Humans ; Female ; Hypertension/complications* ; Kidney Neoplasms/pathology* ; Juxtaglomerular Apparatus/pathology*

Humans ; Female ; Hypertension/complications* ; Kidney Neoplasms/pathology* ; Juxtaglomerular Apparatus/pathology*

Bartter's syndrome is a rare tubular disorder characterized by hypokalemic, hypochloremic metabolic alkalosis, hyperreninemic hyperaldosteronism, hyporesponsiveness to pressor agents, and juxtaglomerular apparatus hyperplasia. We report here a case of Bartter's syndrome in a 5 month-old male infant who improved with potassium supplements. In addition to a case report, brief review of related literatures was done.
Alkalosis ; Bartter Syndrome* ; Humans ; Hyperaldosteronism ; Hyperplasia ; Infant ; Juxtaglomerular Apparatus ; Male ; Potassium

Bartter's syndrome is a rare tubular disorder characterized by hypokalemic, hypochloremic metabolic alkalosis, hyperreninemic, hyperaldosteronism, hyporesponsiveness to pressor agents and juxtaglomerular apparatus heperplasia. We report a case of Bartter's syndrome of a 5 month-old male infant with subdural hematoma who was confirmed by characteristic clinical, laboratory findings and kidney biopsy.
Alkalosis ; Bartter Syndrome* ; Biopsy ; Epilepsy* ; Hematoma, Subdural* ; Humans ; Hyperaldosteronism ; Infant ; Juxtaglomerular Apparatus ; Kidney ; Male ; Seizures*

Bartter syndrome is a rare disorder characterized by the association of hypokalemic hypochloremic metabolic alkalosis, hyperreninemia, hyperaldosteronemia, short stature and nephrocalcinosis. This disorder presents with hyperplasia of juxtaglomerular apparatus on renal biopsy. We experienced a case of late-onset Bartter syndrome with nephrocalcinosis in a 9-year-old boy, whose chief pictures were muscle weakness, short stature, persistent sterile pyuria and microscopic hematuria. We report this case with a brief review of related literatures.
Alkalosis ; Bartter Syndrome* ; Biopsy ; Child ; Hematuria ; Humans ; Hyperplasia ; Juxtaglomerular Apparatus ; Male ; Muscle Weakness* ; Nephrocalcinosis ; Pyuria

Juxtaglomerular cell tumor is a rare cause of secondary hypertension. Focal segmental glomerulosclerosis (FSGS) is a clinicopathological entity associated with renal insufficiency and proteinuria. The exact diagnosis and proper management are important in both juxtaglomerular cell tumor and FSGS. We experienced a 26-year-old male who complained of a palpable abdomen mass associated with proteinuria and hypertension. Ultimately, he was diagnosed with a juxtaglomerular cell tumor combined with FSGS after nephrectomy. After operation, his hypertension normalized, while his renal function deteriorated.
Abdomen ; Adult ; Glomerulosclerosis, Focal Segmental ; Humans ; Hypertension ; Juxtaglomerular Apparatus ; Male ; Nephrectomy ; Proteinuria ; Renal Insufficiency

To elucidate the argument on the hypertensive effect of repeated stress, morphological study on the juxtaglomerular apparatus of pain-stressed mice was carried out. Following the repeated stress on mice by subcutaneous injections of formalin every day, animals were sacrificed on 1, 3, 6, 12 hours and 1, 2, 3, 6 days after the 5th injection. Kidneys fixed in 10%, Zenker-formol solution were routinely processed, and 4um sections were stained by Bowie's stain. For the precise interpretation, following protocols were applied; Comparing the amount of secretory granules, juxtaglomerular granulation index (JGI) was weighted on every 1000 glomeruli according to their granulaities. Comparing the proportional appearance of juxtaglomerular apparatus, granulation cell index (GCI) was counted as a mean number of granular juxtaglomerular apparatus among every 100 glomeruli. Comparing the cellularities of a single juxtaglomerular apparatus, juxtaglomerular granular cell count (JGCC) was counted as a mean number of granular juxtaglomerular cells appearing in a longitudinally. sectioned afferent arteriole. Comparing the functional relationship between the juxtaglomerular cells and macula densa cells, macula densa cell count (MD count) was counted as the mean number of macular densa cells having contacts with granular juxtaglomerular cells. Result of this experiment was processed and interpreted as followings. 1. Repeated pain-stress give rise to the prominant effect to juxtaglomerular cells, as high as double granular amount as compared to normal one, during 3-6 hours after the last stress. 2. Functional relationship between the distal convoluted tubule and Juxtaglomerular cells, checked out by MD contacts, was also highest during 3 to 6 hours term. 3. The effects were decreased afterwards, but still most counts were higher than those of normal data until 6th day, the last term of this experiment. 4. Present study show that the repeated pain-stress initiate the significant pressure-rising effect to animals during the first day after the last stress, and these effects may be latent during some days afterwards. 5. As the precise mechanism involved in this aspect remains to be elucidated, further studies includingelectron microscopic and histochemical methods shall be suggested.
Animals ; Arterioles ; Cell Count ; Formaldehyde ; Injections, Subcutaneous ; Juxtaglomerular Apparatus ; Kidney ; Mice* ; Secretory Vesicles

We report a case of renin-secreting juxtaglomerular cell tumor which developed in a hypertensive 47-yr-old Korean man. Presumptive clinical diagnosis was made before surgery based on the high level of plasma renin and the radiologic evidence of renal mass. Grossly, a round, bulging, well-encapsulated mass of 3x3 cm was located in the mid-portion of the right kidney. On microscopic examination, the tumor was composed of ovoid to polyhedral cells with bland nuclei, indistinct nucleoli and light eosinophilic cytoplasm. The immunostaining for renin showed strong positivity in the cytoplasm of tumor cells. The characteristic rhomboid shaped renin protogranules were observed in ultrastructural analysis.
Human ; Hypertension, Renal/*etiology/pathology ; Juxtaglomerular Apparatus/*pathology ; Kidney Neoplasms/*complications/*pathology/secretion ; Male ; Middle Age ; Renin/blood/secretion

The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma includes hypokalemia, metabolic alkalosis, and features secondary to the increased activation of the renin-angiotensin system without renal artery stenosis. We report a case of reninoma in a female adolescent with a typical clinical presentation, in which surgical removal of the tumor completely cured hypertension. We discuss here the clinical features, imaging studies, and immunohistochemical examination of the tumor used to establish the diagnosis of reninoma and for the management of the condition.
Adolescent ; Alkalosis ; Child ; Diagnosis ; Humans ; Hypertension ; Hypertension, Renal ; Hypokalemia ; Juxtaglomerular Apparatus ; Renal Artery Obstruction ; Renin ; Renin-Angiotensin System ; Young Adult

OBJECTIVETo study the morphologic characteristics and immunophenotype of juxtaglomerular cell tumor of the kidney (JGCT), with discussion on its diagnostic clues and possible histogenesis.

METHODSThe clinical, pathologic and immunohistochemical features of 5 cases of JGCT were evaluated. In addition, 5 cases of hemangiopericytoma and 5 cases of cutaneous glomus tumor were selected for comparative immunohistochemical analysis.

RESULTSThe JGCT cases came from 4 females and 1 male (mean age at diagnosis = 32 years). All of them manifested symptoms of systemic hypertension. Four of the patients received partial nephrectomy and the remaining patient was treated by radial nephrectomy. All of them were followed up for a period of 4 to 66 months (average = 27 months). There was no evidence of local recurrence or distant metastases. On gross examination, these JGCTs were well-circumscribed and situated in the renal cortex and measured 4.4 cm in greatest dimension on average. Histologically, the tumor was characterized by the following three features: (1) solid sheets of relatively uniform polygonal to round cells with lightly eosinophilic cytoplasm, sometimes containing PAS-positive intracytoplasmic granules; (2) absence of or very scanty mitotic figures; (3) interstitium rich in thin-walled capillaries, associated with focal hyaline change and hemangiopericytoma-like architectural pattern. Under electron microscopy, characteristic rhomboid-shaped renin granules were found in the cytoplasm. All JGCTs were immunoreactive for renin, CD34, actin, and calponin. In contrast, all glomus tumors were negative for renin and all hemangiopericytomas were negative for actin.

CONCLUSIONSJGCT is a rare benign renal neoplasm typically found in young adults and manifests as systemic hypertension. The tumor cells may be originated from modified vascular smooth muscle cells. The identification of renin granules by electron microscopy and demonstration of the characteristic immunophenotype is the key to correct pathologic diagnosis.

Adult ; Antigens, CD34 ; analysis ; Calcium-Binding Proteins ; analysis ; Female ; Humans ; Immunohistochemistry ; Juxtaglomerular Apparatus ; chemistry ; pathology ; ultrastructure ; Keratins ; analysis ; Kidney Neoplasms ; pathology ; Male ; Microfilament Proteins ; Microscopy, Electron ; Middle Aged