• Neonatal Medicine
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• v.16 no.1
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• pp.85-88
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• 2009

The amniotic band syndrome is an uncommon congenital fetal abnormality, presumably due to fetal entanglement in strands of ruptured amniotic sac. The defects caused by this syndrome vary from simple limb defects to major visceral and craniofacial defects. We cared for a newborn infant with this syndrome, who showed constriction rings of the right leg and right axilla, right club foot, thoracic scoliosis, polydactyly, absence of the right thumb and aplasia cutis of the scalp. We report this case with a brief review of the literature.

• Neonatal Medicine
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• v.16 no.1
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• pp.89-93
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• 2009

Maternal diabetes is known to have teratogenic effects which increase the risk for congenital anomalies, such as caudal dysplasia, cardiac defects, hydronephrosis, and small left colon syndrome. Infants of diabetic mothers have a 10-fold higher frequency of anomalies in the central nervous system and a 5-fold higher frequency of congenital heart defects. However, jejunal atresia combined with multiple anomalies of the face, ears, and hands has rarely been reported. Herein we report a neonate born to a diabetic mother, who had hemifacial microsomia, displacement of the lacrimal ducts, polydactyly of the right hand, microtia of the right ear and proximal jejunal atresia presenting as bile regurgitation on the 1st day of life.

• Neonatal Medicine
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• v.16 no.1
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• pp.94-98
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• 2009

Listeria monocytogenes (L. monocytogenes) is a foodborne anaerobic gram-positive rod and the third most common pathogen for neonatal meningitis. Although the mortality and morbidity of L. monocytogenes infections are high, thus causing serious problems in Western populations, neonatal listeriosis is relatively rare in Eastern countries, including Korea. Possible routes for intrauterine infection or vertical transmission of L. monocytogenes include infected placentas and the reproductive tract. Intrauterine infections may cause chorioamnionitis, preterm labor, spontaneous abortion, stillbirth, or neonatal infection. A high index of suspicion and early empirical antibiotic treatment are critical to achieve a favorable prognosis for neonatal listeriosis. We managed a case of L. monocytogenes sepsis and pneumonia in a premature neonate born at 26 weeks of gestational age from an asymptomatic mother with culture-proven placental infection. The neonate was successively treated with ampicillin and gentamicin.

• Neonatal Medicine
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• v.16 no.1
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• pp.76-80
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• 2009

A male baby with intrauterine growth retardation had a short neck, small hands and feet, hypospadia, both grade I hydronephrosis, type II atrial septal defect, and moderate valvular pulmonary stenosis. The routine chromosome and banding analyses revealed a 46,XY,rec(8)del(8)(p21)dup(8) (q24.1)inv(8)(p21q24.1)pat chromosome constitution. His mother has normal chromosomes, but the father had 46,XY,inv(8)(p21q24.n Also his uncle had an inv(8) chromosome constitution. We used lymphocytes and examined 40 mitotic cells. All mitotic cells showed deletion of 8p21-->pter and duplication of 8q24.1 -->qter. Because Sp21 involves secretion of macrophage and lymphocyte against cancer cells, long-term follow-up for cancer will be needed.

• Neonatal Medicine
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• v.15 no.2
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• pp.119-122
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• 2008

Clinical research is a necessity, not an option, for developing better and new medicines and therapeutic modalities. But in the course of clinical research, there are rules and guidelines that should be followed to ensure the due respect for persons, beneficence, and justice for persons who voluntarily participate in the research as described in the Belmont Report. Good Clinical Practice (GCP) is an "international scientific and ethical quality standard for designing, conducting, recording, and reporting" clinical trials. The main purposes of GCP would be to protect rights, safety, and well-being of trial subjects, in compliance with the principles of Declaration of Helsinki, and to assure that the data obtained from clinical trials are credible. In order to achieve these, investigators must be fully aware of the meanings as well as actual procedures involved in the research and should make the best effort to comply with GCP. For those individuals who belong to vulnerable populations, such as neonates, in addition to the general principles of GCP, further measures to ensure added protection should be implemented. It is our duty to develop and provide better care through clinical research even for neonates. But in doing so, we have to make sure that the importance of protecting the rights, safety, and well-being of the subjects supersede the interests of science and society.

• Neonatal Medicine
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• v.15 no.2
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• pp.172-175
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• 2008

Hemangioma is the most common benign tumor of infancy. Greater than 60% of hemangiomas occur on the head and neck, and have an uncomplicated course. In contrast, most complicated hemangiomas develop in the urogenital or anogenital areas. These lesions are frequently associated with pain, bleeding, recurring infections, and ulcerations. Sometimes, perianal ulcerative hemangiomas are difficult to treat with multiple therapies, such as laser and steroid therapy. We managed a case of a severe perianal ulcerative hemangioma in a male newborn who did not respond to conservative management. He was successfully treated after a colostomy.

• Neonatal Medicine
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• v.15 no.2
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• pp.183-189
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• 2008

Infantile hepatic hemangioendotheliomas (IHHEs) are benign vascular tumors, but can be associated with the life-threatening complications, such as congestive heart failure, disseminated intravascular coagulation, and massive bleeding. Various therapeutic options have been developed and the treatment response depends on the patient's clinical status and the nature of the lesion. In the case of a symptomatic IHHE, a non-invasive and precise diagnosis should be performed promptly before the therapeutic method is chosen. Additionally, it should be kept in mind that the residual lesions have malignant potential. We report a case of a congenital giant IHHE that was successfully reduced in size by interferon-$\alpha$ and completely removed by surgical tumor resection with a hepatic lobectomy.

• Neonatal Medicine
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• v.15 no.2
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• pp.196-199
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• 2008

The interstitial cells of Cajal are the pacemakers in the gastrointestinal tract that modulate gastrointestinal motility. A case of a neonate with intestinal pseudo-obstruction caused by a decreased number of the interstitial cells of Cajal is presented. A premature male infant born at 32 weeks of gestation showed progressive abdominal distention beginning 3 days after initiation of enteral feeding at 15 days of life. No etiologic factors were identified on radiologic studies, a gastrographin enema, and an intestinal biopsy other than a markedly decreased number of the intestinal cells of Cajal. An ileostomy, followed by repair of the ileostomy was done, which resulted in but a limited improvement of the abdominal gas pattern. Respiratory distress, pancytopenia, and abdominal distention persisted, and the infant expired on 142 days of life.

• Neonatal Medicine
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• v.15 no.2
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• pp.200-206
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• 2008

Human chromosome 9 is characterized by a high degree of morphologic heteromorphisms, including variation in the size of the heterochromatin. We present a case of a de novo short arm addition of chromosome 9, [46, XY, add(9)(p13)], associated with multiple anomalies, including trigonocephaly, upward slant of the palpebral fissures, patent ductus arteriosus, pulmonary hypertension, hypertrophic cardiomyopathy, umbilical hernia, ambiguous genitalia, and sensorineural hearing and visual loss. This mutation affects the pericentric region of the heterochromatin. This patient exhibited a clinically important breakpoint of the heterochromatic region of chromosome 9 short arm and the associated anomalies.

• Neonatal Medicine
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• v.15 no.2
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• pp.207-211
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• 2008

Congenital syphilis is a rare cause of non-immune hydrops fetalis. We cared for a neonate with hydrops fetalis who was delivered by emergency Cesarean section due to prolonged fetal bradycardia and ascites at 34 weeks of gestation. He had anemia, purpura, and hepatosplenomegaly, and the serologic tests revealed congenital syphilis (high titers of serum VDRL and TPHA, and a positive serum FTA-ABS IgM). He survived after aspiration of ascitic fluid, ventilator care, and intravenous penicillin therapy. We report a case of non-immune hydrops fetalis due to congenital syphilis with a brief review of literature.