Postoperative Complications of Esophageal Atresia and Role of Endoscopic Balloon Dilatation in Anastomotic Strictures / 대한소아소화기영양학회지

Purpose@#Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a congenital anomaly that can cause frequent digestive and nutritional problems, even after repair. The most common complication is anastomotic stricture, for which reoperation or balloon dilatation is performed. This study aimed to evaluate the postoperative complications of EA and the role of endoscopic balloon dilatation (EBD) in cases of anastomotic stricture. @*Methods@#We retrospectively analyzed patients diagnosed with EA with or without TEF between January 2000 and February 2021. Patients’ baseline characteristics, associated anomalies, and postoperative complications were reviewed. @*Results@#Among 26 patients, 14 (53.8%) were male, 12 (46.2%) had coexisting anomalies, and the median follow-up was 6.1 years (range, 1.2–15.7 years). In univariate analysis, prematurity, low birth weight, and long-gap EA were associated with postoperative complications in 12 (46.2%) patients. Among the 10 (38.5%) patients with anastomotic stricture, nine (90.0%) required EBD. Regarding the first EBD, it was performed at a median of 3.3 months (range, 1.2–7.6 months) post-repair, while the average patient weight was 4.6 kg. The mean diameter ranged from 3.3 to 9.1 mm without major complications. In univariate analysis, long-gap EA alone was significantly associated with EBD. @*Conclusion@#Approximately half of the patients experienced complications after EA repair.In particular, patients with a long-gap EA had a significantly increased risk of complications, such as anastomotic strictures. EBD can be safely used, even in infants.

Leri-Weill dyschondrosteosis in a newborn presenting with respiratory failure due to severe micrognathia

Short stature homeobox-containing gene (SHOX) is a well-known causative gene for the short stature in Turner syndrome. The clinical manifestation of SHOX gene related disorders varies from SHOX haploinsufficiency, presenting with idiopathic short stature, disproportionate short stature, or Leri-Weill dyschondrosteosis (LWD) to recessive form of extreme dwarfism and limb deformity in Langer mesomelic dysplasia. LWD is usually diagnosed upon suspicion based on short stature and skeletal abnormalities, and it is rarely accompanied with respiratory failure in the neonatal period. Here, we report the case of a newborn infant with LWD presenting with severe micrognathia that caused respiratory distress, which was diagnosed using microarray testing. Even when the manifestation of Madelung deformity is not yet apparent, LWD should be considered as one of underlying diseases related to congenital micrognathia.

Leri-Weill dyschondrosteosis in a newborn presenting with respiratory failure due to severe micrognathia

Short stature homeobox-containing gene (SHOX) is a well-known causative gene for the short stature in Turner syndrome. The clinical manifestation of SHOX gene related disorders varies from SHOX haploinsufficiency, presenting with idiopathic short stature, disproportionate short stature, or Leri-Weill dyschondrosteosis (LWD) to recessive form of extreme dwarfism and limb deformity in Langer mesomelic dysplasia. LWD is usually diagnosed upon suspicion based on short stature and skeletal abnormalities, and it is rarely accompanied with respiratory failure in the neonatal period. Here, we report the case of a newborn infant with LWD presenting with severe micrognathia that caused respiratory distress, which was diagnosed using microarray testing. Even when the manifestation of Madelung deformity is not yet apparent, LWD should be considered as one of underlying diseases related to congenital micrognathia.

A neonate with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome from a consanguineous Pakistani family

Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare autosomal recessive urea cycle disorder. HHH is caused by a deficiency of the mitochondrial ornithine transporter protein, which is encoded by the solute carrier family 25, member 15 (SLC25A15) gene. Recently, government supported Korean newborn screening has been expanded to include a tandem mass spectrometry (MS/MS) measurement of ornithine level. We report a case of a neonate with HHH syndrome showing a normal MS/MS measurement of ornithine level. A female newborn was admitted to neonatal intensive unit due to familial history of HHH syndrome. Her parents were consanguineous Parkistani couple. The subject's older sister was diagnosed with HHH syndrome at age of 30 months based on altered mental status and liver dysfunction. Even though the subject displayed normal ammonia and ornithine levels based on MS/MS analysis, a molecular test confirmed the diagnosis of HHH syndrome. At 1 month of age, amino acid analysis of blood and urine showed high levels of ornithine and homocitrulline. After 11 months of follow up, she showed normal growth and development, whereas affected sister showed progressive cognitive impairment despite no further hyperammonemia after protein restriction and standard therapy. Our report is in agreement with a previous Canadian study, which showed that neonatal samples from HHH syndrome patients demonstrate normal ornithine levels despite having known mutations. Considering the delayed rise of ornithine in affected patients, genetic testing, and repetitive metabolic testing is needed to prevent patient loss in high risk patients.

Modification of nutrition strategy for improvement of postnatal growth in very low birth weight infants / 소아과

PURPOSE: To identify the effects of modified parenteral nutrition (PN) and enteral nutrition (EN) regimens on the growth of very low birth weight (VLBW) infants. METHODS: The study included VLBW infants weighing <1,500 g, admitted to Chungnam National University Hospital between October 2010 and April 2014, who were alive at the time of discharge. Subjects were divided according to 3 periods: period 1 (n=37); prior to the PN and EN regimen being modified, period 2 (n=50); following the PN-only regimen modification, period 3 (n=37); following both PN and EN regimen modification. The modified PN regimen provided 3 g/kg/day of protein and 1 g/kg/day of lipid on the first day of life. The modified EN regimen provided 3.5-4.5 g/kg/day of protein and 150 kcal/kg/day of energy. We investigated growth rate, anthropometric measurements at 40 weeks postconceptional age (PCA) and the incidence of extrauterine growth restriction (EUGR) at 40 weeks PCA. RESULTS: Across the 3 periods, clinical characteristics, including gestational age, anthropometric measurements at birth, multiple births, sex, Apgar score, surfactant use and PDA treatment, were similar. Growth rates for weight and height, from time of full enteral feeding to 40 weeks PCA, were higher in period 3. Anthropometric measurements at 40 weeks PCA were greatest in period 3. Incidence of weight, height and head circumference EUGR at 40 weeks PCA decreased in period 3. CONCLUSION: Beginning PN earlier, with a greater supply of protein and energy during PN and EN, is advantageous for postnatal growth in VLBW infants.

Clinical Features and Role of Viral Isolates from Stool Samples of Intussuception in Children / 대한소아소화기영양학회지

PURPOSE: To detect major acute gastroenteritis virus (rotavirus, norovirus, astrovirus, and enteric adenovirus) and non-enteric type of adenovirus (AdV) in the stools of intussusception patients and to investigate the clinical role of detected viruses. METHODS: From March 2012 to February 2013, major acute gastroenteritis virus and non-enteric type of AdV were isolated from stool samples that collected from 44 patients treated for intussusception in Chungnam National University Hospital. Patients were divided according to age and isolated virus. RESULTS: Virus was detected in 28 (63%) stool specimens. The virus detection rate was significantly lower in patients aged under 12 months (p = 0.04). Twenty-two patients (78.6%) had non-enteric adenovirus, 4 (14.3%) had norovirus, 1 (3.6%) had sapovirus, and 1 (3.6%) had astrovirus. AdV subgroup C (AdV 1, 2, 5, and 6) comprised the majority with 20 cases (90.9%). A monthly increment-and-decrement pattern of intussusception was similar to that of viral detection in the stool samples. Enema reductions were successful in 39 patients and surgical manual reductions were performed in 5 patients. Virus was detected in 24 patients (61.5%) of enema reduction group and 4 patients (80.0%) of surgical manual reduction group. All of the detected viruses were non-enteric adenovirus subgroup C (AdV 1, 5, and 6) in surgical reduction patients. CONCLUSIONS: The virus detection rate was high in the stools of intussusception patients. The pattern of seasonal intussusception occurrence rate was parallel with seasonal these viral detection rate in the stool samples. These findings suggest that viral infection plays an important role in the development of intussusception and further research is warranted.

Eradication of Helicobacter pylori in Patients with S-2 Stage Duodenal Ulcer Scar an Interim Report / 대한소화기내시경학회지

BACKGROUND/AIMS: It is recommended that duodenal ulcer patients who are infected with H. pylori should be treated with eradication therapy, whether the ulcer is active or in remission. However, there has been no report on the effect of eradication treatment in patients with incidentally found S-2 stage duodenal ulcer scar. METHODS: We prospectively enrolled 80 H. pylori-positive patients with S-2 stage duodenal ulcer scar who have no past history of ulcer treatment. Treatment group received triple therapy consisted of omeprazole, amoxicillin, and clarithromycin for 2 weeks, whereas control group received no treatment. The follow-up endoscopy was performed every 1 year and when the patients have symptoms of ulcer disease. Fifty-three patients were followed up for more than 1 year. RESULTS: The eradication rate of the treatment group was 92.9%. During the follow-up period of 14.7 months, 20% (5/25) of patients in the control group (2 gastric ulcers and 3 duodenal ulcers) and 3.6% (1/28) of patients in the treatment group (1 duodenal ulcer) developed active or healing stage peptic ulcers (p=0.089). CONCLUSIONS: Our results suggest that H. pylori eradication may he effective in preventing peptic ulcers in patients with S-2 stage duodenal ulcer scar.

The roles of inducible nitric oxide synthase expression in pancreatic cancer / 대한내과학회지

BACKGROUND: The finding of frequent inducible nitric oxide synthase (iNOS) expression in human cancer indicates that nitric oxide has a pathological role in tumor progression. Increased expression of iNOS in human pancreatic cancer cells was also recently reported, but the clinicopathological and biological significance of the iNOS expression remains unclear. The aim of our study was to look for possible roles and clinical significance of iNOS expression in pancreatic cancer. METHODS: 72 pancreatic adenocarcinoma tissue specimens were obtained from surgical resection. We investigated the immunohistochemical expression of iNOS in respect to variable clinicopathological characteristics, proliferation activity (assayed by Ki-67 expression), apoptosis (by TUNEL stain), and microvessel density (by CD34 expression; angiogenesis). RESULTS: Immunohistochemical positivity for iNOS in pancreatic epithelial cells was observed in 48/72 (66.7%). Apoptotic index (AI) of positive iNOS expressions were significantly higher than for negative expression (p <0.001) and increasing intensity of COX-2 expression showed a trend with increasing AI (p<0.001). No significant association was found between iNOS expression and proliferation index or microvessel density in pancreatic cancer. The expression of iNOS protein did not correlated with age, bilirubin, CA 19-9, location, size, AJCC stage, differentiation, distant metastasis or patient survival. CONCLUSION: The expression of iNOS enzyme in pancreatic cancer contributes to apoptosis of tumor cells. However, we could not find any correlation between iNOS expression and cell proliferation, angiognesis or clinical characteristics. Further in vivo investigations are necessary to determine the putative role of the iNOS expression for tumor progression in human pancreatic cancer.

Thyroid autoantibodies and thyroid dysfunction in rheumatoid arthritis / 대한내과학회지

BACKGROUND: Autoimmune thyroid diseases have been shown to occur in association with connective tissue disorders. This study was undertaken to elucidate the prevalence of thyroid autoantibodies and thyroid dysfunction in Korean patients with rheumatoid arthritis (RA). METHODS: We have compared the prevalence of thyroid autoantibodies in 108 RA patients with 81 age-matched controls. Antimicrosomal antibody (AMA) and antithyroglobulin antibody (ATA) were measured by passive hemagglutinin assay. T3, T4, Free T4, and TSH were measured by chemiluminescence method. Rheumatoid factor was measured by nephelometry. Statistical analysis was performed by chi-square, Fisher's exact test, and Pearson correlation test. RESULTS: 1) The presence of AMA/ATA in patients with RA was significantly higher (28.7%/26.7% in 108 patients) than that of controls (11.1%/9.9% in 81 controls). The positive rates of AMA/ATA in female patients were also higher (28.9%/28.9% in 97 patients) than those of female controls (15.5%/12.1% in 58 controls). 2) Thirty three (17.6%) of 187 patients with RA had the abnormalities of thyroid function; 14 (7.5%) revealed subclinical hypothyroidism, 5 (2.7%) revealed biochemical primary hypothyroidism, 7 (3.7%) revealed sick euthyroid syndrome, and 7 (3.7%) revealed hyperthyroidism. CONCLUSION: These results suggest that autoimmune thyroiditis is highly associated with RA in Korean patients, showing the increased prevalence of thyroid autoantibodies and thyroid dysfunctions.

The Role of Autonomic Dysfunction in Patients with Functional Dyspepsia / 대한소화관운동학회지

BACKGROUND/AIMS: The role of autonomic dysfunction in patients with functional dyspepsia has not been completely understood. The purposes of our study are (1) to prospectively assess the abnormalities of the autonomic function in patients with functional dyspepsia and (2) to assess whether the presence of autonomic dysfunction in patients with functional dyspepsia correlates with the presence of visceral hypersensitivity or with the severity of dyspeptic symptoms. METHODS: Twenty eight patients with functional dyspepsia (4 men and 24 women; age range, 29-57) and 14 healthy volunteers without gastrointestinal symptoms (6 men and 8 women; age range, 23-61) were included in this study. All patients and controls were submitted to a battery of five standard cardiovascular autonomic reflex tests and gastric barostat tests. A modified version of the Glasgow Dyspeptic questionnaire was used in this study. RESULTS: (1) Autonomic function tests showed that both sympathetic and parasympathetic scores of dyspeptic patients were significantly higher than those of the control group. (2) Visceral hypersensitivity could be confirmed in some of our dyspeptic patients in response to proximal gastric distension, demonstrating lower pain threshold in this group. (3) We could not find significant association between the presence of autonomic dysfunction and the presence of visceral hypersensitivity or severity of dyspeptic symptoms in patients with functional dyspepsia. CONCLUSION: Autonomic dysfunction was more prevalent in dyspeptic patients than in the control group. However, it did not correlate with the presence of visceral hypersensitivity or severity of dyspeptic symptoms. It is suggested that a defect in the spinal region or at the CNS level may be a major mechanism of visceral hypersensitivity in functional dyspepsia.

Clinical characteristics of primary biliary cirrhosis based on the analysis of 16 patients / 대한내과학회지

BACKGROUND: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown etiology. The disease is relatively common in western countries, but so far, only about 20 cases have been reported in Korea. Thus, the purpose of our prospective study was to evaluate the clinical characteristics of PBC in Korea. METHODS: Between October 1994 and February 1999, 16 patients diagnosed as PBC at our department were enrolled in this study. We analyzed these patients for the distribution of age and sex, initial symptoms and signs, associated disorders, laboratory, endoscopic, and radiologic and histologic findings. RESULTS: Of the 16 patients, the ratio of male to female was 1:7 and the average age was 57.5 years. Pruritus (37.5%) was the most frequent presenting symptom followed by xerostomia and xerophthalmia (12.5%), jaundice (6.3%), chronic fatigue (6.3%), melena (6.3%). Associated disorders were Sj gren's syndrome (25%), arthropathy (12.5%), Raynaud's phenomenon (6.3%) and diabetes (6.3%). Abnormalities of liver function were found frequently. Antimitochondrial antibody (100%), antinuclear antibody (31.25%) and rheumatoid factor (31.25%) were found. Long-term administration of UDCA improved both clinical and biochemical signs in most patients, however, 2 patients experienced exacerbation during UDCA therapy. CONCLUSIONS: We experienced 16 cases of PBC within a short period. It is expected that we can find these patients more frequently if we suspect this disease in patients with pruritus and unexplained obstructive pattern of liver function abnormality.

A Case of Intramural Pancreatic Pseudocyst of the Duodenum / 대한소화기내시경학회지

Pancreatic pseudocysts are a well-recognized complication of pancreatitis. Most occur in or adjacent to the pancreas. Occasionally, duodenal involvement may occur due to the nonperitonealized posterior surface of the duodenum is in direct contact with the head of the pancreas. But there is little awareness of the intramural and major extrinsic involvement of the duodenum with also occurs. A case was recently experienced involving an intramural pancreatic pseudocyst of the duodenum.

Total Hip Replacement in Unreduced Hip Dislocation / 대한정형외과학회잡지

Total hip arthroplasty in patients with chronically dislocated hips poses many challenging technical problems. Abnormal relationship of bones and soft tissues occurs in chronic complete dislocation of the hip (grade IV, Crowe's classification). Problems in placing the acetabular component in the true acetabulum include, first how to cover the acetabular component sufficiently, second how to fix it securely and last how to avoid excessive stretching of the neurovascular structures such as the sciatic nerve. The authors have introduced a technique of one stage distal femoral shortening in order to avoid excessive stretching of the neurovascular structures. From December 1988 to May 1996, the total hip arthroplasties combined with one stage distal femoral shortening were carried out in chronic complete dislocation of 44 hips (grade IV, Crowe's classification) and followed-up more than two years. This method has several advantages: It preserves wide and healthy proximal metaphysis of the femur, does not need greater trochanteric osteotomy, and provides many chances to choose the appropriate stem size and shape, especially in cementless total hip arthroplasty.

On Pattern of Birth and Death in Seoul City / 예방의학회지

A survey was conducted by the staff of the College of Medicine and School of Public Health, Seoul National University in cooperation with Seoul Special City from 1 December 1967 through 28 February 1968, on such events as delivery, death, abortion and pregnancy. The survey directed to a total population of 47,811 residing in 9,157 households led us to the following findings: 1. Two year averages of crude birth rate, crude death rate and natural increase rate were 30.1, 5.6 and 24.5, respectively. 2. Of all deliveries, home and hospital deliveries constituted 61.1 per cent and 35.5 per cent, respectively. 3. Deliveries other than hospital deliveries were found to be attended more often by mother-in-laws(26.5 per cent) than by doctors or midwives(23.4 per cent). 4. About 52 per cent of all women having experiences in pregnancy during the last two years had an experience of consulting a doctor at least one time throughout whole period of pregnancy. 5. In most cases scissors were used to cut umbilical cords, of which 71.0 per cent were not sterilized and only 28.3 per cent sterilized. 6. In many cases placenta was incinerated(48,2 per cent) and on many other occasions it was thrown away into water(28.3 per cent). 7. Cement page(37.4 per cent), gauze and absorbent cotton(29.8 per cent)were found to be most frequently used to receive new-born babies. 8. In 1966 8.8 per cent of the women had at least one abortion induced and in 1967 the percentage was 9.2 per cent. 9. Nearly all(95.8 per cent) of the induced abortions reportedly were done at doctor's clinics. 10. Of all the abortions induced 65.3 per cent were done by specialists in obstetrics, 30.3 per cent by general practitioners and 2.7 per cent by midwives. 11. Those who experienced spontaneous abortions were 1.9 per cent of all women both in 1966 and 1967. 12. About 9.2 per cent of women investigated were found to be currently pregnant. 13. Age specific death rate turned out to be highest among those under 1 year of age. 14. Ten major causes of death in their order of frequency were: 15. Places of death can be classified into homes(75.3 per cent) and hospitals(13.2 per cent). 16. Method of disposing of corpses comprised burials(54.2 per cent) and cremations(44.6 per cent). 17. Infant, neonatal and hebdomadal mortality rates have been computed at 32.2, 18.9 and 13.7, respectively. 18. Infants were found to have died either at homes(81.5 per cent) or at hospitals(18.5 per cent). 19. Birth registrations had been done for about 18.5 per cent of the dead infants.