• Neonatal Medicine
• /
• v.18 no.1
• /
• pp.34-41
• /
• 2011

Neonatal coagulation disorders and thromboembolism require timely management. Failure to treat these conditions at the appropriate time may lead to death or the development of significant long-term sequelae. However, most current guidelines for managing neonatal coagulation disorders and thromboembolism are empiric and not based on randomized clinical trials. Thus, it is not easy to choose an appropriate management strategy for these conditions in clinical settings. In this review, therapeutic guidelines currently utilized in clinics and novel therapeutic options still under investigation are presented and reviewed.

• The Korean Journal of Pain
• /
• v.23 no.4
• /
• pp.266-269
• /
• 2010

Spinal cord stimulation (SCS) is used to manage chronic pain syndromes and it is accepted a cost-effective therapy. Child-bearing women who had SCS become or choose to become pregnant despite these policies pregnancy is a relative contraindication. A 32-year-old woman had SCS as a treatment for the CRPS I of the left lower extremity, During various check up tests, we happen to find out that her serum beta-hCG was positive and confirmed pregnancy. SCS is not recommended in pregnancy because the effects of SCS on pregnancy and nursing mothers had not been confirmed. However, many female patients suffering from chronic pain may expect future pregnancy and we think that they must be informed about the possibility of pregnancy and the effects of SCS device implantation in the course of pregnancy. First of all, a good outcome requires a multidisciplinary team approach, including obstetrics, neonatology, pain medicine and anesthesia, as was used from an early pregnancy. Unfortunately, she had a misabortrion after 6 weeks.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.20 no.1
• /
• pp.55-60
• /
• 2017

Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to $Omegaven^{TM}$, a fat emulsion comprised of omega-3 fatty acids. $Omegaven^{TM}$ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with $Omegaven^{TM}$ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of $Omegaven^{TM}$. No current guidelines for the diagnosis and management of hypoganglionosis are available. We recommend a multidisciplinary approach and the use of novel therapies such as fat emulsions composed of omega-3 fatty acids for improved patient outcomes. Appropriate compassionate use protocols should be obtained from the Food and Drug Administration prior to initiation of $Omegaven^{TM}$.

• Neonatal Medicine
• /
• v.17 no.1
• /
• pp.123-126
• /
• 2010

Hepatitis A virus (HAV) infection is a common cause of acute hepatitis, but is rarely responsible for neonatal hepatitis. HAV infection is usually transmitted by the fecal-oral route, but during the neonatal period can be transmitted by the intrauterine vertical route or postnatal horizontal route. HAV infection is usually self-limited, but it can be potentially life-threatening in adults. The clinical course and symptoms are mild and the infection can be asymptomatic in newborns. Recently, we experienced a case of symptomatic neonatal hepatitis A. To our knowledge, this is the first case of neonatal hepatitis with HAV infection in Korea.

• Neonatal Medicine
• /
• v.17 no.1
• /
• pp.132-135
• /
• 2010

Although the majority of abnormal bleeding during the neonatal period results from acquired coagulation disorders, inherited coagulation disorders can also manifest at this time. Hemophilia is the most common of inherited coagulation disorder. Although 40-70% of cases with hemophilia are diagnosed in the neonatal period, few cases have been reported in premature infants. We report a case of a premature infant born at 31 weeks of gestation, diagnosed with hemophilia A by blood coagulation test, coagulation factor assay and study of the F8 gene. The baby was treated with recombinant factor VIII (Recombinate$^{(R)}$, USA) because of repeated seizures and intramuscular hematoma.

• Neonatal Medicine
• /
• v.17 no.1
• /
• pp.147-151
• /
• 2010

Giant congenital melanocytic nevi are very rare, with an estimated incidence of 1 in 20,000 live births. They have a high risk of malignant melanoma transformation and neurological deficits such as neurocutaneous melanocytosis and epilepsy. Early evaluation, surgical intervention and careful long term follow up are recommended to monitor for malignant transformation. We report one case of giant congenital melanocytic nevi diagnosed at birth with the related literatures.

• Neonatal Medicine
• /
• v.17 no.1
• /
• pp.116-122
• /
• 2010

Kasabach-Merritt syndrome is a rare thrombocytopenic consumptive coagulopathy associated with a giant hemangioma. We experienced a case of unexplained ascites with thrombocytopenia in a 32 week premature infant. An exploratory laparotomy was performed to determine the cause of the refractory ascites and thrombocytopenia. An intestinal hemangioma was found, but, surgical removal was not performed due to the extensive involvement. Hemangioma was confirmed by SPECT (single-photon emission computed tomography) and the thrombocytopenia was treated with steroid therapy. It is recommended that hemangioma of the visceral organs should be suspected when unexplained thrombocytopenia and disseminated intravascular coagulopathy persist.

• Neonatal Medicine
• /
• v.17 no.1
• /
• pp.127-131
• /
• 2010

Supernumerary derivative (22) syndrome is a rare genomic syndrome. It is characterized by severe mental retardation, microcephaly, failure to thrive, preauricular tag or sinus, ear abnormalities, cleft and/or high-arched palate, micrognathia, kidney abnormalities, congenital heart defects, and genital abnormalities in males. In 99% of the cases, one of the parents is a balanced carrier of a translocation between chromosome 11 and chromosome 22. To date, there have been about 100 case reports of supernumerary derivative (22) syndrome. In most of the cases, supernumerary derivative (22) syndrome was the result of 3:1 meiotic segregation in the maternal 11;22 translocation carrier. We now report a case of 47,XX, + der(22)t(11;22)(q23;q11.2) resulting from 3:1 meiotic segregation of the paternal translocation carrier.

• Neonatal Medicine
• /
• v.16 no.2
• /
• pp.101-109
• /
• 2009

Pulmonary surfactant (PS) therapy in premature infants has a remarkable impact on improving survival and outcomes in neonatal respiratory distress syndrome (RDS). Early PS therapy involves instillation of PS upon delivery of very premature infants or if there is evidence of RDS, such as an increased requirement of oxygen 2 hours after birth, especially in infants <30 weeks gestation. Early PS treatment in very premature infants results in a significant reduction in the severity of RDS, mortality, and incidence of pneumothorax, pulmonary interstitial emphysema, and bronchopulmonary dysplasia in comparison with late PS treatment. According to European and American consensus guidelines on the management of neonatal RDS, early PS instillation should be considered for infants <30 weeks gestation, infants with a birth weight <1,250 g, or if the mother has not received antepartum corticosteroids. We suggest that the Korean health insurance policy on RDS be modified so that PS can be used for better clinical outcomes of very premature infants.

• Neonatal Medicine
• /
• v.16 no.2
• /
• pp.121-130
• /
• 2009

Over the past 20 years, neonatal mortality rates for preterm infants, particularly those born extremely preterm and with a very low birth weight, have decreased steadily. As more very immature preterm infants survive, provision of enteral feeding has become a major focus of concern. According to many experts on neonatal nutrition, the goal for the nutrition of preterm infants should be to achieve a postnatal growth rate approximating that of a normal fetus of the same gestational age. Total parenteral nutrition for maintaining nutritional integrity is mandatory before successful transition to enteral feeding. Early initiation of trophic enteral feeding is vital for postnatal adaptation. Recently published randomized controlled trials provide no evidence to support the practice of postponing enteral feeding to reduce the incidence of necrotizing enterocolitis. Early trophic feeding yields demonstrable benefits and there is currently no evidence of any adverse effects following early feeding. Preterm milk from the infant's own mother is the milk of choice, which can always be supplemented with a human milk fortifier. Here we review over 50 randomized controlled trials and over seven systematic reviews published on neonatal parenteral and enteral feeding of preterm infants. Neonatologists must make use of the evidence from these studies as a reference for feeding protocols for preterm infants in their NICUs are to be based.