Case report: Development of central precocious puberty in a girl with late-diagnosed simple virilizing congenital adrenal hyperplasia complicated with Williams syndrome.

Congenital adrenal hyperplasia (CAH) and Williams Syndrome (WS; MIM # 194050) are distinct genetic conditions characterized by unique clinical features. 21-Hydroxylase deficiency (21-OHD; MIM #201910), the most common form of CAH, arises from mutations in the CYP21A2 gene, resulting in virilization of the external genitalia in affected females, early puberty in males, and short stature. Williams syndrome, caused by a microdeletion of 7q11.23, presents with distinctive facial features, intellectual disability, unique personality traits, early puberty, and short stature. This case report describe the clinical features of a 4-year-old girl referred due to progressive virilization and developmental delay. Genetic analysis confirmed concurrent CAH and WS, identifying a novel mutation in the CYP21A2 gene (c.1442T>C). Following corticosteroid therapy initiation, the patient developed central precocious puberty. This case report delves into the pubertal change patterns in a patient affected by overlapping genetic conditions, providing valuable insights in to the intricate clinical manifestation and management of these rare complex disorders.

Korean clinical practice guidelines for diagnostic and procedural sedation.

Safe and effective sedation depends on various factors, such as the choice of sedatives, sedation techniques used, experience of the sedation provider, degree of sedation-related education and training, equipment and healthcare worker availability, the patient's underlying diseases, and the procedure being performed. The purpose of these evidence-based multidisciplinary clinical practice guidelines is to ensure the safety and efficacy of sedation, thereby contributing to patient safety and ultimately improving public health. These clinical practice guidelines comprise 15 key questions covering various topics related to the following: the sedation providers; medications and equipment available; appropriate patient selection; anesthesiologist referrals for high-risk patients; pre-sedation fasting; comparison of representative drugs used in adult and pediatric patients; respiratory system, cardiovascular system, and sedation depth monitoring during sedation; management of respiratory complications during pediatric sedation; and discharge criteria. The recommendations in these clinical practice guidelines were systematically developed to assist providers and patients in sedation-related decision making for diagnostic and therapeutic examinations or procedures. Depending on the characteristics of primary, secondary, and tertiary care institutions as well as the clinical needs and limitations, sedation providers at each medical institution may choose to apply the recommendations as they are, modify them appropriately, or reject them completely.

Two-center randomized controlled trial comparing oral chloral hydrate and intranasal combination of dexmedetomidine and ketamine for procedural sedation in children: study protocol.

BACKGROUND: Oral chloral hydrate is widely used in pediatric sedation. Intranasal dexmedetomidine has been increasingly used for pediatric sedation; however, its improvement is warranted. The combination of dexmedetomidine with ketamine can improve onset and hemodynamic stability while maintaining sedative efficacy. This study aims to determine the efficacy and safety of intranasal combination of dexmedetomidine and ketamine compared to oral chloral hydrate. METHODS: This is a prospective, parallel-arm, single-blinded, two-center, superiority randomized controlled trial with 1:1 allocation, designed to compare the effects of intranasal combination of dexmedetomidine and ketamine with those of oral chloral hydrate. We shall enroll 136 patients aged < 7 years old in this study. Prior to the procedure, we shall randomize each patient into the control group (oral chloral hydrate 50 mg/kg) or study group (intranasal dexmedetomidine 2 µg/kg and ketamine 3 mg/kg). The primary outcome will be the rate of achieving an adequate sedation level (6-point Pediatric Sedation State Scale 1, 2, or 3) within 15 min. In addition, we shall measure the sedation time, sedation failure rate, completion of procedure, adverse events, patient acceptance, and physician satisfaction. DISCUSSION: This study will provide evidence of the efficacy and safety of the intranasal combination of dexmedetomidine and ketamine in comparison with oral chloral hydrate. TRIAL REGISTRATION: ClinicalTrials.gov , NCT04820205. Registered on 19th March 2021.

Dynamic Contrast-Enhanced Magnetic Resonance Lymphangiography and Lymphatic Interventions for Pediatric Patients with Various Lymphatic Diseases.

Background: To demonstrate the magnetic resonance lymphangiography (MRL) imaging findings of lymphatic diseases and the clinical outcomes of lymphatic embolization in pediatric patients. Methods and Results: This retrospective study included 10 consecutive pediatric patients who underwent MRL for lymphatic diseases between June 2017 and June 2021. Nine patients underwent dynamic contrast-enhanced MRL with bilateral inguinal lymph node injection of diluted gadolinium, and one patient underwent nonenhanced MRL with a heavily T2-weighted image. The etiology of lymphatic disease was classified into three categories based on the magnetic resonance findings. The resolution of chylous fluid and weight-adjusted amounts of chylous fluid collected from a drainage tube were evaluated as outcomes. Patients were classified as postoperative lymphatic leak (n = 3), pulmonary lymphatic perfusion syndrome (n = 3), central lymphatic flow disorder (CLFD; n = 3), and primary lymphatic dysfunction (Gorham-Stout syndrome; n = 1). Three patients underwent radiological lymphatic intervention, and one CLFD patient underwent surgical intervention. In patients with postoperative lymphatic leak, the median chest tube drainage decreased significantly after the intervention [from 87.9 to 12.4 mL/(kg·d); p = 0.02]. However, in one CLFD patient, the amount of chylous fluid did not decrease until 7 days after intervention. Conclusion: The etiology of lymphatic disease in pediatrics can be recognized on MRL, and lymphatic intervention can be performed for cessation of lymphatic leak, even though the treatment outcomes may differ according to the underlying etiology. MRL can play an important role in classifying lymphatic disease, and in planning treatment on the basis of the lymphatic anatomy and underlying etiology.

Factors Associated with Occurrence of Atelectasis during Sedation for Imaging in Pediatric Patients: A Retrospective Single Center Cohort Study.

Sedation can induce atelectasis which may cause suboptimal image quality. This study aimed to identify factors associated with the occurrence of atelectasis during sedation for imaging in pediatric patients. Patients < 18 years who had undergone whole-body magnetic resonance imaging (MRI) under sedation with propofol or dexmedetomidine were included in this study. The development of atelectasis was visually and quantitatively assessed by coronal short tau inversion recovery images of the thoracic level. Multivariable logistic regression was performed to identify the independent factors associated with the development of atelectasis. Ninety-one patients were included in the analysis. In the multivariable analysis, administration of supplemental oxygen was the only factor significantly associated with the occurrence of atelectasis (adjusted odds ratio, 4.84; 95% confidence interval, 1.48-15.83; p = 0.009). Univariable analysis showed that the use of dexmedetomidine was associated with a lower incidence of atelectasis; however, this could not be verified in the multivariable analysis. Among the pediatric patients who had undergone imaging under sedation, additional oxygen supplementation was the only independent factor associated with atelectasis occurrence. A prospective clinical trial is required to identify the cause-effect relationship between oxygen administration and occurrence of atelectasis during sedation.

Efficacy and Safety of Inhalation Sedation during Office Probing for Congenital Nasolacrimal Duct Obstruction.

We compared the effectiveness of inhaled sevoflurane versus physical restraint during probing in children with congenital nasolacrimal duct obstruction (CNLDO). We performed a retrospective review of children with CNLDO who underwent office probing procedures by a single surgeon under sedation or restraint. Patients' characteristics at the time of probing, including age, sex, laterality, previous non-surgical treatment, presence of dacryocystitis, outcomes of probing, and complications were compared between the sedation and restraint groups. A multivariable logistic regression analysis was performed to investigate the prognostic factors associated with the success of probing. A subgroup analysis by 12 months of age was also conducted. The overall success rate was 88.6% in 202 eyes of 180 consecutive children (mean age, 15.1 ± 7.7 months). The sedation group had a marginally higher success rates than the restraint group (93.8% vs. 85.1%, p = 0.056). The success rate was not significantly different between the two groups in children aged <12 months (90.9% vs. 93.1%, p = 0.739), but it was significantly higher in the sedation group (94.7% vs. 77.8%. p = 0.006) in children aged ≥12 months. Inhalation sedation was the most potent factor associated with success (adjusted odds ratio = 5.56, 95% confidence interval = 1.33-23.13, p = 0.018) in children aged ≥12 months. There were no surgical or sedation-related complications intra- and postoperatively. Inhaled sevoflurane sedation resulted in more successful, controlled, painless probing, particularly in children aged ≥12 months. It represents a safe, efficient alternative to general anesthesia.

Intramuscular dexmedetomidine and oral chloral hydrate for pediatric sedation for electroencephalography: A propensity score-matched analysis.

BACKGROUND: Intramuscular dexmedetomidine can be used for pediatric sedation without requiring intravenous access and has advantages for electroencephalography by inducing natural sleep pathway, but only a limited number of studies compared the efficacy of intramuscular dexmedetomidine with oral chloral hydrate. AIMS: To compare the efficacy and safety of intramuscular dexmedetomidine and oral chloral hydrate used for sedation during electroencephalography in pediatric patients. METHODS: We reviewed the medical records of pediatric patients who underwent sedation for electroencephalography between January 2015 and December 2016. Initial doses of dexmedetomidine and chloral hydrate were 3 mcg/kg and 50 mg/kg, respectively; second doses (1 mcg/kg and 50 mg/kg, respectively) were administered if adequate sedation was not achieved. Demographic data, time of sedative administration, time of sedation and awakening, and time of arrival at recovery room and discharge were analyzed. RESULTS: Out of a total of 1239 patients, 125 patients had received dexmedetomidine and 1114 had received chloral hydrate. After 1:1 propensity score matching, the dexmedetomidine and chloral hydrate groups each had 118 patients. Testing completion rate with a single dose of medication was higher in the dexmedetomidine group (91.5% vs 71.2%; mean difference [95% CI] 20.3 [10.8-29.9]; P < .0001; Pearson chi-square value = 16.09). Sedation onset time was shorter in the dexmedetomidine group as well (16.6 ± 13.0 minutes vs 41.5 ± 26.8 minutes; mean difference [95% CI] 24.8 [19.1-30.6]; P < .0001; T = 8.27). On the contrary, the duration of recovery was longer in the dexmedetomidine group (35.5 ± 40.2 minutes vs 18.5 ± 30.7 minutes; mean difference [95% CI] 18.6 [8.8-28.5]; P = .0002; T = -2.82). Total residence time was not significantly different between the two groups (125.8 ± 40.6 minutes vs 122.1 ± 42.2 minutes, mean difference [95% CI] 5.21 [6.1-16.5], P = .3665 T = 0.04). CONCLUSIONS: Intramuscular dexmedetomidine showed higher sedation success rate and shorter time to achieving the desired sedation level compared with oral chloral hydrate and thus may be an effective alternative for oral chloral hydrate in pediatric patients requiring sedation for electroencephalography.

Relationship between perioperative thyroid function and acute kidney injury after thyroidectomy.

Thyroid dysfunction may alter kidney function via direct renal effects and systemic haemodynamic effects, but information on the effect of thyroid function on postoperative acute kidney injury (AKI) following thyroidectomy remains scarce. We reviewed the medical records of 486 patients who underwent thyroidectomy between January 2010 and December 2014. Thyroid function was evaluated based on the free thyroxine or thyroid stimulating hormone levels. The presence of postoperative AKI was determined using the Kidney Disease: Improving Global Outcomes (KDIGO) criteria. AKI developed in 24 (4.9%) patients after thyroidectomy. There was no association between preoperative thyroid function and postoperative AKI. Patients with postoperative hypothyroidism showed a higher incidence of AKI than patients with normal thyroid function or hyperthyroidism (19.4%, 6.7%, and 0%, respectively; P = 0.044). Multivariable logistic regression analysis showed that male sex (OR, 4.45; 95% CI, 1.80-11.82; P = 0.002), preoperative use of beta-blockers (OR, 4.81; 95% CI, 1.24-16.50; P = 0.016), low preoperative serum albumin levels (OR, 0.29; 95% CI, 0.11-0.76; P = 0.011), and colloid administration (OR, 5.18; 95% CI, 1.42-18.15; P = 0.011) were associated with postoperative AKI. Our results showed that postoperative hypothyroidism might increase the incidence of AKI after thyroidectomy.

Frequency of Self-Monitoring of Blood Glucose during the School Day Is Associated with the Optimal Glycemic Control among Korean Adolescents with Type 1 Diabetes.

BACKGROUND: This study aimed to evaluate the relationship between the frequency of self-monitoring of blood glucose (SMBG) and glycosylated hemoglobin (HbA1c) levels among Korean adolescents with type 1 diabetes mellitus (T1DM). Factors affecting the SMBG frequency were analyzed in order to improve their glycemic control. METHODS: Sixty-one adolescents aged 13 to 18 years with T1DM were included from one tertiary center. Clinical and biochemical variables were recorded. Factors associated with SMBG frequency were assessed using structured self-reported questionnaires. RESULTS: Average total daily SMBG frequency was 3.8±2.1 and frequency during the school day was 1.3±1.2. The mean HbA1c level was 8.6%±1.4%. As the daily SMBG frequency increased, HbA1c levels declined (P=0.001). The adjusted odds of achieving the target HbA1c in participants who performed daily SMBG ≥5 significantly increased 9.87 folds (95% confidence interval [CI], 1.58 to 61.70) compared with those performed SMBG four times a day. In the subjects whose SMBG frequency <1/day during the school day, an 80% reduction in the adjusted odds ratio 0.2 (95% CI, 0.05 to 0.86) showed compared to the group with performing two SMBG measurements in the school setting. The number of SMBG testing performed at school was significantly high for individuals assisted by their friends (P=0.031) and for those who did SMBG in the classrooms (P=0.039). CONCLUSION: Higher SMBG frequency was significantly associated with lower HbA1c in Korean adolescents with T1DM. It would be necessary to establish the school environments that can facilitate adequate glycemic control, including frequent SMBG.

Comparison of the effect of spinal anesthesia and general anesthesia on 5-year tumor recurrence rates after transurethral resection of bladder tumors.

Non-muscle invasive bladder tumors are early-stage tumors with high recurrence rates. Transurethral resection of bladder tumor (TURB) is performed under spinal or general anesthesia; however, the effect of the two anesthetic techniques on non-muscle invasive bladder tumor recurrence is unknown. Thus, we compared their effects on tumor recurrence rates five years after TURB. Data from 876 patients who underwent TURB from 2000 to 2007 was reviewed. Patients received spinal or general anesthesia based on their choice or the clinician's preference. Tumor recurrence five years after TURB was assessed using multivariate Cox regression model, propensity score analysis (matching and inverse probability of treatment weighting), and Kaplan-Meier method. The five-year tumor recurrence rate after TURB was 42% and 53% for spinal and general anesthesia groups, respectively (P = 0.013). Hazard ratios for tumor recurrence in the spinal anesthesia group compared to that in the general anesthesia group were 0.619 (P <0.001), 0.642 (P = 0.001), and 0.636 (P <0.001) in the Cox regression model, Cox regression model with propensity score matching, and adjusted analysis with inverse probability of treatment weighting, respectively. The five-year tumor recurrence rate was significantly lower in the spinal anesthesia group than in the general anesthesia group in both the unadjusted (P = 0.002) and adjusted Kaplan-Meier curves (P <0.001). Therefore, spinal anesthesia for non-muscle invasive bladder tumor resection was associated with a lower five-year tumor recurrence rate than general anesthesia. This finding provides useful information for an appropriate selection of anesthetic technique for TURB.

Change in pulse transit time in the lower extremity after lumbar sympathetic ganglion block: an early indicator of successful block.

Objective To investigate the change in pulse transit time (PTT)-time between the electrocardiographic R wave and the highest point of the corresponding plethysmographic wave-after lumbar sympathetic ganglion block (LSGB) and evaluate PTT as an indicator of successful LSGB. Methods Sixteen cases of sympathetically mediated lower extremity neuropathic pain treated with LSGB were studied. Correlations between the changes in PTT and temperature were used to identify the cutoff point indicating successful LSGB. Results PTT rate of change at 5 min relative to the baseline PTT (dPTT5/PTT0) significantly correlated positively with the temperature change at 20 min (correlation coefficient 0.734). The dPTT5/PTT0 ratios of the Success and Failure groups were 6.46 ± 2.81% and 2.77 ± 1.72%, respectively. The dPTT5/PTT0 cutoff indicating successful LSGB, based on receiver operating characteristic curve analysis, was 4.23%. Conclusion PTT measurement 5 min after local anesthetic injection was an early, objective indicator of successful or failed LSGB.

Efficacy of Adjuvant 10% Hypertonic Saline in Transforaminal Epidural Steroid Injection: A Retrospective Analysis.

BACKGROUND: Chronic lower back pain with or without radiculopathy represents an important medical, social, and economic problem. Many treatment modalities and techniques, including surgery and epidural administration of steroids, have been used to manage this pain. Hypertonic saline, which has been used as an adjunct to percutaneous epidural adhesiolysis, can also be injected via a transforaminal approach in expectation of longer-lasting effects. OBJECTIVES: This study aimed to determine the effect of adding hypertonic saline to conventional transforaminal epidural steroid injections (TFEI) to provide pain relief for chronic radiculopathy patients. STUDY DESIGN: A retrospective study. SETTING: Pain clinic of a university hospital. METHODS: Between January 2010 and December 2013, the medical records of 246 patients (94 in the hypertonic group, 153 in the control group) who received transforaminal epidural block were reviewed and analyzed. The hypertonic group received 10% sodium chloride solution added to lidocaine, triamcinolone, and hyaluronidase. Outcomes on pain reduction were measured using a numerical rating scale (NRS) and the responder rate at baseline, one, 3, and 6 months after procedure. RESULTS: The estimated difference in NRS scores from baseline throughout a 6-month follow-up period in the hypertonic group were significantly higher (P = 0.0003). The proportion of substantial responders (41.9% vs. 34.6% at one month, 40.9% vs. 26.8% at 3 months, and 33.3% vs. 14.4% at 6 months, respectively, P = 0.0058) and substantial/moderate responders (71.0% vs. 58.8% at one month, 65.6% vs. 40.4% at 3 months, and 48.4% vs. 20.3% at 6 months, respectively, P < 0.0001) were significantly higher in the hypertonic group. The Oswestry disability index (ODI) was not different between the groups (P = 0.2697). LIMITATIONS: Retrospective design without a control group. CONCLUSIONS: Hypertonic saline provides more superior and longer lasting pain relieving effects when added to TFEIs.Key words: Back pain, epidural injections, epidural steroids, hypertonic saline, lumbar, radiculopathy, transforaminal.

Method to Reduce the False-Positive Rate of Loss of Resistance in the Cervical Epidural Region.

Background. The cervical epidural space can be detected by the loss of resistance (LOR) technique which is commonly performed using air. However, this technique using air has been associated with a high false-positive LOR rate during cervical interlaminar epidural steroid injections (CIESIs). Objective. We investigated whether the detection of LOR with contrast medium might reduce the false-positive LOR rate on the first attempt. Methods. We obtained data retrospectively. A total of 79 patients were divided into two groups according to the LOR technique. Groups 1 and 2 patients underwent CIESI with the LOR technique using air or contrast medium. During the procedure, the injection technique (median or paramedian approach), final depth, LOR technique (air or contrast), total number of LOR attempts, and any side effects were recorded. Results. The mean values for the total number of LOR attempts were 1.38 ± 0.65 (Group 1) and 1.07 ± 0.25 (Group 2). The false-positive rate on the first attempt was 29.4% and 6.6% in Groups 1 and 2, respectively (P = 0.012). Conclusions. The use of contrast medium for LOR technique is associated with a lower rate of false-positivity compared with the use of air.

The Diagnostic Value of Pelvic Ultrasound in Girls with Central Precocious Puberty.

The gonadotropin-releasing hormone (GnRH) stimulation test is the gold standard for differentiating central precocious puberty (CPP) from exaggerated thelarche (ET). Because of this test's limitations, previous studies have clarified the clinical and laboratory factors that predict CPP. The present study investigated the early diagnostic significance of pelvic ultrasound in girls with CPP. The GnRH stimulation test and pelvic ultrasound were performed between March 2007 and February 2015 in 192 girls (aged <8 years) with signs of early puberty and advanced bone age. Ninety-three of 192 patients (48.4%) were diagnosed as having CPP and the others (51.6%) as having ET. The CPP group had higher uterine volumes (4.31±2.79 mL) than did the ET group (3.05±1.97 mL, p=0.03). No significant differences were found in other ultrasonographic parameters. By use of receiver operating characteristic curve analysis, the most predictive parameter for CPP was a uterine volume of least 3.30 mL, with an area under the curve of 0.659 (95% confidence interval: 0.576-0.736). The CPP group had significantly higher uterine volumes than did the ET group, but there were no reliable cutoff values in pelvic ultrasound for differentiating between CPP and ET. Pelvic ultrasound should be combined with clinical and laboratory tests to maximize its diagnostic value for CPP.

Differential Postoperative Effects of Volatile Anesthesia and Intraoperative Remifentanil Infusion in 7511 Thyroidectomy Patients: A Propensity Score Matching Analysis.

Although remifentanil is used widely by many clinicians during general anesthesia, there are recent evidences of opioid-induced hyperalgesia as an adverse effect. This study aimed to determine if intraoperative remifentanil infusion caused increased pain during the postoperative period in patients who underwent a thyroidectomy. A total of 7511 patients aged ≥ 20 years, who underwent thyroidectomy between January 2009 and December 2013 at the Asan Medical Center were retrospectively analyzed. Enrolled patients were divided into 2 groups: group N (no intraoperative remifentanil and only volatile maintenance anesthesia) and group R (intraoperative remifentanil infusion including total intravenous anesthesia and balanced anesthesia). Following propensity score matching analysis, 2582 patients were included in each group. Pain scores based on numeric rating scales (NRS) were compared between the 2 groups at the postoperative anesthetic care unit and at the ward until 3 days postoperation. Incidences of postoperative complications, such as nausea, itching, and shivering were also compared. The estimated NRS pain score on the day of surgery was 5.08 (95% confidence interval [CI] 4.97-5.19) in group N patients and 6.73 (95% CI 6.65-6.80) in group R patients (P < 0.001). There were no statistically significant differences in NRS scores on postoperative days 1, 2, and 3 between the 2 groups. Postoperative nausea was less frequent in group R (31.4%) than in group N (53.5%) (P < 0.001). However, the incidence of itching was higher in group R (4.3%) than in group N (0.7%) (P < 0.001). Continuous infusion of remifentanil during general anesthesia can cause higher intensity of postoperative pain and more frequent itching than general anesthesia without remifentanil infusion immediately after thyroidectomy. Considering the advantages and disadvantages of continuous remifentanil infusion, volatile anesthesia without opioid may be a good choice for minor surgeries, such as thyroidectomy.

Comparison of Acute Kidney Injury After Robot-Assisted Laparoscopic Radical Prostatectomy Versus Retropubic Radical Prostatectomy: A Propensity Score Matching Analysis.

Acute kidney injury (AKI) is associated with extended hospital stay, a high risk of progressive chronic kidney diseases, and increased mortality. Patients undergoing radical prostatectomy are at increased risk of AKI because of intraoperative bleeding, obstructive uropathy, older age, and preexisting chronic kidney disease. In particular, robot-assisted laparoscopic radical prostatectomy (RALP), which is in increasing demand as an alternative surgical option for retropubic radical prostatectomy (RRP), is associated with postoperative renal dysfunction because pneumoperitoneum during RALP can decrease cardiac output and renal perfusion. The objective of this study was to compare the incidence of postoperative AKI between RRP and RALP.We included 1340 patients who underwent RRP (n = 370) or RALP (n = 970) between 2013 and 2014. Demographics, cancer-related data, and perioperative laboratory data were evaluated. Postoperative AKI was determined according to the Kidney Disease: Improving Global Outcomes criteria. Operation and anesthesia time, estimated blood loss, amounts of administered fluids and transfused packed red blood cells, and the lengths of the postoperative intensive care unit and hospital stays were evaluated. Propensity score matching analysis was performed to reduce the influence of possible confounding variables and adjust for intergroup differences between the RRP and RALP groups.After performing 1:1 propensity score matching, the RRP and RALP groups included 307 patients, respectively. The operation time and anesthesia time in RALP were significantly longer than in the RRP group (both P < 0.001). However, the estimated blood loss and amount of administered fluids in RALP were significantly lower than in RRP (both P < 0.001). Also, RALP demonstrated a significantly lower incidence of transfusion and smaller amount of transfused packed red blood cells than RRP (both P < 0.001). Importantly, the incidence of AKI in RALP was significantly lower than in RRP (5.5% vs 10.4%; P = 0.044). Furthermore, the length of hospital stay in RALP was also significantly shorter (P < 0.001).The incidence of AKI after RALP is significantly lower than after RRP. RALP can therefore be a better surgical option than RRP in terms of decreasing the frequency of postoperative AKI.

Peripheral facial palsy, the only presentation of a primitive neuroectodermal tumor of the skull base.

INTRODUCTION: Peripheral facial palsy is rarely caused by primary neoplasms, which are mostly constituted of tumors of the central nervous system, head and neck, and leukemia. PRESENTATION OF CASE: A 2-month-old male infant presented with asymmetric facial expression for 3 weeks. Physical examination revealed suspicious findings of right peripheral facial palsy. Computed tomography of the temporal bone revealed a suspicious bone tumor centered in the right petrous bone involving surrounding bones with extension into the middle ear cavity and inner ear. Subtotal resection of the tumor was performed due to crucial structures adjacent the mass. Histopathology and immunohistochemistry of the resected tumor was consistent with primitive neuroectodermal tumor. CONCLUSION: We report a rare case of a primitive neuroectodermal tumor located at the skull base presenting with only peripheral facial palsy.

Iron deficiency anemia in infants and toddlers.

BACKGROUND: In Korea, the prevalence of anemia and iron deficiency anemia (IDA) among older infants and young children remains high. To detect IDA early and to reduce its adverse impact, we assessed the characteristics of infants and young children who had IDA or were at risk of developing IDA, or who exhibited characteristics associated with severe anemia. METHODS: Among the 1,782 IDA-affected children aged 6 months to 18 years who visited the hospital, we retrospectively analyzed the medical records and laboratory data of 1,330 IDA-affected children aged 6-23 months who were diagnosed between 1996 and 2013. We excluded patients with a C-reactive protein level ≥5 mg/dL. RESULTS: IDA was predominant in boys (2.14:1) during infancy and early childhood. The peak IDA incidence was noted among infants aged 9-12 months. Only 7% patients exhibited symptoms of IDA, while 23.6% patients with severe IDA demonstrated classic symptoms/signs of IDA. Low birth weight (LBW) infants with IDA demonstrated low adherence to iron supplementation. In a multivariate analysis, prolonged breastfeeding without iron fortification (odds ratio [OR] 5.70), and a LBW (OR 6.49) were identified as risk factors of severe anemia. CONCLUSION: LBW infants need more attention in order to increase their adherence to iron supplementation. For the early detection of IDA, nutritional status of all infants, and iron batteries of high-risk infants (LBW infants, infants with prolonged breastfeeding, picky eaters, and/or infants with the presence of IDA symptoms) should be evaluated at their health screening visits.

Photoprotective Potential of Penta-O-Galloyl-ß-DGlucose by Targeting NF-κB and MAPK Signaling in UVB Radiation-Induced Human Dermal Fibroblasts and Mouse Skin.

Exposure of the skin to ultraviolet radiation can cause skin damage with various pathological changes including inflammation. In the present study, we identified the skin-protective activity of 1,2,3,4,6-penta-O-galloyl-ß-D-glucose (pentagalloyl glucose, PGG) in ultraviolet B (UVB) radiation-induced human dermal fibroblasts and mouse skin. PGG exhibited antioxidant activity with regard to intracellular reactive oxygen species (ROS) generation as well as ROS and reactive nitrogen species (RNS) scavenging. Furthermore, PGG exhibited anti-inflammatory activity, inhibiting the activation of nuclear factor-kappaB (NF-κB) and mitogen-activated protein kinase (MAPK) signaling, resulting in inhibition of the expression of pro-inflammatory mediators. Topical application of PGG followed by chronic exposure to UVB radiation in the dorsal skin of hairless mice resulted in a significant decrease in the progression of inflammatory skin damages, leading to inhibited activation of NF-κB signaling and expression of pro-inflammatory mediators. The present study demonstrated that PGG protected from skin damage induced by UVB radiation, and thus, may be a potential candidate for the prevention of environmental stimuli-induced inflammatory skin damage.

Retrospective study of epidural blood patch use for spontaneous intracranial hypotension.

BACKGROUND AND OBJECTIVES: Spontaneous intracranial hypotension (SIH) is characterized by a severe and disabling headache that is usually orthostatic in nature. Cisternography is a useful diagnostic test for evaluating the presence and location of cerebrospinal fluid (CSF) leakage, and a targeted epidural blood patch (EBP) based on the cisternography findings is a very effective treatment modality for SIH. However, the effects of EBPs are not predictable, making repeat EBPs essential in some cases. The aim of the present study was to find the relationship between the EBP response and cisternographic findings, hypothesizing that the number of required EBPs would increase with an increased number of CSF leakage levels as determined by radionuclide cisternography. METHODS: All patients who underwent an EBP and had been discharged with significant improvements in symptoms of SIH during 2006 to 2011 were enrolled. Patients who had no radionuclide cisternographic results were excluded. The demographic variables, number of EBPs, cisternographic findings (location, bilaterality, and number of leakage sites), and preprocedural and postprocedural pain scores were reviewed. RESULTS: There was no correlation found between the cisternographic findings and the number of EBPs. Only the preprocedural pain scores showed a statistically significant correlation with the number of EBPs. CONCLUSIONS: Our study suggests that the response to the EBP is related to the severity of symptoms but not to the number and locations of cisternographic CSF leakages.