No abstract available.
Incontinentia Pigmenti*

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.
Acetaminophen ; Congenital Abnormalities ; Fascia ; Female ; Humans ; Hyperostosis, Cortical, Congenital ; Infant ; Mandible ; Muscles ; Osteogenesis ; Recurrence ; Skeleton ; Tibia

No abstract available.
Asthma* ; Child* ; Dermatitis, Atopic* ; Humans ; Immunoglobulin E* ; Rhinitis*

No abstract available.
Sepsis* ; Serratia marcescens* ; Serratia*

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.
Anal Canal ; Critical Period (Psychology) ; Ductus Arteriosus, Patent ; Esophageal Atresia ; Humans ; Hydronephrosis ; Infant, Newborn ; Kidney ; Male ; Organogenesis ; Pregnancy ; Tracheoesophageal Fistula

No abstract available.
Progeria*

No abstract available.
Diabetes Insipidus ; Holoprosencephaly

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.
Child ; Cicatrix ; Follow-Up Studies* ; Humans ; Incidence ; Pediatrics ; Protestantism ; Sex Distribution ; Technetium Tc 99m Dimercaptosuccinic Acid ; Ultrasonography ; Ureter ; Urinary Tract Infections* ; Urinary Tract* ; Urology ; Vesico-Ureteral 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.
Child ; Cicatrix ; Follow-Up Studies* ; Humans ; Incidence ; Pediatrics ; Protestantism ; Sex Distribution ; Technetium Tc 99m Dimercaptosuccinic Acid ; Ultrasonography ; Ureter ; Urinary Tract Infections* ; Urinary Tract* ; Urology ; Vesico-Ureteral Reflux*

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.
Birth Weight ; Female ; Humans ; Hyperbilirubinemia, Neonatal ; Incidence ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Critical Care ; Intensive Care, Neonatal ; Medical Records ; Obstetric Labor, Premature ; Parturition ; Pregnancy ; Protestantism ; Risk Factors ; Sepsis ; Survival Rate ; Toxemia