Effect of gonadotropin-releasing hormone agonist treatment on near final height in girls with central precocious puberty and early puberty

Purpose@#The aim of this study was to examine whether gonadotropin-releasing hormone (GnRH) agonist treatment is effective in preserving final height in patients with central precocious puberty (CPP) or early puberty (EP). @*Methods@#The medical records of 40 patients with CPP and 206 patients with EP who completed GnRH agonist treatment following diagnosis were analyzed retrospectively. Height and height standard deviation (height SDS) scores based on bone age (BA) were measured and calculated at baseline, after treatment completion, and at final follow-up to compare changes within and between groups. Predicted adult height (PAH) was estimated by the height corresponding to height SDS for BA in girls at 18 years 11 months of age based on the growth chart. @*Results@#PAH at baseline did not differ significantly between the CPP group (153.67±4.95) and the EP group (154.77±3.72). In the CPP group, PAH significantly increased at treatment completion (156.01±4.61) and at final follow-up (158.52±6.04) compared to baseline. In the EP group, PAH significantly increased at treatment completion (157.7±3.60) and at final follow-up (159.31±4.26) compared to baseline. The increase in PAH at all timepoints compared to baseline did not significantly differ between the CPP and EP groups. @*Conclusion@#Both CPP and EP groups had significantly greater PAH after treatment, with no difference in the amount of increase between groups. These results show that GnRH agonist treatment can help increase final height even in patients diagnosed with EP after the age of 8 years.

Renal Infarction in Vascular Ehlers-Danlos Syndrome / 대한내과학회지

Renal infarction is a condition caused by sudden disturbance of renal arterial blood flow, which occurs frequently in cardioembolic disease, renal artery injury (trauma, Marfan syndrome, rarely Ehlers-Danlos syndrome), and in association with a hypercoagulable state. Ehlers-Danlos syndrome is a rare hereditary connective tissue disease characterized by skin fragility, hyperelasticity, hypermobility of small joints, and easy bruising. Among the subtypes, vascular Ehlers-Danlos syndrome is a very rare genetic disease caused by a mutation in the COL3A1 gene. As blood vessels are fragile due dysfunctional collagen synthesis, complications of bleeding due to arterial dissection or rupture are common in patients with this disease. Here, we report a case of vascular Ehlers-Danlos syndrome diagnosed based on a renal infarction caused by thrombus and renal artery injury; we also present a review of the relevant literature.

Nutcracker Syndrome with Thin Basement Membrane Disease: A Case Report / 대한내과학회지

Nutcracker syndrome is caused by external compression of the left renal vein and is characterized by various symptoms, such as hematuria and left flank pain. However, long-standing gross hematuria is uncommon. We report the case of a 23-year-old woman who had had painless gross hematuria for several months. Kidney biopsy revealed thin basement membrane disease, but this did not explain the gross hematuria with proteinuria. Renal venography and Doppler ultrasonography showed increased diameter and velocity ratios between the left renal vein and inferior vena cava. Cystoscopy revealed that the hematuria originated from the left ureteral orifice. These results indicate coexisting Nutcracker syndrome. Nutcracker syndrome with thin basement membrane disease should be considered in the differential diagnosis of a patient who has long-standing gross hematuria.

The association of pancreatic cancer incidence with smoking status and smoking amount in Korean men / 한국역학회지

OBJECTIVES@#Our study examined the dose-response relationship between smoking amounts (pack-years) and the risk of developing pancreatic cancer in Korean men. @*METHODS@#Of 125,743 participants who underwent medical health checkups in 2009, 121,408 were included in the final analysis and observed for the development of pancreatic cancer. We evaluated the associations between smoking amounts and incident pancreatic cancer in 4 groups classified by pack-year amounts. Cox proportional hazards models were used to estimate the adjusted hazard ratios (HRs) and 95% confidence intervals (CIs) of incident pancreatic cancer by comparing groups 2 (40 pack-year smokers) with group 1 (never smokers). @*RESULTS@#During 527,974.5 person-years of follow-up, 245 incident cases of pancreatic cancer developed between 2009 and 2013. The multivariate-adjusted HRs (95% CIs) for incident pancreatic cancer in groups 2, 3, and 4 were 1.05 (0.76 to 1.45), 1.28 (0.91 to 1.80), and 1.57 (1.00 to 2.46), respectively (p for trend=0.025). The HR (95% CI) of former smokers showed a dose-response relationship in the unadjusted model, but did not show a statistically significant association in the multivariate-adjusted model. The HR (95% CI) of current smokers showed a dose-response relationship in both the unadjusted (p for trend=0.020) and multivariate-adjusted models (p for trend=0.050). @*CONCLUSIONS@#The risk of developing pancreatic cancer was higher in current smokers status than in former smokers among Korean men, indicating that smoking cessation may have a protective effect.

Relationship between seafood consumption and bisphenol A exposure: the Second Korean National Environmental Health Survey (KoNEHS 2012–2014)

BACKGROUND: This study aimed to identify the relationship between exposure to bisphenol A (BPA) and seafood consumption using a nationally representative data of the general Korean population.METHODS: This study was conducted on 5,402 adults aged 19 years and older (2,488 men, 2,914 women) based on the second Korean National Environmental Health Survey (2012–2014). We stratified the data according to gender and analyzed urinary BPA concentrations in terms of sociodemographic characteristics, health behavior, dietary factor, and seafood consumption. In the high and low BPA exposure groups, the odds ratios (ORs) were calculated using logistic regression analysis according to the top 75th percentile concentration.RESULTS: In men, large fish and tuna and other seafood categories had significantly higher ORs before and after adjustment in the group who consumed seafood more than once a week than in the group who rarely consumed seafood, with an adjusted value of 1.97 (95% confidence interval [CI]: 1.12–3.48) and 1.74 (95% CI: 1.10–2.75), respectively. In the shellfish category, the unadjusted OR was 1.61 (95% CI: 1.00–2.59), which was significantly higher in the group who consumed seafood more than once a week than in the group who rarely consumed seafood. However, the OR after adjusting for the variables was not statistically significant. In women, the frequency of seafood consumption and the concentration of urinary BPA were not significantly associated.CONCLUSIONS: BPA concentration was higher in men who frequently consumed large fish and tuna, shellfish and other seafood in this study.

Relationship between seafood consumption and bisphenol A exposure: the Second Korean National Environmental Health Survey (KoNEHS 2012–2014)

BACKGROUND@#This study aimed to identify the relationship between exposure to bisphenol A (BPA) and seafood consumption using a nationally representative data of the general Korean population.@*METHODS@#This study was conducted on 5,402 adults aged 19 years and older (2,488 men, 2,914 women) based on the second Korean National Environmental Health Survey (2012–2014). We stratified the data according to gender and analyzed urinary BPA concentrations in terms of sociodemographic characteristics, health behavior, dietary factor, and seafood consumption. In the high and low BPA exposure groups, the odds ratios (ORs) were calculated using logistic regression analysis according to the top 75th percentile concentration.@*RESULTS@#In men, large fish and tuna and other seafood categories had significantly higher ORs before and after adjustment in the group who consumed seafood more than once a week than in the group who rarely consumed seafood, with an adjusted value of 1.97 (95% confidence interval [CI]: 1.12–3.48) and 1.74 (95% CI: 1.10–2.75), respectively. In the shellfish category, the unadjusted OR was 1.61 (95% CI: 1.00–2.59), which was significantly higher in the group who consumed seafood more than once a week than in the group who rarely consumed seafood. However, the OR after adjusting for the variables was not statistically significant. In women, the frequency of seafood consumption and the concentration of urinary BPA were not significantly associated.@*CONCLUSIONS@#BPA concentration was higher in men who frequently consumed large fish and tuna, shellfish and other seafood in this study.

Relationship between seafood consumption and bisphenol A exposure: the Second Korean National Environmental Health Survey (KoNEHS 2012–2014)

BACKGROUND@#This study aimed to identify the relationship between exposure to bisphenol A (BPA) and seafood consumption using a nationally representative data of the general Korean population.@*METHODS@#This study was conducted on 5,402 adults aged 19 years and older (2,488 men, 2,914 women) based on the second Korean National Environmental Health Survey (2012–2014). We stratified the data according to gender and analyzed urinary BPA concentrations in terms of sociodemographic characteristics, health behavior, dietary factor, and seafood consumption. In the high and low BPA exposure groups, the odds ratios (ORs) were calculated using logistic regression analysis according to the top 75th percentile concentration.@*RESULTS@#In men, large fish and tuna and other seafood categories had significantly higher ORs before and after adjustment in the group who consumed seafood more than once a week than in the group who rarely consumed seafood, with an adjusted value of 1.97 (95% confidence interval [CI]: 1.12–3.48) and 1.74 (95% CI: 1.10–2.75), respectively. In the shellfish category, the unadjusted OR was 1.61 (95% CI: 1.00–2.59), which was significantly higher in the group who consumed seafood more than once a week than in the group who rarely consumed seafood. However, the OR after adjusting for the variables was not statistically significant. In women, the frequency of seafood consumption and the concentration of urinary BPA were not significantly associated.@*CONCLUSIONS@#BPA concentration was higher in men who frequently consumed large fish and tuna, shellfish and other seafood in this study.

A Novel c.6766_6767insAA Mutation in the Neurofibromin Gene in a Patient with Neurofibromatosis Type 1-Associated Glioblastoma / 대한소아신경학회지

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous syndrome caused by mutations in the neurofibromin gene. NF-1 patients have a high risk of tumors, and optic glioma is the most commonly observed central nervous system tumor in these patients. However, glioblastoma is extremely rare in pediatric NF-1 patients. Here we report the discovery of a novel heterozygous c.6766_6767insAA (p.Ser2256Lysfs*4), pathogenic mutation in the neurofibromin gene in a 17-year-old boy with NF-1-associated glioblastoma.