Experience with Real-Time Continuous Glucose Monitoring in Newborns with Congenital Hyperinsulinemic Hypoglycemia.

BACKGROUND: Effective treatment and close monitoring of hypoglycemia in children with congenital hyperinsulinemic hypoglycemia (CHH) is vital to prevent brain damage. The current use of capillary sampling schedules does not provide a comprehensive assessment of glycemic status and fails to detect asymptomatic hypoglycemia episodes. AIM: To investigate the efficacy and accuracy of a real-time continuous glucose monitoring system (RT-CGMS) in neonates with CHH. METHODS: A sensor connected to RT-CGMS was inserted into the newborn patients and maintained for at least 6 days during their stay in the hospital. We compared the readings of CGMS with capillary blood glucose values using Bland-Altman analysis. RESULTS: A total of 110 blood glucose values were compared to readings from the CGMS. All results were calculated and plotted for CGMS values at 0-4, 5-9, 10-14, 15-19, 20-24, and 25-29 min after capillary blood glucose sampling. CGMS readings were highly correlated with blood glucose values, especially during normoglycemia. In case of hypoglycemia, the mean difference between the CGMS and capillary glucose values was higher. Although the false positive rate for hypoglycemia was relatively high in CGMS, RT-CGMS may show some episodes of hypoglycemia earlier than blood measurement. CONCLUSION: RT-CGMS is accurate during normoglycemia, and it can reduce the number of capillary blood samples in children with CHH.

A Rare Complication of Patent Ductus Arteriosus Ligation: Inadvertent Ligation of the Left Pulmonary Artery.

BACKGROUND: The inadvertent ligation of the left pulmonary artery (LPA) is a rarely seen surgical complication that has been presented in the literature in a limited number of cases after patent ductus arteriosus (PDA) ligation surgery. Case Report: A PDA closure operation was performed on our patient, a 28-week-old preterm. In the postoperative follow-up, we identified on echocardiography taken on the same postoperative day that the ductus space was still present. On CT angiography, we determined that not only was the ductus space still continuing, but, in addition, ligation of the LPA had been performed inadvertently. An LPA reconstruction operation was performed on the patient 46 days after the first operation. However, owing to severe tissue damage in LPA, LPA reperfusion did not occur in the postoperative period. Conclusion: Although inadvertent ligation of the left pulmonary artery during PDA ligation surgery is rarely seen in patients who have undergone closure surgery, this complication should be kept in mind in the postoperative follow-up period. Patient findings such as physical examination, lung angiography and postoperative echocardiography should be assessed with this in mind.

Low-cost home-use light-emitting-diode phototherapy as an alternative to conventional methods.

BACKGROUND: The aim of our study was to evaluate whether a portable, light-weight, light-emitting-diode phototherapy unit designed for home use is as effective as conventional blue-light fluorescent phototherapy (CFP) for treating hyperbilirubinemia in neonates. METHODS: A total of 50 patients were recruited sequentially for treatment using CFP (n = 25) and the home-type phototherapy unit (n = 25). RESULTS: The average rate of decrease in bilirubin levels was 0.17 ± 0.02 and 0.20 ± 0.01 mg/dL/hours at the end of 24 hours in the groups receiving phototherapy by CFP and home-type phototherapy units, respectively. There was no statistically significant difference in the rate of the decrease in bilirubin levels between the groups (p = 0.104). CONCLUSIONS: It has been shown that the home-type phototherapy unit is as effective as CFP units in the treatment of neonatal hyperbilirubinemia and has the potential to become a standard of care for treatment of jaundiced neonates.

Is (99m)Tc-MDP whole body bone scintigraphy adjuvant to (18)F-FDG-PET for the detection of skeletal metastases?

PURPOSE: Due to the fact that fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG-PET/CT) and technetium-99m-methylenediphosphonate ((99m)Tc-MDP) whole body scans identify bone metastases by different mechanisms, i.e. by using glucose metabolism and osteoblastic response in the bone, respectively, it can be expected that there may be some differences between these two methods in the number of lesions identified. The aim of this study was to compare the sensitivity, specificity, accuracy, positive predictive value (PPV) and negative predictive value (NPV) in detecting bone metastases between (18)F-FDG-PET/CT and conventional (99m)Tc-MDP whole body scans. METHODS: Between 2006-2009, 121 patients with malignancies (62 male and 59 female, mean age 59.3±10.8 years, range 37-84) were examined with (18)F-FDG-PET/CT and conventional (99)Tc-MDP whole-body scans for detection of bone metastases. RESULTS: For (18)F-FDG-PET/CT and for (99m)TC-MDP, sensitivity, specificity, accuracy, PPV and NPV for detecting all studied bone metastases were 88.3, 83.6, 86.7, 91.7, 77.8% and 91.7, 71.0, 84.9, 86.6, 80.8%, respectively. For bone metastases of breast and lung cancers, the specificity and accuracy of PET/CT was higher than that of bone scintigraphy. On the other hand, the sensitivity of bone scintigraphy was higher than PET/CT in breast and lung cancers groups and all patients. In the detection of osteolytic and osteosclerotic metastases no difference was found between the two methods, while for osteolytic lesions the mean standardized uptake value (SUV) max was higher than for osteosclerotic lesions. CONCLUSION: For the detection of bone metastases the specificity and accuracy of (18)F-FDG-PET/CT were higher compared to bone scintigraphy, while the sensitivity was lower. It is the opinion of the authors that both studies are complementary to final diagnosis.

Effect of partially hydrolyzed synbiotic formula milk on weight gain of late preterm and term infants-a multicenter study.

Introduction: Data on the effectiveness of hydrolyzed infant formula containing both pre- and probiotics (synbiotic formula) on the growth of infants is still scarce. This retrospective study was designed to evaluate the effect of a partially hydrolyzed synbiotic formula on growth parameters and the possible occurrence of major gastrointestinal adverse events or morbidities in infants born via cesarean section (C-section) delivery. Methods: C-section-delivered term and late preterm infants who received either partially hydrolyzed synbiotic formula, standard formula, or maternal milk and followed at seven different hospitals from five different regions of Turkey, during a 1-year period with a minimum follow-up duration of 3 months were evaluated retrospectively. All the included infants were evaluated for their growth patterns and any kind of morbidity such as diarrhea, constipation, vomiting, infection, or history of hospitalization. Results: A total of 198 infants (73 in the human milk group, 61 in the standard formula group, and 64 in the partially hydrolyzed synbiotic formula group) reached the final analysis. The groups were similar regarding their demographic and perinatal characteristics. No difference was observed between the three groups regarding gastrointestinal major side effects. Growth velocities of the infants in the human milk and partially hydrolyzed synbiotic formula groups during the first month of life were similar whereas the weight gain of infants in the standard formula group was significantly less than these two groups (p < 0.001). Growth velocities were similar among the three groups between 1st and 3rd months of age. Discussion: A partially hydrolyzed synbiotic formula provided better weight gain in late-preterm and term infants who were delivered via C-section delivery compared to the standard formula during the first month of life. This weight gain was similar to the infants receiving exclusively human milk. This difference was not observed in length and head circumference gain. No difference was observed in any of the parameters during the 1st-3rd months of age. Specially formulated partially hydrolyzed synbiotic formulas may reverse at least some of the negative impacts of C-section delivery on the infant and help to provide better growth, especially during the early periods of life.

An observational, multicenter, registry-based cohort study of Turkish Neonatal Society in neonates with Hypoxic ischemic encephalopathy.

BACKGROUND: Hypoxic ischemic encephalopathy (HIE) is a significant cause of mortality and short- and long-term morbidities. Therapeutic hypothermia (TH) has been shown to be the standard care for HIE of infants ≥36 weeks gestational age (GA), as it has been demonstrated to reduce the rates of mortality, and adverse neurodevelopmental outcomes. This study aims to determine the incidence of HIE in our country, to assess the TH management in infants with HIE, and present short-term outcomes of these infants. METHODS: The Turkish Hypoxic Ischemic Encephalopathy Online Registry database was established for this multicenter, prospective, observational, nationally-based cohort study to evaluate the data of infants born at ≥34 weeks GA who displayed evidence of neonatal encephalopathy (NE) between March, 2020 and April 2022. RESULTS: The incidence of HIE among infants born at ≥36 weeks GA (n = 965) was 2.13 per 1000 live births (517:242440), and accounting for 1.55% (965:62062) of all neonatal intensive care unit admissions. The rates of mild, moderate and severe HIE were 25.5% (n = 246), 58.9% (n = 568), and 15.6% (n = 151), respectively. Infants with severe HIE had higher rates of abnormal magnetic resonance imaging (MRI) findings, and mortality (p<0.001). No significant difference in mortality and abnormal MRI results was found according to the time of TH initiation (<3 h, 3-6 h and >6 h) (p>0.05). TH was administered to 85 (34.5%) infants with mild HIE, and of those born of 34-35 weeks of GA, 67.4% (n = 31) received TH. A total of 58 (6%) deaths were reported with a higher mortality rate in infants born at 34-35 weeks of GA (OR 3.941, 95% Cl 1.446-10.7422, p = 0.007). CONCLUSION: The incidence of HIE remained similar over time with a reduction in mortality rate. The timing of TH initiation, whether <3 or 3-6 h, did not result in lower occurrences of brain lesions on MRI or mortality. An increasing number of infants with mild HIE and late preterm infants with HIE are receiving TH; however, the indications for TH require further clarification. Longer follow-up studies are necessary for this vulnerable population.

The use of chloride-sodium ratio in the evaluation of metabolic acidosis in critically ill neonates.

Acid-base disturbances have been usually evaluated with the traditional Henderson-Hasselbach method and Stewart's physiochemical approach by quantifying anions of tissue acids (TA). It is hypothesized that an increase in tissue acids during metabolic acidosis would cause a compensatory decrease in the plasma chloride (Cl) relative to sodium (Cl-Na ratio) in order to preserve electroneutral balance. Therefore, we aimed to investigate the use of Cl-Na ratio as a bedside tool to evaluate the identifying raised TA in neonates as an alternative to complex calculations of Stewart's physiochemical approach. This retrospective study was conducted between January 2008 and December 2009. Infants were included in the study when blood gas analysis reveals a metabolic acidosis; pH <7.25 and sHCO(3) concentration was <22 mEq/L. The Cl-Na ratio, sodium-chloride difference (Diff(NaCl)), anion gap (AG), albumin-corrected AG (AG(corr)), strong ion difference (SID), unmeasured anions (UMA), and TA were calculated at each episode of metabolic acidosis. A total of 105 metabolic acidosis episodes occurred in 59 infants during follow-up. Hypochloremic metabolic acidosis occurred in 17 (16%) of samples, and all had increased TA. The dominant component of TA was UMA rather than lactate. There was a negative correlation between the Cl-Na ratio and SID, AG(corr), UMA, and TA. Also, there was a positive correlation between Diff(NaCl) and SID, AG(corr), UMA, and TA. Base deficit and actual bicarbonate performed poorly in identifying the TA. In conclusion, our study suggested that Diff(NaCl) and Cl-Na ratio are simple and fast, and may be an alternative method to complex Stewart's physiochemical approach in identifying raised UMA and TA in critically ill neonates.

Salmonella typhimurium infections in BALB/c mice: a comparison of tissue bioluminescence, tissue cultures and mice clinical scores.

In response to systemic infection, mice usually present specific behaviors such as reduced activity and feeding, ruffled fur, hunched position, ataxia and tremor. We aimed to compare tissue bioluminescence, tissue cultures and clinical scores of BALB/c mice as potentially complementary outcome measures of Salmonella disease progression In Balb/c mice. The clinical status of the mice was assessed by visual examination for motility, ruffled fur, hunched position, feeding, ataxia and tremor. Patterns of bioluminescent light emission indicated the progression of infection from the abdominal region (initial site) to secondary tissue sites, which was indicative of systemic infection. As the severity and progression of infection increased, the bioluminescence signal became both more prominent and more anatomically disseminated. Bioluminescent Imaging (BLI) of Salmonella that have been genetically engineered to be bioluminescent is a new method that gives the opportunity to track Salmonella dissemination in mice. BLI is a helpful method to estimate tissue Salmonella concentration and may reduce the number of mice used in experiments, providing the opportunity to obtain serial assessments of disease progression in a single mouse subject. Clinical scores helped us to assess the clinical status of BALB/c mice in systemic Salmonella infections.

High-Resolution Computed Tomography Scores in Cases of Bronchopulmonary Dysplasia.

BACKGROUND: Bronchopulmonary dysplasia (BPD) carries a risk of long-term pulmonary sequelae. High-resolution computed tomography (HRCT) is a method of detecting such structural changes. This study is aimed at characterizing structural abnormalities associated with BPD and at evaluating the clinical findings in the newborn period associated with HRCT scores. METHODS: 28 patients born with a mean gestation age of 30 ± 2.9 weeks and diagnosed as BPD in their neonatal period were reevaluated when they were between the postnatal ages of 6 and 12 months. HRCT was performed in 20 patients with a history of moderate and severe BPD. Scans were interpreted by one radiologist using a scoring system. RESULTS: Patients were 9.8 ± 2.3 months at the time of reevaluation. The average HRCT score of patients was, respectively, 7.20 ± 4.05 with moderate and 7.40 ± 2.84 with severe BPD. The difference between them was not significant (p = 0.620). When moderate and severe groups were collected as a whole on the basis of physical findings and drug treatment, 6 had normal physical examination findings, no oxygen and no drug requirement; 14 had at least one finding at the time of reevaluation. No significant difference was detected in terms of HRCT score between the two groups (6.50 ± 3.83 versus 7.64 ± 3.30). CONCLUSIONS: More studies are needed in terms of the role of HRCT in the assessment of BPD prognosis. A contemporary definition of BPD that correlates with respiratory morbidity in childhood is needed. Also, a new lung ultrasound technique for predicting the respiratory outcome in patients with BPD can be used instead of HRCT.

Association Between Umbilical Cord Levels of Glypican-1, Glypican-3, Syndecan-1, WISP1, Leptin and Birth Weight of Small, Appropriate, and Large for Gestational Age Infants.

BACKGROUND: Many factors are affecting intrauterine growth. The role of Wingless-type (Wnt) inducible signaling pathway protein-1 (WISP1), a novel adipokine and placental proteoglycans in intrauterine growth, is not known. We aimed to measure umbilical cord blood levels of glucose, insulin, leptin, WISP1, and placental proteoglycans [glypican-1 (GPC1), glypican-3 (GPC3), and syndecan-1 (SDC1)] which are thought to have an important role in fetal growth and investigate their relation with birth weight. METHODS: Full-term neonates were included in this prospective, cross-sectional study and classified as appropriate for gestational age (AGA), small for gestational age (SGA), and large for gestational age (LGA) according to their birth weight. Umbilical cord blood levels of glucose, insulin, leptin, WISP1, GPC1, GPC3, and SDC1 were measured. RESULTS: Leptin levels were higher in LGA newborns compared to AGA and SGA newborns, while WISP1, GPC1, GPC3, and SDC1 levels were not different between the three groups. Leptin and GPC1 levels were higher in infants of mothers with gestational diabetes mellitus compared to infants of non-diabetic mothers, while WISP1, GPC3, and SDC1 were not different between the groups. Leptin was positively correlated with insulin, birth weight, and maternal weight. While there was a strong correlation between the WISP1, GPC1, GPC3, and SDC1 levels; there was no correlation between the birth weight, maternal weight, glucose, insulin, and WISP1, GPC1, GPC3, and SDC1 levels. CONCLUSION: Umbilical cord blood levels of GPC1, GPC3, SDC1, and WISP1 were not different between SGA, AGA, and LGA infants. The significance of serum levels of these adipokines and proteoglycans remains to be elucidated.

Prenatal diagnosis of mobile flap-like tissue on the ventricular septal defect in a newborn with trisomy 18.

Trisomy 18 is the second most common autosomal trisomy in liveborn infants. Various congenital malformations, mental retardation, and high rate of infant mortality in the first year of life are characteristic features of trisomy 18. Congenital heart disease occurs in over 90% of these patients and the most common cardiac lesions are ventricular septal defect, patent ductus arteriosus and atrial septal defect. This is a case report of a baby born with trisomy 18 (postnatal diagnosis) in whom there was an unusual echocardiographic appearance of a mobile structure ("flap-like") around the area of a VSD-which was imaged prenatally.

Bilateral renal hypoplasia and cystic dysplasia: a new phenotype of Thomas syndrome or a new syndrome?

Thomas syndrome is a rare syndrome including Potter sequence, renal anomalies, heart defects, cleft palate with other oropharyngeal anomalies. Here, we report a newborn with Potter sequence, bilateral renal hypoplasia and cystic dysplasia, multiple cardiovascular malformations, long large ears, frontal bossing, small lips, partial simple toe syndactyly, and cleft palate. To our best knowledge, this patient may be considered as a new variant of Thomas syndrome or a new syndrome.

Y Chromosome Material in Turner Syndrome.

Background Turner Syndrome (TS) is a frequently identified chromosomal disease in humans characterized by short stature, sexual infantilism, streak gonads, primary amenorrhea, and a number of somatic anomalies. Approximately 55% of TS individuals have a nonmosaic 45,X karyotype. In addition, a cell line with a Y chromosome is present in 5% of patients, which is undetectable by the standard cytogenetic analysis. The identification of Y chromatin in some TS individuals has been associated with the development of gonadoblastoma. Therefore, it is important to exclude the presence of Y chromatin in TS individuals. In this study, it was planned to detect cases with mosaicism in terms of Y chromatin with the help of Y whole chromosome probes (WCP) from individuals with TS by fluorescence in situ hybridization (FISH) analysis. Methodology Forty-four patients with Turner syndrome, who were being followed up in the Genetics Unit, were contacted and invited for the study. Of the 44 invited patients, 28 responded to the invitation. In this study, it was planned to detect TS patients with mosaicism in terms of Y chromatin with the help of the Y whole chromosome probe. Results The majority of the cases (71.42%) included in the study carried pure X monosomy, which is the classical Turner syndrome karyotype. Other structural X chromosome aberrations, in isolated or mosaic forms, were less frequently represented. Y chromosome sequences were searched in 28 cases with Turner syndrome by the FISH method using Y whole chromosome probe. Y chromosome sequence was detected in one (3.5%) case of 28 cases. Conclusions It is recommended that individuals with Turner syndrome be screened for Y chromatin. Detection of this will provide information and guidance to individuals with Turner syndrome, especially in terms of the risk of developing gonadoblastoma, with advanced clinical consultation. This study was conducted to emphasize the importance of this.

Ileal atresia and severe cerebral injury after fetoscopic laser photocoagulation treatment for twin-to-twin transfusion syndrome.

BACKGROUND: Twin-to-twin transfusion syndrome (TTTS) is a significant cause of perinatal morbidity and mortality. Fetoscopic laser photocoagulation (FLP) is the optimal treatment option for twin-to-twin transfusion syndrome; but can cause central nervous system, extremity and intestinal system injury. CASE: We report the case report of ileal atresia and severe cerebral infarction co-occurrence after fetoscopic laser photocoagulation treatment. It is uncertain as to whether ileal atresia occurred due to ischemia associated with TTTS, the treatment with FLP, or a combination of both. CONCLUSIONS: Cases with prenatal ultrasonographic abnormalities after FLP should have a close assessment to detect bowel complications. Despite many developments in its management, TTTS remains an important risk factor for cerebral injury.

The effects of omega-3 fatty acids on the newborn rat hyperoxic lung injury.

Objective: Bronchopulmonary dysplasia (BPD) is an important cause of morbidity in preterms. Inflammation plays a central role in the pathogenesis of the disease while omega-3 fatty acids are known to have anti-inflammatory effects. In this study, we examined the effects of supplementary omega-3 fatty acids on hyperoxic lung injury.Methods: Experimental hyperoxic lung injury induced newborn 3-day-old rats were monitored in a confined hyperoxic environment with an oxygen concentration of 90-95% for a 2-week period. Rats were divided into three groups as placebo, low-dose Omega-3, and high-dose Omega-3. During the 2-week study period, low and high-dose Omega-3 groups were given 300 and 600 mg/kg/day omega-3 docosahexaenoic acid (DHA) and eicosapentaenoic acid (EPA) respectively, while those in placebo received the same amount of serum physiologic. At the end of the 2-week study, lungs of all the rats were removed and morphologic evaluation under light microscopy was performed. Mean cord length (Lm), alveolar surface area (SA), and alveolar wall thickness (Wt) were calculated to find out whether a statistically significant difference between groups existed.Results: Similar alveolar development was observed between groups. No difference was seen between mean Lm values. Although the alveolar surface area was found to be higher in high-dose omega-3 group, the difference was not considered to be statistically significant. While the widest alveolar wall thickness was observed in the placebo group, alveolar wall thickness difference between high-dose omega-3 group and placebo group was found to be statistically significant (placebo Wt=17,8 ± 2.3 µm, low-dose omega -3 Wt=15,6 ± 2,5 µm, high-dose omega -3 Wt=14,2 ± 2 µm) (p < .05).Conclusions: Omega-3 fatty acids were observed to prevent alveolar wall thickness to some extent, though with no noticeable effect on hyperoxic lung injury.

Comparison of Changes in Breast Milk Macronutrient Content During the First Month in Preterm and Term Infants.

Purpose: This study aims to achieve a better understanding of the trend of maternal milk macronutrient levels by analyzing protein, lipid, carbohydrate, and energy content of the breast milk and investigate the possible confounders of macronutrient content during the first 4 weeks of lactation to meet the specific needs of babies, guide optimum fortification of maternal milk. Patients and Methods: Breast milk from 39 mothers who had delivered preterm infants and 21 mothers of term infants were collected longitudinally for the first 4 weeks of lactation. Fresh milk samples were obtained on day 3, 7, 14, and 28 of lactation. The samples are analyzed using mid-infrared milk analyzer (MIRIS Human Milk Analyzer, HMA; Miris AB, Uppsala, Sweden). Results: Colostral milk protein concentrations of mothers of both preterm and term infants were significantly higher and the protein content of the samples decreased in time, according to the week of lactation during the study period. In contrast, fat, carbohydrate, and energy content were lowest in the colostral milk in both groups. When preterm and term milks were compared, fat and carbohydrate levels on 28th day and energy levels on 14th and 28th days were significantly higher in term milks, whereas no difference in protein contents were observed between the two groups on each time period. Mode of delivery was found to have statistically significant correlation with protein content of the milk. Conclusion: This longitudinal study revealed significant changes in analyzed macronutrient contents of mother's milk over the first 4 week period.

Primary cutaneous aspergillosis in an extremely low birth weight preterm.

Aspergillosis is an uncommon infection in neonates. However, it has been an emerging problem for preterm infants in recent years because of long-term parenteral nutrition, multiple-antibiotic therapy and immune deficiency due to prematurity. We report a preterm neonate with disseminated cutaneous lesions due to primary cutaneous aspergillosis. She died despite an early treatment with liposomal amphotericin B. Fungal infections should be remembered in preterms whose clinical conditions and laboratory tests for infection deteriorate, despite an appropriate antibiotic and supportive therapy.

Retinopathy of prematurity risk factors: Does human milk prevent retinopathy of prematurity?

Akyüz-Ünsal AI, Key Ö, Güler D, Bekmez S, Sagus M, Akcan AB, Kurt-Omurlu I, Anik A, Oruç-Dündar S, Türkmen M. Retinopathy of prematurity risk factors: Does human milk prevent retinopathy of prematurity? Turk J Pediatr 2019; 61: 13-19. The aim of this study was to investigate the risk factors for Retinopathy of Prematurity (ROP) development and the potential effect of human breast milk among these factors. For this purpose, infants admitted to a tertiary referral clinic for ROP screening and treatment between April 2013 and May 2015, were included in this retrospective study. The demographic data, accompanying diseases, previous surgery, type of feeding and duration of human breast milk intake were recorded. According to the ROP screening examination results, infants were divided into two groups as those with ROP (infants at any stage of ROP) and those without ROP. Relationship between the risk factors and ROP were evaluated. The comparison of 221 infants without ROP and 99 infants with ROP; revealed that gestational age at birth, birth weight, mechanical ventilation support, bronchopulmonary and cardiac diseases, hydrocephaly, any previous surgery, infections, steroid treatment percentages were high and human breast milk intake percentage was low among infants with ROP. Mean breast feeding time for infants with ROP (3.81 ± 2.33 month) was shorter than the infants without ROP (5.51 ± 1.43 month) (p < 0.001). In logistic regression analysis, the duration of breast feeding was inversely related with ROP (OR 0.744; 95% CI 0.621-0.891; p < 0,001). These results suggest that gestational age at birth and accompanying diseases are the main risk factors for the development of ROP. As the duration of the breast feeding of the infants without ROP was longer than the infants with ROP; breast feeding may have a preventive effect on ROP development.

A Systemic-Onset Juvenile Idiopathic Arthritis Patient with Reduced Anakinra Treatment Admitted with an Attack.

Interleukin-1 plays an important role in the pathogenesis of systemic-onset juvenile idiopathic arthritis (SoJIA), and the use of anti-interleukin-1 therapy has been increasing. We report a case of a 14-year-old male patient with SoJIA. He was in remission with anakinra treatment for almost 2 years. When we extended the therapeutic range and decreased the dose (1 mg/kg twice a week), he developed symptoms mimicking pulmonary embolism and cardiac ischemia. Increased cardiac enzyme levels and echocardiographic findings were interpreted as myopericarditis. Pulmonary computed tomography angiography revealed no thrombus. An SoJIA attack was considered because of high level of acute-phase reactants and clinical findings. Intensive immunosuppressive therapy with 2 mg/kg/day anakinra was reinitiated. Clinical and laboratory parameters began to improve on the fifth day of treatment. Thus, anti-interleukin-1 therapy is very important in patients with SoJIA. Although the treatment dose was gradually reduced and the therapeutic range was extended, it is noteworthy that the case progressed to a severe clinical condition. Broad prospective studies regarding whether, how long, and for what reasons the dosages of these drugs should be reduced in patients with SoJIA with no genetic disorders are required.