males' Symptom and Androgen Deficiency in the Aging Male questionnaires. However, no significant differences in the International Index of Erectile Function score, International Prostatic Symptom Score score, blood test results, and body mass index were observed between the experimental and control groups. CONCLUSION: Lespedeza cuneata extract safely alleviates andropause symptoms without any significant side effects, suggesting its potential for the treatment of the aging male syndrome.

The prognosis of refractory hypotension and severe intraventricular hemorrhage in very low birth weight infants.

BACKGROUND: The increased survival rate among very low birth weight infants has resulted in a higher risk for developing neuro-complications such as intraventricular hemorrhage (IVH), periventricular leukomalacia (PVL), and adverse neurodevelopmental outcomes. PURPOSE: We examined refractory hypotension experienced within a week of life in association with severe IVH (grades 3-4) among very low birth weight infants (VLBWIs). METHOD: Between Jan 2014 and Dec 2017, the clinical data of 191 VLBWIs were retrospectively chart reviewed. Of a total of 191 VLBWIs, 71.2% (136/191) had IVH, and 28.7% (55/191) had severe IVH. RESULTS: The VLBWI with severe IVH group (grade 3-4) presented with a significantly lower gestational age along with higher use of postnatal hydrocortisone for refractory hypotension within a week of life. Resuscitation at delivery, pulmonary hemorrhage, neonatal seizure, and PVL were significantly more frequent in the severe IVH group (P < .05). Higher mortality occurred in the VLBWI with severe IVH group (P < .001). The multivariable logistic regression analysis consistently showed that refractory hypotension within a week of life and neonatal seizures were significantly associated with severe IVH. Those in the severe IVH and refractory hypotension groups had significantly lower composite cognitive, language, motor scores in Bayley Scales of Infant and Toddler Development III scores at corrected 18-24 months. CONCLUSION: Refractory hypotension within a week of life and seizures were consistently associated with severe IVH and developmental delay at corrected 18-24 months. VLBWI who experienced refractory hypotension within a week of life may indicate a more vulnerable clinical setting with a higher risk for developmental delay.

Predictive Value of Heat-Shock Protein Gene Expression on Severe Neonatal Hypoxic-Ischemic Encephalopathy.

This study aims to evaluate significant gene expression in severe hypoxic ischemic encephalopathy (HIE) in newborns, which can be used as a predictable measure for high-risk HIE infants. The study prospectively recruited 77 inborn near-term or term HIE newborns between January 2018 and December 2020. We measured six different genes within 6 h of life among the HIE infants and compared the gene levels between the mild- and severe-HIE groups. Among these, 64 HIE infants (83.1%) did not receive therapeutic hypothermia (TH) because they were categorized as mild HIE, and the 13 remaining (16.9%) infants were categorized as ≥ moderate-HIE group and received TH. More abnormal MRI findings, seizure, and use of anti-convulsant were more found in the ≥ moderate = HIE group along with longer mechanical ventilation days and hospitalization. Heat-shock protein 70 family 1 A (HSPA1A) and serpin family H member 1 (SERPINH1) genes, which encode heat-shock protein (HSP) 70 and 47, respectively, were significantly elevated in the ≥ moderate-HIE, seizure, and abnormal MRI groups. HSP 70 and 47 were significantly elevated in the severe-HIE group, possibly playing protective roles in inhibiting exacerbated neuroinflammation and maintaining a cellular homeostasis. At 18-24 months, ≥ moderate-HIE group manifested a significant language delay.

Risk factors associated with prolonged mechanical ventilation after surgical patent ductus arteriosus ligation in preterm infants.

BACKGROUND: One of the main pathophysiologies of a hemodynamically significant patent ductus arteriosus (hsPDA) involves pulmonary over-circulation. However, PDA treatment does not present with uniform effects on pulmonary outcomes. We aimed to evaluate the clinical characteristics - in particular, respiratory parameters - associated with prolonged mechanical ventilation after PDA ligation. METHODS: Preterm infants ≤32 weeks gestation were included in the study. Infants who underwent PDA ligation were grouped depending on whether the infant successfully was extubated ≤14 d after ligation or required prolonged invasive mechanical ventilation >14 d after ligation. The clinical characteristics, including the parameters concerning the respiratory illness severity and hemodynamical significance of PDA shunt, were compared between the two groups. RESULTS: Among 172 preterm infants, 36 (20.9%) infants underwent surgical PDA ligation. Fifteen (41.6%) infants were successfully extubated at ≤14 d after ligation, and 21 (58.3%) infants required prolonged invasive mechanical ventilation for >14 d after ligation. In the univariable analysis, the infants who required prolonged mechanical ventilation was significantly smaller in terms of gestational age (GA) and birth weight and tended to present a greater respiratory illness severity [represented by the use of high-frequency oscillatory ventilation (HFOV) and greater RSS (respiratory severity score)/kg] with a larger PDA size prior to PDA ligation. In the multivariable logistic regression analysis, peak preoperative RSS/kg (p = 0.012, OR = 0.207, 95% CI = 0.060-0.706) was the only significant factor associated with prolonged mechanical ventilation after PDA ligation. CONCLUSION: Preterm infants with the compromised respiratory condition may be prone to prolonged mechanical ventilation after PDA ligation. The respiratory status during the early phase of life should be considered when evaluating the effect of PDA treatment. With an extended view, distinguishing infants with such risk factors may lead to more polished treatment strategies toward hsPDA.

Compositional Differences of Meconium Microbiomes of Preterm and Term Infants, and Infants That Developed Necrotizing Enterocolitis or Feeding Intolerance.

The primary aim of this study was to investigate the compositional differences of the first passed meconium microbiome in preterm and term infants, and the secondary aim was to compare the meconium microbiomes of preterm and term infants that later developed necrotizing enterocolitis (NEC)/Feeding intolerance (FI) compared to those that did not develop NEC/FI. During the study period, a total of 108 preterm and term newborns' first passed meconium occurring within 72 hours of birth were collected and microbiome analyzed. Meconium microbiomes showed a disruption in the percentages of the core microbiome constituents in both the phylum and genus levels in infants born < 30 weeks of gestational age (GA) compared to those born ≥ 30 weeks of GA. In the phylum level, Bacteroidetes and Firmicutes, and in the genus level, Prevotella and Bacteroides, were predominant, with Prevotella accounting for 20−30% of the relative abundance. As GA increased, a significant increase in the relative abundance of Bacteroidetes (P for trend < 0.001) and decrease in Proteobacteria (P for trend = 0.049) was observed in the phylum level. In the genus level, as GA increased, Prevotella (P for trend < 0.001) and Bacteroides (P for trend = 0.002) increased significantly, whereas Enterococcus (P for trend = 0.020) decreased. Compared to the control group, the meconium of infants that later developed NEC/FI had significantly lower alpha diversities but similar beta-diversities. Furthermore, the NEC/FI group showed a significantly lower abundance of Bacteroidetes (P < 0.001), and higher abundance of Firmicutes (P = 0.034). To conclude, differences were observed in the composition of the first passed meconium in preterm and term infants that later develop NEC/FI compared to those that did not.

Clinical seizures and unfavorable brain MRI patterns in neonates with hypoxic ischemic encephalopathy.

ABSTRACT: The aim was to examine whether clinical seizures and amplitude-integrated electroencephalogram (aEEG) patterns in infants with hypoxic ischemic encephalopathy (HIE) can predict the extent of brain injury on magnetic resonance images (MRI) and the long-term neurodevelopmental outcomes at 18∼24 months of age.HIE infants who underwent therapeutic hypothermia (TH) between June 2014 and March 2017 were included in this study. Infants with clinical seizure were analyzed for aEEG patterns and the extent of brain injury on MRI findings. Clinical seizure, aEEG, and brain MRI were assessed and compared with neurodevelopmental outcomes at 18∼24 months of age.Among the 97 HIE infants enrolled in this study with brain MRI scans, 78 (73.1%) TH-treated HIE infants exhibited clinical seizures. More abnormalities on a EEGs and more significant use of first and second antiepileptic drugs (AEDs) were significantly higher in the clinical-seizure group with longer hospitalized days. At a corrected 18 to 24 months of age, HIE infants in the clinical-seizure group with more extension of injury lesions on diffusion-weighted MRI scans exhibited significantly more delayed neurodevelopment. A risk factor analysis indicated that male infants who stayed in the hospital for more than 11 days were at a higher risk of having clinical seizures. The lesion size in MRI greater than 37 pixels was a risk factor with an 81.8% accuracy.Seizures in HIE infants may predict abnormal brain MRI scans and abnormal neurodevelopment at 18 to 24 months of age.

Interleukin 8 may predict surgical necrotizing enterocolitis in infants born less than 1500 g.

Necrotizing enterocolitis (NEC) often leads to gastrointestinal emergency resulting high mortality in very low birth weight infants (VLBWIs) requiring surgery. To date, few studies have explored the role of serum cytokines in the development of feeding intolerance (FI) or NEC outcomes in VLBWIs. Infants born weighing <1500 g or of 32 weeks of gestational age were prospectively enrolled from May 2018 to Dec 2019. We measured several cytokines routinely within 72 h of life, even before NEC-like symptoms developed. NEC or FI group comprised 17 (27.4%) infants, and 6 (9.7%) infants had surgical NEC. The gestational age and birth weight were significantly lower in the NEC or FI group with more prematurity-related complications. The surgical NEC group also demonstrated significantly lower gestational age and birth weight along with more infants experiencing refractory hypotension within a 1 week of life, pulmonary hypertension, and patent ductus arteriosus. IL-10 levels were significantly higher in the NEC or FI group, whereas IL-8 levels were significantly higher in the infants with surgical NEC. Our findings indicated to IL-8 can predict surgical NEC while increased IL-10 can predict NEC development in VLBWIs.

The prognosis of brain magnetic resonance imaging injury pattern for outcomes of hypothermia-treated infants.

Magnetic resonance imaging (MRI) can be a tool that allows the observation of structural injury patterns after cooling. The aim of this study was to determine the early pattern of brain injury in the MRIs of infants with hypoxic ischemic encephalopathy (HIE) after cooling and to search for any clinical factors related to abnormal MRI findings.The study retrospectively recruited 118 infants who were treated with therapeutic hypothermia (TH) between 2013 and 2016.Forty-three patients had normal brain MRI, and 75 had abnormal brain MRI findings. The TH-treated infants with abnormal brain MRI readings showed significantly more clinical seizures and the use of additional antiepileptic drugs (AEDs) than the normal MRI group. As a long-term outcome, more lesions in the basal ganglia and thalamus, posterior limb of internal capsule, or severe white matter lesions were associated with abnormal neurodevelopmental outcomes at 18 to 24 months of age.A higher frequency of clinical seizures and AED use were related to abnormal brain injury on MRI. A significant risk for poor long-term outcomes was found in the abnormal brain MRI group.

Respiratory Severity Score with Regard to Birthweight during the Early Days of Life for Predicting Pulmonary Hypertension in Preterm Infants.

OBJECTIVE: The respiratory severity score (RSS) has been demonstrated to be associated with the oxygenation index in intubated newborn infants. We aimed to evaluate the usefulness of RSS with regard to birthweight (BW) in preterm infants for predicting an association with future pulmonary hypertension (PH). METHODS: Preterm infants with <30 weeks' gestation and <1250 g BW were reviewed and grouped into two (PH vs. no PH). Basic perinatal characteristics, comorbidities and parameters reflecting lung condition, including duration of invasive mechanical ventilator care, were compared. Respiratory support parameters (FiO2, RSS and RSS/kg) during the first 5 days of birth (0-120 h) were compared between the groups, and receiver operating characteristics curves were produced to evaluate the association with PH. RESULTS: RSS/kg, of the three respiratory support parameters, demonstrated significantly higher values in the PH group in the early days of life. Particularly, RSS/kg at day 2 of life had the greatest area under the curve [0.762, 95% confidence interval (CI) 0.655-0.869, p < 0.001] and maintained statistical significance [odds ratio (OR) 1.352, 95% CI 1.055-1.732, p = 0.017] in the multivariable analysis for a potential association with PH, along with gestational age (OR 0.996, 95% CI 0.993-0.999, p = 0.015) and preterm premature rupture of membrane >18 h (OR 4.907, 95% CI 1.436-16.765, p = 0.011). CONCLUSION: RSS/kg is a potential marker associated with the development of PH. Future studies could verify its usefulness as a reliable surrogate for predicting respiratory morbidity in clinical settings.

Enlarged subarachnoid space on cranial ultrasound in preterm infants: Neurodevelopmental implication.

The role of enlarged subarachnoid space (ESS) in preterm infants has not been described in concrete. We aimed to evaluate whether ESS should be considered a risk factor potentially associated with adverse neurodevelopmental outcomes in prematurity. Electronic medical records of 197 preterm infants (median 32.1 weeks' gestation) including cranial ultrasound (cUS) images, head circumferences, and Korean Developmental Screening Tests for Infants and Children (K-DST) results at 18-24 months corrected age were reviewed. The clinical characteristics and K-DST results were compared in infants with and without ESS (sinocortical width > 3.5 mm). A multivariable logistic regression analysis was performed to identify potential risk factors associated with positive K-DST results. At a median corrected age of 39.0 weeks, 81/197 (41.1%) infants presented ESS. A significantly greater percent of infants in the ESS group screened positive on the K-DST than in the no ESS group (27.2% vs 12.1%, p = 0.007). Within the ESS group, micro-/macrocephaly at term-equivalent age was not different with regard to the K-DST results. From the multivariable logistic regression analysis, gestational age (p = 0.016, OR = 0.855, 95% CI = 0.753-0.971) and ESS (p = 0.019, OR = 1.310, 95% CI = 1.046-1.641) were two significant risk factors associated with positive K-DST results. ESS identified on cUS at term-equivalent age in preterm infants is associated with possible developmental delays. Macrocephaly at term-equivalent age does not guarantee a benign prognosis. Future studies are required to verify ESS as a potential marker for neurodevelopmental delay in preterm infants.

Patent ductus arteriosus ligation on neurodevelopmental outcomes at corrected 2 years.

BACKGROUND: Our aim in this study was to evaluate whether very low birth weight infants (VLBWI) ligated for patent ductus arteriosus (PDA) were associated with worse neurodevelopmental outcomes at corrected 2 years. The ligated group was subdivided into ≤2 weeks of life (early) and ligated > 2 weeks of life (late) groups and compared the in-hospital morbidities and long term outcomes. METHODS: Between Dec 2013 and Dec 2015, VLBWI diagnosed with hs PDA were evaluated. RESULTS: Of the 191 VLBW infants with hs PDA, 28 (14.7%) infants had surgical ligation for PDA; 11 (39%) infants had EL and 17 (61%) infants had LL. Surgical ligation of hs PDA group had higher morbidities and mortality. Among the142 (83.0%) infants of 171 VLBWI with PDA survived, infants who were ligated had significantly lower scores of Bayley Scales of Infant and Toddler Development III at corrected age of 18 months. However, among the ligated group, there was little evidence of differences between the EL and LL groups. In a multivariable logistic regression analysis, only longer exposure of hs PDA and mechanical ventilation were consistently associated with worse neurodevelopmental outcomes. CONCLUSION: Our results suggest that surgical ligation for hs PDA may not increase risk for poor neurodevelopmental outcomes at corrected 2 years of age. The early surgical ligation may not be a risk factor.

Preoperative metabolic acidosis and acute kidney injury after open laparotomy in the neonatal intensive care unit.

BACKGROUND: This study evaluated potential risk factors associated with acute kidney injury (AKI) in infants undergoing bedside open laparotomy in the neonatal intensive care unit (NICU), and analyzed the association between postoperative AKI and outcomes. METHODS: Retrospective data, including neonatal characteristics, perioperative findings (i.e. vital signs and fluid status), postoperative AKI incidence, and postoperative mortality rate of infants who underwent bedside open laparotomy in the NICU between May 2013 and May 2018 were collected and analyzed. RESULTS: A total of 53 cases (26 in AKI group vs 27 in non-AKI group) were analyzed. On univariable analysis, transfusion, pre- and postoperative blood gas analysis and number of inotropic agents, cumulative postoperative percentage fluid overload (48 h), and preoperative hourly urine output were associated with the development of postoperative AKI. On multivariable logistic regression analysis, preoperative acidosis (pH <7.15 or base deficit >10; P = 0.002; OR, 11.067; 95%CI: 2.499-49.017) and preoperative urine output (P = 0.035; OR, 0.548; 95%CI: 0.314-0.959) were significant factors associated with postoperative AKI. Postoperative mortality rate 30 days after surgery was higher in the AKI group, but the difference was not significant. CONCLUSIONS: Preoperative metabolic acidosis and urine output are important factors potentially associated with the development of postoperative AKI in neonates undergoing bedside open laparotomy. Strategies such as alkali therapy, which protect the kidney from further injury, should be validated in future studies. A decreasing urine output may suggest deteriorating kidney function prior to surgery, potentially amplifying the risk of postoperative AKI.

Clinical implications in laboratory parameter values in acute Kawasaki disease for early diagnosis and proper treatment.

PURPOSE: This study aimed to analyse laboratory values according to fever duration, and evaluate the relationship across these values during the acute phase of Kawasaki disease (KD) to aid in the early diagnosis for early-presenting KD and incomplete KD patients. METHODS: Clinical and laboratory data of patients with KD (n=615) were evaluated according to duration of fever at presentation, and were compared between patients with and without coronary artery lesions (CALs). For evaluation of the relationships across laboratory indices, patients with a fever duration of 5 days or 6 days were used (n=204). RESULTS: The mean fever duration was 6.6±2.3 days, and the proportions of patients with CALs was 19.3% (n=114). C-reactive proteins (CRPs) and neutrophil differential values were highest and hemoglobin, albumin, and lymphocyte differential values were lowest in the 6-day group. Patients with CALs had longer total fever duration, higher CRP and neutrophil differential values and lower hemoglobin and albumin values compared to patients without CALs. CRP, albumin, neutrophil differential, and hemoglobin values at the peak inflammation stage of KD showed positive or negative correlations each other. CONCLUSION: The severity of systemic inflammation in KD was reflected in the laboratory values including CRP, neutrophil differential, albumin, and hemoglobin. Observing changes in these laboratory parameters by repeated examinations prior to the peak of inflammation in acute KD may aid in diagnosis of early-presenting KD patients.

Therapeutic hypothermia in infants with hypoxic-ischemic encephalopathy and reversible persistent pulmonary hypertension: short-term hospital outcomes.

AIM: Neonatal hypoxic ischemic encephalopathy (HIE) patients are at times accompanied by persistent pulmonary hypertension (PPHN), which is by itself another risk factor of adverse outcomes. We aimed to assess the outcome of therapeutic hypothermia (TH) in these patients whom we managed to reverse the shunt flow, as they are expected to be at much higher risk of adverse neurodevelopmental outcome. METHODS: We reviewed the medical records of 116 HIE infants (13 with PPHN and 103 without PPHN) who underwent TH between 2012 and 2016. We analyzed the short-term hospital outcomes and brain study results (electroencephalogram and magnetic resonance imaging) of TH in these patients. RESULTS: While infants with PPHN were significantly more likely to be outborn or have meconium aspiration syndrome, and required a longer duration of inotrope and intensive care support, electroencephalographic and brain magnetic resonance findings did not significantly differ according to PPHN status. CONCLUSION: Based on our study, the hospital outcomes of infants with HIE accompanied by reversible PPHN who underwent TH were in general not significantly graver than those not accompanied by PPHN. Our results suggest that undergoing TH may be more beneficial in HIE infants with PPHN and the risks for possible adverse effects may not be as so high.

Eosinophil-derived neurotoxin as a biomarker for disease severity and relapse in recalcitrant atopic dermatitis.

BACKGROUND: Eosinophils are encountered in many skin diseases, but the role of eosinophils in atopic dermatitis (AD) remains uncertain. OBJECTIVE: To examine the role of serum eosinophil-derived neurotoxin (EDN), eosinophil cationic protein (ECP), and total IgE as a biomarker of disease severity and relapse in severe recalcitrant AD. METHODS: We enrolled 99 patients with AD: 37 with severe recalcitrant AD, 20 with severe AD, and 42 with mild to moderate AD. We examined the difference in serum level of total IgE, ECP, and EDN between the groups and whether any correlation existed between disease severity and ECP or EDN. Lastly, difference in levels of ECP or EDN between those who experienced relapse was examined in the severe recalcitrant group. RESULTS: Serum levels of total IgE, ECP, and EDN were significantly higher in the severe recalcitrant AD group and severe AD group compared with the mild to moderate AD group. No significant difference was found in serum levels of total IgE, ECP, and EDN between the severe recalcitrant group and severe group. EDN had a significant positive correlation with the SCORing Atopic Dermatitis. No significant correlation was found between EDN and ECP. In the severe recalcitrant group, 29.7% of patients experienced relapse, and EDN was significantly higher in those who experienced relapse. The cutoff value of EDN for predicting relapse was 64.5. CONCLUSION: EDN correlated with the disease severity of AD. EDN may predict relapse in severe recalcitrant AD. The EDN serum level could be considered a candidate molecule as a clinical biomarker for evaluating AD disease activity and a predictor of relapse.

Recurrent macrophage activation syndrome since toddler age in an adolescent boy with HLA B27 positive juvenile ankylosing spondylitis.

Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3. We were unable to identify the cause for the HLH. He remained symptom-free until the development of back pain and right ankle joint pain with swelling at 15 years of age. He was diagnosed with HLA B27-positive AS with bilateral active sacroiliitis. He showed symptom aggravation despite taking naproxen and methotrexate, and the symptoms improved with etanercept. On admission, his laboratory data showed leukopenia with high ferritin and triglyceride levels. Bone marrow biopsy examination showed histiocytic hyperplasia with hemophagocytosis. There was no evidence of infection. He received naproxen alone, and his symptoms and laboratory data improved without any other immunomodulatory medications. Genetic study revealed no primary HLH or inflammasome abnormalities. In this case, underlying autoimmune disease should have been considered as the cause of recurrent MAS in the young patient once primary HLH was excluded.

Fatal systemic adenoviral infection superimposed on pulmonary mucormycosis in a child with acute leukemia: A case report.

BACKGROUND: Although adenovirus (ADV) infection usually causes self-limiting respiratory disorders in immune competent children; severe and systemic ADV infection in children undergoing chemotherapy for leukemia has been continuously reported. Nevertheless, there has been no consensus on risk factors and treatment strategies for severe ADV infection in children undergoing chemotherapy. CASE SUMMARY: We report a case of a 15-year-old boy with a fatal systemic ADV infection. He had received reinduction chemotherapy for relapsed acute lymphoblastic leukemia under continuing antifungal therapy for previously diagnosed fungal pneumonia. He complained of fever and right shoulder pain 4 days after completing the reinduction chemotherapy. In spite of appropriate antibiotic and antifungal therapy, pneumonia was aggravated and gross hematuria was accompanied. A multiplex polymerase chain reaction test for respiratory viruses was positive for ADV in a blood sample, and a urine culture was positive for ADV. He received oral ribavirin, intravenous immunoglobulin, and intravenous cidofovir therapy; however, he eventually died. Relapsed leukemia, concurrent fungal pneumonia, and delayed cidofovir administration were considered the cause of the grave outcome in this patient. CONCLUSION: ADV may cause severe infections not only in allogeneic hematopoietic cell transplant recipients, but also in patients undergoing chemotherapy for acute leukemia. The risk factors for severe ADV infection in patients undergoing chemotherapy should be determined in the future studies, and early antiviral therapy should be administered to immune compromised patients with systemic ADV infection.

Clinical Application of the Threshold Equalizing Noise Test in Patients with Hearing Loss of Various Etiologies: A Preliminary Study.

BACKGROUND AND OBJECTIVES: We aimed to make a preliminary assessment of the prevalence of cochlear dead regions (DRs) and the factors affecting the results of the threshold-equalizing noise (TEN) test in patients with hearing loss of various etiologies. SUBJECTS AND METHODS: Between May and July 2014, 109 patients (191 ears) with hearing loss who visited our outpatient clinic were prospectively enrolled. Pure tone audiometry and TEN (HL) test were performed for all the patients. DR at each frequency was indicated by masked thresholds of ≥10 dB above the TEN level and ≥10 dB above the absolute threshold. RESULTS: DR was present in 15.7% (n=30) of the 191 ears. According to disease entity, 16.6% of patients with sensorineural hearing loss had a DR. However, DR was absent in patients with chronic otitis media. According to audiometric configurations, DR was most common in moderately severe, flat hearing loss. Significantly worse hearing thresholds for both mean hearing level and hearing threshold at each frequency were found in the presence of DR (p<0.001). Logistic regression analysis showed that only the mean hearing level (odds ratio: 1.053, 95% confidence interval: 1.021-1.085) affected the presence of DR. CONCLUSIONS: Although performance of the TEN test is limited by frequencies and hearing levels, it provides additional information regarding DRs and may therefore have the potential to be used as a prognostic tool for diverse diseases causing hearing loss.

Missed spermatic cord torsion in an old man.

The fate of testicular salvage in spermatic cord torsion depends on the duration of ischemia and the degree of torsion. Even though spermatic cord torsion (SCT) can occur at any age, it is rarely reported in older patients. If the physician does not pay close attention to this unusual situation, the lack of suspicion for SCT may result in a missed or delayed diagnosis. We report a very uncommon case of missed SCT occurring in a 63-year-old man.