Aplasia cutis congenita represents a congenital absence of all skin layers, and it may occasionally extend through the bone and dura of the skull. Since the first report was described in the extremity by Cordon 1767, and Campbell 1826, approximately over 500 cases have been reported. About eighty five percents of all cases are found in the scalp, with 15 to 30 percents involving the skull as well. Fifteen percents of all cases involve nonscalp locations and are often bilateral symmetrical. It has been relatively rarely reported disorder abroad as well as domestically. It has several clinical subtypes classified by the location and pattern of skin absence, the presence of associated malformation and the mode of inheritance with unknown cause. We had a new born female infant with this disorder, who presented with a full thickness skin defect on scalp and skull defect. No skin defect were reported in other family members, including a first child born several years previously. Chromosomal analysis revealed as normal female karyotype, but she had double pelvis and double ureter of both kidney The legions healed for five weeks by conservative treatment as moist wound dressing and systemic antibiotic administrations. As a relatively uncommon skin anomaly with congenital anomaly, one case of aplasia cutis congenita involving scalp and skull is reported with the review of reference.
Bandages ; Child ; Ectodermal Dysplasia* ; Extremities ; Female ; Humans ; Infant ; Karyotype ; Kidney ; Kidney Pelvis* ; Pelvis ; Scalp* ; Skin ; Skull ; Ureter* ; Wills ; Wounds and Injuries

The improved survival rate of premature infants requiring intensive care, shows an increased risk for nosocomial infections such as disseminated fungal infection. Renal candidasis usually occurs secondary to systemic disease, and can Iead to obstructive uropathy by fungus ball. A male neonate was born in week 28 of the gestational period. His birth weight was 1200gm. He required mechanical ventilation and surfactant for respiratory distress syndrome, umbilical artery and vein catheterization, percutaneous central veneous catheterization(PCVC) for parenteral nutrition, steroid, aminophylline and broad spectrum anibiotics. Hypertension developed on the 29th hospital day, but was not controlled by diuretics and antihypertensive drugs. on the 40th hospital day, he had abdominal distension, anuria, and azotemia. A Renal ultrasonogram showed that the ureteropelvic junction of the left kidney was completely obstructed with fungus balls. A percutaneous nephrostorny tube, made in a pigtail shape by hand, was inserted under fluoroscopy guidance, and the obstruction of the pelvis was resolved by wire manipulation. Parenteral amphotericin B and oral flucytocine were started, and the left renal pelvis was directly drained and irrigated by percutaneous nephrostomy tube. Candida albicans(C. albicuns) was cultured from urine and a percutaneous central venous catheter tip. His general condition improved, and follow up urine culture revealed no fungus. On follow-up renal ultrasonogram, renal cortex echogenicity and fungus ball had disappeared except for mild left renal calyectasis and pelvic thickening. This report describes a case of obstructive uropathy by fungus ball in systemic candidiasis of prematurity, and reviews the related literature.
Aminophylline ; Amphotericin B ; Antihypertensive Agents ; Anuria ; Azotemia ; Birth Weight ; Candida ; Candidiasis ; Catheterization ; Catheters ; Central Venous Catheters ; Cross Infection ; Diuretics ; Fluoroscopy ; Follow-Up Studies ; Fungi ; Hand ; Humans ; Hypertension ; Infant, Newborn ; Infant, Premature ; Critical Care ; Kidney ; Kidney Pelvis ; Male ; Nephrostomy, Percutaneous* ; Parenteral Nutrition ; Pelvis ; Respiration, Artificial ; Survival Rate ; Ultrasonography ; Umbilical Arteries ; Veins

No abstract available.
Aneurysm ; Aneurysm, Ruptured* ; Cerebral Veins* ; Veins*

Neonatal alloimmune thrombocytopenia(NAIT) is a rare disease caused by maternal alloimmunization against fetal platelet surface antigen, which is mainly platelet specific alloantigen or human leukocyte antigen(HLA). During routine hemotology, we accidentally discovered thrombocytopenia in a female fullterm newborn admitted due to jaundice. We excluded NAIT due to human platelet alloantigen(HPA), because the HPA of the mother and baby were the same on PCR-RFLP (polymerase chain reaction-restriction fragment length polymorphism). Mother's serum was tested for lyrnphocytotoxity against 36 donor lymphocytes, and anti-HLA A2, A24 and B58 were found. HLA typing of the father and baby revealed A2 antigen which was not present on the mothers lymphocytes. The patient received concentrated platelet and intravenous globulin. Her platelet count increased to 222,000/mm from 3,000/mm on the 11th day of life. We described a case of NAIT due to anti-HLA A2 antibody with a detailed clinical feature. (J Korean Pediatr Soc 1999;43:861-865)
Antigens, Surface ; Blood Platelets ; Fathers ; Female ; Histocompatibility Testing ; Humans ; Infant, Newborn ; Isoantigens ; Jaundice ; Leukocytes ; Lymphocytes ; Mothers ; Platelet Count ; Rare Diseases ; Thrombocytopenia ; Thrombocytopenia, Neonatal Alloimmune* ; Tissue Donors

Candidiasis of the central nervous system (CNS) is a rare condition and like other opportunistic fungal infections, most commonly occurs in immune-compromised patients. Because of the increasing use of antibiotics and the improving survival rate of premature infants requiring intensive care, the incidence of fungal infections in the brain has increased. We report the findings of ultrasonography and MR imaging in two cases of candidiasis of the CNS in premature infants.
Anti-Bacterial Agents ; Brain ; Candidiasis* ; Central Nervous System ; Humans ; Incidence ; Infant, Newborn ; Infant, Premature* ; Critical Care ; Magnetic Resonance Imaging* ; Nervous System* ; Survival Rate ; Ultrasonography

No abstract available.

No abstract available.

No abstract available.
Goldenhar Syndrome*

PURPOSE: Urinary tract infection(UTI) in children may be cause of end stage renal failure and hypertension. So, early detection of renal scar, vesicoureteral reflux(VUR) and anomaly, and proper management are important. We carried this study to observe the significance of 24 hours urine beta2-microglobulin(beta2-MG) for predicting renal injury and correlation of beta2-MG with the grade of VUR. METHODS: We evaluated 52 patients with UTI who were admitted to the department of Pediatrics, Sung-Ae General Hospital from April 1994 to December 1994. All patients were evaluated with 99mTc-2,3-dimercaptosuccinic acid (DMSA) renal scan, voiding cystourethrogram(VCUG) and 24 hours urine beta2-MG. We divided them into 3 groups, group A; renal scarring in DMSA scan and no reflux in VCUG(14 patients), group B; renal scarring and reflux (10 patients), and group C; no renal scarring and reflux (24 patients). RESULTS: 1) Among 52 patients, 31 patients(59.6%) were less than 1 year of age and 21 patients(40.4%) were older than 1 year. The sex ratio of male to female was 1.6:1. 2) Among 24 patients with renal scarring, 15 patients(63%) were less than 1 year of age, and vesicoureteral reflux was observed in 10 patients (41.6%). 3) Vesicoureteral reflux was seen in 14 patients (26.9%): one with grade I, four with grade II, six with grade III, one with grade IV, and two with grade V. 4) The values of 24 hours urine beta2-MG in group A and B were 3.94+/-6.13mg /g Cr and 3.72+/-5.38mg/g Cr respectively, while 0.85+/-0.63mg/g Cr in group C, showing significant difference between group A and group C(p<0.05). However, there was no significant difference between group A and B(p>0.05). The grade of vesicoureteral reflux was also significantly correlated with the level of 24 hours urine beta2-MG. 5) 24 hours urine beta2-MG was significantly decreased with improvement of renal scarring after successful treatment of urinary tract infection. CONCLUSIONS: Measurement of 24 hours urine beta2-MG can be used as a sensitive marker of the severity of UTI and also useful for the evaluation of successful treatment and prognosis of patients with urinary tract infection.
beta 2-Microglobulin ; Child ; Cicatrix ; Female ; Hospitals, General ; Humans ; Hypertension ; Male ; Pediatrics ; Prognosis ; Renal Insufficiency ; Sex Ratio ; Succimer ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux

OBJECTIVES: The objective of this study was to evaluate the thermic effects, the macronutrient oxidation rates and the satiety of medium-chain triglycerides (MCT). METHODS: The thermic effects of two meals containing MCT or long-chain triglycerides (LCT) were compared in ten healthy men (mean age 24.4 +/- 2.9 years). Energy content of the meal was 30% of resting metabolic rate of each subject. Metabolic rate and macronutrient oxidation rate were measured before the meals and for 6 hours after the meals by indirect calorimetry. Satiety was estimated by using visual analogue scales (VAS) at 8 times (before the meal and for 6 hours after meal). RESULTS: Total thermic effect of MCT meal (42.8 kcal, 8.0% of energy intake) was significantly higher than that (26.8 kcal, 5.1% of energy intake) of the LCT meal. Mean postprandial oxygen consumption was also significantly different between the two types of meals (MCT meal: 0.29 +/- 0.35 L/min, LCT meal: 0.28 +/- 0.27 L/min). There were no significant differences in total postprandial carbohydrate and fat oxidation rates between the two meals. However, from 30 to 120 minutes after consumption of meals, the fat oxidation rate of MCT meal was significantly higher than that of the LCT meal. Comparison of satiety values (hunger, fullness and appetite) between the two meals showed that MCT meal maintained satiety for a longer time than the LCT meal. CONCLUSIONS: This study showed the possibility that long-term substitution of MCT for LCT would produce weight loss if energy intake remained constant.
Calorimetry, Indirect ; Energy Intake ; Humans ; Male ; Meals ; Oxygen Consumption ; Triglycerides* ; Weight Loss ; Weights and Measures