A Case of Sporadic Caffey's Disease(Infantil Cortical Hyperostosis) Without Mandibular Involvement

Caffey's disease(infantile cortical hyperostosis) is an uncommon benign and self limited disorder of unknown etiology, affecting the skeleton and its contiguous fasciae and muscles. Its occurrence in isolated cases or in multiple members in families suggests the existence of two different forms, namely a sporadic form and a familial form. The tibia is the predominant bone known to be affected in the familial form, while the mandible is mostly affected in the sporadic form. This benign and self-limited disorder has rarely late recurrence or persistence of symptoms with deformity. The patient in this case had immediate and complete resolution of her symptoms while receiving acetaminophen with no recurrent bone formation during treatment with antiinflammatory drugs. We describe sporadic, nonfamilial Caffey's disease, affecting both radii and tibiae, but not involving the mandibular in a 4-month-old female infant.

A Case of Juvenile Granulosa Cell Tumor of the Ovary

Juvenile granulosa cell tumor (JGCT) is one of the sex cord stromal tumors of the ovary ocurring in the first two decades of life. These tumors are different from adult granulosa cell tumor (AJCT) with regard to clinical and pathological fetures. Follicles are often irregular, Call-exner bodies are rare, and luteinization is frequent. The tumor may be solid, cystic, or both. The most common presenting symptoms are abnormal uterine bleeding and pain. Breast swelling, pain and tenderness may also be associated with unopposed estrogen secretion by granulosa cell tumors. The tumor should be removed as soon as the diagnosis is estabilished. The juvenile granulosa cell tumor has a good overall prognosis because fewer than 5% of these tumors in children are malignant.

Follow-Up Study of Urinary Tract Infection Associated with Vesicoureteral Reflux

PURPOSE: Vesicoureteral reflux (VUR) is the major cause of urinary tract infection (UTI) in children. Prolonged untreated UTI associated with VUR may result in serious complications, such as renal scarring, renal atropy, and decreased renal function. Therefore, follow up must be continued until UTI is cured completely, to prevent of serious complication. The authors conducted this study to evaluate results of the follow-up of UTI associated with VUR. METHODS: During the period from March 1991 to July 1996, we had 47 children with urinary tract infection associated with vesicoureteral reflux who were admitted to the Department of Pediatrics and Urology, Presbyterian Medical Center. The patients were managed medically or surgically. RESULTS: 1) There were no differences in sex distribution in the 47 cases with VUR. The intensity of 67 refluxing ureters, classified by the International Reflux Study Committee, grade I, 10 cases; grade II, 11 cases; grade III, 20 cases; grade IV, 17 cases; grade V, 9 cases. 2) The most common causative agent in UTI was E. coli. Fourty-six cases underwent ultrasonography and 24 showed abnormal findings. Fourty-four cases underwent a 99mTc-DMSA renal scan, and 25 showed abnormal findings. The incidence of renal scarring showed a direct correlation with the severity of VUR. 3) The incidence of urinary tract infection during follow-up did not differ between the groups medical management versus surgical management. 4) Fourty-four refluxing ureters were treated primary medically and 38 of them were followed up. Twenty-five of the 38 ureters were disappeared or improved, and 9 persisted. The rest showed aggravation of reflux. Twenty-five of the 32 refluxing ureters showed disappearance or improvement of reflux before 5 years of age, The spontaneous cure rate of vesicoureral reflux seemed to be higher in the cases with a milder grade of reflux. 5) Although refluxing ureter disappeared completely on VCUG, we found that follow-up is continued until the disappearance of reflux is confirmed on two serial VCUG. 6) Thirty-three refluxing ureters were treated surgically and one of them was stationary on follow-up VCUG, while the others were cleared. CONCLUSIONS: As a result of follow-up the 47 patients with VUR, the incidence of urinary tract infection did not differ between the groups medical versus surgical management. Rates of disappearance of VUR in medical and surgical management were 66%, 97%, respectively. The spontaneous cure rate of VUR seemed to be higher in the cases with a milder grade of reflux and before 5 years of age. We found that follow-up is continued until the disappearance of reflux is confirmed on two serial VCUG.

Follow-Up Study of Urinary Tract Infection Associated with Vesicoureteral Reflux

PURPOSE: Vesicoureteral reflux (VUR) is the major cause of urinary tract infection (UTI) in children. Prolonged untreated UTI associated with VUR may result in serious complications, such as renal scarring, renal atropy, and decreased renal function. Therefore, follow up must be continued until UTI is cured completely, to prevent of serious complication. The authors conducted this study to evaluate results of the follow-up of UTI associated with VUR. METHODS: During the period from March 1991 to July 1996, we had 47 children with urinary tract infection associated with vesicoureteral reflux who were admitted to the Department of Pediatrics and Urology, Presbyterian Medical Center. The patients were managed medically or surgically. RESULTS: 1) There were no differences in sex distribution in the 47 cases with VUR. The intensity of 67 refluxing ureters, classified by the International Reflux Study Committee, grade I, 10 cases; grade II, 11 cases; grade III, 20 cases; grade IV, 17 cases; grade V, 9 cases. 2) The most common causative agent in UTI was E. coli. Fourty-six cases underwent ultrasonography and 24 showed abnormal findings. Fourty-four cases underwent a 99mTc-DMSA renal scan, and 25 showed abnormal findings. The incidence of renal scarring showed a direct correlation with the severity of VUR. 3) The incidence of urinary tract infection during follow-up did not differ between the groups medical management versus surgical management. 4) Fourty-four refluxing ureters were treated primary medically and 38 of them were followed up. Twenty-five of the 38 ureters were disappeared or improved, and 9 persisted. The rest showed aggravation of reflux. Twenty-five of the 32 refluxing ureters showed disappearance or improvement of reflux before 5 years of age, The spontaneous cure rate of vesicoureral reflux seemed to be higher in the cases with a milder grade of reflux. 5) Although refluxing ureter disappeared completely on VCUG, we found that follow-up is continued until the disappearance of reflux is confirmed on two serial VCUG. 6) Thirty-three refluxing ureters were treated surgically and one of them was stationary on follow-up VCUG, while the others were cleared. CONCLUSIONS: As a result of follow-up the 47 patients with VUR, the incidence of urinary tract infection did not differ between the groups medical versus surgical management. Rates of disappearance of VUR in medical and surgical management were 66%, 97%, respectively. The spontaneous cure rate of VUR seemed to be higher in the cases with a milder grade of reflux and before 5 years of age. We found that follow-up is continued until the disappearance of reflux is confirmed on two serial VCUG.

A Case of Nonfamilial Generalized Gastrointestinal Juvenile Polyposis with Pancytopenia

In contrast to the child with a solitary juvenile polyp, generalized gastrointestinal juvenile polyposis have a much different clinical course. In generalized juvenile polyposis blood loss can be considerable and iron-deficiency anemia is a common observation. Although it is usually regarded as a benign condition, juvenile polyposis may predispose to cancer of the gastrointestinal tract. We experienced a case of nonfamilial generalized gastrointestinal juvenile polyposis with pancytopenia in a 15 year-old male patient who had hospitalized because of intermittent rectal bleeding with anemia for three years. Histological examination of polyps from affected patient revealed a spectrum of change from juvenile polyps, to juvenile polyps with adenomatous changes. Subtotal colectomy, rectal mucosectomy, and endorectal pull-through procedure were successfully performed. No complication has occured since the discharge. A brief review of related literatures is also presented.

A Clinical Study of Febrile Convulsion and Factors Related to Recurrence

PURPOSE: To investigate the risk factors of recurrence after their first febrile convulsions. And to evaluate effect of anticonvulsant therapy with recurrent febrile convulsion infants and children. METHODS: We have studied the relationship of their clinical pictures and factors related to the risk of recurrence of 178 patients with primary or recurrent febrile convulsion, who were admitted to department of pediatrics, or visited to emergency room, chonju presbyterian medical center from january 1983 to december 1992. The 178 patients were followed up and consisted of the 97 patients with primary febrile convulsion and the others 81 patients with recurrent febrile convulsions. RESULTS: 1) In sex distribution, the boys(59%) outnumbered the girls(41%) and the ratio was 1.4:1. 2) 95% of overall patients were occurred febrile convulsion under 5 year-old and recurrent rate was 45.5%. In recurrent cases, the first episode under the 12 months was 50.6%(p<0.005). 3) There were family history of febrile convulsion in 28.4% of recurrent cases, compared to 10.9% of primary cases(p<0.001). 4) There was no significant difference with duration and type of convulsion in both groups. 5) Small proportion of 27 children were prescribed anticonvulsants (phenobarbital), but it's not reduced the recurrence and epilepsy significantly. CONCLUSIONS: The risk factor of recurrent febrile convulsion were the first episode under 12 month and familial history of febrile convulsion.

Clinical Study of Foreign Body Aspiration in Infants and Children

A clinical study was done on 42 cases of inpatients, who had been admitted for foreign body aspiration form January 1979 to June 1992. We obtained the following results by analysing the records of their treatment during that time. 1) 31(73.8%) of these cases were below the age of 3 years. 25(59.4%) were below the age of 2. The ratio of male to female was 2:1 2) In 23 cases(54.8%) onset of the symptoms and diagnosis was less than 24 hours after aspiration. 3) Most of the cases(83.3%) had a history of foreign body aspiration; 7 other cases did not. 4) The most common symptoms of bronchial foreign bodies were cough, dyspnea and fever, whereas those of laryngotracheal foreign bodies were dyspnea and cough, in that order. 5) Common complications were obstructive emphysema, pneumonia and atelectasis, in that order. These complications were observed more frequently in the cases of vegetable foreign bodies. 6) Chest X-ray at the time of admission showed obstructive emphysema (42.9%) and pneumonia(40.5%). 11 cases(26.2%) were normal. 7) The most comon site of foreign body enlodgement was the right main bronchus(38.1%), and 4 cases(9.5%) were discovered in other bronchial sites. 8) Vegetable foreign bodies occupied 54.8% of the cases, peanuts being the most common.

Relation between Airway Responsiveness and Serum IgE in Children with History of Asthma, Allergic Rhinitis, and Atopic Dermatitis

No abstract available.

Diagnostic Value and Relationship of the between Stable Microbubble Rating Test and Shake Test for the Prediction of Neonatal Respiratory Distress Syndrome

Respiratory distress syndrome (RDS) in the newborn infants remains a major cause of mortality and morbidity in the newborn period despite much improvements in neonatal intensive care and artificial ventilatory techniques. Gastric fluid was obtained from 151 patients within 6 hours after delivery. The sensitivity, specificity, and predictive value of the simple shake test (133 cases) and stable microbubble rating (SMR) test (151 cases) were assessed in the diagnosis of RDS, as well as the relation between both tests and RDS. We carried out both tests of on gastric aspirates all newborn who admitted to NICU of Presbyterian Medical Center from June 1991 to August 1992. The results were summarized as follows: 1) Among the total 151 cases, RDS were found in 41 cases(27.2%). 2) RDS occurence rate of the simple shake test was 11/11 in 0 group, 17/26 in +1 group, 8/28 in +2 group, 2/41 in +3 group, and 2/27 in +4 group. RDS occurence rate was high the 0 and +1 group. 3) RDS occurence rate of the SMR test was 4/4 in very weak group, 32/36 in weak group, 1/33 in medium group, and 4/78 in strong group. RDS occurence rate was high in the very weak and weak group. 4) Among the positive group of the SMR test 95 cases, positive group of the shake test were found in 87 cases. Among negative group of the SMR test 38 cases, negative of the shake test were found in 29 cases (correlation coefficient=0.763). 5) Sensitivity of the shake test and SMR test were 70%, 87.8% respectively. Specificity of the shake test and SMR test were 93.3%, 96.4% respectively. Positive predictability were 75.7%, 90% respectively and negative predictability were 87.5%, 95.5% respectively. The shake test, as Well as SMR test, has significant value to diagnosis of the RDS. We predict RDS occurence rate of the SMR test was significantly higher than shake test.

Clincial Study on Very Low Birth Weight Infants

Medical records of very low birth weight infants weighing less than 1500 grams at birth were reviewed and analyzed. One hundred and forty three infants who were admitted to neonatal intensive care unit of Presbyterian Medical Center, including those who were born and transferred from other hospitals, from January 1987 to December 1991 were examined and the following results were obtained; 1) The incidence of very low birth weight infant was 1.21% 2) The most common maternal risk factor was premature labor and the next was toxemia. 3) The most common disease of very low birth weight infant was neonatal hyperbilirubinemia and the next was septicemia. 4) Twenty five infants (38.5%) of intensive care group and 67 infants (85.9%) of Not-ventilated group survived with overall survival rate of 64.6%. 5) The heavier birth weight and longer intrauterine period were factors offering better chance for survival. 6) Among 43 (30%) infants expired, 7 (16.3%) died within 24 hours after birth, 12 (27.9%) died in second or third day, 4 (9.3%) died between fourth and seventh day, and 20 (46.5%) died between eighth.and twenty eighth day.

A case of VATER syndrome

The VATER syndrome is a group of congenital anomalies with a nonrandom tendency for concurrence. Defects include vertebral, anorectal malformation, tracheoesophageal fisutla with esophageal atresia, radial-limb, vascular, and renal abnormalities. The critical period of organogenesis is at or before the sixth or seventh week of gestation. We experienced one case of VATER syndrome in a 1 day old male neonate having vertebral anomalies, esophageal atresia with tracheoesophageal fistula to the distal esophageal segment, imperforated anus, left renal dysplasia with hydronephrosis of the right kidney and both hydroureter, patent ductus arteriosus. We report a case of VATER syndrome with brief review of related literature.

A case of holoperosencephaly, alobar type

No abstract available.

Clinical Bacteriologic Study of Serratia Marcescens Septicemia

No abstract available.

A case of Hutchinson Gilford Progeria Syndrome

No abstract available.

A case of Incontinentia Pigmenti

No abstract available.

A Case of Histiocytic Medullary Reticulosis in 17 Month-old Child

No abstract available.