• Journal of the korean academy of Pediatric Dentistry
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• v.39 no.4
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• pp.412-417
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• 2012

Mucopolysaccharidosis (MPS) is a disorder which is caused by the defect of the lysosomal enzyme that is essentially needed for resolution of glycosaminoglycans (GAGs). Metabolite of GAGs will accumulate in the lysosome of cells and will result in the dysfunction of cells, tissues, and organs. Eventually, patients will manifest both mental retardation and physical disorders. In worst cases, mucopolysaccharidosis can cause premature death. The current clinical types have been classified as MPS from type I to type IX according to the defect of certain enzyme. The dental complications have been reported as delay of eruption, enamel hypoplasia, microdontia, malocclusion, condylar defects, gingival hyperplasia and dentigerous cystlike follicle. This clinical report presents the case of a boy with MPS type II, Hunter Syndrome which has various dental complications.

• Advances in pediatric surgery
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• v.17 no.1
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• pp.15-22
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• 2011

Meconium obstruction (MO) in neonates arises from highly viscid meconium and the poor motility of the premature gut. Recently the incidence of the MO in neonates has been Increasing, but, the diagnosis and treatment of this disease have not yet been clarified. Between March 2004 and April 2010, 24 neonates were treated for MO at Severance Children's Hospital. Their clinical characteristics and treatment were reviewed retrospectively. Twenty neonates were diagnosed with MO and 4 neonates were diagnosed with Hirschsprung's disease (HD). The mean birth weight and gestational age of the 20 neonates with MO were $1.45{\pm}0.90kg$ and $31.1{\pm}4.6$ weeks, respectively. Thirteen neonates (65 %) diagnosed with MO weighed less than 1.5 kg and 10 neonates (50 %) weighed less than 1 kg. Half of the neonates with MO were treated by non-operative methods and the other half were treated by operative methods. Compared with the group that weighed over 1.5 kg, the group that weighed less than 1.5 kg were more frequently operated upon (61.5% vs. 28.5%), and contrast enemas were performed later and more frequently. Also the group that weighed less than 1.5 kg had a higher mortality rate (15.4% vs. 0%). Three of the four neonates with HD were diagnosed with long-segment aganglionosis. In conclusion, MO occurred in very low birth weight neonates more often and must be differentiated from HD. Also, MO in very low birth weight neonates should be treated with special attention due to more a complicated clinical course.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.556-562
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• 2009

Ectopic eruption of the maxillary first permanent molar means that the molar erupts out of the normal position and is arrested in its eruption by the second primary molar. This local eruption disturbance results in a premature atypical resorption on the distal part of the second primary molar. In most irreversible cases, the second primary molar is lost prematurely, either by spontaneous exfoliation or by extraction, In cases of doubt as to whether the eruption is of the irreversible type or not, careful radiographic observation period for a few months would be valuable in evaluating the possibilities of the tooth's freeing itself. The purpose of this study was to determine the characteristics and occurrence of the ectopic eruption of the maxillary first permanent molar. A descriptive, observational, retrospective study was done using the radiographs of 25 conseutive patients, who were in the first phase of mixed dentition. A method was designed to evaluate the amount of pathologic resorption of the second maxillary primary molar and the mesial angulation of the first permanent molar. The study showed that the most important etiologic factor was the eruption path or mesial angulation of the first permanent molars relative the chosen reference lines.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.2
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• pp.357-366
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• 2008

Posterior cross-bite is a relatively frequent malocclusion in primary and early mixed dentition and the reported prevalence of posterior cross-bite varies from 7% to 23%. It has been defined as a transverse discrepancy in arch relationship which the palatal cusp of the upper posterior teeth do not occlude in the central fossa of the opposing lower teeth, and can be manifested in a single tooth or in a group of teeth. Posterior cross-bite does not often self-correct and therefore immediate treatment is recommended. Occlusal adjustment to eliminate premature contact that causes mandibular deviation, expansion of narrow maxillary arch, arrangement of the individual teeth to treat asymmetry within the dental arch are the methods of treating cross-bite. In the present case, functional posterior cross-bite was observed in the primary and the early mixed dentition children. The children were treated by the slow maxillary expansion and occlusal adjustment. The outcome of periodic examinations after the correction of cross-bite was favorable.

• Journal of Yeungnam Medical Science
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• v.11 no.1
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• pp.35-41
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• 1994

Newborns with ileal atresia freqently present with abdominal distension, bilious vomiting. and failure to pass meconium. Diagnosis is usually established on plain x-ray of the abdomen by the findings of distended small bowel loops and air-fluid levels. In the period of October 1988 to February 1994, 8 patients with congenital ileal atresia were operated and the following results were obtained. 1. Eight patients were comprised of 4 males and 4 females, the ratio of male and female was 1 : 1. 2. Six patients(75%) had been admittted to our hospital during three days of life. 3. Congenital ileal atresia was in 8 cases : Type I in two(25%), Type II in two(25%), Type IIIa in three(37.5%), Type IIIb in one(12.5%). 4. There was one premature patient who was small for gestational age. 5. Overall, abdominal distension and bilious vomiting occurring in seven patients, were frequent presenting complaints. 6. Diagnosis was possible with clinical symptom and simple abdomen. 7. Operative treatment was undertaken as soon as the diagnosis was made. In seven cases a primary end-to-end anastomosis was performed after resection of dilated proximal loop. 8. A total of four associated congenital anomalies were found in one patient. 9. Postoperative complications occurred in three cases(37.5%).

• Journal of Yeungnam Medical Science
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• v.11 no.1
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• pp.115-126
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• 1994

The recent availability of recombinant human erythropoietin has opened new perspectives in the management of a variety of anemias. Clinical trials have been initiated in several countries using different approaches and methodology. We randomly assigned twelve premature infants(gestational age < 32 week) at high risk of requiring erythrocyte transfusion for anemia of prematurity with either subcutaneous recombinant human erythropoietin or a placebo. Treatment with rHuEPO was initiated at a dose of 100 units/kg day for 3 days a week. All patients were given supplemental oral iron therapy at a dose of 3 mg/kg per day, as tolerated and oral vitamin E at a dose of 25 units per day. Treated and control babies did not differ with respect to weight, hematocrit, overall mean reticulocyte count or rate of growth respectively. However, reticulocyte counts increased earlier in patients given rHuEPO. We conclude that rHuEPO administration is safe and feasible at the dose studied.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.12
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• pp.4915-4920
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• 2015

Background: : Familial adenomatous polyposis (FAP) is an autosomal dominant inherited disease mainly caused by mutations of the adenomatous polyposis coli (APC) gene with almost complete penetrance. These colorectal polyps are precancerous lesions that will inevitable develop into colorectal cancer at the median age of 40-year old if total proctocolectomy is not performed. So identification of APC germline mutations has great implications for genetic counseling and management of FAP patients. In this study, we screened APC germline mutations in Chinese FAP patients, in order to find novel mutations and the APC gene germline mutation characteristics of Chinese FAP patients. Materials and Methods: The FAP patients were diagnosed by clinical manifestations, family histories, endoscope and biopsy. Then patients peripheral blood samples were collected, afterwards, genomic DNA was extracted. The mutation analysis of the APC gene was conducted by direct polymerase chain reaction (PCR) sequencing for micromutations and multiplex ligation-dependent probe amplification (MLPA) for large duplications and/or deletions. Results: We found 6 micromutations out of 14 FAP pedigrees, while there were no large duplications and/or deletions found. These germline mutations are c.5432C>T(p. Ser1811Leu), two c.3926_3930delAAAAG (p.Glu1309AspfsX4), c.3921_3924delAAAA (p.Ile1307MetfsX13), c3184_3187delCAAA(p.Gln1061AspfsX59) and c4127_4126delAT (p.Tyr1376LysfsX9), respectively, and all deletion mutations resulted in a premature stop codon. At the same time, we found c.3921_3924delAAAA and two c.3926_3930delAAAAG are located in AAAAG short tandem repeats, c3184_3187delCAAA is located in the CAAA interrupted direct repeats, and c4127_4128 del AT is located in the 5'-CCTGAACA-3', 3'-ACAAGTCC-5 palindromes (inverted repeats) of the APC gene. Furthermore, deletion mutations are mostly located at condon 1309. Conclusions: Though there were no novel mutations found as the pathogenic gene of FAP in this study, we found nucleotide sequence containing short tandem repeats and palindromes (inverted repeats), especially the 5 bp base deletion at codon 1309, are mutations in high incidence area in APC gene,.

• Neonatal Medicine
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• v.18 no.1
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• pp.14-22
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• 2011

Neonatal bleeding is a common problem encountered in nursery rooms or neonatal intensive care units, especially among premature infants. Furthermore, owing to recent remarkable improvement of neonatology, survival rates of preterm neonates have increased; hence, neonatal bleeding cannot be emphasized enough. Since the total blood volume of neonates is small, bleeding can be one of the causes of morbidities and mortalities. Therefore, rapid diagnosis and immediate therapy is urgently needed. The patient's medical history including a familial history of a bleeding disorder or of a previously affected infant who suffered from bleeding along with maternal and neonatal drugs can provide important diagnostic clues. Presence of bleeding with or without petechiae and ecchymoses in a healthy term or late preterm infant with thrombocytopenia but normal prothrombin time and activated partial thromboplastin time strongly suggests a congenital bleeding disorder. For a sick infant who is bleeding from multiple sites, an acquired disorder such as disseminated intravascular coagulation is suspected. Intracranial hemorrhage in term or late preterm infants without a history of birth trauma is highly suggestive of coagulation disorders. The purpose of this review is to summarize recent advances in diagnostic methods is as well as basic concepts of neonatal hemostatic disorders. First, an outline of background information will be presented followed by a discussion of primary and secondary hemostatic disorders as well as inherited and acquired disorders.

• Neonatal Medicine
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• v.18 no.1
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• pp.158-162
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• 2011

Preterm infants with oligohydramnios after preterm premature rupture of membranes can present with severe respiratory distress immediately after birth, and the most common cause is pulmonary hypoplasia. Unlike infants with pulmonary hypoplasia, some cases have shown dramatic improvement with aggressive ventilatory support during the initial 1-2 days of distress: those patients have been defined as having dry lung syndrome. It is assumed that oligohydramnios leads to functional pulmonary hypoplasia by compression of the fetal lungs: some of the improvement in dry lung syndrome may thus have resulted from inflation of compressed lung tissue and increase of lung compliance. We report two incidences of dry lung syndrome that were treated successfully with high inflation pressure and inhaled nitric oxide (NO): these are the first dry lung syndrome cases to be reported in Korean infants.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.3
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• pp.1295-1301
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• 2015

Background: Cancer imposes a significant economic burden on individuals, families and society. The purpose of this study was to estimate the economic burden of cancer using the healthcare claims and cancer registry data in Korea in 2009. Materials and Methods: The economic burden of cancer was estimated using the prevalence data where patients were identified in the Korean Central Cancer Registry. We estimated the medical, non-medical, morbidity and mortality cost due to lost productivity. Medical costs were calculated using the healthcare claims data obtained from the Korean National Health Insurance (KNHI) Corporation. Non-medical costs included the cost of transportation to visit health providers, costs associated with caregiving for cancer patients, and costs for complementary and alternative medicine (CAM). Data acquired from the Korean National Statistics Office and Ministry of Labor were used to calculate the life expectancy at the time of death, age- and gender-specific wages on average, adjusted for unemployment and labor force participation rate. Sensitivity analysis was performed to derive the current value of foregone future earnings due to premature death, discounted at 3% and 5%. Results: In 2009, estimated total economic cost of cancer amounted to $17.3 billion at a 3% discount rate. Medical care accounted for 28.3% of total costs, followed by non-medical (17.2%), morbidity (24.2%) and mortality (30.3%) costs. Conclusions: Given that the direct medical cost sharply increased over the last decade, we must strive to construct a sustainable health care system that provides better care while lowering the cost. In addition, a comprehensive cancer survivorship policy aimed at lower caregiving cost and higher rate of return to work has become more important than previously considered.