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1.  The surgical experience for retroperitoneal, mesenteric and omental cyst in children 
Intra-abdominal cystic masses originating from the retroperitoneum, mesentery or omentum are very rare and mostly benign tumors, but sometimes present as a complicated cyst encasing the major organs.
We analyzed the clinical findings, histologic diagnosis, and surgical outcomes in children who underwent operation for retroperitoneal, omental, and mesenteric cyst from 1998 to 2010, retrospectively.
Twenty-three patients (male, 12; female, 11) underwent the operation at a median age of 46 months (range, 9 days to 16 years). Among them, 17 cysts presented one or two symptoms such as abdominal mass, abdominal pain or abdominal distension. The median duration of symptoms was 7 days (range, 1 day to 365 days). Five were detected prenatally. Ten cysts were found in retroperitoneum, 8 in the omentum and 5 in the mesentery. The median diameter was 13 cm (range, 3 to 30 cm). Twenty cysts were completely removed. Five mesenteric cysts required bowel resection and anastomosis. Three of retroperitoneal cysts were impossible to complete excise because of location and extensiveness. Pathologically, 20 cysts were lymphangioma and 3 were pseudocyst. The morbidity was one of adhesive ileus and the mortality was one who had extensive retroperitoneal cyst with mesenteric cyst. He died from sepsis. During follow-up period, there was no recurrence.
Preoperative diagnosis and localization for these cysts are very difficult. Complete excision was possible in almost all cases despite the size, bringing a favorable outcome. The possibility of this disease entity should be considered as the cause of acute abdomen.
PMCID: PMC3412181  PMID: 22880185
Retroperitoneal cyst; Mesenteric cyst; Omental cyst; Lymphangioma; Pseudocyst
2.  Clinical experience with persistent cloaca 
Persistent cloaca is one of the most severe types of anorectal malformation. Appropriate initial drainage is difficult due to their various malformations and hydrocolpos or dilated urinary bladder. Corrective surgery also differs among individual patients. We describe our experiences with the surgical management of children with persistent cloaca.
We retrospectively reviewed 16 children diagnosed with persistent cloaca at Asan Medical Center.
Sixteen patients were managed in their neonatal period. Twelve patients had enlarged bladder or vagina at birth. Three patients, who did not undergo cystostomy or vaginostomy at first operation, had earlier complications after surgery or required drainage tube insertion. One patient who did not undergo hydrocolpos drainage died of sepsis and complications. Nine patients underwent corrective surgery; posterior sagittal anorectovaginourethroplasty using the Pena method. Three patients required additional operations due to complications after surgery.
Patients found to have anatomical malformations before colostomy, as well as hydrocolpos and bladder enlargement, require a vaginostomy with or without a cystostomy to reduce complications. Follow-up is required in patients with hydrocolpos and bladder enlargement to determine whether vaginal drainage improves dilated bladder. Continuous long-term follow-up examination is required to determine the long-term results of corrective surgery.
PMCID: PMC3204684  PMID: 22066071
Persistent cloaca; Anorectal malformation; Hydrocolpos; Posterior sagittal anorectovaginourethroplasty
4.  All-trans-Retinoic Acid Ameliorates Hepatic Steatosis in Mice via a Novel Transcriptional Cascade 
Hepatology (Baltimore, Md.)  2014;59(5):1750-1760.
Mice deficient small heterodimer partner (SHP) are protected from diet induced hepatic steatosis due to increased fatty acid oxidation and decreased lipogenesis. The decreased lipogenesis appears to be a direct consequence of very low expression of peroxisome proliferator activated receptor gamma 2 (PPARγ2), a potent lipogenic transcription factor, in the SHP−/− liver. The current study focuses on the identification of a SHP dependent regulatory cascade that controls PPARγ2 gene expression, thereby regulating hepatic fat accumulation. Illumina BeadChip array and real-time polymerase chain reaction were used to identify genes responsible for the linkage between SHP and PPARγ2 using hepatic RNAs isolated from SHP−/− and SHP-overexpressing mice. The initial efforts identify that hairy and enhancer of split 6 (Hes6), a novel transcriptional repressor, is an important mediator of the regulation of PPARγ2 transcription by SHP. The Hes6 promoter is specifically activated by the retinoic acid receptor (RAR) in response to its natural agonist ligand all-trans retinoic acid (atRA), and is repressed by SHP. Hes6 subsequently represses hepatocyte nuclear factor 4 alpha (HNF4α) activated-PPARγ2 gene expression via direct inhibition of the HNF4α transcriptional activity. Furthermore, we provide evidences that atRA treatment or adenovirus-mediated RARα overexpression significantly reduced hepatic fat accumulation in obese mouse models as observed in earlier studies and the beneficial effect is achieved via the proposed transcriptional cascade.
Our study describes a novel transcriptional regulatory cascade controlling hepatic lipid metabolism that identifies retinoic acid signaling as a new therapeutic approach to non-alcoholic fatty liver diseases.
PMCID: PMC4008145  PMID: 24038081
Lipid homeostasis; Nuclear Hormone Receptors; Fsp27; SHP; Hes6
5.  Hiatal hernia in pediatric patients: laparoscopic versus open approaches 
The aim of this study was to evaluate the surgical outcomes of laparoscopic approach for hiatal hernia (HH) in pediatric patients.
This was a retrospective study of 33 patients younger than 18 years who underwent an operation for HH between January 1999 and December 2012.
The HH symptoms were various and included regurgitation, vomiting, weight loss, cough, hoarseness, and cyanosis. Among the 33 patients, there were 25 sliding types, 1 paraesophageal type, and 7 mixed types. Open surgery (OS) and laparoscopic surgery (LS) were used in 16 and 17 patients, respectively. There were no statistically significant differences in sex, age, or body weight between the groups. The median operating time was longer in the LS group (150 minutes; range, 90-250 minutes vs. 125 minutes; range, 66-194 minutes; P = 0.028). Time to oral intake was shorter in the LS group than in the OS group (1 day; range, 1-3 days vs. 2 days; range, 1-7 days; P = 0.001) and time to full feeding was shorter in the LS group than in the OS group (6 days; range, 3-16 days vs. 10 days; range, 3-33 days; P = 0.048). There were no differences in length of hospital stay and complications between the two groups. There was no perioperative mortality or recurrence of HH.
A good surgical outcome for laparoscopic correction of HH was seen in pediatric patients.
PMCID: PMC4024937  PMID: 24851228
Hiatal hernia; Child; Laparoscopy; Fundoplication; Minimal invasive surgical procedures
6.  Long-Term Survival of a Patient with Pulmonary Artery Intimal Sarcoma after Sequential Metastasectomies of the Thyroid and Adrenal Glands 
Endocrinology and Metabolism  2013;28(1):46-49.
Cancer metastases to the thyroid or adrenal gland are uncommon. Furthermore, cases showing long-term survival after surgical resection of those metastatic tumors are rare. We report a case of pulmonary artery intimal sarcoma with metastases to the thyroid and adrenal glands sequentially that was successfully treated with sequential metastasectomies. A 62-year-old woman presented with a 4-week history of dyspnea on exertion and facial edema in November 1999. Echocardiography and chest computed tomography (CT) revealed an embolism-like mass in the pulmonary trunk. Pulmonary artery endarterectomy with pulmonary valve replacement was performed, and histopathology revealed pulmonary artery intimal sarcoma. A thyroid nodule was found by chest CT in November 2001 (2 years after initial surgery). During follow-up, this lesion showed no change, but we decided to obtain fine needle aspiration cytology (FNAC) in August 2004 (4.7 years after initial surgery). FNAC revealed atypical spindle cells suggestive of metastatic intimal sarcoma. She underwent total thyroidectomy. During follow-up, a right adrenal gland mass was detected by chest CT in March 2006 (6.3 years after initial surgery), and adrenalectomy was done, which also revealed metastatic sarcoma. She has been followed up without any evidence of recurrent disease until May 2012 (12.5 years after initial surgery).
PMCID: PMC3811804  PMID: 24396650
Endocrine organ; Metastasectomy; Pulmonary artery intimal sarcoma
7.  Ileo-Pelvic Anastomosis and Augmentation Cystoplasty for Treatment of Encrusted Pyelitis in a Transplanted Kidney 
Korean Journal of Urology  2013;54(2):143-146.
Infection stones are more likely to form after urinary diversion as the result of urinary stasis. To prevent urinary stasis due to encrusted pyelitis in a transplanted kidney, we describe an alternative a surgical treatment: ileo-pelvic anastomosis. In our patient with a transplanted kidney, the ileal conduit had previously been anastomosed end-to-side owing to renal tuberculosis with an atrophied bladder; the transplanted ureter was anastomosed to the ileum in the left lower abdomen with an ileal conduit on the opposite side. Routine check-up revealed hydronephrosis with infected pyelitis and ureteritis in the transplanted kidney. We performed ileo-pelvic end-to-end anastomosis to prevent urinary stasis by lengthening the ileal conduit and performed augmentation cystoplasty to support the atrophied bladder following tuberculosis. We suggest that this approach may be useful in similar cases.
PMCID: PMC3580306  PMID: 23550263
Kidney transplantation; Pyelitis; Urinary diversion
8.  Adrenocortical tumors in children 18 years old and younger 
Pediatric adrenocortical tumors (ACTs) are rare. We reviewed findings in 8 children, 18 years of age or younger, diagnosed with ACT in our institution over the past 15 years.
We retrospectively reviewed 8 children with ACTs treated between 1996 and 2010.
Three girls and 5 boys were treated for ACTs; their median age at presentation was 144 months (range, 28 months to 18 years). Seven patients showed signs of endocrine dysfunction, 4 with Cushing syndrome, 2 with virilization, and 1 with hyperaldosteronism. One patient, with symptoms of hematuria, underwent a computed tomography scan, which showed an adrenal mass. The median duration of symptoms prior to resection was 6 months (range, 1 to 24 months). Five patients had adenomas and 3 had carcinomas. All underwent complete resection of the tumor, with laparoscopic adrenalectomy performed on 3 patients with adenoma and 1 with carcinoma. The median tumor weight was 12.5 g (range, 1 to 130 g) and the median tumor volume was 18.3 cm3 (range, 2.2 to 299.2 cm3). At a median follow-up of 5.1 years (range, 4 months to 15 years), all 8 patients remain alive with no recurrence of disease.
The characteristics of pediatric ACTs vary considerably. Laboratory findings, clinical hormonal features, and tumor size could not distinguish adenomas from carcinomas before surgery. Complete tumor resection was successful, with no tumor recurrence. However, the small number of patients and short follow-up period limit assessments of prognosis.
PMCID: PMC3319779  PMID: 22493766
Adrenocortical adenoma; Carcinoma; Child
9.  An 18-year experience of tracheoesophageal fistula and esophageal atresia 
Korean Journal of Pediatrics  2010;53(6):705-710.
To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit.
A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007.
The rate of prenatal diagnosis was 12%. The average gestational age and birth weight were 37+2 weeks and 2.5±0.7 kg, respectively. Thirty-one infants were born prematurely (32%). Type C was the most common. The mean gap between the proximal and distal esophagus was 2 cm. Esophago-esophagostomy was performed in 72 patients at a mean age of 4 days after birth; gastrostomy or duodenostomy were performed in 8 patients. Forty patients exhibited vertebral, anorectal, cardiac, tracheoesophageal, renal, limb (VACTERL) association with at least 2 combined anomalies, and cardiac anomaly was the most common. The most common post-operative complications were esophageal stricture followed by gastroesophageal reflux. Balloon dilatation was performed for 1.3 times in 26 patients at a mean age of 3 months. The mortality and morbidity rates were 24% and 67%, respectively, and the most common cause of death was sepsis. The weight of approximately 40% patients was below the 10th percentile at 2 years of age.
Mortality and morbidity rates of patients with TEF and EA are high as compared to those of infants with other neonatal surgical diseases. Further efforts must be taken to reduce mortality and morbidity and improve growth retardation.
PMCID: PMC2994136  PMID: 21189942
Tracheoesophageal fistula; Esophageal atresia; VACTERL association; Mortality; Morbidity; Growth
10.  Changes in Serum Prostate-Specific Antigen Levels after Potassium-Titanyl-Phosphate (KTP) Laser Vaporization of the Prostate 
Korean Journal of Urology  2010;51(2):111-114.
The prostate-specific antigen (PSA) level decreases after transurethral resection of the prostate (TURP). However, changes in the PSA level after potassium-titanyl-phosphate (KTP) laser vaporization of the prostate are not well known. The aim of this study was to investigate the effect of KTP laser vaporization of the prostate on PSA levels in patients with benign prostatic hyperplasia (BPH).
Materials and Methods
Serum PSA levels were checked before and 1, 3, 6, and 12 months after the procedure in patients who underwent KTP laser vaporization between October 2004 and August 2008. Patients with prostate cancer, a history of urinary retention, or prostatitis during the follow-up period were excluded. The results for 278 patients were studied.
The mean age of the patients was 69.0±6.7 years (range, 50-91 years) and the mean preoperative PSA level was 2.72±2.93 ng/ml. The PSA level tended to be increased at 1 month after the operation (3.18±3.23 ng/ml, p=0.032) but decreased within 3 months and became stabilized after 6 months at 1.79±1.82 ng/ml (p<0.001).
PSA levels may increase after KTP laser vaporization for a certain period of time, but eventually decrease and become stabilized after 6 months. Therefore, it may be appropriate to wait up to 3 months if the PSA level rises after the procedure, and further investigation should be considered if the PSA level still remains high after 6 months.
PMCID: PMC2855487  PMID: 20414423
Prostate-specific antigen; Potassium titanyl-phosphate; Prostatic hyperplasia
11.  Krüppel-like zinc finger protein Gli-similar 2 (Glis2) represses transcription through interaction with C-terminal binding protein 1 (CtBP1) 
Nucleic Acids Research  2005;33(21):6805-6815.
Glis2 is a member of the Gli-similar (Glis) subfamily of Krüppel-like zinc finger transcription factors. It functions as an activator and repressor of gene transcription. To identify potential co-activators or co-repressors that mediate these actions of Glis2, we performed yeast two-hybrid analysis using Glis2 as bait. C-terminal binding protein 1 (CtBP1) was identified as one of the proteins that interact with Glis2. This interaction was confirmed by mammalian two-hybrid analysis. CtBP1 did not interact with other members of the Glis subfamily suggesting that this interaction is specific for Glis2. Pulldown analysis with GST-CtBP1 demonstrated that CtBP1 physically interacts with Glis2. Analysis of CtBP1 and Glis2 deletion mutants identified several regions important for this interaction. CtBP1 repressed transcriptional activation induced by Glis2(1–171). Repression by Glis2 appears to involve the recruitment of both CtBP1 and histone deacetylase 3 (HDAC3). Confocal microscopic analysis demonstrated that Glis2 localized to nuclear speckles while in most cells CtBP1 was found diffusely in both cytoplasm and nucleus. However, when CtBP1 and Glis2 were co-expressed, CtBP1 was restricted to nuclear speckles and co-localized with Glis2. Our observations suggest that the co-repressor CtBP1 and HDAC3 are part of transcription silencing complex that mediates the transcriptional repression by Glis2.
PMCID: PMC1301595  PMID: 16326862

Results 1-11 (11)