Background: Mass spectrometry (MS) methods exhibit higher accuracy and comparability in measuring serum C-peptide concentrations than immunoassays. We developed and validated a novel isotope dilution-ultraperformance liquid chromatography-tandem MS (IDUPLC-MS/MS) assay to measure serum C-peptide concentrations. Methods: Sample pretreatment involved solid-phase extraction, ion-exchange solid-phase extraction, and derivatization with 6-aminoquinolyl-N-hydroxysuccinimidylcarbamate (Cayman Chemical, Ann Arbor, Michigan, USA). We used an ExionLC UPLC system (Sciex, Framingham, MA, USA) and a Sciex Triple Quad 6500 + MS/MS system (Sciex) for electrospray ionization in positive-ion mode with multiple charge states of [M+3H]3+ and multiple reaction monitoring transitions. The total run time was 50 mins, and the flow rate was 0.20 mL/min. We evaluated the precision, trueness, linearity, lower limit of quantitation (LLOQ), carryover, and matrix effects. Method comparison with electrochemiluminescence immunoassay (ECLIA) was performed in 138 clinical specimens. Results: The intra- and inter-run precision coefficients of variation were < 5% and the bias values for trueness were < 4%, which were all acceptable. The verified linear interval was 0.050–15 ng/mL, and the LLOQ was 0.050 ng/mL. No significant carryover or matrix effects were observed. The correlation between this ID-UPLC-MS/MS method and ECLIA was good (R = 0.995, slope = 1.564); however, the ECLIA showed a positive bias (51.8%). Conclusions The developed ID-UPLC-MS/MS assay shows acceptable performance in measuring serum C-peptide concentrations. This will be useful in situations requiring accurate measurement of serum C-peptide in clinical laboratories.

Constipation is a common gastrointestinal disorder and is often accompanied by alteration in the gut microbiota. Recently, several studies have disclosed its association with an increased risk of cardiovascular disease and mortality in the general population. Despite the high prevalence of constipation, data on the clinical impact of constipation in patients with chronic kidney disease are limited. We aimed to explore the prevalence of constipation and its association with cardiovascular disease in chronic kidney disease using a nationally representative cohort of hemodialysis patients. Methods: This study used hemodialysis quality assessment and health insurance claims data from patients undergoing maintenance hemodialysis in South Korea. Chronic constipation was defined using the total number of laxatives prescribed during the 1-year baseline period. The primary outcome was a composite of acute ischemic stroke, hemorrhagic stroke, myocardial infarction, or all-cause death. Secondary outcomes were the individual components of the primary outcome. Results: Among 35,230 patients on hemodialysis, 9,133 (25.9%) were identified as having constipation. During a median follow-up of 5.4 years, patients with constipation had a 15% higher incidence of the composite outcome, 16% higher incidence of ischemic stroke, and 14% higher all-cause mortality, after multivariate adjustment. Conclusion: Chronic constipation requiring laxatives was associated with a higher risk of the composite outcome of cardiovascular events or all-cause death in patients on hemodialysis. Further studies are needed to confirm whether constipation is an independent predictor or a possible causal factor of cardiovascular disease.

Primary aldosteronism is a group of disorders in which the autonomous secretion of aldosterone is associated with hypertension and hypokalemia. It is crucial to determine the laterality of aldosterone hypersecretion because treatment options differ accordingly. Adrenal venous sampling (AVS) is considered the most reliable method for assessing the laterality of primary aldosteronism. This procedure is often technically challenging because of the small size and varied locations of the adrenal veins. A better understanding of anatomical variations and careful review of imaging studies would improve sampling success. This report presents three cases of anatomical variations encountered during AVS.

Purpose: Pregnant women with diabetes mellitus (DM) and their babies are affected by complications and congenital abnormalities during pregnancy. This study aimed to examine the risk factors for cardiac anomalies and the characteristics of pregestational DM and GDM in mothers and babies in Korea. Methods: This retrospective study used medical records from Bucheon St. Mary's Hospital between January 2013 and July 2021. We studied 1983 infants, with 335 neonates from mothers with DM. Results: Body weight and body mass index were high in mothers with pregestational DM at delivery (p<0.005). The rate of preterm births was lower in the pregestational DM group than in the GDM group. Furthermore, the cardiac anomaly rate was high in the pregestational DM group (p<0.001). The rate of hyperbilirubinemia in the DM group was higher than that in the control group (p<0.001). Multivariate logistic regression analysis revealed no significant risk factors for congenital heart disease. Conclusion DM in pregnant women is associated with congenital anomalies. Therefore, the appropriate management of mothers with DM is important for the prognosis of their neonates.

Branchio-oto-renal (BOR) Syndrome is a rare autosomal dominant genetic disorder characterized by features such as branchial arch anomalies, hearing loss, preauricular fistula, auricular malformations, and renal abnormalities. Also, BOR syndrome without renal anomalies is referred to as branchio-otic syndrome. In this report, we present a case of a 50-year-old female patient with bilateral type 2 branchial arch anomalies, both preauricular fistula, bilateral inner ear malformations, bilateral sensorineural hearing loss, and right auricular malformation. The patient underwent surgical treatment. This report is the first documentation in Korea of auricular malformation in BOR syndrome, where the patient’s family exhibited a typical autosomal dominant inheritance pattern.

Primary aldosteronism is a group of disorders in which the autonomous secretion of aldosterone is associated with hypertension and hypokalemia. It is crucial to determine the laterality of aldosterone hypersecretion because treatment options differ accordingly. Adrenal venous sampling (AVS) is considered the most reliable method for assessing the laterality of primary aldosteronism. This procedure is often technically challenging because of the small size and varied locations of the adrenal veins. A better understanding of anatomical variations and careful review of imaging studies would improve sampling success. This report presents three cases of anatomical variations encountered during AVS.

Purpose: Pregnant women with diabetes mellitus (DM) and their babies are affected by complications and congenital abnormalities during pregnancy. This study aimed to examine the risk factors for cardiac anomalies and the characteristics of pregestational DM and GDM in mothers and babies in Korea. Methods: This retrospective study used medical records from Bucheon St. Mary's Hospital between January 2013 and July 2021. We studied 1983 infants, with 335 neonates from mothers with DM. Results: Body weight and body mass index were high in mothers with pregestational DM at delivery (p<0.005). The rate of preterm births was lower in the pregestational DM group than in the GDM group. Furthermore, the cardiac anomaly rate was high in the pregestational DM group (p<0.001). The rate of hyperbilirubinemia in the DM group was higher than that in the control group (p<0.001). Multivariate logistic regression analysis revealed no significant risk factors for congenital heart disease. Conclusion DM in pregnant women is associated with congenital anomalies. Therefore, the appropriate management of mothers with DM is important for the prognosis of their neonates.

Although advances in neonatology have reduced the mortality rate of high-risk infants, sick newborns or pre-mature infants undergo more intensive monitoring, pain-ful procedures, and lengthy hospitalization, leading to pro-longed separation from their parents. In recent decades, the importance of parent-infant closeness early in life has become more apparent, especially in preterm infants who are prone to neurodevelopmental deficits. There is an increasing body of evidence regarding the benefits of family-centered care (FCC) in neonatal intensive care units. Key aspects related to neonatal FCC include the parents’ presence in the ward and their participation in infants’ daily care and decision-making processes. In addition, an environment that supports a private and comfortable space for each family member and infant, such as a single-family room, should be provided. To successfully implement FCC in neonatal intensive care units, the culture of care and hospital policies should be changed to successfully implement FCC in neonatal intensive care units, and appropriate training for medical staff is also required.