Fetal bilateral renal agenesis is a lethal congenital anomaly. An early and reliable prenatal diagnosis is extremely important as it may offer options for pregnancy termination as early as possible. The criteria for the ultrasonographic diagnosis of bilateral renal agenesis are severe oligohydramnios, nonvisualization of the bladder, and the empty renal fossa. However, severe oligohydramnios makes it difficult to diagnose the disease because of poor sonographic resolution. We present two cases of bilateral renal agenesis, one is diagnosed by ultrasonography after amnioinfusion at 24 weeks gestation, the other is diagnosed postnatally after term delivery.
Diagnosis ; Female ; Oligohydramnios ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography ; Urinary Bladder

No abstract available.

Although viruses are the most common cause of acute gastroenteritis (AGE) in humans, details about the causative viruses in AGE are largely unknown because many causative viruses are unable to be cultured by current culture techniques. In our study, fecal samples from 10 children under five years of age with unexplained AGE and 10 healthy children were investigated for RNA viruses using random priming (RP)-mediated sequence-independent single primer amplification (SISPA). The causative viruses in cases of cryptogenic diarrhea were then assessed for their potential diagnostic value. Of the 1,129 viral clones identified, rotavirus was most commonly associated with AGE (125 sequences, 22.4%). In contrast, bacteriophage was most common (43 sequences, 13.6%) in healthy children. The remaining 515 viral clones were unidentifiable. These findings suggest that investigation of cases or outbreaks of unexplained diarrhea using a metaviromic strategy is a new avenue for diagnosis.
Bacteriophages ; Child ; Clone Cells ; Culture Techniques ; Diagnosis ; Diarrhea ; Disease Outbreaks ; Feces* ; Gastroenteritis* ; Humans ; Parechovirus ; RNA Viruses ; Rotavirus

PURPOSE: To observe MR findings of vascular abnormality in Sturge-Weber syndrome and to determine the value of MRI in diagnosis. MATERIALS AND METHODS: Ten patients with Sturge-Weber syndrome (age : 3 months-32 years)were evaluated by MR imaging ; in six and four cases, respectively, the results were correlated with those of CT and angiography. We retrospectively analysed changes in the cortical vein and deep venous system, including the medullary and subependymal vein, as well as an largement of the choroid plexus, leptomeningeal enhancement, and changes in diploic space. RESULTS: In all cases except one, in which non-contrast enhanced study had been performed, varying degrees of leptomeningeal enhancement were seen. In nine cases the cortical vein became smaller; enlargement of the choroid plexus was seen in eight cases, change in the diploic space in seven (including three in which there was angiomatous involvement), and enlargement of the deep venous system in seven. In younger patients, collateral pathways were less developed and leptomeningeal angiomatous changes were more pronounced than in those who were older. CONCLUSION: MRI is a useful modality for the evaluationn of vascular changes in Sturge-Weber syndrome. These changes vary according to a patient's age and the duration of the disease.
Angiography ; Choroid Plexus ; Diagnosis ; Humans ; Magnetic Resonance Imaging ; Retrospective Studies ; Sturge-Weber Syndrome* ; Veins

Since G.N. Hounsfield's clinical use of computed tomography in 1971, digital imaging technique using computers has shown an eye opening progress. Progress has made 3-dimensional understanding of not only facial bones but muscles and other connective tissues possible through 3-dimensional reconstruction of preexisting tomographical images. Also, quantitative analysis of density, distance, volume has become possible, allowing objective analysis of preoperative and postoperative states through imaging. The authors measured the masseter muscle volume of 20 normal individuals and 8 female patients through 3-D reconstructive CT imaging and made a statistical analysis of the measurements. The method used in our study may be applied to the diagnosis of disease causing the change of the facial volume and presurgical design as a useful tool to provide objective information on the evaluation of surgery outcome.
Connective Tissue ; Diagnosis ; Facial Bones ; Female ; Humans ; Masseter Muscle* ; Muscles

No abstract available.
Antibodies ; Guillain-Barre Syndrome

We report on 2 siblings with a partial trisomy of 7q (7q22-->qter) and concomitant partial monosomy of 8p (8p23.3-->pter), which were shown by FISH using probes located at the telomere region of each chromosome. All the balanced translocation carriers (father and a sister) in this family had a normal phenotype. The 2 siblings with the same abnormal karyotype had similar multiple congenital anomalies and dysmorphic features. During the follow-up, the first male patient died in the neonatal period, but the female sibling is still alive at 2 years and 6 months of age.
Abnormal Karyotype ; Chromosome Deletion ; Female ; Follow-Up Studies ; Humans ; Male ; Phenotype ; Siblings ; Telomere ; Trisomy

Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children younger than 4 years and is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. HUS associated with diarrheal prodrome is usually caused by Shiga toxin-producing Escherichia coli O157:H7 or by Shigella dysenteriae, which generally has a better outcome. However, atypical cases show a tendency to relapse with a poorer prognosis. HUS has been reported to be associated with acute lymphoblastic leukemia (ALL) in children. The characteristics and the mechanisms underlying this condition are largely unknown. In this study, we describe the case of an 11-year-old boy in whom the diagnosis of ALL was preceded by the diagnosis of atypical HUS. Thus, patients with atypical HUS should be diagnosed for the possibility of developing ALL.
Acute Kidney Injury ; Anemia, Hemolytic ; Child ; Hemolytic-Uremic Syndrome ; Humans ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Recurrence ; Shiga-Toxigenic Escherichia coli ; Shigella dysenteriae ; Thrombocytopenia

BACKGROUND AND OBJECTIVES: The conventional adenoidectomy is performed via the transoral approach and can be accomplished with adenoid curette, adenotome, and adenoid punch. This method has been associated with many complications. We performed an adenoidectomy using an electric adenotome under the operating microscope. The aim of this study was to compare the electric adenotome adenoidectomy with the conventional adenoidectomy and to evaluate the effectiveness and outcome of the electric adenotome surgery. MATERIALS AND METHODS: One hundred cases of microscopic adenoidectomy using the electric adenotome and one hundred cases of conventional methods using curette or punch forceps were examined. RESULTS: There was less incidence of intraoperative and postoperative bleeding in the electric adenotome adenoidectomy group. There were no differences regarding the postoperative outcome and recurrence rate between the electric adenotome method group and the conventional method group. CONCLUSION: Compared to the conventional method, the electric adenotome method has similar postoperativc result but has a lower incidence of intraoperative and postoperative bleeding. We suggest that adenoidectomy using an electric adenotome is an attractive surgical method in children with symptomatic adenoid vegetation,
Adenoidectomy* ; Adenoids ; Child ; Hemorrhage ; Humans ; Incidence ; Recurrence ; Surgical Instruments

BACKGROUND: Growth impairment is a common complication after hematopoietic stem cell transplantation (SCT). The aim of this study was to evaluate the final adult height of patients who underwent SCT in childhood and to identify the factors that influence long-term growth in these patients. METHODS: A retrospective review of 15 children who underwent SCT before puberty at Chonnam National University Hospital and reached final adult height was undertaken. To assess the severity of height reduction and to monitor the height changes longitudinally, height measurements of each patient both at the time of SCT and the final height were expressed as the height standard deviation score (SDS). RESULTS: Seven children were males and eight were females with a median age of 12.8+/-2.4 years (range, 6.3~14.7) at SCT. The median follow-up period was 7.1+/-2.0 years (range, 4.5~11.1) and their final height was achieved at 18.1+/-1.5 years (range, 17.0~21.8). Final height SDS values were within normal for the healthy population in all except two who had short stature (below -2.0 SDS). No patient achieved height values greater than +2.0 SDS. The final height SDS value (-0.5+/-1.2) was not decreased from the height SDS value at SCT (-0.8+/-0.8). The younger age group at SCT (6.1~10.0 years, n=5) showed significantly lower final height SDS and greater Delta SDS than the older age group (10.1~15.0 years, n=10) (-1.5+/- 0.6 vs. -0.1+/-1.1, P<.05; -1.2+/-0.7 vs. 0.5+/-0.8, P<.05, respectively). The irradiation-based conditioning (n=6) had negative effects on the Delta SDS (P>.05) and the final height SDS (P<.05). The gender, type of disease, donor type or the presence of chronic graft-versus-host disease did not influence height. CONCLUSION: Growth impairment may be encountered in children after SCT. A younger age at transplant and irradiation were found to be factors associated with reduced final height. However, most patients (13/15) reached a final adult height within normal limits for the general healthy population.
Adolescent ; Adult ; Child* ; Female ; Follow-Up Studies ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Jeollanam-do ; Male ; Puberty ; Retrospective Studies ; Stem Cell Transplantation* ; Stem Cells* ; Tissue Donors