We describe a case of cutaneous angiomyolipoma found in the ear lobe, that is not associated with tuberous sclerosis. The lesion developed on the youngest patient yet reported in the literature.
Angiomyolipoma* ; Ear ; Humans ; Tuberous Sclerosis

Some sexual differentiation disorders are associated with gonadal neoplasia and increased incidence of testicular tumors has been discribed in the patients with, XY gonadal dysgenesis. The incidence of testicular tumors in infants and children are rare, representing only 1% of all pediatric solid tumors. In general, gonadal stromal tumors are one of the most characteristic endocrine tumors of the testis, endocrine activity occurs in at least 10-20%, among them Leydig cell tumors and Sertoli cell tumors are clinically important. Although the exact pathogenesis is unknown, endocrine activity due to estrogen secretion can be manifested clinically with gynecomastia or precocious puberty. We experienced and reported a child who visited for sexual precocity and had XY gonadal dysgenesis with Sertoli cell tumor.
Child ; Disorders of Sex Development ; Estrogens ; Gonadal Dysgenesis ; Gonads ; Gynecomastia ; Humans ; Incidence ; Infant ; Leydig Cell Tumor ; Male ; Puberty, Precocious ; Sertoli Cell Tumor* ; Testicular Neoplasms ; Testis

BACKGROUND: Lichen planus is a papulosquarnous disease characterized by pruritic violaceous papules most commonly an the extremities of middle-aged adults. It may or may not be accompanied by oral and genital mucous membrane involvement. Its course is generally self-limited for a period of several months to years, but it may last indefinitely. OBJECTIVE: This study was performed to find the clinical characteristics of lichen planus. METHODS: Thirty-five cases of lichen planus comfirrned clinically and histopathologically were analyzed. RESULTS: The incidenc of lichen planus was 0.073% of the total number of dermatologic patients. The age distribution was in the range of 14-63 years old being most prevalent in the 6th decade and there was ariequal sex ratio. The most common site of involvement was the mucous membrane, and the extremities were more common than the trunk. Pruitus or pain and discomfort developed in 28 pat,ients(80% ). In most cases, the treatment was topical corticosteroid and antihistamine. Sixteen cses improved within 1 month to 2 years. Five cases recurred in the improved cases. CONCLUSION: The clinical analysis of 35 patients with lichen planus was performed. The incidence of lichen planus was 0.073% of the total number of dermatologic patients. Males and females were equally affected Mucous membrane involvement was detected in 21 patients(60%).
Adult ; Age Distribution ; Extremities ; Female ; Humans ; Incidence ; Lichen Planus* ; Lichens* ; Male ; Mucous Membrane ; Sex Ratio

The tibiofemoral angle is widely used for the diagnosis and treatment of knee problems, especially in degenerative joint disease of the knee. Among the various methods of measurement of tibiofemoral angle, Bauers method is being used most frequently. But measurement by Bauers method has seemed to show wide individual differences. Therefore we designed a ruler which can be used very easily for the measurement of the tibiofemoral angle with little differences between persons who measure the angle. To testify the advantages of our method, we selected 4 orthopedic surgeons and let them measure the tibiofemoral angles of 60 knees of 30 patients by Bauers method and by our method using the ruler. And 20 orthopedic surgeons measured tibiofemoval angles of 2 left knees by Bauers method and by our method using the ruler. The values of the tibiofemoral angle by two methods were compared and analyzed statistically. There was no significant differences between mean values. But, the tibiofemoral angles by our new method showed little differences among measurers. (P < 0.05)
Diagnosis ; Humans ; Individuality ; Joint Diseases ; Knee ; Linear Energy Transfer ; Methods ; Orthopedics ; Surgeons

Ureterosciatic hernia is extremely rare. In ureteral herniation, ureter prolapses occur through either the greater or lesser sciatic foramen. Atrophy of the piriformis muscle, hip joint diseases, and defects in the parietal pelvic fascia are predisposing factors for the development of ureterosciatic hernia. Most symptomatic patients have been treated surgically, with conservative treatment reserved only for asymptomatic patients. To the best of our knowledge, long-term follow-up outcomes after ureterosciatic hernia management are sparse. In this paper, we report the case of a 68-year-old woman who presented with colicky left abdominal pain. After computed tomography (CT) scan and anterograde pyelography, she was diagnosed ureterosciatic hernia with obstructive uropathy. We performed ureteral balloon dilatation and double-J ureteral stent placement. After this minimally invasive procedure, CT scan demonstrated that the left ureter had returned to its normal anatomical position without looping into the sciatic foramen. The patient remained asymptomatic with no adverse events 7 years after the minimally invasive procedures. This brief report describes ureterosciatic hernia successfully managed with minimally invasive procedures with long-term follow-up outcomes.

PURPOSE: The purpose of this study was to calculate the operating characteristics of narrowband imaging (NBI) cystoscopy versus traditional white light cystoscopy (WLC) in common clinical scenarios involving suspicion of bladder urothelial carcinoma (UC). MATERIALS AND METHODS: Sixty-three consecutive patients initially underwent WLC and then NBI in a single session for evaluation of microscopic hematuria (group I, n=20), gross hematuria (group II, n=19), and follow-up for prior UC (group III, n=24), by an experienced urologist. All lesions that were abnormal in contrast with adjacent normal mucosa were diagnosed as positive and biopsied. RESULTS: Sixty-six biopsies from 47 patients were performed. Pathologic examination showed 17 cases of UC from 21 sites. While the overall sensitivity of NBI was similar to that of WLC (100% vs. 94.1%), the specificity of NBI was significantly lower than that of WLC (50% vs. 86.9%, p < 0.001), particularly in group III (38.9% vs. 88.9%, p=0.004). Based on identification by NBI only, 23 additional biopsies from 18 cases were performed for identification of one patient with UC, who belonged to group III. In this group, to identify this specific patient, 15 additional biopsies were performed from 10 patients. All seven cases with positive findings from NBI within 2 months after the last intravesical therapy were histologically proven as negative. CONCLUSION: In evaluation for recurrence early after intravesical instillation, the decision based on NBI increased unnecessary biopsy in the absence of an established standard for judging NBI.
Administration, Intravesical* ; Biopsy* ; Cystoscopy* ; Follow-Up Studies ; Hematuria ; Humans ; Mucous Membrane ; Narrow Band Imaging* ; Recurrence ; Sensitivity and Specificity ; Urinary Bladder Neoplasms ; Urinary Bladder*

Neonatal Gaves disease is a relatively rare condition due to transplacental passage of Thyroid-stimulating antibody(TSAb) from a mother with active or inactive Graveses disease or autoimmune thyroiditis. A 11-day-old female newborn was referred to our department of pediatrics from a local clinic because of low level T4(3.55microg/dl) concurrent with high level TSH (501.74uIU/ml) on the 5th day neonatal metabolic screening. But, our repeated laboratory data showed very high serum T4(59.6microg/dl), T3(1,600ng/dl), suppressed TSH(0.43uIU/ml), and the presence of TSH receptor antibody. Her mother was treated with propylthiouracil(PTU) for Graves disease during pregnancy. Therefore, we thought it was a delayed-onset neonatal hyperthyroidism, because the fetal thyroid gland was initially suppressed by antithyroid drug taken during pregnancy. After initiating antithyroid drug therapy for the hyperthyroid nature, TSH levels became elevated again, while thyroid hormone levels decreased. Maternal and infant blood samples at the 23th day after birth were examined for serum autoantibodies directed towards the TSH receptor(Thyrotropin-binding inhibitory immunoglobulin:TBII, Thyroid-stimulating antibody:TSAb, Thyroid-stimulating blocking antibody:TSBAb) and high levels of TBII and TSAb were detected. About 2 months after birth, TBII and TSAb decreased within normal limit, and then we could stop antithyroid medication in safety. We report here a case of neonatal Graveses disease with very high level of T4 and T3, but firstly presented as hypothyroid nature on neonatal screening because of the maternally transferred antithyroid drug, PTU.
Autoantibodies ; Drug Therapy ; Female ; Graves Disease ; Humans ; Hyperthyroidism ; Infant ; Infant, Newborn ; Mass Screening ; Mothers ; Neonatal Screening ; Parturition ; Pediatrics ; Pregnancy ; Receptors, Thyrotropin ; Thyroid Gland ; Thyroiditis, Autoimmune

PURPOSE: The popular use of prenatal ultrasonography has lead to an increase in the diagnosis of hydronephrosis in fetuses. However, there is little data available, in Korea, on the natural progress of prenatal hydronephrosis. Therefore, to help in the management of patients, the follow-up data on fetuses, with antenatally diagnosed hydronephrosis, were analyzed. MATERIALS AND METHODS: Fifty-five (71 renal units) of 102 patients (118 renal units), with antenatally diagnosed hydronephrosis, were available for followed up from the 27th to the 36th fetal week. The follow-up postnatal ultrasonographs were retrospectively reviewed to try and determine the etiology, natural progress, prognosis and treatment of prenatal hydronephrosis. Diuretic 99mTc-DTPA renal and 99mTc-DMSA renal scans, intravenous pyelography and voiding cystourethrography were performed in the unimproved cases. RESULTS: The etiologies of prenatal hydronephrosis were ureteropelvic junction obstruction, VACTERL syndrome, vesicoureteral reflux, duplicated ureter, ureterovesical junction obstruction and polycystic kidney. During the follow-up period, 38 renal units, with prenatal hydronephrosis, improved spontaneously, but 18 units were aggravated. Operations were performed in 13 units. CONCLUSIONS: Many of the fetal hydronephrosis, diagnosed antenatally, disappeared postnatally. It is suggested that fetal, or neonatal, hydronephrosis does not necessarily imply the presence of obstructive uropathy or significant functional abnormality. The fetal antenatally diagnosed hydronephrosis, with clinical symptoms of pyuria, hematuria or a palpable abdominal mass, were often aggravated to a significant uropathy, which required a follow up evaluation and treatment.
Diagnosis ; Fetus ; Follow-Up Studies ; Hematuria ; Humans ; Hydronephrosis* ; Korea ; Polycystic Kidney Diseases ; Prenatal Diagnosis ; Prognosis ; Pyuria ; Retrospective Studies ; Technetium Tc 99m Dimercaptosuccinic Acid ; Ultrasonography ; Ultrasonography, Prenatal ; Ureter ; Urography ; Vesico-Ureteral Reflux

Ring chromosome is a structural abnormality that is thought to be the result of fusion and breakage in the short and long arms of chromosome. Wolf-Hirschhorn syndrome (WHS) is a well-known congenital anomaly in the ring chromosome 4 with a partial deletion of the distal short arm. Here we report a 10-month-old male of mosaic ring chromosome 4 with the chief complaint of severe short stature. He showed the height of -4 standard deviation, subtle hypothyroidism and mild atrial septal defect/ventricular septal defect, and also a mild language developmental delay was suspected. Brain magnetic resonance imaging showed multifocal leukomalacia. Chromosomal analysis of the peripheral blood showed the mosaic karyotype with [46,XY,r(4)(p16q35)[84]/45,XY,-4[9]/91,XXYY, dic r(4;4)(p16q35;p16q35)[5]/46,XY,dic r(4;4)(p16q35;p16q35)[2]]. FISH study showed the deletion of the 4p subtelomeric region with the intact 4q subtelomeric and WHS region. Both paternal and maternal karyotypes were normal. We compared the phenotypic variation with the previously reported cases of ring chromosome 4. The ring chromosome 4 with the subtelomeric deletion of short arm seems to be related with the phenotype of short stature.
*Chromosome Deletion ; *Chromosomes, Human, Pair 4 ; Growth Disorders/*diagnosis/genetics ; Humans ; In Situ Hybridization, Fluorescence ; Infant ; Karyotyping ; Male ; *Ring Chromosomes ; Telomere

Although syringoma is a relatively common adnexal tumor, syringoma of the hand has not been reported previously in Korea. We report a case of acral syringoma that appeared as grouped yellow-brownish papules limited to the dorsum of the right hand in a 40 year-old woman. The histopathologic findings were typical of syringoma and were characterized by the presence of numerous solid strands of basophilic epithelial cells and small cystic ducts embedded in a fibrous connective tissue stroma in the dermis. The classical "tadpole" or "comma-like tail" structures were also observed. Our case of acral syringoma presented a distinctive clinical picture, and this condition should be considered in the differential diagnosis of papular lesions of the hands.
Adult ; Basophils ; Connective Tissue ; Cystic Duct ; Dermis ; Diagnosis, Differential ; Epithelial Cells ; Female ; Hand* ; Humans ; Korea ; Syringoma*