Laryngeal atresia is a very rare congenital anomaly requiring immediate tracheotomy. We present a case of laryngeal atresia with tracheoesophageal fistula who showed immediate respiratory difficulty after ligation of umbilical cord and died of aspiration pneumonia at 8 days of age. The atretic portion of larynx is composed of irregulary arranged cartilaginous tissue, bundles of intrinsic muscle and soft tissue without epithelium-lined lumen. The lungs show normal development and evidences of aspiration pneumonia.
Infant, Newborn ; Humans

The umbilical cord length may be a reliable indicator of fetal activity during gestation because it grows in response to tensile forces related to fetal movement. But there has been little attention to normal range of the umbilical cord length. We analyzed the umbilical cord length of 2342 cases de1ivered in Seoul National University Hospital to determine mean values according to the gestational age. The umbilical cord length increased linearly from 15.5cm of 13 week to 50.6 cm of 44 week. After 42 week, the growth was nearly stopped. There was no difference by fetal sex. The length of umbilical cord was variable even within same gestational age.

Infantile myofibromatosis with systemic involvement is a very rare disease and is characterized by numerous nodules composed of spindle cells of a myofibroblastic nature. There are often disseminated throughout the subcutis, muscle, skeleton and viscera. We report an autopsy case of infantile myofibromatosis in a stillborn female fetus of 32 weeks of gestation. The nodules, Imm to 2 cm, were found over the whole body and viscera. The involved viscera were the heart, tongue, esophagus, gastrointestinal tract, portal areas of the liver, spleen anc pancreas. There were also associated malformations, viz., frontal meningoencephalocele, flexion defer-mities, syndactyly, cleft palate, agenesis of corpus callosum, pachygyria, diaphragmatic hemia, renal hypoplasia, etc. Multiple basaloid follicular hamartomas of the skin were noted on the face and extremeties. There are no previous reports in the literature of infantile myofibromatosis in conjunction with the above skin lesion and congenital malformations.
Infant ; Male ; Female ; Humans ; Hamartoma

Two cases of urachal anomaly (1 urachal cyst and 1 patent urachus) are reported in a neonate and an eleven-year old boy, respectively. In case 1, the patient was born after an uncomplicated pregnancy to a mother who had taken progesterone during the first trimester. Because of breech presentation, cesarian section was elected to deliver a male baby weighing 2.3 kg who showed abdominal distension. The patient died of respiratory difficulty several minutes after birth. At autopsy, there was a large cyst in the midpoint of the abdominal and pelvic cavity. This round cyst was composed of two components, urachus and urinary bladder. No area of umbilicocystic fistula was present. The lining epithelium was chiefly of transitional type. Assocaited anomalies were segmental stenosis of posterior urethra, absence of abdominal musculature, bilateral polycystic kidney of Potter type IV, hydroureter, and hypoplasia of lungs. Low set ears, micrognathia and club foot were also present. In case 2 the patient was 11-year old boy. He had suffered from intermittent urinary dribbling from umbilicus since early infancy, whenever the abdominal pressure was increased. The patency of urachus was confirmed by fistulography. And the urachal anomaly was surgically removed. Histopathologically the resected patent urachus consisted of pseudostratified columnar to transitional epithelium resting on fibrous stroma mixed with well formed smooth muscle bundles.
Pregnancy ; Female ; Male ; Infant, Newborn ; Humans ; Cysts

No abstract available.
Brain Infarction* ; Brain* ; Nephrotic Syndrome*

PURPOSE: This study was performed to investigate the prevalence of Staphylococcus aureus (S. aureus) nasal carriage in Korean children attending day care centers. METHODS: During September and October 2009, a survey for nasal carriage of S. aureus and methicillin-resistant S. aureus (MRSA) was conducted among children attending day care centers located in Seoul with questionnaire survey for evaluation of risk factors of acquisition of MRSA was obtained from their guardians. A culture of the anterior nares swabs using enrichment broth was executed for isolating S. aureus and oxacillin susceptibility was assessed by the disk diffusion method. RESULTS: Out of the 428 children enrolled whose mean age was 55 months old, 163 (38.1%) were colonized with S. aureus. Of the 163 isolates, 40 (24.5%) were MRSA. The nasal carriage rate of S. aureus showed an increasing trend with increase of age. Based on the answer to the questionnaire, 9.2% and 3.6% of children had a recent history of hospitalization and surgery, respectively, and approximately 40% of children had a history of prescription of antibiotics within 1 year prior to enrollment. Of the 428 subjects, 40 (9.3%) were MRSA nasal carriers. CONCLUSION: S. aureus and MRSA carriage rate of children attending day care center in Korea was 38.1% and 9.3%, respectively. Continued surveillance for nasal carriage rate of S. aureus and MRSA (especially community-associated MRSA) is mandatory.
Anti-Bacterial Agents ; Child ; Colon ; Day Care, Medical ; Diffusion ; Hospitalization ; Humans ; Korea ; Methicillin Resistance ; Methicillin-Resistant Staphylococcus aureus ; Oxacillin ; Prescriptions ; Prevalence ; Surveys and Questionnaires ; Risk Factors ; Staphylococcus ; Staphylococcus aureus

No abstract available.
Graft vs Host Disease*

The association of major fetal malformations with amniotic bands has been known for many years. However, we are apt to ignore the possibility of amniotic band syndrome. In this case, fetal anencephaly was diagnosed at 17 weeks, menstrual age on the basis of sonographic findings. Following pregnancy termination, examination of the abortus rev- ealed the cerebral remnant which is similar to that found in dysraphic anencephaly, but collateral evidence of amniotic band was found. Therefore, when confronted with severe cranial or cerebral malformation amniotic band syndrome should be in the differential dia- gnosis.
Amniotic Band Syndrome* ; Anencephaly* ; Infant, Newborn ; Pregnancy ; Skin* ; Ultrasonography

Invasive infection of the Streptococcus bovis group in a neonate is rare. In cases reported to date, the pathogen of neonatal S. bovis infections is usually Streptococcus gallolyticus subsp. pasteurianus (S. bovis biotype II/2). Streptococcus lutetiensis (S. bovis biotype II/1) was identified using 16S rRNA and tuf gene sequence analysis of the isolates from blood and cerebrospinal fluid (CSF) of a fever-presenting 28-day-old male. Blood culture analysis was performed using automatic equipment (VITEK 2) and identified Streptococcus infantarius supsp. infantarius, yet we were unable to get accurate results from the CSF culture. The fever subsided on the second day of hospitalization, and the patient was discharged without neurologic complication after 14 days of antibiotic therapy. In this case, we were able to accurately identify the pathogen using molecular genetic methods. To our knowledge, this is the first case of late onset neonatal bacteremia and meningitis caused by S. lutetiensis.
Bacteremia* ; Cerebrospinal Fluid ; Fever ; Hospitalization ; Humans ; Infant, Newborn ; Male ; Meningitis* ; Molecular Biology ; Sequence Analysis ; Streptococcus bovis ; Streptococcus*

No abstract available.
Autopsy* ; Cardiomyopathies* ; Humans ; Infant, Newborn*