Diagnosis and Treatment > Investigation > Renin plasma activity
You are looking at 1 - 6 of 6 items
Search for other papers by Takuya Higashitani in
Google Scholar
PubMed
Search for other papers by Shigehiro Karashima in
Google Scholar
PubMed
Search for other papers by Daisuke Aono in
Google Scholar
PubMed
Department of Internal Medicine, Keiju Medical Center, Nanao, Ishikawa, Japan
Search for other papers by Seigoh Konishi in
Google Scholar
PubMed
Search for other papers by Mitsuhiro Kometani in
Google Scholar
PubMed
Search for other papers by Rie Oka in
Google Scholar
PubMed
Search for other papers by Masashi Demura in
Google Scholar
PubMed
Search for other papers by Kenji Furukawa in
Google Scholar
PubMed
Search for other papers by Yuto Yamazaki in
Google Scholar
PubMed
Search for other papers by Hironobu Sasano in
Google Scholar
PubMed
Department of Health Promotion and Medicine of the Future, Kanazawa University, Kanazawa, Ishikawa, Japan
Search for other papers by Takashi Yoneda in
Google Scholar
PubMed
Search for other papers by Yoshiyu Takeda in
Google Scholar
PubMed
Summary
Renovascular hypertension (RVHT) is an important and potentially treatable form of resistant hypertension. Hypercortisolemia could also cause hypertension and diabetes mellitus. We experienced a case wherein adrenalectomy markedly improved blood pressure and plasma glucose levels in a patient with RVHT and low-level autonomous cortisol secretion. A 62-year-old Japanese man had been treated for hypertension and diabetes mellitus for 10 years. He was hospitalized because of a disturbance in consciousness. His blood pressure (BP) was 236/118 mmHg, pulse rate was 132 beats/min, and plasma glucose level was 712 mg/dL. Abdominal CT scanning revealed the presence of bilateral adrenal masses and left atrophic kidney. Abdominal magnetic resonance angiography demonstrated marked stenosis of the left main renal artery. The patient was subsequently diagnosed with atherosclerotic RVHT with left renal artery stenosis. His left adrenal lobular mass was over 40 mm and it was clinically suspected the potential for cortisol overproduction. Therefore, laparoscopic left nephrectomy and adrenalectomy were simultaneously performed, resulting in improved BP and glucose levels. Pathological studies revealed the presence of multiple cortisol-producing adrenal nodules and aldosterone-producing cell clusters in the adjacent left adrenal cortex. In the present case, the activated renin-angiotensin-aldosterone system and cortisol overproduction resulted in severe hypertension, which was managed with simultaneous unilateral nephrectomy and adrenalectomy.
Learning points:
-
Concomitant activation of the renin-angiotensin-aldosterone system and cortisol overproduction may contribute to the development of severe hypertension and lead to lethal cardiovascular complications.
-
Treatment with simultaneous unilateral nephrectomy and adrenalectomy markedly improves BP and blood glucose levels.
-
CYP11B2 immunohistochemistry staining revealed the existence of aldosterone-producing cell clusters (APCCs) in the adjacent non-nodular adrenal gland, suggesting that APCCs may contribute to aldosterone overproduction in patients with RVHT.
Search for other papers by Skand Shekhar in
Google Scholar
PubMed
Search for other papers by Rasha Haykal in
Google Scholar
PubMed
Search for other papers by Crystal Kamilaris in
Google Scholar
PubMed
Search for other papers by Constantine A Stratakis in
Google Scholar
PubMed
Search for other papers by Fady Hannah-Shmouni in
Google Scholar
PubMed
Summary
A 29-year-old primigravida woman with a known history of primary aldosteronism due to a right aldosteronoma presented with uncontrolled hypertension at 5 weeks of estimated gestation of a spontaneous pregnancy. Her hypertension was inadequately controlled with pharmacotherapy which lead to the consideration of surgical management for her primary aldosteronism. She underwent curative right unilateral adrenalectomy at 19 weeks of estimated gestational age. The procedure was uncomplicated, and her blood pressure normalized post-operatively. She did, however, have a preterm delivery by cesarean section due to intrauterine growth retardation with good neonatal outcome. She is normotensive to date.
Learning points:
-
Primary aldosteronism is the most common etiology of secondary hypertension with an estimated prevalence of 5–10% in the hypertensive population.
-
It is important to recognize the subtypes of primary aldosteronism given that certain forms can be treated surgically.
-
Hypertension in pregnancy is associated with significantly higher maternal and fetal complications.
-
Data regarding the treatment of primary aldosteronism in pregnancy are limited.
-
Adrenalectomy can be considered during the second trimester of pregnancy if medical therapy fails to adequately control hypertension from primary aldosteronism.
Search for other papers by Yasufumi Seki in
Google Scholar
PubMed
Search for other papers by Satoshi Morimoto in
Google Scholar
PubMed
Search for other papers by Naohiro Yoshida in
Google Scholar
PubMed
Search for other papers by Kanako Bokuda in
Google Scholar
PubMed
Search for other papers by Nobukazu Sasaki in
Google Scholar
PubMed
Search for other papers by Midori Yatabe in
Google Scholar
PubMed
Search for other papers by Junichi Yatabe in
Google Scholar
PubMed
Search for other papers by Daisuke Watanabe in
Google Scholar
PubMed
Search for other papers by Satoru Morita in
Google Scholar
PubMed
Search for other papers by Keisuke Hata in
Google Scholar
PubMed
Search for other papers by Tomoko Yamamoto in
Google Scholar
PubMed
Search for other papers by Yoji Nagashima in
Google Scholar
PubMed
Search for other papers by Atsuhiro Ichihara in
Google Scholar
PubMed
Summary
Primary aldosteronism (PA) is more common than expected. Aberrant adrenal expression of luteinizing hormone (LH) receptor in patients with PA has been reported; however, its physiological role on the development of PA is still unknown. Herein, we report two unique cases of PA in patients with untreated Klinefelter’s syndrome, characterized as increased serum LH, suggesting a possible contribution of the syndrome to PA development. Case 1 was a 39-year-old man with obesity and hypertension since his 20s. His plasma aldosterone concentration (PAC) and renin activity (PRA) were 220 pg/mL and 0.4 ng/mL/h, respectively. He was diagnosed as having bilateral PA by confirmatory tests and adrenal venous sampling (AVS). Klinefelter’s syndrome was suspected as he showed gynecomastia and small testes, and it was confirmed on the basis of a low serum total testosterone level (57.3 ng/dL), high serum LH level (50.9 mIU/mL), and chromosome analysis. Case 2 was a 28-year-old man who had untreated Klinefelter’s syndrome diagnosed in his childhood and a 2-year history of hypertension and hypokalemia. PAC and PRA were 247 pg/mL and 0.3 ng/mL/h, respectively. He was diagnosed as having a 10 mm-sized aldosterone-producing adenoma (APA) by AVS. In the APA, immunohistochemical analysis showed co-expression of LH receptor and CYP11B2. Our cases of untreated Klinefelter’s syndrome complicated with PA suggest that increased serum LH levels and adipose tissues, caused by primary hypogonadism, could contribute to PA development. The possible complication of PA in hypertensive patients with Klinefelter’s syndrome should be carefully considered.
Learning points:
-
The pathogenesis of primary aldosteronism is still unclear.
-
Expression of luteinizing hormone receptor has been reported in aldosterone-producing adenoma.
-
Serum luteinizing hormone, which is increased in patients with Klinefelter’s syndrome, might contribute to the development of primary aldosteronism.
Memorial University Medical Center, Savannah, Georgia, USA
Search for other papers by Himangshu S Bose in
Google Scholar
PubMed
Pediatric Endocrinology and Diabetes Center, Kalispell Regional Medical Center, Kalispell, Montana, USA
Search for other papers by Alan M Rice in
Google Scholar
PubMed
Search for other papers by Brendan Marshall in
Google Scholar
PubMed
Laboratory of Pathology, Memorial University Medical Center, Savannah, Georgia, USA
Search for other papers by Fadi Gebrail in
Google Scholar
PubMed
Search for other papers by David Kupshik in
Google Scholar
PubMed
Search for other papers by Elizabeth W Perry in
Google Scholar
PubMed
Summary
Steroid hormones are essential for the survival of all mammals. In adrenal glands and gonads, cytochrome P450 side chain cleavage enzyme (SCC or CYP11A1), catalyzes conversion of cholesterol to pregnenolone. We studied a patient with ambiguous genitalia by the absence of Müllerian ducts and the presence of an incompletely formed vagina, who had extremely high adrenocorticotropic hormone (ACTH) and reduced pregnenolone levels with enlarged adrenal glands. The testes revealed seminiferous tubules, stroma, rete testis with interstitial fibrosis and reduced number of germ cells. Electron microscopy showed that the patient’s testicular mitochondrial size was small with little SCC expression within the mitochondria. The mitochondria were not close to the mitochondria-associated ER membrane (MAM), and cells were filled with the microfilaments. Our result revealed that absence of pregnenolone is associated with organelle stress, leading to altered protein organization that likely created steric hindrance in testicular cells.
Learning points:
-
Testes revealed seminiferous tubules, stroma, rete testis with interstitial fibrosis and reduced number of germ cells;
-
Testicular mitochondrial size was small with little SCC expression within the mitochondria;
-
Absence of pregnenolone is associated with organelle stress.
Search for other papers by Mads Ryø Jochumsen in
Google Scholar
PubMed
Search for other papers by Peter Iversen in
Google Scholar
PubMed
Search for other papers by Anne Kirstine Arveschoug in
Google Scholar
PubMed
Summary
A case of follicular thyroid cancer with intense focal Methionine uptake on 11C-Methionine PET/CT is reported here. The use of 11C-Methionine PET in differentiated thyroid cancer is currently being investigated as a surrogate tracer compared to the more widely used 18F-FDG PET. This case illustrates the potential incremental value of this modality, not only in the localizing of parathyroid adenoma, but also indicating that 11C-Methionine PET might have a potential of increasing the pretest likelihood of thyroid malignancy in a cold nodule with highly increased Sestamibi uptake.
Learning points:
-
11C-Methionine PET/CT and 18F-Fluorocholine PET/CT often visualizes the parathyroid adenoma in case of negative Tc-99m-MIBI SPECT/CT.
-
A cold nodule in Tc-99m Pertechnetat thyroid scintigraphy with a negative Sestamibi scintigraphy has a very low probability of being malignant.
-
However, the pretest likelihood of thyroid cancer in a cold nodule with increased Sestamibi uptake is low.
-
11C-Methionine PET might have a potential incremental value in increasing the pretest likelihood of thyroid malignancy in a cold nodule with highly increased Sestamibi uptake.
Search for other papers by Sudeep K Rajpoot in
Google Scholar
PubMed
Search for other papers by Carlos Maggi in
Google Scholar
PubMed
Search for other papers by Amrit Bhangoo in
Google Scholar
PubMed
Summary
Neonatal hyperkalemia and hyponatremia are medical conditions that require an emergent diagnosis and treatment to avoid morbidity and mortality. Here, we describe the case of a 10-day-old female baby presenting with life-threatening hyperkalemia, hyponatremia, and metabolic acidosis diagnosed as autosomal dominant pseudohypoaldosteronism type 1 (PHA1). This report aims to recognize that PHA1 may present with a life-threatening arrhythmia due to severe hyperkalemia and describes the management of such cases in neonates.
Learning points
-
PHA1 may present with a life-threatening arrhythmia.
-
Presentation of PHA can be confused with congenital adrenal hyperplasia.
-
Timing and appropriate medical management in the critical care unit prevented fatality from severe neonatal PHA.
