Heung Bae Kim
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View article: Pediatric segmental Caroli disease: A case report and review of the literature
Pediatric segmental Caroli disease: A case report and review of the literature Open
Introduction: Caroli disease is a rare congenital disorder causing nonobstructive segmental dilatation of intrahepatic bile ducts. Caroli disease can affect the liver diffusely or be confined to a single segment or lobe, although localized…
View article: Staple aneurysmorrhaphy and suture venoplasty for repair of large bilateral external iliac vein aneurysms in an adolescent
Staple aneurysmorrhaphy and suture venoplasty for repair of large bilateral external iliac vein aneurysms in an adolescent Open
Aneurysms of the iliac veins are very rare; thus, the best approach to management has not yet been defined. We have presented the case of a 17-year-old boy with incidentally identified large bilateral external iliac vein aneurysms. Given t…
View article: Outcomes of internal biliary diversion using cholecystocolostomy for patients with severe Alagille syndrome
Outcomes of internal biliary diversion using cholecystocolostomy for patients with severe Alagille syndrome Open
Alagille syndrome (AGS) is a disorder that leads to increased serum cholesterol and bile acids, which can result in debilitating xanthomas and pruritus. External biliary drainage and transplantation are effective treatments for AGS. Intern…
View article: Early Experiences Designing a Scalable COVID-19 Reusable Elastomeric Respirator
Early Experiences Designing a Scalable COVID-19 Reusable Elastomeric Respirator Open
The COVID-19 pandemic created an unprecedented shortage of personal protective equipment (PPE) for healthcare workers—especially respirators. In response to a lack of commercial respirator equipment, a multidisciplinary prototyping hackath…
View article: Prenatal diagnosis of rapidly enlarging choledochal cyst with gastric outlet obstruction
Prenatal diagnosis of rapidly enlarging choledochal cyst with gastric outlet obstruction Open
Choledochal cysts are congenital malformations of the biliary tract that involve aberrant configurations of the pancreaticobiliary ductal system. The pathology exists on a spectrum from fusiform dilation of the common bile duct to multiple…
View article: A large and complex saccular external jugular vein aneurysm in an adolescent girl
A large and complex saccular external jugular vein aneurysm in an adolescent girl Open
Venous aneurysms of the head and neck region are very uncommon. Aneurysms of the external jugular vein in children are exceedingly rare, and consequently the best approach to diagnosis and management has not been clearly defined. We presen…
View article: The re‐occurrence of cardiomyopathy in propionic acidemia after liver transplantation
The re‐occurrence of cardiomyopathy in propionic acidemia after liver transplantation Open
Cardiomyopathy is a frequent complication of propionic acidemia (PA). It is often fatal, and its occurrence is largely independent of classic metabolic treatment modalities. Liver transplantation (LT) is a treatment option for severe PA as…
View article: Impact of standardized protocols for cytomegalovirus disease prevention in pediatric solid organ transplant recipients
Impact of standardized protocols for cytomegalovirus disease prevention in pediatric solid organ transplant recipients Open
End‐organ disease caused by CMV is a significant cause of morbidity and mortality in pediatric SOT recipients. Pediatric transplant centers have adopted various approaches for CMV disease prevention in this patient population. We observed …
View article: Management of symptomatic neonatal aortic thrombosis: When is surgery indicated?
Management of symptomatic neonatal aortic thrombosis: When is surgery indicated? Open
Neonatal aortic thrombosis is a rare but potentially life-threatening condition. Treatment is patient-specific and may include expectant management, therapeutic anticoagulation, systemic thrombolysis or surgical thrombectomy. The majority …
View article: Internal surgical drainage for bile lakes following hepatoportoenterostomy for biliary atresia
Internal surgical drainage for bile lakes following hepatoportoenterostomy for biliary atresia Open
Bile lakes may be present at time of diagnosis or develop later following hepatoportoenterostomy (HPE) in patients with biliary atresia (BA). We report a patient with recurrent cholangitis following HPE who developed multiple large biliary…
View article: Whole-Exome Sequencing Enables a Precision Medicine Approach for Kidney Transplant Recipients
Whole-Exome Sequencing Enables a Precision Medicine Approach for Kidney Transplant Recipients Open
Significance Statement Case reports describe scenarios in which previously undiagnosed genetic disorders, such as primary hyperoxaluria type 1, caused early allograft failure in kidney transplant recipients. Whole-exome sequencing (WES) ha…
View article: Transduodenal cystoduodenostomy for pancreatic pseudocyst in two young children
Transduodenal cystoduodenostomy for pancreatic pseudocyst in two young children Open
Pancreatic pseudocysts are uncommon in children and tend to arise secondary to trauma or an episode of acute or chronic pancreatitis. Those that fail initial conservative management and become too large and/or complicated often require int…
View article: Acute multi-visceral thrombosis and ischemia in a 3-year-old child
Acute multi-visceral thrombosis and ischemia in a 3-year-old child Open
Acute multi-visceral ischemia secondary to multi-vessel thrombosis is rare in the pediatric population and to our knowledge it has not been previously reported. We present a 3-year old boy with acute thrombosis of the celiac trunk, superio…
View article: Whole Exome Sequencing Reveals a Monogenic Cause of Disease in ≈43% of 35 Families With Midaortic Syndrome
Whole Exome Sequencing Reveals a Monogenic Cause of Disease in ≈43% of 35 Families With Midaortic Syndrome Open
Midaortic syndrome (MAS) is a rare cause of severe childhood hypertension characterized by narrowing of the abdominal aorta in children and is associated with extensive vascular disease. It may occur as part of a genetic syndrome, such as …
View article: Ileal angiodysplasia causing chronic, occult gastrointestinal bleeding in a 14-year-old boy
Ileal angiodysplasia causing chronic, occult gastrointestinal bleeding in a 14-year-old boy Open
A 14-year old boy with anemia due to chronic gastrointestinal bleeding required recurrent transfusion. An extensive non-invasive evaluation that included detection of fresh blood in the small bowel followed by laparotomy with on-table pan-…
View article: YAP Subcellular Localization and Hippo Pathway Transcriptome Analysis in Pediatric Hepatocellular Carcinoma
YAP Subcellular Localization and Hippo Pathway Transcriptome Analysis in Pediatric Hepatocellular Carcinoma Open
Pediatric hepatocellular carcinoma (HCC) is a rare tumor which is associated with an extremely high mortality rate due to lack of effective chemotherapy. Recently, the Hippo pathway and its transcriptional co-activator Yes-associated prote…
View article: Successful Recovery and Transplantation of 11 Organs Including Face, Bilateral Upper Extremities, and Thoracic and Abdominal Organs From a Single Deceased Organ Donor
Successful Recovery and Transplantation of 11 Organs Including Face, Bilateral Upper Extremities, and Thoracic and Abdominal Organs From a Single Deceased Organ Donor Open
We report on the to date largest recovery of 11 organs from a single deceased donor with the transplantation of face, bilateral upper extremities, heart, 1 lung, liver (split for 2 recipients), kidneys, pancreas, and intestine. Although lo…