• Journal of Appropriate Technology
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• v.7 no.2
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• pp.206-210
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• 2021

Hyperbilirubinemia is the most common neonatal disease and is observed in about 80% of newborns worldwide in neonatal period within a week after birth. Untreated infant hyperbilirubinemia may lead to brain damage and even death, so it is very important to diagnose it quickly and accurately. In this study, a total bilirubin measurement system was developed that is portable and easy to use without pre-processing using a commercial smartphone. This system measures using the LED and camera of the smartphone without the need for additional devices, and because a small amount of blood is injected without pre-treatment, anyone can easily measure it in the field. In a comparative study with Cobas c111 results, accuracy meets CLIA guidelines with 94% (17/18) within ±0.4 mg/dL below 3 mg/dL and 98% within ±20% above 3 mg/dL (276/282). This system offers a simple, fast and accurate diagnosis for jaundice in infants and young children in low-resource settings.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.31-37
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• 2017

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an autosomal recessive disorder of the urea cycle that causes hyperammonemia. Two forms of CPS1D are recognized: a lethal neonatal type and a less severe, delayed-onset type. Neonatal CPS1D cases often present their symptoms within the first days of life. Delayed-onset cases are predominantly adolescents or adults, and infantile delayed-onset cases are rare. Severe hyperammonemia in the neonatal period leads to serious brain damage, coma, and death if not treated promptly. Therefore, early diagnosis and acute treatment are crucial. Despite the improvement of treatments, including continuous hemodialysis, ammonia-lowering agents, and a low-protein diet, the overall outcome of severe forms of hyperammonemia often remains disappointing. As the liver is the only organ in which ammonia is converted into urea, liver transplantation has been considered as an elegant and radical alternative therapy to classical dietary and medical therapies. However, liver transplantation has many disadvantages, such as a considerable risk for technical complications and perioperative metabolic derangement, especially in neonates. Additionally, there is a lack of suitable donor organs in most countries. According to recent studies, liver cell transplantation is a therapeutic option and serves as a bridge to liver transplantation. Here, we report a Korean CPS1D patient with novel mutations in CPS1 who was treated by liver cell transplantation after being diagnosed in the neonatal period and showed a good neurodevelopmental outcome at the last follow-up at six months of age.

• Clinical and Experimental Pediatrics
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• v.51 no.2
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• pp.219-221
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• 2008

Intestinal obstruction is not uncommon in infants. The common causes of intestinal obstruction in the neonatal period are Hirschsprung disease, intestinal atresia, meconium ileus, and intussusception. However, small bowel obstruction caused by a congenital band is very rare. We report a 27-day-old baby who was admitted with abdominal distension and fever. The abdominal X-ray revealed massive bowel dilatation and the contrast gastrografin enema suggested a distal small bowel obstruction. The explolaparotomy showed small bowel entrapment due to an unusual anomalous congenital band.

• Archives of Reconstructive Microsurgery
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• v.21 no.2
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• pp.137-142
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• 2012

Purpose: To report the clinical and radiological result of the vascularized fibular epiphyseal transplantation in the treatment of humeral head deformity by septic arthritis Material & Methods: A 3 years old male who has humeral head deformity and bone defect by septic arthritis on neonatal period. We replaced bone defect as vascularized fibular epiphyseal transplantation and lengthened humerus shaft for humerus discrepancy. We followed it up for 14 years. Result: We saw the callus formation 2 months after surgery and obtained bone union, one year after surgery. The transplanted fibular bone got hypertrophy. We could check full range of motion on lt. shoulder and The bone deformity was not worsened and The graft did not displaced on last follow up. Conclusion: Humeral head reconstruction by vascularized fibular epiphyseal transplantation showed good clinical outcome.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.38
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• pp.8.1-8.4
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• 2016

Congenital alveolar synechiae is a rare anomaly mostly presenting in association with cleft palate. Owing to reduced mouth opening, feeding difficulties, and compromised airway in extreme cases along with presentation in early neonatal period, these patients present unique challenges to the surgeon as well as the anesthetist. Here, we discuss the surgical and anesthetic management of this entity in a 12-month-old female child.

• Journal of Chest Surgery
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• v.28 no.11
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• pp.1045-1048
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• 1995

Pulmonary atresia with VSD is uncommon congenital anomaly with high mortality in neonatal period.Recently we experienced surgical correction of pulmonary atresia with VSD. The case was 2 month old male patient diagnosed as pulmonary atresia with VSD and PDA. Atretic pulmonary artery segment from Rt ventricular infundibulum to pulmonary artery was lcm in length. The pulmonary trunk tapered toward Right ventricular infundibulum and resulted in blind pouch with diameter of lmm. The left pulmonary artery was stenosed at just proximal and distal part to which PDA was connected. Total correction was undertaken which consisted of PDA ligation, dacron patch closure of VSD, establishment of continuity between right ventricle and pulmonary artery with autogenous pericardium. Postoperative systolic fight ventricular pressure and left ventricular pressure ratio was 0.7. In patient with pulmonary atresia with VSD it is advisable to perform a corrective operation, whenever the size and anatomy of pulmonary artery are acceptable for it.

• Journal of Chest Surgery
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• v.20 no.4
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• pp.825-828
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• 1987

Authors carried out a retrospective study in 22 infants with Bochdaleck`s hernia who were operated upon, the results are; 1] the male to female ratio was 2:1 and the incidence of left side was threefold prevalent than right, 2] the chief complaint of those who were operated upon in neonatal period was dyspnea and diagnosis was possible by chest A-P, 3] primary repair was possible except one patient in whom a prosthesis was used. 4] complication occurred in 41% and pulmonary complication was most common including 5 pneumothorax, 5] mortality rate was 14%, the deaths occurred in infants who were operated upon within 48 hours of life and the lesion was left without sac, the defect was larger than average and all died within 72 hours after operation.

• Clinical and Experimental Pediatrics
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• v.53 no.6
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• pp.669-679
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• 2010

The risk of mortality and morbidity of patients with congenital heart defects (CHDs) is highest during neonatal period and increases when diagnosis and proper management are delayed. Neonates with critical CHDs may present with severe cyanosis, respiratory distress, shock, or collapse, all of which are also frequent clinical presentations of various respiratory problems or sepsis in the newborn. Early diagnosis and stabilization and timely referral to a tertiary cardiac center are crucial to improve the outcomes in neonates with CHDs. In this review, the clinical presentation of critical and potentially life-threatening CHDs is discussed along with brief case reviews to help understand the hemodynamics of these defects and ensure proper decision-making in critically ill patients.

• Journal of Chest Surgery
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• v.49 no.2
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• pp.115-118
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• 2016

Williams syndrome (WS) is a developmental disorder characterized by vascular abnormalities such as thickening of the vascular media layer in medium- and large-sized arteries. Supravalvular aortic stenosis (SVAS) and peripheral pulmonary artery stenosis (PPAS) are common vascular abnormalities in WS. The natural course of SVAS and PPAS is variable, and the timing of surgery or intervention is determined according to the progression of vascular stenosis. In our patient, SVAS and PPAS showed rapid concurrent progression within two weeks after birth. We report the early manifestation of SVAS and PPAS in the neonatal period and describe the surgical treatment for stenosis relief.

• Advances in pediatric surgery
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• v.3 no.2
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• pp.164-167
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• 1997

Conjoined twins are one of the rarest and most challenging congenital anomalies in pediatric surgery. Successful surgical separation is difficult because it the majority of conjoined twins in is technically typical to separate shared vital organs successfully. The timing of separation is variable, but separation is usually delayed until such infants are relatively mature(i.e, 9-12 months of age). Operative survival was 50 % in the neonatal period, but 90 % in those over 4 months of age. The present case was successfully separated early beacase of cardiac problems in one of the twins. These twins were omphalopagus and only the liver was shared through a bridge. The vascular and biliary trees were independent from each other. Successful surgical separation was on 11th day of life, but one died of sepsis 18 days after operation.