We report 1 case of Fanconi's anemia which has severe pancytopenia, PDA, hypoplatic right thumb and cryptorchism with a brief review of related literature and references.
Cryptorchidism ; Fanconi Anemia* ; Male ; Pancytopenia ; Thumb

Authors experienced the cases like to Muco-Cutaneous Lymph node Syndrome (MCLS) admitted to the department of Pediatrics, St. Benedict Hospital from Jan. 1978 to Dec. 1979. Here Authors presented the clinical datas of twenty cases simulating this syndrome as reported by Kawasski. 1. The peak age group was from 1 to 2 years of age and the youngest case was 3 months old infant. Most of them (95%) was under 3 yesrs old. The ratio of male and female was 13 to 7. The seasonal incidence was higher in May to June and November to February. The annual incidence of 1979 was more than 2 times as that of 1978. 2. The clinical symptoms on admission were as follow : fever (100%), cough (70%), diarrhea (65%), vomiting (45%), irritability (40%), So, on admission, Authors had used to overlook as respiratory disease or acute gastroenteritis. 3. The 70% of them showed at least 5 items of the principal symptoms of MCLS. At acute phase, 14 cases (70%) also showed the erythematous desquamation of anogenital region, and 2 cases (10%) showed a transitory hydrocele. The appearance of rashes was observed in 10 cases (50%), and 7 cases of the remainder showed the desquamation of digits during convalescent phase. 7 of 15 cases (46.7%) showed only the desquamation of fingertips. 4. 8 cases (40%) had sinus tachycardia, and I case had gallop rhythm. Most of them showed normal EKG, and 4 cases (20%) showed low voltage of limb leads. 5. In the laboratory findings, most of them had leucocytosis, elevated ESR, positive CRP, normal ASO, and 5 cases (25%) had slight increase of serum transminase. Hepatomegaly was observed in 7 cases (35%). 6. In spite of the intensive treatment, fever was continued for about 5 days in 5 cases (25%).
Cough ; Diarrhea ; Electrocardiography ; Exanthema ; Extremities ; Female ; Fever ; Gastroenteritis ; Hepatomegaly ; Humans ; Incidence ; Infant ; Lymph Nodes* ; Male ; Pediatrics ; Seasons ; Tachycardia, Sinus ; Vomiting

Tow patients with severe glomerulonephitis and their serum creatinine clearance of 6.0ml/min/1.73m2 in case 2 were treated with high dosage intravenous methylprednisolone, 30mg/kg given over one hour period on each of six slternate days. Follwing the ?ulse therapy, both patients with rapidly deteriorathin renal funtion began to inprove and theit serum creatinine clearance levels showed over douvble by sixdays In both patients treated early in the curse of their disease, creatinine clearance increased to 80ml/min/1.73m2 in case 2 after one year follow-up and in recent study, clinical and laboratory findings are within normal limit.
Creatinine ; Follow-Up Studies ; Humans ; Methylprednisolone*

Clinical and statistical analysis were performed on 573 cases of neonates who were admitted in the special care baby unit, St. Benedict Hospital, from January 1978 to June 1979. The results were as following: 1) Sex ratio of male to female was 1.04:1. 2) According to the route of admission, The ratio of "non-referred patients" to "referred patients was 1.86:1. 3) About the parity incidence, the first baby was 57.1%, the second was 29.7%, and the third was 9.6%. 4) About the age on admission, the babies of the one day I\of age was 45.5% and 86.9% was within 1 week of age. 5) According to the duration of admission, 1-7 days group was the most common(59.6%) and the next were 8-14 days group(24.6%), 15-21 days group(6.1%) 6) According to the duration of admission, 1-7 days group was the most common(59.6%) and the next were 8-14 days group(24.6%), 15-21 days group(6.1%). 7) About the maternal education of non-referred patients, group of high school educated was the most common(45.6%), followed by college(22.3%), middle school(15.3%), and primary school(9.7%). 8) On distribution of the maternal age of non-referred patients, the group of 20-30 years of age was the most common(51.7%), followed by 20-25 years of age (23.3%). Admission rate according to maternal age, higher incidence was seen over 31 years of age. 9) The most common placentas weight of non-refered patients was 600-800 gm. Group(39.9%) and the highest incidence of pre-term was the group of 200-400gm. 10) The most frequent problem was hyperbilirubinemia, followed by prematurity, infectious diseases, respiratory tract diseases, congenital anomalies, diseases associated with metabolism and birth injury. 11) According to the age of initiation of treatment in hyperbilirubinemic patients, the most common age was 4 day(30.5%) and the next was 3 days(25.1%), Suspected causes ware idiopathic(51.2%), blood group incompatibility(17.1%), prematurity(15.3%) and so on. 12) The main diseases associated with prematurity were hyperbilirub-inemia(47.9%), pneumonia(15.1%), RDS(8.4%). 13) Mortality rate were 26.6% in pre-term, 7.4% in post-term, 2.6% in full-term and mortality rate of all admission was 8.0%. Generally, the shorter gestational age and lower birth weight, the higher the mortality rate of premature infants was observed. 14) The leading causes of death were immaturity(48.5%), RDS(24.2%), pneumonia(21.2%) in pre-term, pneumonia(27.3%), kernicterus(18.2%) in full-term and anencephaly & placental dysfunction syndrome in postern 2 cases.
Anencephaly ; Birth Injuries ; Birth Weight ; Cause of Death ; Communicable Diseases ; Education ; Female ; Gestational Age ; Humans ; Hyperbilirubinemia ; Incidence ; Infant, Newborn ; Infant, Premature ; Male ; Maternal Age ; Metabolism ; Mortality ; Parity ; Placenta ; Respiratory Tract Diseases ; Sex Ratio

No abstract available.
Bilirubin* ; Infant, Newborn ; Jaundice, Neonatal*

No abstract available.
Phototherapy*

We experienced one case of Beckwith syndrome. This one day aged male neonate manifested macroglossia, umbilical hernia, visceromegaly, hypoglycemia, gigantism and polycythemia. A brief review of related literature is also presented.
Gigantism ; Hernia, Umbilical ; Humans ; Hypoglycemia ; Infant, Newborn ; Macroglossia ; Male ; Polycythemia

Choledochal cyst, or cystic dilatation of the common bile duct, is generally considered to be an uncommon indiopathic congenital condition that may produce symptoms in infancy but more of ten is discovered at some time during the first two decades of life. Female patients have outnumbered males 3 or 5 to1, and almost cases reported by many authors had more than two symptoms among the classic triad of abdominal mass, jaundice and upper abdominal pain. Authors would like to submit here with the brief review of the literature and report on 2 cases of idiopathic dilatation of the common bile duct that they had experiences among the Pediatric inpatient at Busan St. Benedict Hospital, and the diagnosis was established by clinical, laboratory, X-ray and surgical findings. Cases I in 14 year old girl was complained of severe abdominal distension, jaundice, pruritus and abdominal mass. On 7th admission day, exploratory laparatomy was done and choledochal cyst of 1722cm in size was confirmed and has been followed up for 1 year after Roux-en-Y choledochojejunsotomy without any complications. Cases II in 9 year old boy was complained of intermittent fever, jaundice, colicky abdominal pain and upper abdominal mass. On 6th admission day, exploratory laparatomy was done and choledochal cyst of 1015cm in size was confirmed and has been followed up for 1 year after choledochoduodenostomy with cholecystectomy without any complications.
Abdominal Pain ; Adolescent ; Busan ; Child ; Cholecystectomy ; Choledochal Cyst ; Choledochostomy ; Common Bile Duct* ; Diagnosis ; Dilatation* ; Female ; Fever ; Humans ; Inpatients ; Jaundice ; Male ; Pruritus

A case of congenital hypothyroidism in 7 month old infant was presented. This baby was admitted because of growth retardation, feeding difficulties, and constipation. We diagnosed this case by clinical features as well as thyroid scintigram, X-ray survey for bone age and skeletal maturation and biochemical studies. The patient was treated with desiccated thyroid, but was died of intercurrent infection on 20th hospital day. The review of literature was made briefly.
Congenital Hypothyroidism* ; Constipation ; Humans ; Infant ; Thyroid Gland

The authors observed thirty two cases of nephrotic syndrome admitted at Pediatric Department of St. Benedict Hospital, Busan, Korea. No specific cause for nephrotic syndrome was found in all patients. Respnse to the initial continuous steroid therapy, relapsing patterns and onset of diuresis between steroid only and steroid combined with furosemide especially in severely illed patients were evaluated in this study. The mean period of observation was 3.2 years. 1) The mean age of onset was 7.1 years. Most of them(97%) showed under the 10 years of age at onset. The ratio of male and female was 4.3 to 1. The seasonal incidence showed prevalence from September to October (31%). 2) Upper respiratory infection (21.9%) was the most common associated infection at onset. 3) Complete remission was showed in 23 children (71.8%) after initial continuous steroid therapy. Partial remission in which mild biochemical abnormalities and residual proteinuria wer still present after initial continuous steroid therapy was showed in 6 patients (18.8%), but biochemical abnormalities and residual proteinuria returned to normal within subsequent several months. Three patients (9.4%) were restant to initial steroid therapy. 4) Proteinuria abolished in most of them (87.4%) after 3 weeks of prednisolone therapy in complete remitted patient group, and also abolished after not more than 6 months treatment and their subsequent course in partial remitted patient group. 5) 10 patients (45.4%) had an episode of relapses more than one time, though most of them once or twice, 7 patients (31.8%) relapsed at first year and 2 patients (9%) at second year of follow up period. 6) Steriod responsiveness in relapsing cases was the same as first attack except one. 7) Hematuria, hypertension and transient azotemia were observed in 18.7%, 18.7% and 21.8% of he 32 patiets respectively. These findings at onset of illness were present more frequently in patients with poor steroid response and partial remitted patients than with complete remitted patients, and these episodes were also obtained to correlation with a less favorable initial steroid response and outcome. 8) Duration of illness before institution of steroid therapy had no influences on the steroid response. 9) Diruesis was achieved by 5.2 days in group steroid only and by 4.8 days in group of steroid combined with furosemide, 1~2mg/kg/day, p.o. 10) Two cases were remitted after combined therapy with cyclophosphamide (2mg/kg/day for 4~8 weeks) and prednisolone (60mg/m(2)/day as a single dose on alternate days). One of them was early steroid resistant patient and the other frequent relapsing patient. 11) In one patient, chronic renal insufficiency developed progressively during the follow up period. The other one, whohad no responsiveness to initial steroid therapy and youngest at onset, expired with itercurrent infection.
Age of Onset ; Azotemia ; Busan ; Child* ; Cyclophosphamide ; Diuresis ; Female ; Follow-Up Studies ; Furosemide ; Hematuria ; Humans ; Hypertension ; Incidence ; Korea ; Male ; Nephrotic Syndrome* ; Prednisolone ; Prevalence ; Proteinuria ; Recurrence ; Renal Insufficiency, Chronic ; Seasons