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

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

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

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

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*

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

Bartter's syndrome is characterized by hyperreninemia, hyperaldosteronism, hypokalemic hypochlorenlic alkalosis, normal blood pressure, juxtaglomerular apparatus hyperplasia, general weakness, and muscle weakness. We experienced a case of hypokalemic myopathy associated with Bartter's syndrome in 15 years old male. He had experienced paroxysmal muscle weakness without sensory change and myalgia since 10 years old. Subsequently, he had complaints of progressive muscle weakness, especially proximal muscles. Prominent juxtaglomerular apparatus with cellular proliferation biopsy was seen in the kidney. And there were mild perivascular inflammatory cell infiltration, small degenerating and/or regenerating muscles fibers, and normal muscle fiber distribution without evidence of chronic myopathy in the muscle biopsy. The patient was sucessfully managed with indomethacin and oral potassium chloride.
Adolescent ; Alkalosis ; Bartter Syndrome* ; Biopsy ; Blood Pressure ; Cell Proliferation ; Child ; Humans ; Hyperaldosteronism ; Hyperplasia ; Indomethacin ; Juxtaglomerular Apparatus ; Kidney ; Male ; Muscle Weakness ; Muscles ; Muscular Diseases* ; Myalgia ; Potassium Chloride