Comparison between transient and permanent congenital hypothyroidism on a thyroid function test after re-evaluation.

PURPOSE: Congenital hypothyroidism (CH) is diagnosed with neonatal screening and is treated early in the neonatal period. Among these patients, transient CH (TCH) is included and requires re-evaluation. The purpose of this study was to find the best way to discontinue levothyroxine and to find trends in thyroid function tests (TFTs) after re-evaluation. METHODS: We retrospectively reviewed 388 patients diagnosed with CH. They were classified as permanent CH (PCH) and TCH. The total number of the PCH and TCH groups was 83 (51 boys and 32 girls). We compared clinical parameters to predict TCH and to identify the trends of TFT. RESULTS: The first thyroid-stimulating hormone (TSH) value after discontinuation and the average TSH value for 1, 2, and 3 years were all significantly higher in the PCH group (P<0.01). The first fT4 value after discontinuation and the average fT4 value for 1, 2, and 3 years were all significantly higher in the TCH group (P<0.01). The optimal cutoff value on the receiver operating characteristic curve for PCH prediction with an average of 3 years of TSH was greater than 9.05 µIU/mL, which was predicted with a sensitivity of 100% and a specificity of 100%. CONCLUSION: When the TSH value ranges from 10 µIU/mL to 20 µIU/mL, clinicians can discontinue levothyroxine if the next result is around 10 µIU/mL or shows a decreasing trend.

Classic and backdoor pathways of androgen biosynthesis in human sexual development.

Both genes and hormones regulate human sexual development. Although ovarian hormones are not essential for female external genitalia development, male sexual development requires the action of testicular testosterone and dihydrotestosterone (DHT). DHT is the most active endogenous androgen formed by the conversion of testosterone in genital skin. This synthesis route from cholesterol to DHT is called the conventional classic pathway. Recent investigations have reported an alternative ("backdoor") route for DHT formation that bypasses fetal testicular testosterone. This alternative route plays a crucial role in human hyperandrogenic disorders like congenital adrenal hyperplasia caused by P450c21 deficiency, polycystic ovary syndrome, and P450 oxidoreductase deficiency. In addition, mutations in AKR1C2 and AKR1C4, genes encoding 3α-reductases, have been implicated in disorders of sexual development, indicating that both the classic and backdoor routes are required for normal human male sexual development. More recently, androsterone was found to be the primary androgen of the human backdoor route. Androsterone and steroidal substrates specific to the backdoor route are predominantly found in the placenta, liver, and adrenal glands rather than in the testes. These findings are essential to understanding human sexual development.

Severe hypernatremia in soft drink ketoacidosis and hyperglycemic hyperosmolar state at the onset of type 2 diabetes mellitus: a case series of three adolescents.

Diabetic ketoacidosis (DKA) and hyperglycemic hyperosmolar state (HHS) are diabetic emergencies. Some patients with a hyperglycemic crisis can present with an overlap of DKA and HHS. The coexistence of DKA and HHS is associated with higher mortality than in isolated DKA and HHS. In addition, electrolyte derangements caused by global electrolyte imbalance are associated with potentially life-threatening complications. Here, we describe three cases of mixed DKA and HHS with severe hypernatremia at the onset of type 2 diabetes mellitus. All patients had extreme hyperglycemia and hyperosmolarity with acidosis at the onset of diabetes mellitus. They consumed 2 to 3 L/d of high-carbohydrate drinks prior to admission to relieve thirst. They showed severe hypernatremia with renal impairment. Two patients recovered completely without any complications, while one died. Severe hypernatremia with mixed DKA and HHS is rare. However, it may be associated with excess carbohydrate beverage consumption. Reduced physical activity during the COVID19 pandemic and unhealthy eating behaviors worsened the initial presentation of diabetes mellitus. We highlight the impact of lifestyle factors on mixed DKA and HHS.

Recombinant growth hormone therapy in children with Turner Syndrome in Korea: a phase III Randomized Trial.

BACKGROUND: Short stature is the most consistent characteristic feature of Turner syndrome (TS). To improve final heights of children with TS effectively, it is important to provide them with early and appropriate treatment using growth hormone (GH). The objective of this study was to assess the efficacy and safety of a new recombinant human GH, Growtropin®-II (DA-3002, Dong-A ST Co., Ltd) versus a comparator (Genotropin®, Pfizer Inc.) for Korean children with TS. METHODS: This open-label, active-controlled, parallel-group, randomized controlled phase III trial was conducted at 11 hospitals in Korea. Eligible patients (n = 58) were randomized to two groups: 1) DA-3002 group (administrated with DA-3002 at 0.14 IU [0.0450-0.050 mg] /kg/day); and 2) comparator group (administrated with the comparator at 0.14 IU [0.0450-0.050 mg] /kg/day). RESULTS: The change from baseline in annualized height velocity (HV) after a 52-week treatment period was 4.15 ± 0.30 cm/year in the DA-3002 group and 4.34 ± 0.29 cm/year in the comparator group. The lower bound of 95% two-sided confidence interval for group difference in the change of annualized HV (- 1.02) satisfied the non-inferiority margin (- 1.5). The change in height standard deviation score (HtSDS) at 52-week was 0.70 ± 0.23 for the DA-3002 group and 0.66 ± 0.39 for the comparator group, showing no significant (p = 0.685) difference between the two groups. The change of skeletal maturity defined as change in bone age/change in chronological age between the two groups was not significantly different (1.25 ± 0.58 for the DA-3002 group and 1.47 ± 0.45 for the comparator group, p = 0.134). Changes from baseline in serum insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3) after 52 weeks of treatment did not differ significantly between the two groups (p = 0.565 and p = 0.388, respectively) either. The occurrence of adverse events was not statistically different between groups. CONCLUSIONS: This study demonstrates that the efficacy and safety of GH treatment with DA-3002 in children with TS are comparable with those of the comparator. It is expected to analysis the long-term effect of DA-3002 on the increase of final adult height in children with TS and possible late-onset complications in the future. TRIAL REGISTRATION: The study was registered at ClinicalTrials.gov. ClinicalTrials.gov identifier: NCT01813630 (19/03/2013).

Risk factors for renal involvement in Henoch-Schönlein purpura

Abstract Objective: Henoch-Schönlein purpura is a systemic vasculitis that mainly occurs in children. Renal impairment is a major complication of Henoch-Schönlein purpura, but there is no established predictive marker for renal involvement. Thus, in this study, we investigated the risk factors for renal involvement in children with Henoch-Schönlein purpura. Method: The medical records of children newly diagnosed as having Henoch-Schönlein purpura between 2005 and 2020 were reviewed retrospectively. Selected laboratory data were recorded before treatment initiation. The date and the age at diagnosis; sex; and the presence of arthralgia, gastrointestinal and renal involvement were obtained retrospectively. Results: This study included a total of 186 patients with Henoch-Schönlein purpura. Among them, 36.0% had renal involvement; 28.4% had only microscopic hematuria, 53.7% had nonnephrotic range proteinuria, and 17.9% had nephrotic-range proteinuria during follow-up. The mean age was higher (p = 0.016) and female sex was predominant (p = 0.001) in patients with renal involvement than in those without renal involvement. Blood neutrophil/lymphocyte ratio (p = 0.002) and platelet/lymphocyte ratio (p = 0.002) were significantly higher than that of the patients without renal involvement. No statistically significant differences were observed in the hemoglobin concentration, platelet count, presence of arthralgia, and gastrointestinal involvement between patients with and without renal involvement. Logistic regression analysis revealed female sex (odd ratio = 3.213) and neutrophil/lymphocyte ratio (odd ratio = 1.329) as risk factors for renal involvement. Conclusions: Female sex and high neutrophil/lymphocyte ratio were risk factors for renal involvement in Henoch-Schönlein purpura.

Investigating whether serum IGF-1 and IGFBP-3 levels reflect the height outcome in prepubertal children upon rhGH therapy: LG growth study database.

Serum insulin-like growth factor-1 (IGF-I) and IGF binding protein-3 (IGFBP-3) levels can be used to monitor the safety of recombinant human growth hormone (rhGH) therapy. In this study, we evaluated the changes in serum IGF-I and IGFBP-3 levels during rhGH therapy as a marker of height outcome in prepubertal children. Totally, 705 prepubertal children with short stature were enrolled from the LG Growth Study Database. Data for three groups of subjects were obtained as follows: Idiopathic GH deficiency (IGHD; n = 486); idiopathic short stature (n = 66); small for gestational age (n = 153). Serum IGF-I and IGFBP-3 levels at the baseline and after the 1st and 2nd year of rhGH therapy, as well as the Δheight standard deviation score (SDS), were obtained. Δheight SDS after the 1st and 2nd year of rhGH therapy had notably increased compared to that at the baseline for all three groups. IGF-I and IGFBP-3 levels in all three groups were significantly increased compared to those at the baseline (p <0.001). Δheight SDS was positively correlated with ΔIGF-1 SDS after the 1st year of therapy, ΔIGFBP-3 SDS after the 2nd year of therapy in the IGHD group, and ΔIGF-I SDS and ΔIGFBP-3 SDS after the 2nd year of therapy (p < 0.05), regardless of whether the height at the baseline was a covariate. The increase in IGF-I and IGFBP-3 levels during rhGH therapy was related to the growth response in children with IGHD. Therefore, it may be valuable to measure the change in serum IGF-I and IGFBP-3 levels, especially the latter, during rhGH treatment to predict the growth response upon long-term treatment.

Efficacy and adverse events related to the initial dose of methimazole in children and adolescents with Graves' disease.

PURPOSE: The first-line antithyroid drug for children and adolescents with Graves' disease (GD) is methimazole (MMI). This study evaluated the relationship between the initial MMI dose and the clinical course of GD after treatment. METHODS: We studied the efficacy of the initial MMI dose and the relationship between the initial MMI dose and adverse events (AEs). We retrospectively enrolled 22 males and 77 females and divided those subjects into 3 groups according to the initial dose of MMI: <0.4 mg/kg/day (group A; n=32); 0.4-0.7 mg/kg/day (group B; n=39); and >0.7 mg/kg/day (group C; n=28). RESULTS: The mean time to the normalization of free thyroxine (fT4) levels upon initial treatment was 5.64, 8.61, and 7.98 weeks in groups A, B, and C, respectively (P=0.116). The incidence of liver dysfunction, neutropenia, and skin rash was 12.5%, 20.5%, and 42.9% in groups A, B, and C, respectively (P=0.018). Neutropenia, as a severe AE, was absent in group A, but its prevalence was 7.7% in group B and 21.4% in group C (P=0.015). When comparing only groups B and C, the incidences of liver dysfunction and neutropenia were higher in group C (P=0.04 and P=0.021, respectively). CONCLUSION: The mean time to the normalization of fT4 levels did not differ among the 3 groups, but the incidence of AEs was higher in the groups that received high MMI doses. High doses of MMI (>0.7 mg/kg/day) should be reconsidered as an initial treatment for children and adolescents with GD.

Efficacy and safety of the recombinant human growth hormone in short children born small for gestational age: A randomized, multicentre, comparative phase III trial.

OBJECTIVE: Growth hormone (GH) treatment is known to be effective in increasing stature in children with a short stature born small for gestational age (SGA). This multicentre, randomized, open-label, comparative, phase III study aimed to evaluate the efficacy and safety of Growtropin-II (recombinant human GH) and to demonstrate that the growth-promoting effect of Growtropin-II is not inferior to that of Genotropin in children with SGA (NCT ID: NCT02770157). METHODS: Seventy five children who met the inclusion criteria were randomized into 3 groups in a ratio of 2:2:1 (the study group [Growtropin-II, n = 30], control group [Genotropin, n = 30], and 26-week non-treatment group [n = 15]). The study and control groups received subcutaneous injections of Growtropin-II and Genotropin, respectively for 52 weeks, whereas the non-treatment group underwent a non-treatment observation period during weeks 0 to 26 and a dosing period during weeks 27 to 52 and additional dosing till week 78 only in re-consenting children. RESULTS: No significant differences in demographic and baseline characteristics between the groups were observed. The mean ± standard deviation change difference in annualized height velocity (aHV) (study group - control group) was 0.65 ±2.12 cm/year (95% confidence interval [CI], -0.53 to 1.83), whereas the lower limit for the 2-sided 95% CI was -0.53 cm/year. Regarding safety, treatment-emergent adverse events (TEAEs) occurred in 53.33% children in the study group and 43.33% children in the control group; the difference in the incidence of TEAEs between the 2 treatment groups was not statistically significant (P  = .4383). A total of 17 serious adverse events (SAEs) occurred in 13.33% children in the treatment groups, and no significant difference in incidence between groups (P  = .7065) was seen. Two cases of adverse drug reaction (ADR) occurred in 2 children (3.33%): 1 ADR (injection site swelling or pain) occurred in 1 child (3.33%) each in the study and control groups. CONCLUSIONS: This study demonstrates that the change in aHV from the baseline till 52 weeks with Growtropin-II treatment is non-inferior to that with Genotropin treatment in children with short stature born SGA. Growtropin-II is well-tolerated, and its safety profile is comparable with that of Genotropin over a 1-year course of treatment.

Risk factors for renal involvement in Henoch-Schönlein purpura.

OBJECTIVE: Henoch-Schönlein purpura is a systemic vasculitis that mainly occurs in children. Renal impairment is a major complication of Henoch-Schönlein purpura, but there is no established predictive marker for renal involvement. Thus, in this study, we investigated the risk factors for renal involvement in children with Henoch-Schönlein purpura. METHOD: The medical records of children newly diagnosed as having Henoch-Schönlein purpura between 2005 and 2020 were reviewed retrospectively. Selected laboratory data were recorded before treatment initiation. The date and the age at diagnosis; sex; and the presence of arthralgia, gastrointestinal and renal involvement were obtained retrospectively. RESULTS: This study included a total of 186 patients with Henoch-Schönlein purpura. Among them, 36.0% had renal involvement; 28.4% had only microscopic hematuria, 53.7% had non-nephrotic range proteinuria, and 17.9% had nephrotic-range proteinuria during follow-up. The mean age was higher (p = 0.016) and female sex was predominant (p = 0.001) in patients with renal involvement than in those without renal involvement. Blood neutrophil/lymphocyte ratio (p = 0.002) and platelet/lymphocyte ratio (p = 0.002) were significantly higher than that of the patients without renal involvement. No statistically significant differences were observed in the hemoglobin concentration, platelet count, presence of arthralgia, and gastrointestinal involvement between patients with and without renal involvement. Logistic regression analysis revealed female sex (odd ratio = 3.213) and neutrophil/lymphocyte ratio (odd ratio = 1.329) as risk factors for renal involvement. CONCLUSIONS: Female sex and high neutrophil/lymphocyte ratio were risk factors for renal involvement in Henoch-Schönlein purpura.

Acute Kidney Injury in Pediatric Diabetic Ketoacidosis.

OBJECTIVE: To assess the incidence and clinical characteristics of acute kidney injury (AKI) and identify the associated risk factors for AKI in children with type 1 diabetes mellitus (T1DM) and diabetic ketoacidosis (DKA). METHODS: This was a retrospective study performed over 15 y in a single Korean center. Children aged ≤18-y-old with T1DM and DKA were enrolled and divided into 2 groups according to the presence of AKI. RESULTS: This study included 90 episodes of DKA in 58 children with T1DM. AKI occurred in a total of 70 hospitalizations (77.8%) of 44 children: 18 (20.0%) with stage 1 AKI, 39 (43.3%) with stage 2 AKI, and 13 (14.4%) with stage 3 AKI. The number of AKI decreased to 28 (47.4%) and 13 (28.3%) after 12 h and 24 h of admission, respectively. The white blood cell count (P = 0.001) and anion gap levels (P = 0.025) were significantly higher and serum bicarbonate level (P = 0.004) was lower in the AKI group. Logistic regression analysis revealed that a longer duration of TIDM and high anion gap were independent predictors of developing severe AKI in pediatric DKA with T1DM (odds ratio, 1.225, P = 0.013; odds ratio, 1.130, P = 0.038). CONCLUSIONS: AKI frequently occurred in TIDM children with DKA. Longer duration of TIDM and elevated anion gap are associated with occurrence of severe AKI.

Neutrophil-to-lymphocyte ratio to predict gastrointestinal bleeding in Henoch: Schönlein purpura.

BACKGROUND: Henoch-Schönlein purpura (HSP) is a common form of vasculitis in children. It typically involves small vessels of the skin, the gastrointestinal (GI) tract, joints, and kidneys. GI involvement is the most severe short-term complication and GI bleeding is a major complication of HSP, but there is no established predictive marker of GI bleeding. Blood neutrophil-to-lymphocyte ratio (NLR) has been proposed as a potentially useful marker of clinical outcome in diseases with an inflammatory component. The aim of this study was to clarify the association of NLR with HSP and investigate the usefulness of NLR as a marker to predict GI bleeding in children with HSP. METHODS: All patients with newly diagnosed HSP were reviewed retrospectively. White blood cell count, hemoglobin, platelet counts, mean platelet volume, neutrophil and lymphocyte count were evaluated. NLR and platelet-to-lymphocyte ratio (PLR) were calculated using complete blood count data. RESULTS: This study involved 141 HSP patients. GI involvement was found in 65 patients (46.1%), and, of these, 15 (10.6%) had GI bleeding. At the time of diagnosis, NLR was significantly higher (P = 0.001) and PLR significantly lower (P = 0.032) in patients with GI bleeding than in those without GI bleeding. On logistic regression analysis, NLR was the only independent predictor of GI bleeding (P = 0.004). The optimal cut-off of NLR for predicting GI bleeding was 2.86 (sensitivity, 73%; specificity, 68%). CONCLUSIONS: NLR, a simple and easily obtainable parameter, is a potential predictive marker of GI bleeding in children with HSP.

Plasma Neutrophil Gelatinase-Associated Lipocalin as a Predictor of Renal Parenchymal Involvement in Infants With Febrile Urinary Tract Infection: A Preliminary Study.

BACKGROUND: Urinary tract infection (UTI) is the most common bacterial infection in infants. Renal parenchymal involvement is an important prognostic factor; however, early detection of parenchymal involvement in UTI may be difficult during infancy. This study aimed to assess whether a recently established biomarker of UTI, neutrophil gelatinase-associated lipocalin (NGAL), can serve as a useful marker for the detection of cortical defects (CD) and to determine the appropriate diagnostic cut-off value of NGAL in infants with febrile UTI. METHODS: Infants hospitalized for febrile UTI were divided into two groups according to the presence of cortical defects on dimercaptosuccinic acid (DMSA) scintigraphy. Among 64 enrolled infants, 43 (67%) had CD (UTI-CD) and 21 (33%) had no CD (UTI-ND). The white blood cell count, C-reactive protein, and plasma NGAL (pNGAL) levels were determined before antibiotic therapy and compared between the two groups. RESULTS: pNGAL level was significantly higher in the UTI-CD group than in the UTI-ND group (340 µg/L vs 214 µg/L, P=0.002). Multivariate analysis showed that pNGAL level was the only independent predictor of CD (odds ratio 2.759, P=0.039). In the ROC curve analysis, pNGAL showed the highest area under the curve (0.745; 95% confidence interval, 0.561-0.821; P=0.014). The appropriate cut-off value of pNGAL was 267 µg/L (sensitivity, 72.1%; specificity, 71.4%). CONCLUSIONS: pNGAL was found to be a useful marker for early prediction of renal parenchymal involvement in infants with febrile UTI.

Congenital Syphilis Presenting with Only Nephrotic Syndrome: Reemergence of a Forgotten Disease.

Syphilis infection has re-emerged after years of declining incidence. The prevalence of congenital syphilis (CS) has increased in Korea and other countries during the last few decades. Untreated infants develop symptoms such as rhinorrhea, anemia, jaundice, cutaneous lesions, hepatosplenomegaly, and pseudoparalysis within weeks or months. Significant renal disease is uncommon in CS, and clinical renal involvement varies from mild transient proteinuria to frank nephrosis. We report a 2-month-old infant with CS who presented with only nephrotic syndrome (NS). The previously healthy infant presented with NS and showed no other syphilitic manifestations. Remission of the NS was achieved with adequate penicillin treatment. No recurrence of proteinuria was observed during the 1 year of follow-up. Although rare, this long forgotten disease continues to affect pregnant women, resulting in prenatal or postnatal mortality. We still consider the possibility of syphilitic nephropathy and therefore serologic testing for congenital NS.

Urinary tract infection caused by community-acquired extended-spectrum ß-lactamase-producing bacteria in infants / Infecção do trato urinário causada por bactérias produtoras de ß-lactamase- de espectro ampliado adquiridas na comunidade em neonatos

Abstract Objective: Urinary tract infection (UTI) caused by resistant strains of bacteria is increasingly prevalent in children. The aim of this study was to investigate the clinical characteristics and risk factors for UTI caused by community-acquired extended-spectrum β-lactamase (CA-ESBL)-producing bacteria in infants. Methods: This was a retrospective study performed over 5 years in a single Korean center. Hospitalized infants with febrile UTI were enrolled and divided into two groups (CA-ESBL vs. CA non-ESBL UTI). The yearly prevalence was calculated. Baseline characteristics and clinical course such as fever duration, laboratory and radiological findings were compared between the two groups. Risk factors associated with the CA-ESBL UTI were investigated. Results: Among the enrolled infants (n = 185), 31 (17%) had CA-ESBL UTI. The yearly prevalence of ESBL of CA-ESBL UTI increased during the study (0% in 2010, 22.2% in 2015). Infants with CA-ESBL UTI had a longer duration of fever after initiating antibiotics (2.0 ± 1.1 vs. 1.5 ± 0.6 days, p = 0.020). Cortical defects on renal scan and early treatment failure were more frequent in CA-ESBL (64.5 vs. 42.2%, p = 0.023; 22.6 vs. 4.5%, p = 0.001). A logistic regression analysis revealed that urinary tract abnormalities and previous UTI were independent risk factors for CA-EBSL UTI (odds ratio, 2.7; p = 0.025; 10.3; p = 0.022). Conclusion: The incidence of UTI caused by ESBL-producing bacteria has increased in Korean infants. Recognition of the clinical course and risk factors for ESLB-producing UTI may help to determine appropriate guidelines for its management.

Resumo Objetivo: A infecção do trato urinário (ITU) causada por cepas de bactérias resistentes está cada vez mais prevalente em crianças. O objetivo deste estudo foi investigar as características clínicas e os fatores de risco de ITU causada por bactérias produtoras de β-lactamases de espectro ampliado adquiridas na comunidade (ESBL CA) em neonatos. Métodos: Estudo retrospectivo feito por mais de cinco anos em um único centro sul-coreano. Neonatos internados com ITU febril foram inscritos e divididos em dois grupos (ITU por ESBL CA em comparação com não ESBL CA). A prevalência anual foi calculada. As características básicas e o curso clínico, como duração da febre e achados laboratoriais e radiológicos, foram comparados entre os dois grupos. Os fatores de risco associados à ITU por ESBL CA foram investigados. Resultados: Entre os neonatos inscritos (n = 185), 31 (17%) apresentaram ITU por ESBL CA. A prevalência anual de ESBL em ITU por ESBL CA aumentou durante o estudo (0% em 2010, 22,2% em 2015). Os neonatos com ITU por ESBL CA apresentaram maior duração de febre após o início dos antibióticos (2 ± 1,1 em comparação com 1,5 ± 0,6 dias, p = 0,020). Os defeitos corticais no exame renal e a falha precoce no tratamento foram mais frequentes em ESBL CA (64,5 em comparação com 42,2%, p = 0,023; 22,6 em comparação com 4,5%, p = 0,001). Uma análise de regressão logística revelou que as anomalias do trato urinário e a ITU anterior eram fatores de risco independentes de ITU por ESBL CA (razão de chance: 2,7; p = 0,025; 10,3; p = 0,022). Conclusão: A incidência de ITU causada por bactérias produtoras de ESBL aumentou em neonatos sul-coreanos. O reconhecimento do curso clínico e dos fatores de risco de ITU por ESBL poderá ajudar a determinar as diretrizes adequadas de manejo.

A Pediatric Case of Severe Fever with Thrombocytopenia Syndrome in Korea.

Severe fever with thrombocytopenia syndrome (SFTS) is an emerging infectious disease and elderly people living in rural areas have the greatest risk of infection. We report the first pediatric case of SFTS in Korea and the clinical characteristics and disease progression in children. A 10-year-old child from Chonnam province visited the hospital with myalgia and a history of fever over the previous 8 days. Her father noticed a tick on her head and removed it before fever developed. Because the symptoms continued, her father consulted the community health center and SFTS virus was detected both from the tick (Haemaphysalis longicornis) and the patient's blood. On hospitalization, fever and severe myalgia were improved and no gastrointestinal and hemorrhagic symptoms were observed. The patient was successfully treated with a combination of steroids, IVIG, and ribavirin. In this report, a pediatric case of SFTS presents a mild clinical course but close attention must be paid to the screening of children with mild symptoms consisting of SFTS.

Clinical utility of spot urine protein-to-creatinine ratio modified by estimated daily creatinine excretion in children.

BACKGROUND: The spot urine protein-to-creatinine ratio (UPCR) is widely used to predict 24-h urine protein (24-h UP) excretion. In patients with low daily urine creatinine excretion (UCr), however, the UPCR may overestimate 24-h UP. The aim of this study was to predict 24-h UP using UPCR adjusted by estimated 24-h UCr in children. METHODS: This study included 442 children whose 24-h UP and spot UPCR were measured concomitantly. Estimated 24-h UCr was calculated using three previously existing equations. We estimated the 24-h UP excretion from UPCR by multiplying the estimated UCr. The results were compared with the measured 24-h UP. RESULTS: There was a strong correlation between UPCR and 24-h UP (r = 0.801, P < 0.001), and the correlation improved after multiplying the UPCR by the measured UCr (r = 0.847, P < 0.001). Using the estimated UCr rather than the measured UCr, there was high accuracy and strong correlation between the estimated UPCR weighted by the Cockcroft-Gault equation and 24-h UP. Improvement was also observed in the subgroup (proteinuria vs. non-proteinuria) analysis, particularly in the proteinuria group. CONCLUSIONS: The spot UPCR multiplied by the estimated UCr improved the accuracy of prediction of the 24-h UP in children.

Urinary tract infection caused by community-acquired extended-spectrum ß-lactamase-producing bacteria in infants.

OBJECTIVE: Urinary tract infection (UTI) caused by resistant strains of bacteria is increasingly prevalent in children. The aim of this study was to investigate the clinical characteristics and risk factors for UTI caused by community-acquired extended-spectrum ß-lactamase (CA-ESBL)-producing bacteria in infants. METHODS: This was a retrospective study performed over 5 years in a single Korean center. Hospitalized infants with febrile UTI were enrolled and divided into two groups (CA-ESBL vs. CA non-ESBL UTI). The yearly prevalence was calculated. Baseline characteristics and clinical course such as fever duration, laboratory and radiological findings were compared between the two groups. Risk factors associated with the CA-ESBL UTI were investigated. RESULTS: Among the enrolled infants (n=185), 31 (17%) had CA-ESBL UTI. The yearly prevalence of ESBL of CA-ESBL UTI increased during the study (0% in 2010, 22.2% in 2015). Infants with CA-ESBL UTI had a longer duration of fever after initiating antibiotics (2.0±1.1 vs. 1.5±0.6 days, p=0.020). Cortical defects on renal scan and early treatment failure were more frequent in CA-ESBL (64.5 vs. 42.2%, p=0.023; 22.6 vs. 4.5%, p=0.001). A logistic regression analysis revealed that urinary tract abnormalities and previous UTI were independent risk factors for CA-EBSL UTI (odds ratio, 2.7; p=0.025; 10.3; p=0.022). CONCLUSION: The incidence of UTI caused by ESBL-producing bacteria has increased in Korean infants. Recognition of the clinical course and risk factors for ESLB-producing UTI may help to determine appropriate guidelines for its management.

Hashimoto thyroiditis with an unusual presentation of cardiac tamponade in Noonan syndrome.

Noonan syndrome is an autosomal dominant, multisystem disorder. Autoimmune thyroiditis with hypothyroidism is an infrequent feature in patients with Noonan syndrome. A 16-year-old boy was admitted because of chest discomfort and dyspnea; an echocardiogram revealed pericardial effusion. Additional investigations led to a diagnosis of severe hypothyroidism due to Hashimoto thyroiditis. The patient was treated with L-thyroxine at 0.15 mg daily. However, during admission, he developed symptoms of cardiac tamponade. Closed pericardiostomy was performed, after which the patient's chest discomfort improved, and his vital signs stabilized. Herein, we report a case of an adolescent with Noonan syndrome, who was diagnosed with Hashimoto thyroiditis with an unusual presentation of cardiac tamponade.

Frequency of Diabetes and Thyroid Autoantibodies in Patients with Type 1 Diabetes and Their Siblings.

The aim of this study was to better understand the frequency of autoimmune thyroid and diabetes antibodies in patients with type 1 diabetes mellitus (T1DM) compared with their siblings. Glutamic acid decarboxylase antibodies (GADA), islet cell antibodies (ICA), insulin autoantibodies (IAA), and thyroid autoantibodies were studied in all subjects. The rates of positive GADA and IAA were significantly higher in probands compared to in siblings (p<0.001) or controls (p<0.001). All pancreatic autoantibodies were not significantly different between the siblings and the healthy controls. Thyroid antiperoxidase antibody (TPOAb) and antithyroglobulin antibody (TGAb) were significantly different between the probands and the control subjects (p=0.002 and p=0.018, respectively). The rates of TPOAb and TGAb positivity in siblings were higher than in those of the controls, but there was no significant difference between the two groups. However, thyroid autoimmunity (TA) was significantly different among the groups (p=0.004). Siblings of the TA-positive probands were shown to have a greater prevalence of thyroid antibodies than did the controls (p=0.022), but siblings of the TA-negative probands did not have such a prevalence compared with the control subjects. The prevalence of pancreatic and thyroid antibodies positivity in probands was statistically significant compared with the siblings and the controls. Siblings of TA-positive probands revealed a greater prevalence of thyroid antibodies than did the controls. Therefore, the screening for TA in siblings, particularly siblings of TA-positive probands, is as important as it is in probands.