Primary aldosteronism, is defined as a group of disorders characterized by the excess of aldosteron, with suppressed rennin activity, resulting in hypertension and hypokalemia. In most cases, primary aldosteronism is sporadic due to a unilateral adrenal adenoma or bilateral adrenal hyperplasia. Familial hyperaldosteronism is a rare cause of primary aldosteronism and its prevalence has not been established well. We describe two cases of primary aldosteronism in a family involving a sister and brother due to an aldosterone producing adenoma in the left adrenal gland. Their hypokalemia and hypertension were cured by complete resection of the adrenal adenoma. Genetic analyses could not be done because of patients' rejection.
Adenoma ; Adrenal Glands ; Adrenocortical Adenoma ; Aldosterone ; Chymosin ; Humans ; Hyperaldosteronism ; Hyperplasia ; Hypertension ; Hypokalemia ; Prevalence ; Rejection (Psychology) ; Siblings

To express bovine chymosin in yeast, we amplified the prochymosin gene from the plasmid pMD18T-Prochy by PCR, and then cloned the gene into the expression vector pPICZaA, resulting in pPICZaA-Prochy. Pichia pastoris GS115 was used as host cells. Integration of the prochymosin cDNA into the Pichia pastoris genome was confirmed by PCR and sequencing analysis. Chymosin was expressed in Pichia pastoris successfully, and a strong band at about 37 kD was shown by SDS-PAGE. Activity tests showed that the chymosin activity of the culture supernatant was 12.2 SU/mL. This is the first report of successful expression of chymosin in Pichia pastoris. The recombinant Pichia pastoris strain obtained in this study could be further used to produce recombinant chymosin for cheese making.
Animals ; Cattle ; Chymosin ; biosynthesis ; genetics ; Cloning, Molecular ; Enzyme Precursors ; genetics ; Genetic Vectors ; genetics ; Pichia ; genetics ; metabolism ; Recombinant Proteins ; biosynthesis ; genetics

Female phenotype of a 46,XY male may originates from male pseudohermaphroditism due to 17alpha-hydroxylase deficiency. Lack of cortisol increases adrenocorticotropic hormone (ACTH) and mineralocorticoid production, leading to low renin hypertention and hypokalemia. A 41-year-old phenotypic female presented primary amenorrhea and hypertension. In the hormonal profile, the levels of serum estradiol, testosterone, rennin, and cortisol were decreased and ACTH and deoxycorticosterone were increased. Laparoscopic bilateral gonadectomy was performed, and corticosteroid, antihypertensive drugs, and estrogen were administered. We report this case with a brief review of the literatures.
46, XY Disorders of Sex Development* ; Adrenal Hyperplasia, Congenital ; Adrenocorticotropic Hormone ; Adult ; Amenorrhea ; Antihypertensive Agents ; Chymosin ; Desoxycorticosterone ; Estradiol ; Estrogens ; Female ; Humans ; Hydrocortisone ; Hypertension ; Hypokalemia ; Male* ; Phenotype ; Renin ; Testosterone

Prolonged ingestion of licorice can cause hypermineralocorticoidism, with sodium retention, potassium loss and hypertension. Nevertheless, its initial presentation with a very severe degree of hypokalemic paralysis and rhabdomyolysis are exceedingly rare. We describe a patient who experienced hypokalemic paralysis and rhabdomyolysis after licorice ingestion. The patient's initial blood pressure was 160/80mmHg. The major biochemical abnormalities included; hypokalemia(K+ 1.3mEq/L), metabolic alkalosis, with a pH of 7.64, and urine myoglobin > 3000ng/mL. The plasma rennin activity and aldosterone level were suppressed. The 24 hour urine cortisol concentration was normal. The patients, over a 1 month period, had ingested 500g of licorice boiled in water. After quitting the licorice, the hypokalemia and muscle paralysis gradually improved and blood pressure returned to normal
Aldosterone ; Alkalosis ; Blood Pressure ; Chymosin ; Eating ; Glycyrrhiza* ; Glycyrrhizic Acid ; Humans ; Hydrocortisone ; Hydrogen-Ion Concentration ; Hypertension ; Hypokalemia ; Myoglobin ; Paralysis* ; Plasma ; Potassium ; Rhabdomyolysis* ; Sodium ; Water

The renal manifestations of systemic sclerosis include proteinuria, hypertension, azotemia, and renal crisis. Two types of scleroderma renal crisis (SRC) are recognized. Typical SRC is a syndrome consisting of acute-onset malignant hypertension accompanied by rapidly progressive renal failure, hypertensive retinopathy, and elevated plasma renin activity. The other type is normotensive renal failure, which is generally accompanied by antineutrophil cytoplasmic autoantibody (ANCA)-positive crescentic glomerulonephritis. A 51-year-old woman with scleroderma without marked dermatological change developed ANCA-related renal failure. She had neither malignant hypertension nor an elevated plasma rennin concentration. Renal biopsy showed crescentic glomerulonephritis (pauci-immune type), and the myeloperoxidase-specific ANCA (MPO-ANCA) titer was elevated at 1015 AAU. She was cured using steroid pulse therapy, combined with an angiotensin-converting-enzyme inhibitor and angiotensin-II receptor blocker
Antibodies, Antineutrophil Cytoplasmic ; Azotemia ; Biopsy ; Chymosin ; Cytoplasm ; Female ; Glomerulonephritis ; Humans ; Hypertension ; Hypertension, Malignant ; Hypertensive Retinopathy ; Isonipecotic Acids ; Middle Aged ; Plasma ; Proteinuria ; Renal Insufficiency ; Renin ; Scleroderma, Systemic

Chymosin is an important industrial enzyme widely used in cheese manufacture. To improve expression efficiency of recombinant bovine chymosin in Kluyveromyces lactis strain GG799, we designed and synthesized a DNA sequence encoding bovine prochymosin gene (GenBank Accession No. AA30448) by using optimized codons. The synthesized prochymosin gene was amplified by two-step PCR method, and then cloned into the expression vector pKLAC1, resulting in pKLAC1-Prochy. pKLAC1-Prochy was linearized and transformed into K. lactis GG799 by electrotransformation. Positive clones were screened by YEPD plates containing 1% casein. A recombinant strain chyl with highest activities and multi-copy integration which was detected by using specifical integration primers was chosen and fermented in flask. Prochymosin was expressed in K. lactis successfully. SDS-PAGE analysis revealed that the purified recombinant bovine prochymosin had a molecular mass of 41 kDa. After acid treatment, molecular weight of chymosin is about 36 kDa, the same as native bovine chymosin. Activity tests showed that the chymosin activity of the culture supernatant was 99.67 SU/mL after 96 h cultivation. The activities of chymosin were not prominent increased when galactose was used as carbon source instead of glucose, which proved that the fermentation of recombinant strain does not need galactose inducing. The recombinant K. lactis strain obtained in this study could be further used to produce recombinant chymosin for cheese making.
Animals ; Cattle ; Chymosin ; biosynthesis ; genetics ; Enzyme Precursors ; biosynthesis ; genetics ; Gene Expression Regulation, Fungal ; genetics ; Genetic Vectors ; genetics ; Kluyveromyces ; genetics ; growth & development ; metabolism ; Protein Engineering ; Recombinant Proteins ; biosynthesis ; genetics

Primary aldosteronism is a disease that the stimulus for the excessive aldosterone production residues within the adrenal gland. It was first described by conn in 1955. And many cases were reported by physicians at present in the world. But it is relatively rare in Korea, probably due to lack of attention and medical facilities. Only about 13 cases have been reported at present. The clinical, biochemical feature in l case of primary aldosteronism caused by adrenal hyperplasia that was diagnosed at Yeungnam University Hospital was observed and the following result were obtained. 1. Clinical feature: The present case was 27-year-old woman who was admitted due to general weakness and easy fatigability. The above mentioned chief complaints occurred 8 months prior to admission when she delivered of second baby by cesarean section. Symptoms such as above chief complaints, intermittent muscle paralysis and cramping were noticed. Trousseau's sign was also present. The average blood pressure ranged from 170/90 to 200/120 2. Biochemical abnormalities: Severe hypokalemia lower than 2.5 mEq/L was presented and 24 hours urine potassium showed markedly increased urinary loss (228 mEq/day). Plasma rennin activity was decreased under normal range with furosemide administration. (Basal renin; 0.01 ng/ml/hr, stimulated rennin 0.12 ng/ml/hr). Saline suppression test revealed markedly elevated levels of aldosterone higher than normal range (Basal aldosterone; 320.68 pg/ml stimulated aldosterone; 451.86 pg/ml). And posture test showed decreased plasma rennin activity and increased plasma aldosterone level. 3. Adrenal CT scan revealed no abnormal findings. 4. Treatment and course: Spironolactione was given at OPD with regular follow-up. Her blood pressure ranged from 150/90 to 160/100 and symptoms were improved. The effect of treatment was satisfactory and further follow up would be performed.
Adrenal Glands ; Adult ; Aldosterone ; Blood Pressure ; Cesarean Section ; Chymosin ; Female ; Follow-Up Studies ; Furosemide ; Humans ; Hyperaldosteronism* ; Hyperplasia* ; Hypokalemia ; Korea ; Muscle Cramp ; Paralysis ; Plasma ; Posture ; Potassium ; Pregnancy ; Reference Values ; Renin ; Tomography, X-Ray Computed

The role of (pro)rennin receptor (PRR) in cardiomyocytes of a heart failure (HF) rat model was studied. Spontaneously hypertensive rats (SHR) with HF (SHR-HF) or not were identified by two-dimensional (2-D) ultrasound. Age-matched Wistar Kyoto normotensive (WKY) rats were used as controls. PRR short hair RNA (sh-RNA) was injected into the heart of SHR-HF. Simultaneously SHR and controls received the same shRNA injection into the heart. Scramble shRNA was injected into the heart as controls. The expression of PRR mRNA and protein in cardiomyocytes was detected by using real-time PCR and Western blotting respectively. The heart function was evaluated by 2-D ultrasound, including eject fraction (EF%), fractional shortening (FS%), left ventricle thickness (LV), and inter-ventricular septal thickness (IVS). The number of apoptotic cardiomyocytes was counted by using flow cytometry. The results showed that the mRNA and protein expression levels of PRR were significantly higher in cardiomyocytes of SHR-HF group than in those of SHR group or control group. The apoptosis of myocytes in SHR-HF group was increased as compared with SHR group or control group. After knock-down of PRR with shRNA in SHR-HF group, the apoptosis of myocytes was reduced, resulting in the improved heart function. It was suggested that down-regulation of PRR might protect the heart from development of HF in SHR-HF by inhibiting the apoptosis of cardiomyocytes.
Animals ; Apoptosis ; genetics ; physiology ; Blotting, Western ; Chymosin ; metabolism ; Disease Models, Animal ; Enzyme Precursors ; metabolism ; Gene Expression ; Heart Failure ; genetics ; metabolism ; physiopathology ; Male ; Myocardium ; metabolism ; pathology ; Myocytes, Cardiac ; metabolism ; RNA Interference ; Rats ; Rats, Inbred SHR ; Rats, Inbred WKY ; Receptors, Cell Surface ; genetics ; metabolism ; Reverse Transcriptase Polymerase Chain Reaction