Alport syndrome, a rare genetic disorder affecting around 1 in 50,000 individuals, primarily presents as microscopic hematuria and chronic kidney disease (CKD) with associated extrarenal complications. The Alport syndrome results from mutations in COL4A3, COL4A4, and COL4A5 genes, disrupting the formation of the α3–α4–α5 chain in the collagen IV network. The etiology involves X chromosome- related, autosomal dominant, autosomal recessive, and digenic inheritance patterns. The disease primarily manifests as kidney involvement, featuring persistent hematuria, proteinuria, and a progressive decline in renal function. Hearing loss, ocular abnormalities, and extrarenal manifestations further contribute to its complexity. Genotype-phenotype correlations are relatively evident, with distinct presentations in X-linked, autosomal recessive, and autosomal dominant cases. Diagnosis relies on urinalysis, histologic examination, and genetic testing with advancements in next-generation sequencing aiding identification. Although no specific treatment exists, early diagnosis improves outcomes, emphasizing the importance of genetic testing for prognosis and familial screening. The purpose of this review is to advance knowledge and enhance understanding of Alport syndrome.

Purpose: To present a case of oculomotor nerve atrophy complicated by herpes zoster ophthalmicus (HZO).Case summary: A 68-year-old man with a history of type 2 diabetes and dyslipidemia complained of diplopia and deviation in left eye. On examination, visual acuity was 20/25 in right eye and 20/20 in left eye. Prism and alternate cover tests revealed right exotropia and hypertropia in the primary position. Examination of ductions and versions showed limitation of adduction and depression in the right eye. On detailed history taking, we found that his symptoms started after suffering HZO on right side about 2 years ago. He was prescribed antiviral agents from a dermatology clinic and was told that all symptoms would improve after medication so he didn't undergo an ophthalmologic examination at that time. Magnetic resonance imaging confirmed that diffuse atrophy of oculomotor nerve in cistern along with atrophic changes of medial rectus and inferior rectus muscles in right eye. A diagnosis of strabismus complicated by HZO-induced oculomotor nerve atrophy was done. Conclusions Ophthalmoplegia secondary to HZO may not be self-limiting and have a poor prognosis resulting in cranial nerve atrophy. A detailed imaging investigation of extraocular muscle and cranial nerves along with thorough history taking are required for proper diagnosis and better prognosis.

Background: Due to the importance of the cold chain related to vaccine transportation, it is necessary to establish a blood cold chain operation strategy by checking the status of blood transportation from blood donation to transfusion. Methods: The blood transportation system and blood storage facilities were checked by inspecting the Korean Red Cross (KRC) Blood Centers and its affiliated supplier and Hanmaeum Blood Center. The status of the cold chain was evaluated through an interview with the quality control manager or blood supply team leader. For the hospital, the status was investigated from the perspective of the 30-min rule. A questionnaire survey was conducted for medical technicians and nurses working at the hospital. Results: Data on temperature during transport of blood components were computerized, and all standards were met. A nationwide network that could supply blood from the blood supply center to the hospitals within 2 hours was established. The frequency of temperature monitoring in the blood transport box and constant temperature check in the transport box during long-distance transport were evaluated. Conclusion This study confirmed that blood storage and transportation in Korea complied with the cold chain standards of high-income countries or higher. The evaluation of the cold chain is a constantly evolving process requiring continuous attention. When standards for storage and transportation of blood products are established, strict regulations and examinations will be required.

The variant terminal trunk of the superior mesenteric artery (SMA) could be confused with the ileocolic artery (ICA) as it runs on the right side of the superior mesenteric vein. If the variant ileal branch of SMA is mistaken for the ICA, unintentional ligation could cause long-segment ischemia in the ileum. We encountered a rare case of ileal ischemia caused by unintentional ligation of the variant ileal branch of the SMA during laparoscopic right hemicolectomy, which was confirmed by indocyanine green (ICG) angiography and hyperspectral imaging (HSI). Intraoperative real-time perfusion monitoring using ICG angiography and tissue oxygen saturation monitoring using HSI could help detect segments of hypoperfusion and prevent hypoperfusion-related anastomotic complications.

Treatment for early colon cancer has progressed rapidly with endoscopic resection and minimally invasive surgery. Selection of patients without risk of lymph node metastasis is necessary before deciding on endoscopic resection for early colon cancer treatment. We aimed to review the optimal multidisciplinary treatment strategies for early colon cancer, including endoscopy and surgery.Current Concepts: Pathological risk factors include histologic grade of cancer cell differentiation, lymphovascular invasion, perineural invasion, tumor budding, and deep submucosal invasion. These risk factors for predicting lymph node metastasis are crucial for determining the treatment strategy of endoscopic excision and radical resection for early colon cancer. Prediction of the depth of invasion in early colon cancer using endoscopic optical assessments is vital to determine the appropriate treatment method for endoscopic or surgical resection. Furthermore, optical assessment of pit and vascular patterns is useful for estimating the depth of submucosal invasion using magnifying chromoendoscopy and narrow-band imaging endoscopy. Performing an endoscopic and pathologic evaluation of the risk factors for lymph node metastasis is imperative when selecting endoscopic or surgical resection. Endoscopic treatments include cold snare polypectomy, endoscopic mucosal resection, and endoscopic submucosal dissection. In addition, appropriate surgical treatment should be recommended for patients with early colon cancer with a high risk of lymph node metastasis.Discussion and Conclusion: A multidisciplinary approach should be recommended to establish an optimized treatment strategy, minimize the risk of complications, and obtain excellent oncologic outcomes via patienttailored treatment in patients with early colon cancer.

Background: Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the cognitive function of children and adolescents with CKD. This study therefore aimed to investigate the mean intelligence and risk factors for low intelligence in children and adolescents with CKD. Methods: Eighty-one patients with CKD under 18 years old were included in the KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease (KNOW-Ped CKD). Participants completed either the Wechsler Intelligence Scale for Children (6–16 years), or Wechsler Adult Intelligence Scale (> 16 years). Results: The mean full-scale intelligence quotient (IQ) was 91 ± 19; 24.7% of participants scored a full-scale IQ below 80. Participants with a short stature (height Z scores < −1.88), failure to thrive (weight Z scores < −1.65), more severe CKD stage (≥ IIIb), longer duration of CKD (≥ 5 years), and those who were Medicare or Medicaid beneficiaries, had significantly lower mean full-scale IQs. Conclusion On linear regression analysis, the association between the full-scale IQ, and longer duration of CKD and growth failure, remained significant after controlling for demographic and clinical variables. It is therefore necessary to investigate cognitive impairment in pediatric patients with CKD who exhibit growth failure or for a longer postmorbid period. It is believed that early interventions, such as kidney transplantation, will have a positive effect on IQ in children with CKD, as the disease negatively affects IQ due to poor glomerular filtration rate over time.

Background: Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the cognitive function of children and adolescents with CKD. This study therefore aimed to investigate the mean intelligence and risk factors for low intelligence in children and adolescents with CKD. Methods: Eighty-one patients with CKD under 18 years old were included in the KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease (KNOW-Ped CKD). Participants completed either the Wechsler Intelligence Scale for Children (6–16 years), or Wechsler Adult Intelligence Scale (> 16 years). Results: The mean full-scale intelligence quotient (IQ) was 91 ± 19; 24.7% of participants scored a full-scale IQ below 80. Participants with a short stature (height Z scores < −1.88), failure to thrive (weight Z scores < −1.65), more severe CKD stage (≥ IIIb), longer duration of CKD (≥ 5 years), and those who were Medicare or Medicaid beneficiaries, had significantly lower mean full-scale IQs. Conclusion On linear regression analysis, the association between the full-scale IQ, and longer duration of CKD and growth failure, remained significant after controlling for demographic and clinical variables. It is therefore necessary to investigate cognitive impairment in pediatric patients with CKD who exhibit growth failure or for a longer postmorbid period. It is believed that early interventions, such as kidney transplantation, will have a positive effect on IQ in children with CKD, as the disease negatively affects IQ due to poor glomerular filtration rate over time.

Streptococcus mutans and Streptococcus sobrinus play important roles in dental caries. Coptis chinensis is a natural product with antimicrobial activity against enterobacteria; however, its effects on oral streptococci are still unknown.Therefore, the effects of C. chinensis on the growth and biofilm formation of the representative cariogenic bacteria S. mutans and S. sobrinus were investigated for the possible use of C. chinensis as an anticaries agent. The C.chinensis extract was diluted with sterile distilled water, and 0.1–2.5% of the extract was used in the experiment. The effects of the C. chinensis extract on the growth and glucan formation of S. mutans and S. sobrinus were measured by viable cell counting and spectrophotometry at 650 nm absorbance, respectively. Crystal violet staining was also carried out to confirm the C. chinensis extract’s inhibitory effect on biofilm formation. The C. chinensis extract significantly inhibited the growth of S. mutans and S. sobrinus at concentrations of ≥ 0.3% as compared with the control group. The viable cell count of colonies decreased by 1.7-fold and 1.2-fold at 2.5% and 1.25%, respectively, compared with the control group. The biofilm formation of S. mutans and S. sobrinus was inhibited by > 20-fold at C.chinensis extract concentrations of ≥ 1.25% as compared with the control group. In summary, the C. chinensis extract inhibited the growth and biofilm and glucan formation of S. mutans and S. sobrinus. Therefore, C. chinensis might be a potential candidate for controlling dental caries.

A total of 24 SD rats were allotted to four treatment groups such as the control (CON), 1% of cholesterol diet (CHO), 0.5% of coenzyme Q₁₀ (COQ) and 1% of cholesterol plus 0.5% of coenzyme Q₁₀ (CHCQ) groups to determine the effects of coenzyme Q₁₀ (CoQ₁₀) on the antioxidant defense system in rats. The body weight, weight gain, liver weight and abdominal fat pads were unaffected by 0.5% of CoQ₁₀ supplement in the rats. The level of triglyceride and HDL-cholesterol levels in the blood was significantly increased (p< 0.05) by the 1% of cholesterol supplement (CHO), whereas 0.5% of CoQ₁₀ supplement (COQ) did not alter these blood lipid indices. In the mRNA expression, there was a significant effect (P < 0.05) of the CoQ₁₀ supplement on the mRNA expression of superoxide dismutase (SOD), although the mRNA expression of glutathione peroxidase (GPX) and glutathione S-transferase (GST) was unaffected by cholesterol or the CoQ₁₀ supplement. Similar to mRNA expression of SOD, its activity was also significantly increased (P < 0.05) by CoQ₁₀, but not by the cholesterol supplement effect. The activities hepatic GPX and GST were unaffected by CoQ₁₀ and cholesterol supplements in rats. Lipid peroxidation in the CHO group resulted in a significant (p< 0.05) increase compared with that in the other groups, indicating that the CoQ₁₀ supplement to 1% of cholesterol-fed rats alleviated the production of lipid peroxidation in the liver. In conclusion, 0.5% of the CoQ₁₀ supplement resulted in positive effects on the hepatic antioxidant defense system without affecting blood lipid indices in 1% of cholesterol fed rats.