We Reviewed 10 hypertensive children with pheochromocytoma retrospectively and the following results were obtained. 1) Out of 10 patients, 7 were male and 3 female. Age ranged from 5.5 years to 13.8 years and their median age was 9.9 years. 2) They complained of sweating, lethargy, headache. or chest pain and so on. Hypertension were noticed in all patients. Heart murmurs were detected in 7 patients and hypertensive retinopathy in 70%. 3) The three cases arised at extraadrenal gland and bilaterality was seen in 3 patients. In the view of diagnosis, abdominal sonography, computerized tomography and urine VMA test revealed the sensitivity of 100%. But MIBG scan showed 60% in sensitivity. 4) Waiting for operation, their hypertension were controlled by adrenergic blockers or calcium channel blockers. They received tumorectomy successfully except one who was in hypertensive state after operation and followed up through OPD. In conclusion high suspicion for the existence of pheochromocytoma from the clinical manifestations should be entertained in any pediatric patients and biochemical and imaging studies were mandatory. Furthermore, for the accurate localization of tumors, several imaging studies should be collaborated.
3-Iodobenzylguanidine ; Adrenergic Antagonists ; Calcium Channel Blockers ; Chest Pain ; Child* ; Diagnosis ; Female ; Headache ; Heart Murmurs ; Humans ; Hypertension ; Hypertensive Retinopathy ; Lethargy ; Male ; Pheochromocytoma* ; Retrospective Studies ; Sweat ; Sweating

A 70-year-old female who was diagnosed as myxoid chondrosarcoma by fine needle aspiration of a pleural mass is described. She presented with left chest discomfort of 4 months' duration and aggravating dyspnea and chest pain for 2 months. Chest X-ray and CT scan revealed a large lobulated low density mass invading chest wall at the left pleural cavity and massive pleural fluid. Fine needle aspiration was done under the impression of mesothelioma or metastatic cancer. The aspirates from the mass were very cellular and composed of isolated or clustered forms of large plump cells. Abundant cytoplasm was bluish opaque and the margin was rounded in the isolated cells, whereas clustered cells show ill-defined cell borders and aggregating tendency. The nuclei were eccentric, round to ovoid, and had fine chromatin pattern and multiple small nucleoli. Cellular pleomorphism or mitotic figure was not definite. These findings were consistent with cytologic features of chondrosarcoma. Final diagnosis was confirmed as myxoid chondrosarcoma by mediastinoscopic biopsy and the tumor showed strong positivity for S-100 protein.
Aged ; Biopsy ; Biopsy, Fine-Needle ; Chest Pain ; Chondrosarcoma ; Chromatin ; Cytoplasm ; Diagnosis ; Dyspnea ; Eosinophils* ; Female ; Humans ; Mesothelioma ; Peritoneal Dialysis, Continuous Ambulatory* ; Peritonitis* ; Pleural Cavity ; S100 Proteins ; Thoracic Wall ; Thorax ; Tomography, X-Ray Computed

PURPOSE: Steroid-resistant nephrotic syndrome in children is difficult to manage and tends to progress to chronic renal failure. We studied clinicopathological correlations in primary nephrotic syndrome in children resistant to 4-week daily steroid therapy. METHODS: Among children who had been admitted to Seoul National University Children's Hospital during the period between Oct. 1985 and Jul. 1995 and diagnosed as primary nephrotic syndrome, 87 patients were selected for this study. They showed poor response to 4-week daily steroid therapy either initially (initial nonresponder) or subsequently in the disease course (subsequent nonresponder). The medical records including renal pathologic findings were analyzed retrospectively. RESULTS: The mean age at the onset of nephrotic syndrome was 7.3+/-4.1 years and male to female ratio was 62:25. Pathologically, 28 (32%) had minimal change lesion (MCL), 47 (54%) had focal segmental glomerulosclerosis (FSGS) and 12 (14%) had others. There were 15 (54%) initial nonresponders and 13 (46%) subsequent nonresponders in the MCL group, and there were 26 (55%) and 21 (45%), respectively, in the FSGS group. The incidence of hematuria was less frequent in the MCL group. The frequencies of hypertension and azotemia were not significantly different between in the MCL and the FSGS group. Among 10 patients with MCL in whom the steroid therapy were extended to 6 weeks, 3 patients responded subsequently. And 1 of 3 patients among the FSGS group responded to 8-week daily steroid therapy. The 2nd line drug therapy such as oral cyclophosphamide, intravenous pulsed methylprednisolone, enalapril, dipyridamole, etc. was tried in 26 patients with MCL and all 47 patients with FSGS. In the MCL group, 7 of 13 initial nonresponders and 9 of 13 subsequent nonresponders responded to these 2nd line drug therapies. In the FSGS group, 10 of 26 initial nonresponders and 11 of 21 subsequent nonresponders responded to these therapies. While only 1 subsequent nonresponder in the MCL group progressed to chronic renal failure, 9 initial and 4 subsequent nonresponders progressed in the FSGS group. CONCLUSIONS: The FSGS group formed about a half and the MCL group formed about a third of steroid-resistant nephrotic syndrome in children. Although the response to 2nd line drug therapies was not different between 2 groups, the incidence of progression to chronic renal failure was significantly higher in the FSGS group.
Azotemia ; Child ; Cyclophosphamide ; Dipyridamole ; Drug Therapy ; Enalapril ; Female ; Glomerulosclerosis, Focal Segmental ; Hematuria ; Humans ; Hypertension ; Incidence ; Kidney Failure, Chronic ; Male ; Medical Records ; Methylprednisolone ; Nephrotic Syndrome* ; Retrospective Studies ; Seoul

Granular cell tumor is mostly benign and thought to be of Schwann cell origin. The head and neck, particularly tongue, breast, and upper respiratory tract are frequently involved. Recently, we have experienced a case of granular cell tumor of the right thigh in a 30-year old male, diagnosed by fine needle aspiration cytology which revealed distinct cytologic features. The smear revealed cellular aspirates with clear background. The tumor cells showed uniform small nuclei and abundant eosinophilic, granular cytoplasm with hazy cell border. Mitoses were not found.
Adult ; Biopsy, Fine-Needle ; Breast ; Child* ; Cytoplasm ; Eosinophils ; Granular Cell Tumor ; Head ; Humans ; Lupus Nephritis* ; Male ; Mitosis ; Neck ; Paraganglioma ; Respiratory System ; Statistics as Topic* ; Thigh ; Tongue

PURPOSE: This study was designed to aid the diagnosis and to predict the outcorne by understanding the clinical course of nutcracker syndrome in childhood. METHODS: The clinical, laboratory, radiological and cystoscopic data from the medical records of eleven children who were diagnosed as nutcracker syndrome by gross hematuria and pressure gradient criteria (>3mrnHg) were studied retrospectively and analyzed. RESULTS: Sex ratio of the cases was 7:4, and the median age of onset was 12.8 (3-14.3) years. Six cases showed persistent and 5 cases manifested interrnittent, exercise induced hematuria. Left flank pain (64%), abdominal pain (18%), left varicocele (9%) were associated in some of the children, but hematuria was the only symptom in 36Yo. Left renal vein entrapment was documented in 10 cases by ultrasonography. Out of the 5 cases studied by renal Doppler ultrasonography, 4 and 5 cases showed higher (>5) mean left renal vein diameter ratio (Distal/ Aortomesenteric portion) and mean peak velocity ratio respectively. Unilateral bleeding from left ureteral orifice was documented in 7 of the 9 cases at cystoscopy. The mean pressure gradient between proximal left renal vein and inferior vena cava was 4.4+/-1.6 (3-7) mmHg. Hematuria of 25% and 57% of the cases disappeared spontaneously in 3 and 5 years after onset respectively. Proteinuria disappear- ed in 3 of the 5 initial proteinuric cases. CONCLUSION: Nutcracker syndrome must be considered in the differential diagnosis of non-glomerular, especially gross hematuria in childhood, and Doppler ultrasonography can aid diagnosis non-invasively. The renal function remained stable, but 4396 of the cases continued to show hematuria still 5 years after onset.
Abdominal Pain ; Age of Onset ; Child ; Cystoscopy ; Diagnosis ; Diagnosis, Differential ; Flank Pain ; Hematuria* ; Hemorrhage ; Humans ; Medical Records ; Proteinuria ; Renal Veins ; Retrospective Studies ; Sex Ratio ; Ultrasonography ; Ultrasonography, Doppler ; Ureter ; Varicocele ; Vena Cava, Inferior

The causes of sudden death after medullary infarction involve arrhythmia, central respiratory failure, and dysautonomia. Sudden cardiac arrest in a medullary infarction is uncommon. Most of these cases experienced sudden cardiopulmonary arrest within 2 weeks from stroke onset as the extent of lesion increased. Here, we report two cases of medullary infarction presenting as sudden cardiac arrest. These cases indicate that medullary infarction could be one of the causes of sudden cardiac arrest.
Arrhythmias, Cardiac ; Death, Sudden ; Death, Sudden, Cardiac ; Heart Arrest ; Infarction ; Medulla Oblongata ; Primary Dysautonomias ; Respiratory Insufficiency ; Stroke

Mastocytosis is a rare disease which occurs in both children and adults, and it can manifest as a solitary or multiple skin lesions. Both can cause cutaneous or systemic symptoms. Because of the heterogeneity of clinical presentation of mastocytosis and its rare prevalence, it can be hard to suspect the mastocytosis at the first time. Most solitary mastocytomas are about 1–5 cm in diameter and have features of brownish-yellow, minimally elevated plaques with a smooth shiny surface. This article presents a case of solitary mastocytoma which occurred in neonate and that we treated through surgical excision. In histopathological examination, it consisted of c-kit-positive mast cells. Although pediatric cutaneous mastocytosis might regress spontaneously, clinicians should keep in mind that it could be associated with systemic mastocytosis which involves hematopoietic system.
Adult ; Child ; Hematopoietic System ; Humans ; Infant, Newborn ; Mast Cells ; Mastocytoma* ; Mastocytosis ; Mastocytosis, Cutaneous ; Mastocytosis, Systemic ; Parturition* ; Population Characteristics ; Prevalence ; Rare Diseases ; Skin

We report three cases of cystinuria, presenting with urinary stones. A 2-year-old girl presented with urinary difficulty, hematuria, dysuria of sudden onset, and her 7-month-old younger brother also was presented with urinary difficulty, irritability on urination & stone passage. Other 6-month-old boy was admitted due to sudden onset anuria. They had radioopague renal & ureter stones and stone analysis revealed mixed cystine stones. The diagnosis of cystinuria was confirmed metabolic studies and stone analysis. Lrinary amino acid analysis showed excessive excretion of dibasic amino acids(cystine, ornithine, lysine, arginine). And they all had hypercalciuria and hyperuricosuria. They were treated with combination of percutaneous lithotripsy for large obstructing senes a nd an oral drug therapy with sodium bicarbonate for rendering the urine more alkaline, and alpha-mercaptopropionylglycine(ThiolaR). This form of treatment was sucessful in our three cases with elimination of recurrent nephrolithiasis, but in one patient, nephrotic syndrome possibly caused by ThiolaR was developed. The nephrotic syndrome was recovered spontaneously after cessation of Thiola. A review of literatures was also attempted briefly.
Anuria ; Child, Preschool ; Cystine ; Cystinuria* ; Diagnosis ; Drug Therapy ; Dysuria ; Female ; Hematuria ; Humans ; Hypercalciuria ; Infant ; Lithotripsy ; Lysine ; Male ; Nephrolithiasis ; Nephrotic Syndrome ; Ornithine ; Siblings ; Sodium Bicarbonate ; Tiopronin ; Ureter ; Urinary Calculi ; Urination

Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus (SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly improved through the use of plasmapheresis. Although various factors can precipitate pulmonary hemorrhage in SLE, our case was probably caused by an immune mediated mechanism since the hemorrhage responded promptly to plasmapheresis. This case illustrates the importance of plasmapheresis in the treatment of pulmonary hemorrhage which is not improved by methylprednisolone and cyclophosphamide. We report this case with a brief review of the related literatures.
Adolescent ; Child* ; Cyclophosphamide ; Hemorrhage* ; Humans ; Lupus Erythematosus, Systemic* ; Male ; Methylprednisolone ; Plasmapheresis*

Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus (SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly improved through the use of plasmapheresis. Although various factors can precipitate pulmonary hemorrhage in SLE, our case was probably caused by an immune mediated mechanism since the hemorrhage responded promptly to plasmapheresis. This case illustrates the importance of plasmapheresis in the treatment of pulmonary hemorrhage which is not improved by methylprednisolone and cyclophosphamide. We report this case with a brief review of the related literatures.
Adolescent ; Child* ; Cyclophosphamide ; Hemorrhage* ; Humans ; Lupus Erythematosus, Systemic* ; Male ; Methylprednisolone ; Plasmapheresis*