Congenital Syphilis: An Uncommon Cause of Gross Hematuria, Skin Rash, and Pneumonia / 소아감염

Although congenital syphilis can be prevented with prenatal screening, the disease remains problematic. Currently, there are no cases that describe hematuria and pneumonia related to congenital syphilis. We report a case of congenital syphilis that involved nephrotic syndrome and pneumonia alba in a 22-day-old male infant whose mother did not receive adequate prenatal care. The congenital syphilis diagnosis was confirmed with a serologic test and the patient recovered with penicillin treatment. Clinical findings may be subtle in neonates and delayed recognition occurs frequently, thus complete prenatal screening is critical for congenital syphilis prevention. Immediate serologic testing should be performed to obtain a differential diagnosis if an infant is delivered by a mother that has not received appropriate prenatal examinations.

Clinical Study of Influenza B-Associated Myositis / 소아알레르기및호흡기학회지

PURPOSE: Influenza B-associated myositis is an infrequent and poorly known complication of influenza B virus infection in children. The aim of this study was to describe the clinical and laboratory manifestations, diagnosis, and outcomes of influenza B-associated myositis in Korean children. METHODS: A retrospective analysis was conducted in patients aged <16 years who had been diagnosed with influenza B-associated myositis at the Catholic University Bucheon Saint Mary's Hospital between April 2010 and May 2010. RESULTS: Overall, 16 cases were analyzed. Influenza B-associated myositis typically occurred in preschool-aged children with a 3:1 male predominance. The mean age was 4.6 years (range, 3 to 7 years). The median interval between onset of influenza infection and onset of influenza B-associated myositis was 3 days (range, 1 to 7 days). Only the calf muscles were involved in all patients. Blood creatine phosphokinase concentration was elevated in most patients. Median duration to clinical recovery was 3 days (range, 1 to 4 days). No patient had rhabdomyolysis, renal failure, or related sequelae. All patients had a favorable outcome without administration of antiviral drugs. CONCLUSION: Clinical and laboratory findings of influenza B-associated myositis are very characteristic and allow a rapid diagnosis during the influenza season. Outcomes of influenza B-associated myositis are good with only proper supportive treatment.

The Relationship between the Timing of Gestational Diabetes Screening and HbA1c Level and Neonatal Outcome / 대한진단검사의학회지

BACKGROUND: The aim of this study was to observe clinical outcomes of the mother and her infant who were possibly exposed to high blood glucose at least 2-3 months in the early and midterm pregnancy by checking gestational weeks (GW) and the first HbA1c level at initial diagnosis of gestational diabetes (GDM). METHODS: A total of 107 GDM patients and their newborns were subject of this study. GDM patients were newly diagnosed at the Holy Family Hospital of Catholic University from January 2003 until December 2007 and continuously managed in the diabetes center. Patients medical records were retrospectively reviewed to evaluate GW and HbA1c level at the time of diagnosis, and clinical outcomes of mother and newborn baby. RESULTS: The proportion of subjects who had been diagnosed of having GDM according to GW was 7.5%, in less than 24th week of pregnancy; 55.1% in the 24-28th week; 28.0% in the 29-32nd week; and 9.4% 33rd week or more. There were 39 out of 107 subjects (36.4%) with HbA1c levels > or =6.5% and 26 out of 39 subjects (24.3%) with HbA1c levels > or =7.0%. In clinical outcomes of newborn by HbA1c levels, the frequency of delivery of large for gestational age (LGA) infant was higher in mothers diagnosed with GDM after 29th week of pregnancy or with HbA1c levels 7.0% or more (P<0.001). CONCLUSIONS: If the screening test for gestational DM was delayed, HbA1c level and the risk for LGA seemed to be higher, so it may be necessary to screen GDM no later than 24th week of pregnancy.

Perianal Ulcerative Hemangioma Treated with a Colostomy

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.

Infantile Type Choledochal Cyst with Biliary Atresia: Report of two cases

Choledochal cysts in neonates and young infants take two forms: the choledochal cyst associated with biliary atresia (CCBA) and the choledochal cyst in the absence of biliary atresia (CC). Infants in both groups usually have similar clinical symptoms at presentation, but management and prognosis are different. While early portoenterostomy is required for CCBA, cyst excision with hepaticojejunostomy or choledochojejunostomy is usually performed for CC. The former shows a relatively poor prognosis, and the latter shows a better prognosis. Two infants who presented with clinical features of neonatal cholestasis, and clinically suspected to have choledochal cysts, were found on imaging studies and surgery to have extrahepatic bile duct atresia in association with choledochal cysts.

Appropriate Timing of the Screening Test of Gestational Diabetes / 대한주산의학회잡지

OBJECTIVE: Screening tests for Gestational diabetes (GDM) are performed during the 24~28 weeks of pregnancy. Therefore, in many cases, GDM is not detected during the first trimester of pregnancy while most of important organs are developed during this period. In this review, we performed a retrospective study by dividing GDM mothers into 3 groups: diabetic, impaired glucose tolerance (IGT) and normal. The medical records of mothers and neonates from these three groups were compared. METHODS: 136 out of 536 GDM patients in Holy Family Hospital from 1995 to 2005 took a 75 g OGTT 6 weeks after delivery. As a result, 28 were diagnosed as diabetes, 18 had impaired glucose tolerance, and 90 were normal. The antenatal and perinatal history of above groups were retrospetively compared. RESULTS: In the diabetic group, 46.4% of mothers had a positive family history of diabetes, where as 38% of IGT group, 31.8% of normal group had the history. Secondly, the abortion history rate for each group was 21.4%, 16.6%, 13.3%, and each group had an average HbA1C of 7.20, 5.82 and 5.69. Also, the rate of preterm labor for each group was 17.9%, 11.1%, and 4.45%. The Apgar score (1 min/ 5min) was 7.29/8.54, 7.75/8.75 and 7.89/8.97. Lastly the rate of preeclampsia for each group was 14.3%, 11.1% and 6.8%. CONCLUSION: Three groups had distinctive differences in their perinatal morbidity both before and after delivery. Therefore, in order to reduce the perinatal morbidity and mortality rate, the present frequency or timing of GDM screening test, should be reconsidered.

A survey of parental knowledge of vaccination / 소아과

PURPOSE: In this study, we created a questionnaire and collected answers concerning actual conditions of vaccination and parents' knowledge of vaccination issues, in order to find false knowledge of patients. We tried to give correct information and suggested the role of the pediatrician. METHODS: We made questionnaires and collected answers from 466 parents from March 2004 to June 2004. RESULTS: Places of vaccination were pediatric clinics(49.4 percent), health centers(27.7 percent), general/university hospitals(15.0 percent) and other clinics(4.9 percent). We found 38.8 percent of parents thought that the reason for vaccination at pediatric clinics was a belief of speciality, even though there is no difference in the vaccination itself. We also found 15.0 percent of parents thought that there were no differences between pediatricians and other physicians, but 52.0 percent of parents wanted to receive vaccination at pediatric clinics in the future. Our study also found that 62.4 percent of parents wanted to make out a preliminary questionnaire for vaccination. Many parents got vaccination information from vaccination record books(57.9 percent), and 52.6 percent of parents incorrectly believed that Hib vaccination could prevent all kinds of meningitis. CONCLUSION: Our study suggests that pediatricians need to make efforts to give out correct information. It is necessary to use preliminary questionnaires for vaccination and correct vaccination information should be written on the vaccination record book. Also, there needs to be counselling with parents about weaning, growth and development at the time of vaccination, and to point out the differences between pediatricians and other physicians.

A Single Nucleotide Deletion resulting in Frameshift in Two Korean Neonates with Thyroxine-Binding Globulin Deficiency / 소아과

Abnormalities in the levels of thyroxine-binding globulin (TBG) are not associated with clinical disease and they do not require treatment. Congenital TBG deficiency is inherited in an X-linked manner. To date, some complete and partial TBG variants and one polymorphism have been identified by analysis of the TBG gene. Two male neonates were referred to us because of their low T4 levels that were noted on the neonatal screening test. They showed normal levels of free T4 and TSH. Their serum TBG was not detectable and those values of their parents were within the normal ranges. The genomic DNA was extracted from their white blood cells and the four coding exons of the TBG gene were amplified by using polymerase chain reaction. Sequencing of the four coding regions and all the intron/exon junctions revealed a single nucleotide deletion of the first base of the codon 352 of the mature protein in both of the neonates. This mutation resulted in a frameshift and a premature stop codon (TGA) 374. Their mothers were shown to be heterozygotes. We detected a single nucleotide deletion resulting in a frameshift in two male Korean neonates who had complete TBG deficiency.

Detection of Cytokeratin-19 caused by Intestinal Epithelial Cell Damage in Childhood Diarrhea / 소아과

PURPOSE: Acute gastroenteritis in infancy is a very common disease with a frequency of a billion diarrhea per year, leading to 3 to 5 million deaths. Since the etiology is not always clear, the treatment plan can be quite difficult to make. The laboratory examinations of stool culture, along with complete blood counts may sometimes be helpful to find bacterial etiology, while occult blood in stool merely means the presence of gastrointestinal tract bleeding. We evaluated the presence of cytokeratin-19 in stools in differentiating bacterial and non-bacterial causes of childhood diarrhea to establish appropriate treatment plans. METHODS: Among 201(male 124 patients, female 77 patients) patients with diarrhea, cytokeratin-19 examined by ELISA was positive in 40 patients. Laboratory findings including peripheral blood leukocytes count with differential count, ESR, CRP were compared between the cytokeratin-19 positive and negative groups. We also investigated the correlation between cytokeratin-19 and stool culture, leukocytes, rotavirus antegen test, and occult blood in stool. RESULTS: The leukocytes count, neutrophil count in peripheral blood and ESR were higher in cytokeratin-19 positive group(P=0.030). There was significant correlation between serum CRP and cytokeratin-19(P=0.007). Sensitivity of cytokeratin-19 was 0.30 and specificity was 0.86 in serum CRP positive group and negative group. CONCLUSION: Cytokeratin-19 in stool was significantly increased in correlation with leukocytes and neutrophil count in peripheral blood, ESR, serum CRP and the specificity of cytokeratin-19 was 0.86 in the serum CRP positive group and the negative group. Therefore, the quantitification of cytokeratin-19 in stool was considered as indicator of differential diagnosis between clinically bacterial and non-bacterial infection groups.

Colonic Polyps; Experience of 34 Cases in Two Hospitals / 소아과

PURPOSE: Colonic polyps are among the most common causes of rectal bleeding in children. We studied the clinical spectrum, histology, malignant potential and treatment of colonic polyps in our cases. METHODS: We reviewed hospital charts of all patients with colonic polyps, seen over a 15-year period from January 1988 to November 2002. Colonoscopy and/or barium enema were done for diagnosis of colonic polyps. Thirty-four children, aged one to 18 years old with colonic polyps, were enrolled in this study. Clinical spectrum, gross appearance, histologic appearance, malignant potential, treatment and prognosis of the colonic polyps were evaluated. RESULTS:The mean age of these patients was 6.4 years, with a male predominance(M:F=1.9:1). All patients had symptoms of rectal bleeding of a mean duration of five months. Solitary polyps were seen in 82.4%(28/34), more than on polyp(all had two polyps) in 11.8%(4/34), juvenile polyposis syndrome in 2.9%(1/34), and Peutz-Jegher syndrome in 2.9%(1/34) of the cases. All but one of the 36 polyps had typical features of a juvenile polyp on histological examination. Adenomatous change was observed in 2.8%(1/36) of juvenile polyps. 94.4%(34/36) of juvenile polyps were located in the rectosigmoid region, 2.8%(1/36) were in the distal colon, and 2.8%(1/36) were in the proximal colon. False negative result of barium enema alone without colonoscopy were found in 25%(2/8), and rectosigmoidscopy alone could miss proximal polyps. Colonoscopic polypectomy or surgical transrectal polypectomy were performed in all cases without major complications. Surgical polypectomy needed general anesthesia in 100%(11/11) of children compared to colonoscopic polypectomy which needed general anesthesia in 23.8%(5/21) of children. Recurrence of polyps was observed only in 2.9%(1/34) of the children. CONCLUSION: Juvenile polyps may be the most common colonic polyps in children. Juvenile polyps should be removed even if asymptomatic because of their neoplastic potential. Because some cases of colonic polyps may be multiple and proximally located, pancolonoscopic polypectomy is recommended in all cases. Surgical polypectomy is less recommended, because general anesthesia is required in all cases and proximal polyps could be missed.

The Activity of sICAM-1 and sVCAM-1 in Plasma of Preeclamptic Mother and Neonatal Cord Blood

PURPOSE: Current concepts of preeclampsia have been focused on dysfunction of the maternal vascular endothelium, a central pathogenetic feature of the disease. But it is uncertain whether maternal preeclampsia has a harmful effect on fetal or neonatal vascular endothelium. In this study, plasma levels of endothelial adhesion molecules in preeclamptic mother and cord blood were determined to delineate vascular effects of preeclampsia on neonates. METHOD: Quantitative determinations of sICAM-1 and sVCAM-1 were measured from plasma of preeclamptic mother and neonatal cord blood in pairs according to gestational age and was compared to nonpreeclamptic control groups. RESULTS: Plasma ICAM-1 level was significantly higher in the maternal groups compared to corresponding cord groups (P<0.001). Preeclamptic maternal groups showed significantly higher sICAM-1 level compared to control maternal groups (P<0.001) and preterm maternal groups showed higher levels than term maternal groups (P<0.001). The level of sICAM-1 was significantly elevated in preeclamptic preterm cord groups than other cord groups (P<0.001). In respect to plasma sVCAM-1 level, higher value was observed in the preeclamptic preterm cord groups than preeclamptic preterm maternal groups. CONCLUSIONS: Elevation of the plasma sICAM-1 level caused by factors including vascular endotherial damage in preeclamptic mothers was observed in their neonates but with much lesser degree than their mothers. Factors associated with preterm labor other than maternal preeclampsia may seem to influence vascular endothelial injury in the cord blood.

The Effect of Preeclampsia on Neonatal Outcome of Premature Infants Delivered before 35 Weeks Gestation / 대한주산의학회잡지

OBJECTIVE: The purpose of this study was to determine neonatal outcomes according to laboratory and clinical features in preeclampsia before 35 weeks gestation. METHODS: The medical records of all patients delivered at the hospital from January 1, 1996 through March 31, 2002 were reviewed for the occurrence of preeclampsia before 35 weeks gestation. We compared their maternal medical and obstetric courses and neonatal outcomes with those in a group of non preeclamptic patients who delivered at similar gestational ages. RESULTS: The preeclampsia group had lower mean birth weight, 1-minute Apgar score and umbilical arterial pH. But there were no difference in the incidence of neonatal death, respiratory distress syndrome, grade 3 and 4 intraventricular hemorrhage, and culture-proven sepsis. Results were similar when analysis was limited to infants born before 32 weeks. CONCLUSION: Maternal preeclampsia does not improve neonatal outcome of infants born before 35 weeks gestation.

A Case of Congenital Solitary Liver Abscess

Congenital solitary liver abscess in newborns is an extremely rare and serious disease. Only one case was reported in the literature. Most a liver abscesses have a postnatal origin. We encountered a premature infant who had a low. Apgar score and an elevated right diaphragm at birth. Pyogenic solitary liver abscess was diagnosed by ultrasound and computed tomography. Staphylococcus aureus was cultured from ultrasound guided aspiration fluid.

Two Cases of Hydrometrocolpos in Neonate

Imperforated hymen, vaginal atresia or high transverse vaginal septum are caused by incomplete vaginal canalization. The infant may be present with distention of the vagina and the uterus with glandular secretions stimulated by maternal estrogens, known as hydrometrocolpos. We report two cases of hydrometrocolpos. In the first case, distal vaginal atresia with cystovaginal fistula was revealed by a contrast fluoroscope through the percutaneous catheterization. In the second case, urogenital sinus was detected by a fistulogram through a single orifice in the genital area. We decompressed the cystic mass by ultrasonogram guided aspiration, promptly at birth, then achieved the transient drainage of cystic fluids by percutaneous catheterization.

Quantities of Receptor Molecules for Colony Stimulating Factors on Leukocytes in Measles

We analyzed the comparative amounts of granulocyte-colony stimulating factor (G-CSFr) and granulocyte macrophage CSF (GM-CSFr) receptors expressed on neutrophils and monocytes in measles patients to investigate the role of these CSFrs in the development of leukopenia including neutropenia and monocytopenia in measles. EDTA-anticoagulated peripheral blood of 19 measles patients, 10 children with other infections showing leukopenia and 16 children with normal complete blood cell counts (CBC)s were analyzed using flow cytometry and QuantiBRITE. The leukocyte (5260 +/- 2030/uL vs. 9900 + 2680/uL, p=0.000), neutrophil (2580 +/- 960/uL vs. 4250 +/- 2750/uL, p=0.024) and the lymphocyte counts of measles patients (1810 +/- 1430/uL vs. 4530 +/- 3450/uL, p= 0.006) were lower than in the normal controls. The neutrophils of measles patients expressed similar amounts of G- CSFr (1858 +/- 355) as normal children (1764 +/- 477, p= 0.564) and leukopenic patients (1773 +/- 673, p=0.713), but lower levels of GM-CSFr (535 +/- 118) than normal children (957 +/- 344, p=0.000) and leukopenic patients (832 +/- 294, p=0.002). The monocytes of measles patients expressed similar amounts of G-CSFr (916 +/- 336) and GM-CSFr (3718 +/- 906) as normal children (1013 +/- 391 and 4125 (2645, p > 0.05) but less than leukopenic patients (1454 +/- 398 and 5388 +/- 806, p > 0.05). The neutrophil and monocyte counts of measles patients did not correlate with the amount of G-CSFr or GM-CSFr expressed on neutrophils or monocytes (p > 0.05), but in the normal children, the monocyte count correlated with the levels of GM-CSFr on monocytes (r=0.951, p=0.049). In conclusion, neutropenia is one of the more important characteristics of measles patients, which could be due to the decreased GM-CSFr expression on neutrophils. However, the monocytopenia found in measles patients is not due to the decreased expression of CSFr on the monocytes.

Radiographic Findings and Atelectasis in Children Admitted to Hospital with Acute Asthma

PURPOSE: We aimed to evaluate the abnormalities of chest radiographs including atelectasis in children who were admitted with bronchial asthma. METHODS: We retrospectively analyzed the 357 chest radiographs and the clinical and laboratory features of the 144 children with asthma, who were admitted at Daejoen St. Mary's Hospital from April 1994 to May 1998. RESULTS: Clinical and laboratory characteristics were as follows: male to female ratio, 2.1 : 1; mean age, 4.8 years of age; mean numbers of admission, 2.5; mean hospitalization, 5.0 days; mean IgE, 387 IU/mL; mean eosinophil count, 362/mm3. In the abnormal findings of the 357 radiographs, there were 314 cases(88.0%) of hyperinflation, pulmonary infiltration 35.0%, atelectasis 5.3% and pneumomediastinum 0.3%. All(19) cases of atelectasis were observed in the right lung field with mostly segmental and lobular distribution, except one with lobar involvement. Atelectasis was predominant in males and those under 2 years of age. There was a tendency that the right upper lung was more involved under two years while the right lower lung was more involved over seven years of age. CONCLUSION: Radiographs of children admitted to hospital with bronchial asthma showed abnormal findings including pneumonia or atelectasis(5.3%). These abnormal findings can help to determine other therapeutic modalities in addition to asthma treatment.

Effect of the Maternal Hypertension on the Neonatal Status of Lipid Peroxidation and Antioxidant Activity in the Preterm Newborn Rat Lung

PURPOSE: Lipid peroxidation may be linked with the dysfunction of endothelium of hypertensive pregnancy and it may also have effects on antioxidant response of the fetal lung. Acute change of lipid peroxidation and antioxidant enzyme activity after exposure to extrauterine environment has been shown to be involved in the pathogenesis of lung injury in preterm and term lung. We want to study the immediate postnatal possible differences between hypertensive and normal pregnancy in lipid peroxidation and antioxidant response of preterm rat lung. METHODS: Female SHR (essential hypertension, 1b, A, 6-8 weeks, n=20) Sprague- Dawley rats were cohabited. C-section was done on the 20th day of gestation to the half of pregnant SHR rats (preterm SHR group, n=50). Control group was consisted with the premature pups (preterm normal group, n=50) delivered from normal dams. The birth weight, content of malondialdehyde (MDA), and activity of superoxide dismutase (SOD) in the lung tissues were analyzed at birth and 24 hours after birth. RESULTS: Birth weights were significantly low in premature SHR group compared to control group. At birth, MDA content and SOD activity were significantly increased in preterm SHR group. After 24 hours, the MDA value and SOD activity were even more increased in preterm SHR group. CONCLUSION: These results suggest that maternal hypertension during pregnancy is associated with an increased lipid peroxidation of the fetal and immediate postnatal preterm rat lung and that compensatory activation of antioxidant system works in the fetal and newborn rat lung.

Two Cases of Hemorrhagic Necrotizing Enterocolitis as a Presenting Complaint of Hirschsprung's Disease

Hirschsprung's disease (HD) is one of the most common cause of intestinal obstruction in neonates. Enterocolitis is a well known complication of HD. Since a severe form of enterocolitis occurring in HD is the major cause of death and disability of these patients, early diagnosis is essential. Recently, ischemic hemorrhagic necrotizing enterocolitis have been reported as lethal complications of HD. However little distinction is made between inflammatory enterocolitis which often follows HD and hemorrhagic ischemic necrotizing enterocolitis, which is a rare, and often fatal complication of HD. Most of reported cases of hemorrhagic necrotizing enterocolitis developed very early, generally within 3 days of birth except several cases. Which may be confused with necrotizing enterocolitis (NEC) which was not associated with HD. We report here two cases of hemorrhagic necrotizing enterocolitis associated with Hirschsprung's disease, who were initially diagnosed as having NEC.

A Case of 18 Ring Chromosome

A ring 18 chromosome(18r) karyotype is a rare disorder characterized by short stature, obesity, microcephaly, mental retardation, micropenis, cryptorchidism, hypertelorism, epicanthal folds, micrognathia, and small hands with short tapering fingers. A 22-month-old girl was referred to our department of pediatrics because of ptosis and delayed development. Karyotype revealed ring 18 and dicentric ring 18 chromosome(46,XX,r(18)[45]/45,XX,-18[7]/46,XX,dic r(18)[3]). Fluorescent in situ hybridization with 18 centromeric probe showed 18 ring chromosome with 1 signal and 18 dicentric ring chromosome with 2 signals. We report a case of a ring 18 chromosome diagnosed by karyotype and fluorescent in situ hybridization.

A Clinical Study of Atypical Kawasaki Disease: A Rate of Coronary Artery Involvement

PURPOSE: Without a specific diagnostic method, the clinical diagnostic criteria for atypical Kawasaki disease is known as the presence of coronary artery changes in a patient who did not meet the already estabilished clinical criteria. With this criteria, we are put into dilemma because atypical Kawasaki disease should exhibit coronary artery disease. So, we demonstrated the rate of coronary artery involvement in atypical Kawasaki disease by another diagnostic criteria. METHODS: This diagnostic criteria for atypical Kawasaki disease is "the clinical symptoms which fulfilled four out of six diagnostic criteria below of typical Kawasaki disease with or without the presence of coronary artery changes, and were inconsistent under a through clinical investigation with any other illness similar to Kawasaki disease." RESULTS: The incidence of coronary artery involvement in atypical Kawasaki disease during the acute phase (analysis of 30 cases who were admitted before the 15th day of illness), on the 30th day of illness (26 cases) and on the 60th day of illness(26 cases) were 20.0% (dilatation : 13.3%, aneurysm : 6.7%), 19.2% (dilatation : 19.2%, aneurysm : 0.0%), 7.7%(dilatation : 7.7%, aneurysm : 0.0%), respectively. CONCLUSOIN: Kawasaki disease may occur in atypical type, ranging over many severe involvements of vital organs including the coronary artery. Up to the present, there are no infallible diagnostic tests for Kawasaki disease and it is advisable to deemphasize the rigid adherence to the current already proposed diagnostic criteria for Kawasaki disease.