• Child Health Nursing Research
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• v.5 no.1
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• pp.97-106
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• 1999

The purpose of this study was to provide basic data of nursing intervention through understanding disease pattern of pediatric patients. Data were collected from 3,016 patients who visited emergency center of I university Hospital during one year period from January to December, 1997. The results were as follows : 1. The most common type of disease was respiratory disease (26.7%). 2. According to the developmental age, toddler (35%) ranked first followed by preschooler(22.1%), infant(18.5%), schooler(16.0%), adolescent (5.6%), neonate (2.7%). 3. The proportion of males to females, male patients(59.8%) outnumbered female(40.2%) by the ratio of 1.48 ; 1. 4. Visiting patients were seasonally more frequent in spring(29.8%) and summer (29.3%) than fall or winter. 5. Comparing weekly distribution, week end involing holiday outnumbered week day(48.5%). 6. The visiting time predominattly high from 8 : 00 PM to 0 : 00 AM(29.8%) during a day. 7, The majority duration of stay at Emergency center was 1-6 hours. 8. Most patients dischrged from the emergency center in good condition(76.5%).

• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.258-261
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• 2010

Lymphangioma is a rare benign congenital tumor of the lymphatic system, which is commonly diagnosed before 2 years of age. In the natronal report, cystic lymphangioma was usually reported as a huge translucent mass located in the head and neck area. It's occurrence in retropharyngeal space with respiratory obstruction and swallowing difficulty in neonate is extremely rare and postoperative nasopharyngeal reflux has rarely been reported. Complete resection is the standard therapy. However, involvement of the upper airway may be determining prognosis in the extensive lymphangiomas because of the difficulty of complete excision. We present a case of cystic lymphangioma in neonate which was initially asymptomatic but gradually progressed to cause respiratory obstruction due to enlargement. After resection, nasopharyngeal reflux developed with dysfunction of the soft palate and gradually improved with conservative care over 5 months.

• Korean Parent-Child Health Journal
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• v.2
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• pp.83-110
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• 1999

The purpose of this study was to identify the sleep/activity patterns of neonates and to provide a baseline data for the development effective mother-infant relationship. Subjects of this study were 36 newborns aged 1-21 days and stayed in Postpartum Care Center in Taejon during the period of October 28th, 1999 to February 10th, 2000. The data were collected using NCASA developed by Barnard et al. and were analyzed with SPSS 7.5 for window program. The results of this study were as follows: 1. An average day sleep of neonates was 8.0 hours, night sleep was 3.6 hours, and total sleep was 11.6 hours. A longest period of day sleep was 3.8 hours and a longest period of night sleep was 3.4 hours. The regularity of day sleep was 22.1%, night sleep was 23.3%, and total was 22.1%. 2. The frequency of daytime feeding was 6.9, nighttime fee ding was 3.4, resulting in total of 10.3. The regularity of feeding was 37.4%, and the frequency of night awakening was 2.4. 3. Associations among variables related to neonate's sleep/activity records were as follows : - The regularity of total sleep was positively correlated with the frequencies of day sleep(r=.57, P=.00), night sleep (r=.40, P=.01), and total sleep(r=.65, P=.00). - The frequency of total feeding was negatively correlated with the frequencies of day sleep(r=-.29, P=.04), night sleep(r=-.39, P=.02), and total sleep(r=-.42, P=.00), as well as longest day sleep(r=-.50, P=.00). - The regularity of feeding was negatively correlated with the frequency of night sleep(r=-.35, P=.02), longest period of day sleep(r=-.32, P=.04), and longest period of night sleep(r=-.30. P=.04), whereas positively correlated with frequencies of daytime feeding (r=.29, P=.02) and nighttime feeding (r=.32, P=.05). - The frequency of night awakening was negatively correlated with amount of night sleep(r=-.39, P=.02) and frequency of daytime feeding(r=-.42, P=.01) while positively correlated with frequencies of nighttime feeding(r=.68, P=.00) and total feeding(r=.50, P=.00). 4. Although there was no statistical significant difference between sleep pattern and infant's age, following tendency was noted: - As baby gets older, total amount of sleep and longest period of day sleep decreased while longest period of night sleep increased. - As baby gets older, the regularity of day sleep decreased while the regularity of night sleep and total sleep slightly increased. - The frequencies of daytime and total feedings increased while the frequency of nighttime feeding decreased. In conclusion, the author found that the neonates of this study showed less amount of sleep, more night awakening, and more night time feedings than NCAST sample. There was a certain sleep/activity pattern existed: If babies were fed more frequently, daytime or nighttime sleeping hours became shorter, and if babies had longer daytime sleep, frequent nighttime awakenings were noted. This pattern illustrated the significant influence of feeding and sleep periods on each other. Awakening in the night is natural and normal patterns in the newborn period. The result of this study will provide information to parents about what to expect their infants.

• Women's Health Nursing
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• v.2 no.2
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• pp.284-297
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• 1996

A maternity ward in a hospital in Seoul has an educational program for postpartal mothers since its opening, but evaluation on the program has not been done. This study was to find whether the educational program contributed to increase of knowledge and confidence of the post-partal mothers in the area of postpartal self-care and the newborn baby care or not. This study aimed at improvement of the educational program for posrpartal mothers and the newborn babies. Subjects were 40 primiparae who were admitted to the obstetric ward in a general hospitals in Seoul from August 20th to September 10th, 1995. Subjects were those who had no labor pain at the admission time, had no complications during labor and delivery, gave a birth to a healthy baby, and agreed to participate in this study. All subjects were well educated and were in well-to-do group. Most of them received antenatal care adequately. They were tested on knowledge and self-confidence in the area of postpartal self-care and infant care two times, one at the admission time and the other prior to discharge. After the first test, nurses in a maternity ward and nursery taught them on postpartal self-care and infant care. Education consisted of a 1 hour planned program and incident teachings done at bed side. Four tools were developed by authors based literature review. The test tool for knowledge of postpartal self-care consisted of 15 items which included the definition of postpartum, dangerous symptoms in postpartum, lochia, time to begin coitus, postpartum exercise, sitz-bath, and perineal care. The test tool for self-confidence in postpartal self-care included such items as emergency care on dangerous symptoms in postpartum, sexual life and contraception after postpartum, self-confidence in postpartum exercise and perineal care. The test tool for knowledge of newborn baby care consisted of 18 items which included bathing, umbilical cord care, vaccination, breast feeding, abnormal symptoms of neonate. The test tool for self confidence in the newborn baby care included umbilical cord care, vaccination, breast feeding, emergency care for baby. Analysis of demographic data were analyzed with calculation of percentage. Score differences between the first test and the second test were analyzed with paired t-test. SAS / PC (Release 6.04 for DOS) program are as follows. 1. Mothers' knowledge of postpartal self-care increased significantly after education (t=10.04, df=38, p=0.0001). 2. Mothers' self-confidence in postpartal self-care increased significantly after education (t=6.53, df=38, p=0.0001). 3. Mother's knowledge of the newborn abay care increased significantly after education (t=9.74, df=38, p=0.0001). 4. Mothers' self-confidence in the newborn baby care increased significantly after education(t=8.22, df=38, p=0.0001) Suggestions for further studies and nursing practice were as follows. 1. We suggest studies by randomized control-group pretest-posttest design or nonequivalent control group pretest-posttest design will be done. 2. We suggest follow-up studies to find if mothers's confidence will last or not after discharge. 3. We suggest general hospitals to establish a phone-counseling system.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.255-262
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• 2005

Battler and Bartter-like syndromes, which include classic Bartter syndrome(type III), neonatat Bartter syndrome(type I, II or IV) and Gitelman syndrome, are autosomal - recessively inherited renal tubular disorders characterized b)r hypokalemic metabolic alkalosis, salt wasting and normal to low blood pressure. Neonatal Bartter syndrome is characterized by intrauterine polyhydramnios, premature delivery, life-threatening episodes of fever and dehydration, subsequent failure to thrive, and severe hypercalciuria with nephrocalcinosis and osteopenia. It is caused by mutations in NKCC2(type I), ROMK(type II) or BSND(type IV) genes. If diagnosed and treated early, the progression to renal failure can be prevented and catch-up growth and normal development are achieved. We report here a 6 month-old infant with neonatal Bartter syndrome who presented with hypokalemic metabolic alkalosis, polyhydramnios and premature delivery, persistent high fever and dehydration, failure to thrive, hypercalciuria, and nephrocalcinosis. He received indomethacin and potassium chloride per os and show ed catch-up growth and normal developmental profile at 19 months of age. (J Korean Soc Pediatr Nephrol 2005;9:255-262)

• Clinical and Experimental Pediatrics
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• v.56 no.3
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• pp.139-142
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• 2013

X-linked recessive myotubular myopathy (XLMTM) is a severe congenital muscle disorder caused by mutations in the MTM1 gene and characterized by severe hypotonia and generalized muscle weakness in affected males. It is generally a fatal disorder during the neonatal period and early infancy. The diagnosis is based on typical histopathological findings on muscle biopsy, combined with suggestive clinical features. We experienced a case of a newborn who required intubation and ventilator care because of profound hypotonia and respiratory difficulty. The preliminary diagnosis at the time of request for retrieval was hypoxic ischemic encephalopathy, but the infant was clinically reevaluated for generalized weakness and muscle atrophy. Muscle biopsies showed variability in fiber size and centrally located nuclei in nearly all the fibers. We detected an MTM1 gene mutation of c.1261-1C>A in the intron 10 region, and diagnosed the neonate with myotubular myopathy. The same mutation was detected in his mother.

• Neonatal Medicine
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• v.28 no.3
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• pp.133-138
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• 2021

Osteopetrosis refers to a group of genetic skeletal disorders characterized by osteosclerosis and fragile bones. Osteopetrosis can be classified into autosomal dominant, autosomal recessive, or X-linked forms, which might differ in clinical characteristics and disease severity. Autosomal recessive osteopetrosis, also known as malignant osteopetrosis, has an earlier onset, more serious clinical symptoms, and is usually fatal. We encountered a 1-day-old girl who was born full-term via vaginal delivery, which was complicated by meconium-stained amniotic fluid, cephalo-pelvic disproportion, and nuchal cord. Routine neonatal care was provided, in addition to blood tests and chest radiography to screen for sepsis, as well as skull radiography to rule out head injuries. Initial blood tests revealed hypocalcemia, which persisted on follow-up tests the next day. Radiographic examinations revealed diffusely increased bone density and a "space alien" appearance of the skull. Based on radiographic and laboratory findings, the infantile form of osteopetrosis was suspected and genetic testing for identification of the responsible gene. Eventually, a heterozygous mutation of the T cell immune regulator 1, ATPase H+ transporting V0 subunit a3 (TCIRG1) gene (c.292C>T) was identified, making this the first reported case of neonatal-onset malignant osteopetrosis with TCIRG1 mutation in South Korea. Early-onset hypocalcemia is common and usually results from prematurity, fetal growth restriction, maternal diabetes, perinatal asphyxia, and physiologic hypoparathyroidism. However, if hypocalcemia persists, we recommend considering 'infantile of osteopetrosis' as a rare cause of neonatal hypocalcemia and performing radiographic examinations to establish the diagnosis.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.568-574
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• 2009

Tongue-tie is a congenital anomaly characterized by an abnormally short lingual frenum, which may restrict tongue tip mobility, In the neonate and infant, tongue-tie was said to cause difficult breast-feeding, In the older infant, toddler and young child, poor speech has been frequently listed because movement of tongue tip is limit ed. Also as a abnormal tongue position, various orthodontic problems, later mechanical and social manifestations could be developed. Treatment options such as observation, speech therapy, frenotomy and frenectomy have been suggested. The optimal timing for the surgery has not been determined, but early intervention may be appropriate for the children with significant tongue-tie who has the significant potential to speech difficulties and later social and mechanical problems. Sedation is an effective method for incapable of cooperative and the handicapped children, necessary to early intervention. We report three cases, using oral sedation for the frenectomy in young children with severe tongue-tie. After treatments, we could find out sufficient tongue movement and improved speech ability.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.45-48
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• 2016

Short-chain acyl-CoA dehydrogenase deficiency (SCADD) is a rare autosomal recessive mitochondrial disorder of fatty acid ${\beta}$-oxidation, and is associated with mutations in the acyl-CoA dehydrogenase (ACADS) gene. Recent advances in spectrometric screening for inborn errors of metabolism have helped detect several metabolic disorders, including SCADD, without symptoms in the neonate period. This allows immediate initiation of treatment and monitoring, so they remain largely symptomless metabolic disease. Here, we report a 15-month-old asymptomatic male, who was diagnosed with SCADD by newborn screening. Spectrometric screening for inborn errors of metabolism 72 hours after birth revealed an elevated butyrylcarnitine (C4) concentration of $2.25{\mu}mol/L$ (normal, < $0.99{\mu}mol/L$). Urinary excretion of ethylmalonic acid was also elevated, as detected by urine organic acid analysis. To confirm the diagnosis of SCADD, direct sequencing analysis of 10 coding exons and the exon-intron boundaries of the ACADS gene were performed. Subsequent sequence analysis revealed compound heterozygous missense mutations c.164C>T (p.Pro55Leu) and c.1031A>G (p.Glu344Gly) on exons 2 and 9, respectively. The patient is now growing up, unretarded by symptoms such as seizure and developmental delay.

• Journal of Chest Surgery
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• v.25 no.1
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• pp.32-41
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• 1992

From August 1982 to December 1991, 58 consecutive infants with tetralogy of Fallot underwent primary repair. Age ranged from 22 days to twelve months [n=58, 8.7$\pm$2.7 months] and body weight from 3.1 to 13 kilograms [n=58, 7.8$\pm$1.7 kilograms]. Qne infant had absence of the pulmonary valve; one had Ebstein`s anomaly and one had supramitral ring. Thirty-two patients [56%] experienced anoxic spell. Preoperative pulmonary artery indices were measured in 38 cases, ranging 126-552mm2/M2BSA[n=38, 251$\pm$79mm2/M2BSA]. All infants required a right ventricular outflow tract patch; in 41, the patch extended across the pulmonary valve annulus, in 13 of them, monocusps were constructed. All had patch closure of ventricular septal defect. Two infants had REV operation for avoiding injury to the canal branch of the right coronary artery which cross the right ventricular out flow tract. Post repair PRV/LV were measured at operating room in 40 cases, which revealed mean value of 0.49$\pm$0.12 [range: 0.25-0.74]. The hospital mortality was 10.3% [6 patients], and causes of deaths were right heart failure due to sustained right ventricular hypertension[4] and right ventricular outflow tract obstruction, intractablesuraventricular tachyarrhythmia[1], hypoxia[1] due to residual right to left shunt across the atrial septal defect in patient associated with Ebstein`s anomaly. All infants were doing well at follow-up from 1 to 101 months[20.6 months /patient, 1, 072 patient-month] Serial postoperative echocardiograms revealed no residual ventricular septal defects and estimated RVOT gradients between 0 and 40 mmHg except 3 cases [50, 50, 60 mmHg]. There were no late deaths and late ventricular arrhythmias or congestive heart failure. Redo operations were done in 2 cases because of residual right ventricular outflow tract obstruction. This experience with infants with tetralogy of Fallot suggests that, if mortality is tolerable, eletive repair of tetralogy of Fallot could be reasonably undertaken during the first year of life, and even better results could be anticipated along with improvement of methods of myocardial protection and postoperative care.