Combination Therapies of Amiodarone and Digoxin for Refractory Supraventricular Tachycardia Accompanied by Congenital Heart Diseases in a Preterm Infant with Hydrops Fetalis

Fetal tachycardia is at risk for developing low cardiac output, non-immune hydrops fetalis and ultimately fetal death. Spontaneous resolution of supraventricular tachycardia (SVT) is common during the first year of age, but some infants need long-term antiarrhythmic therapy. In almost neonatal tachyarrhythmia including SVT, adenosine is the drug of the first choice. Digoxin is used to treat the SVT which is not controlled with adenosine. Class Ic and III antiarrhythmic drugs are additionally recommended for the disease unresponsive to digoxin. Intravenous amiodarone is highly effective and safe in an infant with refractory or life threatening tachycardia. Some cases have been reported that amiodarone combined with digoxin therapy is effective for treating tachycardia. We herein report a case of a preterm infant-born at 32 weeks of gestational age-with hydrops fetalis and life-threatening refractory SVT accompanied by multiple congenital heart diseases. SVT was initially not responsive to adenosine therapy, however, it was then successfully controlled with combination therapies of amiodarone and digoxin.

Therapeutic Application of Neural Stem Cells for Neonatal Hypoxic-ischemic Brain Injury

Neural stem cells (NSCs) are characterized by a capacity for self-renewal, differentiation into multiple neural cell lineages, and migration toward damaged sites in the central nervous system (CNS). NSCs expanded in culture could be implanted into the brain where they integrate into host neural circuitry and stably express foreign genes. It hence appears that transplantation of NSCs has been proposed as a promising therapeutic strategy in neurological disorders. During hypoxic-ischemic (HI) brain injury, factors are transiently elaborated to which NSCs respond by migrating to degenerating regions and differentiating towards replacement of dying neural cells. In addition, NSCs serve as vehicles for gene delivery and appear capable of simultaneous neural cell replacement and gene therapy (e.g. with factors that might enhance neuronal differentiation, neurites outgrowth, proper connectivity, neuroprotection, and/or immunomodulatory substances). When combined with certain synthetic biomaterials, NSCs may be even more effective in 'engineering' the damaged CNS towards reconstitution. Human NSCs were isolated from the forebrain of an aborted fetus at 13 weeks of gestation and were grown as neurospheres in cultures. After the characterization of human NSCs in preclinical testing and the approval of the IRB, a clinical trial of the transplantation of human NSCs into patients with severe perinatal HI brain injury has been performed. The existing data from these clinical trials have shown to be safe, well tolerated, and of neurologically-some benefits. Therefore, long-term and large scale multicenter clinical study is required to determine its precise therapeutic effect and safety.

Meconium-related Ileus in Extremely Low-birth Weight Newborn Infants Meconium-related Ileus in ELBWI

PURPOSE: Meconium-related ileus (MRI) is one of the major causes of bowel obstruction in extremely low-birth weight newborn infants (ELBWI). Hyperosmolar water-soluble contrast (HWSC) enemas been recognized to be an effective treatment for MRI. The purpose of this study is to observe clinical findings of MRI accompanied by ELBWI and evaluate the therapeutic efficacy and complications of HWSC enemas. METHODS: A total of 15 ELBWI with MRI were treated with HWSC enemas under the guidance of ultrasonography at the bedside in the NICU between 2008 and 2011. Clinical findings of 15 patients were reviewed and compared with those of 48 ELBWI without MRI administered to NICU during the same period. Radiological findings, therapeutic efficacy and complications of HWSC enemas in patients with MRI were also reviewed. RESULTS: Patients with MRI, compared to those without MRI, showed the following significantly lower Apgar score at 1 minute, higher incidence of preeclampsia, bronchopulmonary dysplasia and sepsis, and longer duration of the first meconium passing and non-feeding per oral. Fourteen patients with MRI had resolved bowel obstruction successfully following 1-2 trials of enema. One case was not relieved following 3 trials of enema, showed no clinical improvement, and died of severe intraventricular hemorrhage and multi-organ failure at 45 days old. No complications associated with HWSC enemas were observed in all cases. CONCLUSION: Administration of HWSC enemas under the guidance of abdomen ultrasonography in the NICU is safe and efficacious for the rapid diagnosis and treatment of MRI even accompanied by ELBWI.

A Case of Newborn with Prenatally Detected Cerebral Venous Sinus Thrombosis / 대한주산의학회잡지

Cerebral venous sinus thrombosis (CVST) in pediatric patients is rare, but the incidence of this disease is much higher in neonates. It is one of the important causes of neonatal seizures, and also can cause long term neurologic deficits. So far, there has been only one retrospective study of 10 neonatal CVST patients reported in Korea. However, there are no reported cases of prenatally diagnosed patient. We report a patient prenatally diagnosed with CVST which might cause severe neurologic complication, as we experienced significant decrease in size and number of thrombi without anticoagulation therapy and relatively benign clinical progress from birth to 1-year-old age.

Inflammatory Myofibroblastic Tumor in a 3 Months Old Infant / 임상소아혈액종양

Benign intestinal tumors are rare in infancy, however the authors describe an inflammatory myofibroblastic tumor (IMT) of the mesentery in a 3-month-old infant who was diagnosed incidentally. During operation, huge mass which was attached to proximal jejunum was resected, from which a histological diagnosis of IMT was made. A review of the literature for this rare condition was done to delineate the natural history of this tumor. Because of the risk of local recurrence and malignant transformation, IMT cases needed a long-term follow up.

Inflammatory Myofibroblastic Tumor in a 3 Months Old Infant / 임상소아혈액종양

Benign intestinal tumors are rare in infancy, however the authors describe an inflammatory myofibroblastic tumor (IMT) of the mesentery in a 3-month-old infant who was diagnosed incidentally. During operation, huge mass which was attached to proximal jejunum was resected, from which a histological diagnosis of IMT was made. A review of the literature for this rare condition was done to delineate the natural history of this tumor. Because of the risk of local recurrence and malignant transformation, IMT cases needed a long-term follow up.