Comparisons of clinical outcomes between hypertensive and normotensive living kidney donors: a prospective, multicenter nationwide cohort study.

Background: Living kidney donors with hypertension are potential candidates for solving the donor shortages in renal transplantation. However, the safety of donors with hypertension after nephrectomy has not been sufficiently confirmed. Methods: A total of 642 hypertensive and 4,848 normotensive living kidney donors who were enrolled in the Korean Organ Transplantation Registry between May 2014 and December 2020 were included in this study. The study endpoints were a decreased estimated glomerular filtration rate (eGFR) and proteinuria. Results: In the entire cohort, donors with hypertension had a lower eGFR before nephrectomy in comparison to normotensive donors which remained lower after kidney transplantation. The incidence of proteinuria in hypertensive donors increased during follow-up. In propensity score-matched analysis, the risk of eGFR being <60 mL/min/1.73 m2 (hazard ratio [HR], 0.77; 95% confidence interval [CI], 0.50-1.19) or <45 mL/min/1.73 m2 (HR, 0.50; 95% CI, 0.06-4.03) was not significantly increased in donors with hypertension. However, hypertensive donors were found to have a significantly higher risk of proteinuria than normotensive donors (HR, 2.28; 95% CI, 1.05-4.94). Similar findings were also observed in the analysis of the entire cohort, indicating that hypertensive donors had a significantly higher risk of proteinuria (adjusted HR, 1.77; 95% CI, 1.10-2.85), without a substantial increase in the risk of decreased renal function. Conclusion: The risk of proteinuria after donation was substantially increased in donors with hypertension. These findings underscore the need for careful monitoring of proteinuria in hypertensive donors following donation.

Small graft size and hepatocellular carcinoma outcomes in living donor liver transplantation: a retrospective multicentric cohort study.

INTRODUCTION: This study examined associations between the graft-to-recipient weight ratio (GRWR) for adult-to-adult living donor liver transplantation (LDLT) and hepatocellular carcinoma (HCC) outcomes. MATERIALS AND METHODS: Data from patients in the Korean Organ Transplantation Registry who underwent LDLT for HCC from 2014 to 2021 were retrospectively reviewed. Patients were categorized using the cutoff GRWR for HCC recurrence determined by an adjusted cubic spline (GRWR <0.7% vs. GRWR ≥0.7%). Recurrence-free survival (RFS) and HCC recurrence were analyzed in the entire and a 1:5 propensity-matched cohort. RESULTS: The eligible cohort consisted of 2005 LDLT recipients [GRWR <0.7 ( n =59) vs. GRWR ≥0.7 ( n =1946)]. In the entire cohort, 5-year RFS was significantly lower in the GRWR <0.7 than in the GRWR ≥0.7 group (66.7% vs. 76.7%, P =0.019), although HCC recurrence was not different between groups (77.1% vs. 80.7%, P =0.234). This trend was similar in the matched cohort ( P =0.014 for RFS and P =0.096 for HCC recurrence). In multivariable analyses, GRWR <0.7 was an independent risk factor for RFS [adjusted hazard ratio (aHR) 1.89, P =0.012], but the result was marginal for HCC recurrence (aHR 1.61, P =0.066). In the pretransplant tumor burden subgroup analysis, GRWR <0.7 was a significant risk factor for both RFS and HCC recurrence only for tumors exceeding the Milan criteria (aHR 3.10, P <0.001 for RFS; aHR 2.92, P =0.003 for HCC recurrence) or with MoRAL scores in the fourth quartile (aHR 3.33, P <0.001 for RFS; aHR 2.61, P =0.019 for HCC recurrence). CONCLUSIONS: A GRWR <0.7 potentially leads to lower RFS and higher HCC recurrence after LDLT when the pretransplant tumor burden is high.

Accidental ABO-incompatible pediatric liver transplantation with blood group antigen immune and operational tolerance: a case report with 21 years of follow-up.

Liver transplantation is a critical procedure for patients with end-stage liver disease, but it is often hindered by ABO-incompatibility between the donor and recipient, which can lead to immediate humoral rejection. We present a unique case involving a 10-month-old patient who, by accident, received an ABO-incompatible partial liver transplant from a type A mother without undergoing desensitization. Remarkably, during a 21-year follow-up period, the patient exhibited no signs of humoral or graft rejection, despite nonadherence to medication. This case highlights the possibility of dual tolerance in pediatric ABO-incompatible liver transplantation and provides insights into immune tolerance mechanisms, with implications for enhancing patient care and reducing healthcare costs. Further research is necessary to clarify these mechanisms and to evaluate the long-term durability of tolerance in pediatric transplant recipients.

The Association Between Tachycardia and Mortality in Septic Shock Patients According to Serum Lactate Level: A Nationwide Multicenter Cohort Study

Background@#This study aimed to evaluate whether the effect of tachycardia varies according to the degree of tissue perfusion in septic shock. @*Methods@#Patients with septic shock admitted to the intensive care units were categorized into the tachycardia (heart rate > 100 beats/min) and non-tachycardia (≤ 100 beats/min) groups. The association of tachycardia with hospital mortality was evaluated in each subgroup with low and high lactate levels, which were identified through a subpopulation treatment effect pattern plot analysis. @*Results@#In overall patients, hospital mortality did not differ between the two groups (44.6% vs. 41.8%, P = 0.441), however, tachycardia was associated with reduced hospital mortality rates in patients with a lactate level ≥ 5.3 mmol/L (48.7% vs. 60.3%, P = 0.030; adjusted odds ratio [OR], 0.59, 95% confidence interval [CI], 0.35–0.99, P = 0.045), not in patients with a lactate level < 5.3 mmol/L (36.5% vs. 29.7%, P = 0.156; adjusted OR, 1.39, 95% CI, 0.82–2.35, P = 0.227). @*Conclusion@#In septic shock patients, the effect of tachycardia on hospital mortality differed by serum lactate level. Tachycardia was associated with better survival in patients with significantly elevated lactate levels.

Gastrointestinal AA Amyloidosis following Recurrent SARS-CoV-2 Infection: A Case Report

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has been associated with the overproduction of serum amyloid A protein, resulting in systemic AA amyloidosis. In this report, we describe a case of gastrointestinal (GI) AA amyloidosis following SARS-CoV-2 infection. A 75-year-old male presented to the emergency department with upper abdominal pain 6 weeks post kidney transplantation. He had a history of SARS-CoV-2 infection 4 weeks prior. On day 7 of hospitalization, while receiving conservative management, the patient developed symptoms of cough and fever, leading to a diagnosis of SARS-CoV-2 reinfection. The patient’s abdominal pain persisted, and hematochezia developed on day 30 of hospitalization. Esophagogastroduodenoscopy and colonoscopy revealed multiple ulcers in the stomach and colon, with histologic findings revealing the presence of amyloid A. The patient was managed conservatively and was also given remdesivir for the SARS-CoV-2 infection. His clinical symptoms subsequently improved, and endoscopic findings demonstrated improvement in multiple gastric ulcers. GI amyloidosis may be a subacute complication following SARS-CoV-2 infection in immunocompromised patients.

Antithrombotic therapy has no beneficial effect in conservative treatment of spontaneous isolated superior mesenteric arterial dissection

Purpose@#Initial conservative treatment with selective endovascular or surgical intervention has shown successful outcomes in the treatment of spontaneous isolated superior mesenteric artery dissection (SISMAD). However, the benefits of antithrombotic therapy as a part of conservative treatment have not been clarified. This study aimed to investigate the clinical course of SISMAD patients and determine differences in clinical outcomes between the antithrombotic and noantithrombotic groups. @*Methods@#We retrospectively reviewed 79 cases of SISMAD that were treated conservatively from January 2004 to December 2019 at Chonnam National University Hospital. Clinical outcomes, including the length of hospital stay, pain resolution time, image remodeling, and maximal remodeling time, were compared between the antithrombotic and noantithrombotic groups. @*Results@#There were 30 patients in the no-antithrombotic group and 49 patients in the antithrombotic group. There was no significant difference in clinical characteristics between the 2 groups, except for dyslipidemia (P = 0.011). The follow-up period (32.6 months vs. 14.6 months, P = 0.009) and imaging follow-up period (31.6 months vs. 13.9 months, P = 0.011) were longer in the antithrombotic group than in the no-antithrombotic group. The length of hospital stay (5.1 days vs. 7.7 days, P = 0.002) was significantly shorter in the no-antithrombotic group than in the antithrombotic group because patients in the antithrombotic group required longer hospitalization for warfarin titration. @*Conclusion@#In patients with SISMAD, conservative treatment without antithrombotic therapy may have clinical benefits such as decreased length of hospital stay compared with conservative treatment with antithrombotic therapy.

Prognostic Value of Admission Blood Glucose Level in Critically Ill Patients Admitted to Cardiac Intensive Care Unit according to the Presence or Absence of Diabetes Mellitus

BACKGROUND: Admission blood glucose (BG) level is a predictor of mortality in critically ill patients with various conditions. However, limited data are available regarding this relationship in critically ill patients with cardiovascular diseases according to diabetic status. METHODS: A total of 1,780 patients (595 with diabetes) who were admitted to cardiac intensive care unit (CICU) were enrolled from a single center registry. Admission BG level was defined as maximal serum glucose level within 24 hours of admission. Patients were divided by admission BG level: group 1 (< 7.8 mmol/L), group 2 (7.8–10.9 mmol/L), group 3 (11.0–16.5 mmol/L), and group 4 (≥ 16.6 mmol/L). RESULTS: A total of 105 patients died in CICU (62 non-diabetic patients [5.2%] and 43 diabetic patients [7.9%]; P = 0.105). The CICU mortality rate increased with admission BG level (1.7%, 4.8%, 10.3%, and 18.8% from group 1 to group 4, respectively; P < 0.001). On multivariable analysis, hypertension, mechanical ventilator, continuous renal replacement therapy, acute physiology and chronic health evaluation II (APACHE II) score, and admission BG level significantly influenced CICU mortality in non-diabetic patients (group 1 vs. group 3: hazard ratio [HR], 3.31; 95% confidence interval [CI], 1.47–7.44; P = 0.004; group 1 vs. group 4: HR, 6.56; 95% CI, 2.76–15.58; P < 0.001). However, in diabetic patients, continuous renal replacement therapy and APACHE II score influenced CICU mortality but not admission BG level. CONCLUSION: Admission BG level was associated with increased CICU mortality in critically ill, non-diabetic patients admitted to CICU but not in diabetic patients.

Colon perforation due to embolization coil for internal iliac aneurysm

Coil migration is an extremely rare but hazardous complication of aneurysmal coil embolization. Only 1 case report has described coil migration following endovascular exclusion to gastrointestinal (GI) tract. We report the experience of a case of colon penetration caused by embolization coil placed for internal iliac aneurysm. A 66-year-old man visited the Emergency Department for hematochezia that had persisted for 3 months. Stent insertion and coil embolization of left internal iliac artery aneurysm had been performed on the patient 18 months ago. Colonoscopy was performed. It suggested penetration of sigmoid colon by embolization coil and diverticulum. Angiography revealed extravasation of contrast media at left internal iliac artery. Covered stent deployment was done in the left internal iliac artery. One week after the stent insertion, the patient underwent anterior resection, aneurysm resection, and coil removal. The patient recovered without complications. He was discharged at 2 weeks after the operation.

Vascular Complications Related to Posterior Lumbar Disc Surgery

PURPOSE: To evaluate patients who underwent surgical or endovascular treatment after vascular injury related to posterior lumbar disc surgery. MATERIALS AND METHODS: We retrospectively reviewed seven cases of vascular injuries (four lacerations, one arteriovenous fistula [AVF], and two pseudoaneurysms) related to lumbar disc surgery by a posterior approach from January 1997 to December 2016 at Chonnam National University Hospital. Information of patient characteristics, diagnosis, treatment strategies, and outcomes were analyzed. RESULTS: Five out of seven cases were inhospital cases. In three laceration cases, each patient instantly became hypotensive and a life-threatening arterial injury was suspected. Therefore, the patient was immediately turned to the supine position and surgical repair was performed. The patients with pseudoaneurysm and AVF were treated by endovascular intervention. Remaining two were referred cases under the impression of vascular injuries. One laceration case of them was in preshock condition, and the left common iliac artery was surgically repaired. The other referred patient showed pseudoaneurysm which was treated with stent graft insertion. There was no surgery or endovascular intervention related death and none of the patients suffered any sequela related to vascular injury. CONCLUSION: Vascular injury associated with posterior lumbar disc surgery is not common, but can be fatal. Early recognition, diagnosis, and prompt treatment are essential to prevent fatal outcomes. Recently, endovascular intervention is increasingly and preferably used because of its low morbidity and mortality. However surgery is still the best option for the patients with unstable vital sign and endovascular approach can be applied to stable patients.

Optimal Management of Heart Failure with Preserve Ejection Fraction / 대한내과학회지

More than 50% of patients who are diagnosed with heart failure have preserved ejection fraction (HFpEF), and they have an equally poor prognosis when compared to patients with heart failure with reduced ejection fraction (HFrEF). However, a comprehensive understanding and awareness of heart failure with preserved ejection fraction is still limited and there are currently no optimized treatments to improve morbidity and mortality in these patients. This review summarizes the differences in the epidemiology, pathophysiology, diagnosis, and prognosis between HFpEF and HFrEF. We also review current management strategies of HFpEF patients according to evidence-based treatment guidelines.

Anatomical Features and Early Outcomes of Endovascular Repair of Abdominal Aortic Aneurysm from a Korean Multicenter Registry

PURPOSE: To introduce a nation-based endovascular aneurysm repair (EVAR) registry in South Korea and to analyze the anatomical features and early clinical outcomes of abdominal aortic aneurysms (AAA) in patients who underwent EVAR. MATERIALS AND METHODS: The Korean EVAR registry (KER) was a template-based online registry developed and established in 2009. The KER recruited 389 patients who underwent EVAR from 13 medical centers in South Korea from January 2010 to June 2010. We retrospectively reviewed the anatomic features and 30-day clinical outcomes. RESULTS: Initial deployment without open conversion was achieved in all cases and procedure-related 30-day mortality rate was 1.9%. Anatomic features showed the following variables: proximal aortic neck angle 48.8+/-25.7degrees (mean+/-standard deviation), vertical neck length 35.0+/-17.2 mm, aneurysmal sac diameter 57.2+/-14.2 mm, common iliac artery (CIA) involvement in 218 (56.3%) patients, and median right CIA length 34.9 mm. Two hundred and nineteen (56.3%) patients showed neck calcification, 98 patients (25.2%) had neck thrombus, and the inferior mesenteric arteries of 91 patients (23.4%) were occluded. CONCLUSION: Anatomical features of AAA in patients from the KER were characterized as having angulated proximal neck, tortuous iliac artery, and a higher rate of CIA involvement. Long-term follow-up and ongoing studies are required.

Iliocaval Fistula Presenting with Paradoxical Pulmonary Embolism Combined with High-Output Heart Failure Successfully Treated by Endovascular Stent-Graft Repair: Case Report

A 51-yr-old man presented exertional dyspnea as a consequence of iliocaval fistula combined with paradoxical pulmonary embolism and high-output heart failure. Endovascular stent-graft repair was performed to cover iliocaval fistula and restore the heart function. After the procedure, dyspnea was improved and procedure related complication was not seen. A 6-month follow-up computed tomography showed regression of pulmonary thromboembolism and well-positioned stent-graft without graft migration, aortacaval communication or endoleak. Stent graft implantation should be considered an alternative of open repair surgery for treament of abdominal arteriovenous fisula, especially in patient with high risk for surgery.

Coexistence of AA and AL Cardiac Amyloidosis in a Patient with Ankylosing Spondylitis Accompanying Monoclonal Gammopathy of Undetermined Significance

Amyloidosis is a clinical disorder caused by extracellular deposition of proteinaceous insoluble fibrils in various tissues, resulting in organ compromise. Amyloid L (AL) amyloidosis is the most common type of systemic amyloidosis, which occurs in association with multiple myeloma or monoclonal gammopathy of undetermined significance (MGUS). Secondary amyloid A (AA) amyloidosis is a complication of chronic inflammatory conditions, such as rheumatoid arthritis or ankylosing spondylitis. We report a case of a 49-year-old manwith a 11-year history of ankylosing spondylitis, who was recently diagnosed with MGUS presented with cardiac amyloidosis of both the AA and AL types. We report this case along with a review of relevant literature.

Clinical Analysis of Foreign Bodies in Gastrointestinal Tract in Children / 소아외과

Foreign body ingestion is a common problem among paediatric populations. Most of the ingested foreign bodies spontaneously pass through the gastrointestinal tract, but approximately less then 10% of them remain without being discharged, and trigger complications. Therefore, proper evaluation and treatment according to the situation is required. In this study, clinical progress and complications were analyzed according to the clinical features and treatment in children who ingested foreign bodies. Among pediatric patients under 18 who were admitted to Chonnam National University Hospital after ingesting foreign bodies between January 2008 to June 2012, only the patients who had their foreign body in the gastrointestinal tract were included in this study. Based on medical records, age, type of foreign body, time spent till admission, and whether the endoscopy was done or not, complication were researched retrospectively. According to symptoms and plain abdomen X-ray findings, treatment was chosen and conducted among endoscopy, observation and emergency operation. Among 273 patients, 9 (3.3%) of them had surgical removal. Seven (2.6%) of them had an emergency operation on the day of admission, and the rest 2 (0.7%) had operation during observation. Removal through initial endoscopic approach was tried in 157 (57.5%) patients. Eleven (70.8%) of them had their foreign body removed at the initial trial, and 5 (4.9%) of them at the second trial. Among 109, who were on observation status, 9 (8.3%) of them needed endoscopic removal, and 2 (1.8%) of them suffered from surgical removal. It is thought to be better to approach slowly considering the type, size and symptoms in foreign body ingestion of pediatric patients, rather than immediate and invasive removal.

Alteration of Ankle-Brachial Index after Kidney Transplantation / 대한이식학회지

BACKGROUND: A steno-occlusive disease of the iliac artery can mimic renal vascular hypertension, and is an important cause of renal dysfunction in renal transplant recipients. We assessed the alternation of postanastomotic arterial blood flow of lower extremities by ankle-brachila index (ABI). METHODS: We analyzed 50 patients who underwent kidney transplantation between March, 2010 and November, 2012 at Chonnam National University Hospital. This study was performed prospectively and case selection by renal transplantation patients who got first operation on right iliac fossa. All operational procedures were end to side anastomosis of the external iliac artery to the renal artery. We measured and compared the preoperative and postoperative (1 week, 6 months, 1 year) ABI. We analyzed the diameter of the recipient external iliac artery and renal artery of the transplanted kidney. RESULTS: Among 50 patients, 34 were male (68%) and 16 were female (32%). The mean age of recipients was 44.37+/-11.42 years. The mean preoperative ABI at the right lower extremity was 1.17+/-0.11, postoperative 1 week, 6 months, and 1 year was 1.14+/-0.10, 1.15+/-0.11, 1.17+/-0.15, respectively. Alternation of preoperative ABI and postoperative 1 week, 6 months, and 1 year was P=0.331, P=0.864, and P=0.992, respectively. CONCLUSIONS: Alternation of ABI on the ipsilateral lower extremity was not significant in renal transplanted recipients. We recommend a long-term study with more cases.

Changes in suprarenal and infrarenal aortic angles after endovascular aneurysm repair

PURPOSE: We investigated whether suprarenal and infrarenal aortic angles change after the endovascular aneurysm repair (EVAR) procedure and during follow-up, and investigated the correlation between infrarenal aortic angle after EVAR and type Ia endoleaks. METHODS: Data collected on 70 EVAR procedures for a fusiform infrarenal aortic aneurysm performed between May 2006 and December 2012 were supplemented with a retrospective review of charts and radiographs. RESULTS: The greater the preoperative infrarenal aortic angle, the greater the suprarenal aortic angle (r = 0.72, P < 0.001). The infrarenal aortic angle decreased after the EVAR procedure and continued to decrease slowly thereafter (all P < 0.001). Suprarenal aortic angle decreased immediately after the EVAR procedure and continued to decrease during the first month (P < 0.001). No differences in angulation were observed based on stent graft type. Type Ia endoleaks occurred with significantly greater incidence in patients with a larger post EVAR infrarenal angle (P = 0.037). CONCLUSION: The infrarenal aortic angle decreased significantly immediately after the EVAR procedure and continued to decrease slowly thereafter. Suprarenal aortic angle decreased immediately after the EVAR procedure and continued to decrease during the first month. We found a correlation between infrarenal and suprarenal aortic angle. Type Ia endoleaks occurred with greater incidence in patients with a larger infrarenal angle immediately after EVAR.

MicroRNA profiling of tacrolimus-stimulated Jurkat human T lympocytes / 대한외과학회지

PURPOSE: This study investigated the Jurkat T cell line expresses cytotoxicity when treated with different concentrations of FK506, and analyzed the expression pattern of microRNA when stimulated by FK506 using the microRNAs microarray, as well as the expression pattern of a gene that is related to the differentiation, activation and proliferation of T cells after being affected by the change of microRNAs. METHODS: To investigate the effects of FK506 on microRNA expression, we purified total RNA of Jurkat cells treated with 20 microM FK506 for 72 hours and used to analyze microRNA profiling by using Agilent's chip. RESULTS: These results demonstrated that treatment with FK506 markedly induced the down-regulation of 20 microRNAs as well as the up-regulation of 20 microRNAs in a time-dependent manner. The genes that down-regulated by FK506 include let-7a*, miR-20a*, and miR-487a. Otherwise miR-202, miR-485-5p, and miR-518c* are gradually up-regulated in expression. Sanger Institute and DAVIDs bioinformatics indicated that microRNAs regulated the several transcriptomes including nuclear factor of activated T cell-related, T cell receptor/interleukin-2 signaling, and Ca(2+)-calmodulin-dependent phosphatase calcineurin pathways. CONCLUSION: As a result of treating FK506 to a Jurkat cell line and running the microRNA microarray, it was found that FK506 not only took part in the suppression of T cell proliferation/activation by inhibiting calcineurin in Jurkat apoptosis, but also affected the microRNAs that are involved in the regulation of various signal transduction pathways.

Helicobacter pylori Seropositivity Is Positively Associated with Colorectal Neoplasms / 대한소화기학회지

BACKGROUND/AIMS: Helicobacter pylori is a well known precursor to gastric cancer and gastric mucosa-associated lymphoid tissue lymphoma. This study was to determine whether H. pylori was associated with colorectal neoplasms in Korean subjects undergoing routine checkup. METHODS: A total of 10,082 subjects underwent routine checkups from January 2004 to April 2005. A H. pylori IgG test and stool occult blood test were included in the routine checkup program. Colonoscopy was performed if the stool occult blood test was positive or under subject request. Patients who underwent colonoscopy and had histologically confirmed cases of colorectal neoplasms were designanted as the subject group and those without as the control group. RESULTS: Of the 10,082 subjects, 597 had full colonoscopy. The results identified 9 colorectal carcinomas and 118 adenomas. H. pylori seropositivity was identified in 6 (66%) subjects with colorectal carcinoma, 81 (68.6%) with colorectal adenoma and 248 (52.8%) controls. Subjects having colorectal neoplasms had a significantly higher H. pylori seropositivity rate compared with the controls (OR 1.94, 95% CI 1.28-2.95). This remained significant after adjusting for age, sex, body mass index, HbA1c and total cholesterol (OR 1.90, 95% CI 1.23-2.93). Patients with distal neoplasms also had a significantly higher H. pylori seroposivity rate (OR 1.88, 95% CI 1.17-3.01) which persisted after multivariate adjustment (OR 1.79, 95% CI 1.10-2.94). CONCLUSIONS: Subjects with colorectal neoplasms present an increased H. pylori seroprevalence compared with controls.

Pulmonary Hypertension in Neurofibromatosis Type 1: A Case Report / 대한내과학회지

Neurofibromatosis type I is a genetic disease caused by mutations in the neurofibromin 1 (NF1) gene. Although it is characterized by a number of distinct clinical features, including cafe au lait macules, freckling in the axillary or inguinal regions, neurofibromas, and Lisch nodules (iris harmartomas), it can affect all physiological systems in the body [1]. Neurofibromatosis-related pulmonary hypertension has also been reported, and some patients showed a poor prognosis despite having received proper medical treatment [2-4]. We herein describe a case of pulmonary hypertension in a patient with neurofibromatosis type I who had no identified risk factors of pulmonary hypertension. To our knowledge, this is the first such report in Korea.

Pulmonary Hypertension in Neurofibromatosis Type 1: A Case Report / 대한내과학회지

Neurofibromatosis type I is a genetic disease caused by mutations in the neurofibromin 1 (NF1) gene. Although it is characterized by a number of distinct clinical features, including cafe au lait macules, freckling in the axillary or inguinal regions, neurofibromas, and Lisch nodules (iris harmartomas), it can affect all physiological systems in the body [1]. Neurofibromatosis-related pulmonary hypertension has also been reported, and some patients showed a poor prognosis despite having received proper medical treatment [2-4]. We herein describe a case of pulmonary hypertension in a patient with neurofibromatosis type I who had no identified risk factors of pulmonary hypertension. To our knowledge, this is the first such report in Korea.