A 4 year and 7 month old male patient was admitted with the chief complaint of subgaleal hematoma and recurrent bleeding episodes. The first episode was umbilical cord bleeding on the third day of life. Routine coagulation study including factor assay at that time was within normal limit. The bleeding cleared after whole blood transfusion. He had been admitted... |
Patent urachus is a rare congenital anomaly due to failure of luminal closure of urachus. At many instance, it was discovered by drainage of urine from the umbilicus. Patent urachus is sometimes associated with anomaly of Genitourinary system or such as Prunebelly syndrome. We report 1 case of patent urachus in a 1 day old male newborn, treated by surgical intervention.... |
To evaluate the influence of HBV to fetus, authors studied 817 pregnant women and neonates who were admitted to delivery room of Keimyung University Dongsan medical center from 1882 to 1984. The results were as follows; 1) Percent of HBsAg positive mother was 6.8% (50/817) and child bearing age was 7.8% (72/917). Percent of Anti-HBs positive was 33.9% and... |
The congenital nephrotic syndrome is characterized by large placenta, low birth weight, early clinical findings, delayed growth and development and resistance to treatment. Authors experienced a case of characteristic of congenital nephrotic syndrome in a girl, who was admitted to the Pediatric Department of HUH at 28 days of age because of generalized edema, abdominal distension with vomiting and diarrhea.... |
Congenital hypoplastic anemia is a rare disease characterized by aplasia or severe hypoplasia of the erythron, with onset usually at birth or during early infancy. This report presented three cases of congenital hypoplastic anemia in 3 infants with pallor and irritability which were noticed since one to three months of age. Three cases were treatsd with prednisolone and the courses of... |
We observed a case of congenital hypoplastic anemia in a 10 month old male patient who was diagnosed through bone marrow aspiration. This steroid-unresponsive patient has been maintaining a concentration of hemoglobin compatible with health from supportive transfusion therapy. A brief review of related literature was made. |
A case of Caroli's disease was presented and review of the literature was done. The patient was a 3 year old girl and had recurrent episodes of jaundice, abdominal pain and vomiting. Dilatation of the left intrahepatic bile duct was clearly shown on the operative cholangiography. |
Congenital diaphragamatic anomaly is a rare congenital malformation and is found more frequently on the left side than the right side. We had experienced two cases of congenital diaphragmatic anomaly on the right and the left side. One case was 1 year old male infant which was admitted with chief complaints of fever and mild cough. The diagnosis was confirmed by... |
We observed a case of congenital nonspherocytic hemolytic anemia who was a 24 month old boy with the signs of jaundice 技 pallor. Evidences of the same disease was also found in the patient' s elder brother & father. We diagnosed these cases by history & physical examination, peripheral & B.M. blood smears, osmotic fragility test, autohemolysis test and etc. A... |
The authers experienced two cases of salt losing congenital adrenal hyperplasia. Case 1. was a 22 day old male infant, who had vomiting and lethargy. Increased urinary 17-KS and decreased 17-OHCS were observed. He was been treated with oral hydrocortisone and florinef, successfully. Case 2. was a 30 day old male infant, who had anorexia, convulsion, and diffusely pigmented skin.... |
The occurrence of congenital heart disease can be explained by genetic or environmental aspects. But these factors act separately only in about 10% and most of all, which reaches up to 90%, are inherited by the pattern of multifactorial inheritance, in which there is an underlying genetic predisition, usually produced by the small effects of many genes interacting with an... |
Although pediatrics has been advanced progressively in the past years, congenital malformations are known as a major cause of neonatal death. In spite of every efforts of pediatricians and obstetricians to exclude the possible factors of congenital malformations by antenatal care, the general incidence of congenital malformations has not been decreased yet. So, we surveyed the incidence of congenital malformations... |
Congenital hypothyroidism is one of the important causes of retardation of growth and development. A 4 year 2 month old male patient was admitted to our department of Pediatrics, who showed marked growth failure and developmental retardation. Congenital hypothyroidism was suspected by history and physical examination, and confirmed by thyroid function test and thyroid scan, which showed ectopic lingual thyroid. A brief... |
A heart disease screening program was performed on 15,685 primary school children in Seoul City from the first to sixth grade to estimate the prevalence of heart diseases. Among the 15,685 children examined, 45 were confirmed to have heart disease (2.9/1,000) Out of the 45 children, 38 had congenital heart disease(2.4/1,000) and 7 had rheumatic heart disease(0.4/1,000) and the ratio... |
We experienced a case of congenital tuberculosis in a 10-month-old female patient who had disseminated tuberculosis. She was the 1st born of 30-year-old woman who was diagnosed as tuberculous pleurisy on the 12 th day after delivery. Congenital tuberculosis was suspected because of detection of AFB in gastric aspirates on the 60 days of life. But the treatment was discontinued after... |
A clinical study was done in 42,906 cases of the newborn infant born at Busan National University and II Sin Womens Hospital Delivery Room about congenital anomalies for 6 years from Jan. 1977. The results were summarized as follows: 1)Among total newborns, congenital anomalies were 1,845 cases(4.3%) and the frequency of male congenital anomalies was 5.4% and that of female... |
We experienced a case of congenital fibrosarcoma in 1-month-old male infant who had a mass in the left back since his birth. Diagnosis was based on ultrasonography, CT Scan and pathalogic pictures obtained by percutaneous needle biopsy. Review of literatures was also attempted briefly. |
We experienced 2 cases of salt losing form of congenital adrenal hyperplasia. Case I was 3days old female neonate who had dehydration and large clitoris. Case H was 22 days old male neonate who had large pigmented penis. Both cases had hyponatremia and hyperkalemia. Their 24 hours urinary 17-ketosterod was also increased. Fluid therapy, hydrocortisone and florinef were tried with improvement. A brief review related literatures... |
Congenital adrenal agenesis or hypoplasia is very rare disorder which causes adrenal insufficiency. It is usually the result of an isolated defect of organogenesis. It may be sporadic, or it may express itself as an autosomal recessive or X-linked disorder within families. We experienced a baby who revealed hyperpigmentation, hypoglycemia, hyperkalemia and hyponatremia immediately after birth. Hormone study was compatible with primary adrenal insufficiency. Complete... |
The postmortem finding in a 64 day-old Korean male of asplenia associated with congenital malformation of heart and partial situs inversus of the abdominal viscera has been presented. And results were as follows. 1. Heart 1) abscence of atrial septum with, triangular muscular band. 2) common atrioventricular orfice with, anomalous attachment. 3) univentricle. 2. Respiratory tract 1) bilateral trilobed lung. 2) chronic passive congestion of lung, bilateral. 3. Gastrointestinal tract 1) Right sided stomach, duodenum and pancreas. 2) Enlarsgment of left lobe... |
We experienced a case of congenital monocytic leukemia in a 25 day old female newborn infant. She was admitted because of petechiae, abdominal distension and skin nodules scattered on. whole body. Diagnosis was confirmed with findings of CBC, bone marrow aspiration and cytochemistry. A review of literatures was also presented. |
This is a clinical observation of congenital anomalies in 55 newborn infants associated with hydramnios. These infants were born at Severance Hospital from Sep. 1975 to Jun. 1983. The results were as follows: 1) The frequency of hydramnios was 49 cases in 160,060 deliveries in that period, which was 0.31%. 27 infants (49.1%) had congenital anomalies of 55 infants associated with hydramnios... |
To assess the validity of Echocardiographic criteria for pulmonary arterial hypertension we studied pulmonary valve motion in 75 congenital heart disease children. 75 patients were divided into two groups, A and B. The group A consisted of 39 patients who had normal pulmonary arterial pressure, group B consisted of 36 patients who had pulmonary arterial hypertension(pulmmonary artery mean pressure more... |
Neuroblastoma is the commonest malignant tumor identified during the neonatal period and the majority of neuroblastomas are felt to be congenital in origin. Neuroblastoma is a tumor of particular interest because it is known to arise from cells of the neural crest which possess the potential for maturation of spotaneous regression. This report present the clinical, radiographic and autopsy findings of... |
Isolated congenital tricuspid insufficiency is a rare anomaly and may be caused by abnormal chordae tendinae, papillary muscle, or valve leaflets. The cause of these lesions is generally ascribed to incomplete undermining of primitive endocardial cushion by sinusoidal space, which differentiate the cushion into the various valvular structures. Carvallo’s sign, most characteristic clinical sign in tricuspid insufficiency, is augumentation of systolic... |
Congenital leukemia is rare disease and several cases were reported in this country. We experienced a case of congenital leukemia(myelomonocytic type) in a 27 days old female. She was admitted due to marked abdominal distension, vomiting and generalized petechia. The diagnosis was established by clinical features as well as peripheral blood and bone marrow studies. We report this case with... |
We have experienced a case of Turner’s Syndrome with hydronephrosis and partial duplication of renal pelvis in 71 days old female. The patient manifested the clinical finding of loose skin fold at the nape and palpable mass in the left flank. The chromosomal analysis was 45, XO. She was shown by operation to be a left hydronephrosis, shown by intravenous... |
Six cases of congenital coronary arteriovenous fistula were analyzed. The incidence was 0.2% of confirmed congenital heart disease. Male to female ratio was equal. Most of the cases showed exertional dyspnea and the history of frequent URI. Thrill was palpable in all cases except the case of right coronary artery-right atrium fistula. Continuous murmur on the left or right lower... |
From July 1980 to June 1983, total 468 cases of infants of congenital heart disease were studied, who needed intensive care in infancy at Seoul National University Hospital. 1)Among 468 cases, 285 cases were diagnosed by cardiac catheterization or cardiac surgery and the other 183 cases by only clinical base. 2)Relative frequencies of these individual anomalies were in order of... |
When the adrenogenital syndrome is associated with congenital adrenal hyperplasia, it is caused by an inborn defect in the biosynthesis of adrenal corticoids. Especially, infant with congenital adrenal hyperplasia caused by a 21-hydroxylation. defect in steroidogenesis often present with salt-losing crisis, hyponatremia and dehydration. Vomiting is prominent and disturbances in cardiac rate and rhythm may occur. Without treatment, collapse and death... |