PURPOSE: The purpose of this study was to evaluate the CT findings of acute pyelonephritis (APN) in children and to assess the correlation between these findings, clinical parameters and renal scar development, as seen on follow-up CT scans. MATERIALS AND METHODS: Contrast-enhanced CT scans of thirty children in whom APN had been diagnosed were assigned to one of three groups according to whether an abscess had formed, and then to subgroups on the basis of the number of lesions in the renal parenchyme. Initial CT findings were retrospectively correlated with five clinical parameters (maximal body temperature, fever duration, leukocytosis, pyuria and admission period) and renal scar development, as seen on follow-up CT (n=12). RESULTS: CT scans demonstrated linear, wedge-shaped, low-density renal parenchymal lesions in 35 kidneys of 25 patients and abscesses in seven kidneys of seven patients, but no abnormal lesions in five patients. In the three groups there was correlation between these findings and some clinical parameters (maximal body temperature, fever duration and admission period), but no subgroup showed significant correlation with any clini-cal parameter. Renal cortical scars detected by follow-up CT were more prevalent in patients in whom initial CT demonstrated the presence of an abscess. CONCLUSION: Clinical parameters correlated with the presence of renal parenchymal hypoenhancing lesions and abscess formation, as seen on CT scans, rather than the number of renal parenchymal lesions. Renal cortical scars were more prevalent in patients in whom initial CT revealed the presence of an abscess. Enhanced CT is thought to be useful both for diagnosing APN and for predicting its clinical course in children.
Abscess ; Body Temperature ; Child ; Cicatrix ; Fever ; Follow-Up Studies ; Humans ; Kidney ; Leukocytosis ; Pyelonephritis* ; Pyuria ; Retrospective Studies ; Tomography, X-Ray Computed

PURPOSE: Left renal vein entrapment syndrome has been suggested as an etiology for asymptomatic non-glomerular hematuria since it was reported as a cause of unilateral gross hematuria. Reported diagnostic criteria has been controversial since various degrees of left renal vein entrapment was found in normal children. Some of asymptomatic non-glomerular hematuria was not diagnosed even with renal biopsies but was usually known to have self-limited benign course. We analyzed the relationship between asymptomatic non-glomerular hematuria of unknown origin and the degree of left renal vein entrapment phenomenon. METHODS: The renal doppler sonograpy of 92 children with asymptomatic non-glomerular hematuria [gross hematuria(GH) N=44, microscopic hematuria (MH) N=48] were compared to 30 control children with normal renal function and urinalysis who underwent renal doppler sonography for abdominal pain and enuresis from January, 1999 to Febrary, 2000 at Ewha Womans Mokdong Hospital. The narrowed diameter(ND) of the left renal vein between the aorta and superior mesenteric artery and its maximal velocity(NV), and the dilated diameter(DD) of the left renal vein and its maximal velocity(DV) were measured and the DD/ND and NV/DV ratio were compared with those of the control children and the results of several previous reports. RESULTS: The DD/ND ratio was 3.9+/-1.89 in the GH group, 2.4+/-0.62 in the MH group, and 2.0+/-0.48 in the control group. There was a significant difference among GH, MH and control group(p<0.05). The NV/DV ratio was 3.6+/-2.37 in the GH group was significantly higher than 1.9+/-0.60 in the MH group and 1.7+/-0.55 in the control group(p<0.05). There was no significant differences between MH and control groups. Normal cut off values of DD/ND and NV/DV ratio in this study were 3.0 and 2.8 which was different to previous reports. CONCLUSION: Left renal vein entrapment phenomenon should be considered as one of the etiology of asymptomatic non-glomerular hematuria in children and the sonographic diagnostic criteria for Left renal vein entrapment syndrome needs to be revised.
Abdominal Pain ; Aorta ; Biopsy ; Child* ; Enuresis ; Female ; Hematuria* ; Humans ; Mesenteric Artery, Superior ; Renal Nutcracker Syndrome ; Renal Veins* ; Ultrasonography ; Urinalysis

PURPOSE: Left renal vein entrapment syndrome has been suggested as an etiology for asymptomatic non-glomerular hematuria since it was reported as a cause of unilateral gross hematuria. Reported diagnostic criteria has been controversial since various degrees of left renal vein entrapment was found in normal children. Some of asymptomatic non-glomerular hematuria was not diagnosed even with renal biopsies but was usually known to have self-limited benign course. We analyzed the relationship between asymptomatic non-glomerular hematuria of unknown origin and the degree of left renal vein entrapment phenomenon. METHODS: The renal doppler sonograpy of 92 children with asymptomatic non-glomerular hematuria [gross hematuria(GH) N=44, microscopic hematuria (MH) N=48] were compared to 30 control children with normal renal function and urinalysis who underwent renal doppler sonography for abdominal pain and enuresis from January, 1999 to Febrary, 2000 at Ewha Womans Mokdong Hospital. The narrowed diameter(ND) of the left renal vein between the aorta and superior mesenteric artery and its maximal velocity(NV), and the dilated diameter(DD) of the left renal vein and its maximal velocity(DV) were measured and the DD/ND and NV/DV ratio were compared with those of the control children and the results of several previous reports. RESULTS: The DD/ND ratio was 3.9+/-1.89 in the GH group, 2.4+/-0.62 in the MH group, and 2.0+/-0.48 in the control group. There was a significant difference among GH, MH and control group(p<0.05). The NV/DV ratio was 3.6+/-2.37 in the GH group was significantly higher than 1.9+/-0.60 in the MH group and 1.7+/-0.55 in the control group(p<0.05). There was no significant differences between MH and control groups. Normal cut off values of DD/ND and NV/DV ratio in this study were 3.0 and 2.8 which was different to previous reports. CONCLUSION: Left renal vein entrapment phenomenon should be considered as one of the etiology of asymptomatic non-glomerular hematuria in children and the sonographic diagnostic criteria for Left renal vein entrapment syndrome needs to be revised.
Abdominal Pain ; Aorta ; Biopsy ; Child* ; Enuresis ; Female ; Hematuria* ; Humans ; Mesenteric Artery, Superior ; Renal Nutcracker Syndrome ; Renal Veins* ; Ultrasonography ; Urinalysis

No abstract available.
Fibroma* ; Testis

Forth-nine patients with clinically nonfunctioning pituitary adenomas were evaluated clinically, endocrinologically and morphologically in this study.The results obtained were as follows.1) The mean age was 47.1 years(range 23 to 76 years), and 22 were male(44.9%) and 27(55.1%) female.2) The major clinical manifestations of male patients were visual disturbance(72.7%), headache(54.5%), loss of libido(45.5%), but those of female visual disturbance(59.6%), headache(48.1%), amenorrhea(48.1%), loss of body hair(25.9%), and galactorrhea(22.2%).3) All were macroadenomas evaluated by CT scan, and in the male patients 16(72.7%) were grade III and 6(27.3%) grade IV by Hardy classification, and in the female patients 6(22.2%) were grade II, 12(44.4%) grade III, and 9(33.3%) grade IV.4) The elevation of serum prolactin were observed 7(31.8%) out of male, and 24(88.9%) of female.5) Combined stimulation test revealed that GH insufficiency was 89.6%, ACTH 58.9%, LH 58.7%, FSH 51.1 %, and TSH 50.0% and hormone insufficiency more than 4 pituitary hormone was 54.2%.6) Prolactin response to TRH decreased in 12(70.6%) of 17 patients with normal basal prolactin, and 19(76.0%) of 25 with elevated prolactin.7) Immunohistochemistry revealed that null cell adenoma was 57.1%, gonadotrope adenoma 26.5%, plurihormonal adenoma 8.0%, silent corticotrope adenoma 4.0%, thyrotrope adenoma(2.0%), and lactotrope adenoma(2.0%).8) The ultrastructural characteristics examined by electron-microscopy were similar despite of immunohistochemical differences.In summary, the prevalance of clinically nonfunctioning pituitary adenoma was middle aged men and women, and their main symptoms were visual disturbance and headache. Hyperprolactinemia and pituitary hormone insufficiency more than 4 hormone were observed commonly. Most of them were null cell adenoma and gonadotrope adenoma examined by immunohistochemistry. Further study using modern techniques: cell culture, subunit-immunostaining. And Northern blot analysis of mRNA for pituitary hormone or subunit, will be needed to clarify null cell adenomas.
Adenoma ; Adrenocorticotropic Hormone ; Blotting, Northern ; Cell Culture Techniques ; Classification ; Female ; Headache ; Humans ; Hyperprolactinemia ; Immunohistochemistry ; Lymphocytes, Null ; Male ; Middle Aged ; Pituitary Neoplasms ; Prolactin ; RNA, Messenger ; Tomography, X-Ray Computed

Diabetic mastopathy is a rare disease that occurs in long-term insulin-dependent diabetic patient. It manifests as a hard palpable breast mass that may be clinically indistinguishable from a breast carcinoma. Mammography shows a non-specific, dense, heterogenous glandular opacity in both breasts. Sonography shows a markedly hypoechoic, ill-marginated mass with a posterior acoustic shadowing. We present the mammography, ultrasonography and MRI findings of a 54-year-old woman with diabetic mastopathy.
Acoustics ; Breast ; Breast Neoplasms ; Female ; Humans ; Magnetic Resonance Imaging* ; Mammography ; Middle Aged ; Rare Diseases ; Shadowing (Histology) ; Ultrasonography

Distraction osteogenesis has become an alternative technique to treat craniomaxillofacial anomalies. It was initially used to treat mandibular dysplasia and now it is applied to other regions of the craniofacial skeleton. We now present our clinical experience of midface distraction with the use of rigid external distraction for the treatment of an 8-years-old girl with midface hypoplasia in Crouzon's disease, who had undergone fronto-orbital advancement at the age of 6. We performed midface advancement by Le Fort III osteotomy with rigid external distraction system(RED II, KLS Martin, Jacksonville, FL). The active distraction was initiated on the 3rd postoperative day and was continued until the 20th postoperative day for 18 days. The rate of distraction can be adjusted during this time according to clinical judgment and cephalometric assessment. On completion of distraction, the RED II was left in place without activation for 25 days for rigid retention. The RED II was then removed and an orthodontic facemask was applied with elastic traction for 6wks. The total amount of distraction was 18.5mm, 28.5mm, 10.5mm, 14.5mm at right inferior orbital rim, left inferior orbital rim, right intraoral, left intraoral area respectively. The photography, cephalometry, and 3D CT(3 dimensional computed tomography) show that facial convexity was improved. We could correct midface deficiency successfully by LeFort III osteotomy and rigid external distraction.
Cephalometry ; Craniofacial Dysostosis* ; Female ; Humans ; Judgment ; Orbit ; Osteogenesis, Distraction ; Osteotomy ; Photography ; Skeleton ; Traction

Tetrahydrobiopterin (BH4) deficiency is caused by mutations in genes encoding enzymes involved in the synthesis and regeneration of BH4. The condition is usually accompanied by hyperphenylalaninemia (HPA) and deficiency of neurotransmitter precursors L-dopa and 5-hydroxytryptophan. BH4 deficiency is much rarer than classical phenylketonuria. Dihydropteridine reductase (DHPR) deficiency, an autosomal recessive genetic disorder, is a cause of malignant hyperphenylalaninemia due to BH4 deficiency. When left untreated, DHPR deficiency leads to neurologic deterioration at the age of 4 or 5 months, including psychomotor retardation, tonicity disorders, drowsiness, irritability, abnormal movements, hyperthermia, hypersalivation, and difficulty swallowing. Treatment of DHPR deficiency should be initiated as early as possible with BH4 supplementation and replacement of the neurotransmitter precursors L-dopa and 5-hydroxytryptophan. We report the first case of DHPR deficiency in Korea, a child diagnosed at 9 years of age by genetic testing.
5-Hydroxytryptophan ; Biopterin ; Child ; Deglutition ; Dihydropteridine Reductase ; Dyskinesias ; Fever ; Genetic Testing ; Humans ; Korea ; Levodopa ; Neurotransmitter Agents ; Phenylketonurias ; Regeneration ; Sialorrhea ; Sleep Stages

Childhood intestinal tuberculosis is difficult to diagnose for its protean clinical manifestations, especially in cases without pulmonary involvement. Differential diagnosis with Crohn's disease, inflammatory bowel disease and other malignancy is also important. Surgery has often been required for pathologic confirmation or therapy. Colonoscopy may be performed safely under consciousness sedation in children for bacteriologic and histopathologic confirmation of the biopsy specimen in addition to gross appearance of the lesion. We have experienced a case of intestinal tuberculosis presenting with chronic abdominal pain, diarrhea, weight loss and anemia in a 9 year old girl who was diagnosed by a colonoscopic examination and culture of the biopsy specimen from the ascending colon. The patient was managed with antituberculous drugs and recovered uneventfully.
Abdominal Pain ; Anemia ; Biopsy ; Child ; Colon, Ascending ; Colonoscopy* ; Consciousness ; Crohn Disease ; Diagnosis, Differential ; Diarrhea ; Female ; Humans ; Inflammatory Bowel Diseases ; Tuberculosis* ; Weight Loss

Campomelic syndrome is a very rare skeletal dysplasia with a characteristic pattern of deformity involving the proximal and distal extremities, pelvic and shoulder girdles, thoracic cage and palate. Respiratory compromise often leads to death in early infancy. Etiology has not been determined although evidence suggests genetic heterogeneity. Cytogenetic study revealed high incidence of a 46,XY karyotype in phenotypic females. Recently, we had experienced a case of campomelic dysplasia at amenorrhea 30weeks and termination was done, so we report with a brief review of literature.
Amenorrhea ; Campomelic Dysplasia* ; Congenital Abnormalities ; Cytogenetics ; Extremities ; Female ; Genetic Heterogeneity ; Humans ; Incidence ; Karyotype ; Palate ; Shoulder