Purpose: Pseudohypoparathyroidism is a hereditary disorder characterized by symptoms and signs of hypoparathyroidism, typically in association with distinctive skeletal and developmental defects. Hypoparathyroidism is caused by a insufficient end-organ response to PTH (parathyroid hormone). Hypoparathyroidism consists of four types in which the most common form, pseudohypoparathyroidism-Ia, accompany with Albright's hereditary osteodystrophy. We experienced a case of a woman who had been suffering from calcified mass on left foot, diagnosed Albright's hereditary osteodystrophy. Methods: We present a case of a 24-year-old Korean female who visited plastic surgery department with a painful mass on dorsum of the left foot. On the physical exam, bony hard and painful mass, fixed to dermis, was noted. Plain X-ray films demonstrate suspicious calcification on subcutaneous tissue of dorsum of the left foot. The patient was diagnosed pseudohypoparathyroidism 2 years ago at the plastic surgery department. At the visiting time, the laboratory results were within normal range even though the patient actually had a disease. The reason is because the patient has been treated with Vit.D, calcium replacement therapy and thyroid hormone therapy. Moreover, the patient has been treated with anticonvulsant agents due to epilepsy. On the brain computer tomography (CT), calcification was noted on the basal ganglia and dentate nucleus. So we decided the total excision of entire mass from the left foot. Results: We excised main mass with numerous pinhead sized masses which were scattered around the main mass. The $6.0{\times}4.0{\times}0.5\;cm$ sized main mass was bony hard, and its surface was flat and margin was irregular. The permanent biopsy was confirmed that the main mass and all the scattered tiny masses were heterotopic calcification. The patient did not suffer from the pain after the mass excision. The wound has been healed without any problem. Conclusions: Heterotrophic calcification is often accompanied with pseudohypoparathyroidism, but such a huge one is uncommon. We report a case of pseudohypoparathyroidism with heterotrophic calcification developed in dorsum of left foot who was diagnosed by excisional biopsy.
Kim, Minjeong;Jeong, Ji Seong;Kim, Hyunji;Hwang, Seungwoo;Park, Il-Hyun;Lee, Byung-Chul;Yoon, Sung Il;Jee, Sun Ha;Nam, Ki Taek;Lim, Kyung-Min
Biomolecules & Therapeutics
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v.26
no.5
/
pp.512-519
/
2018
Phthalates widely used in the manufacture of plastics have deeply penetrated into our everyday lives. Recently, a concern over the toxicity of phthalates on thyroid, has been raised but in most of cases, the doses employed were unrealistically high. To investigate the effects of phthalates on thyroid, we investigated the effects of the repeated oral exposure to low to high doses (0.3, 3, 30 and 150 mg/kg) di-2-ethylhexylphthalate (DEHP) from weaning to maturity for 90 days in juvenile rats on the thyroid. The histological examination revealed that DEHP significantly induced hyperplasia in the thyroid from the doses of 30 mg/kg, which was confirmed with Ki67 staining. In line with this finding, increased mRNA expression of thyrotropin releasing hormone (Trh) was observed in the thyroid of female at 0.3 mg/kg and 150 mg/kg as determined by RNAseq analysis. Moreover, significantly increased expression of parathyroid hormone (Pth) in the female at 0.3 mg/kg, and thyroglobulin (Tg) and thyroid hormone responsive (Thrsp) in the male at 0.3 mg/kg were noted in the blood, of which changes were substantially attenuated at 150 m/kg, alluding the meaningful effects of low dose DEHP on the thyroid hormone regulation. Urinary excretion of mono-2-ethylhexyl-phthalate (MEHP), a major metabolite of DEHP was determined to be 4.10 and 12.26 ppb in male, 6.65 and 324 ppb in female at 0.3 and 30 mg/kg DEHP, respectively, which fell within reported human urine levels. Collectively, these results suggest a potential adverse effects of low dose phthalates on the thyroid.
Kim, Hyun-Jung;Kim, Bo Ra;Seo, Yeong-Mi;Cho, Yoon Young;Baek, Jong-Ha;Kim, Kyong Young;Kim, Soo-Kyung;Woo, Seung-Hoon;Jung, Jung Hwa;Jung, Jaehoon;Hahm, Jong Ryeal
Journal of Yeungnam Medical Science
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v.34
no.1
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pp.69-74
/
2017
Background: Chronic kidney disease is considered a risk factor for thyroid nodules as well as thyroid dysfunction such as hypothyroidism. Among patients on hemodialysis, we assessed the size of thyroid nodule and goiter at baseline and 1 year later with ultrasonography. Methods: We prospectively selected 47 patients with hemodialysis at January 2012 and reviewed their medical records. We checked goiter and thyroid nodules at January 2012 and December 2012. Results: In the hemodialysis patients (n=47), 24 patients (51.1%) had thyroid nodules and 33 patients (70.2%) had goiter at baseline. Parathyroid hormone (PTH) was higher in patients with thyroid nodules ($204.4{\pm}102.9$ vs. $129.9{\pm}93.6pg/mL$, p=0.01). Thyroid ultrasonography was conducted in 29 patients after 1 year. The thickness of the thyroid isthmus increased ($2.8{\pm}1.6$ vs. $3.2{\pm}1.9mm$, p=0.003), but the number of nodules did not change ($1.2{\pm}1.9$ vs. $1.4{\pm}2.0$, p=0.109). PTH was associated with the enlargement of thyroid nodules significantly through logistic regression analysis. Conclusion: Thyroid goiter and nodules in hemodialysis patients were more prevalent than in the general population. PTH influenced the production of thyroid nodules in hemodialysis patients. Regular examination with thyroid ultrasonography and thyroid function test should be considered in hemodialysis patients.
Purpose: The aims of this study were to assess the clinical and laboratory profiles of chronic kidney disease-mineral bone disorder (CKD-MBD) and to assess the effects of treatment of active vitamin D analogs on severe hyperparathyroidism (SHPT) in pediatric patients on chronic peritoneal dialysis. Methods: This is a retrospective study included 53 patients who had been undergoing dialysis for more than 1 year, between January 2003 and December 2012. Results: Even after treatment with phosphate binders and active vitamin D analogs, the $mean{\pm}standard$ deviation of the percentage of time during peritoneal dialysis that the patients' serum concentrations of phosphorus, corrected total calcium, and parathyroid hormone (PTH) fell within the Kidney Disease Outcomes Quality Initiative recommended ranges was $25.06{\pm}17.47%$, $53.30{\pm}23.03%$, and $11.52{\pm}9.51%$, respectively. Clinical symptoms or radiological signs of CKD-MBD were observed in 10 patients (18.9%). There were significant differences in percentage of time that the serum intact PTH concentration was outside of the recommended range between patients with and without symptoms or signs of CKD-MBD (below recommended range, $11.74{\pm}7.37%$ vs. $40.77{\pm}25.39%$, P <0.001; above the recommended range, $63.79{\pm}27.86%$ vs. $37.09{\pm}27.76%$, P =0.022). Of the 25 patients with SHPT, high-dose alfacalcidol treatment was required in 13 patients that controlled SHPT in 7 of these patients, without marked complications. Conclusion: Despite our efforts to manage CKD-MBD, patients' met the recommended ranges from relevant guidelines at a low frequency. The treatment of high-dose active vitamin D analogs was required in about half of the patients with SHPT and effective in about half of them.
The effects of different dietary electrolyte balance (DEB) on growth performance, nitrogen (N) metabolism and some blood biochemical parameters were investigated in 2 to 3 months old growing rabbits. A total of 150 growing rabbits of 2 months age were randomly divided into five groups according to average body weight, with 30 rabbits in each group. The DEB levels of the five experimental diets were -154, -3.16, +201, +347, and +500 meq/kg of dry matter (DM), respectively. There was a 7-d adaptation period and a 23-d experimental period. The results showed that the DEB levels had a quadratic affect on the average daily feed intake (ADFI) (p<0.001). The greatest ADFI was achieved when the DEB level was +201 meq/kg DM. Fecal N (FN) content linearly decreased (0.047), while digestible N (DN), retained N (RN), efficiency of intake N converted into digestible N (DN/IN) and the efficiency of intake N converted into retained N (RN/IN) linearly increased with the DEB increase (0.020, 0.004, 0.021, and 0.049, respectively). Serum phosphorus (P) ion content linearly increased with the DEB increase (p = 0.036). The DEB had a quadratic relationship with serum anion gap (AG) (p = 0.002) and serum parathyroid hormone (PTH) content (p = 0.016). The DEB levels quadratically affected base excess (BE) in the plasma (p<0.001). In conclusion, the DEB unaffected growth performance but affected feed intake, N metabolism and some blood biochemical parameters of growing rabbits.
This study was designed to investigate the effect of aromatherapy on skin xerosis and pruritus in patients undergoing maintenance hemodialysis. Twenty one subjects of this study were selected from St. Paul's Hosipital in Seoul. All the subjects were received the mineral oil massage at the arm without fistular three timesper week for 4 weeks. After 2 weeks period of wash-out, the subjects were received the aromatherapy of lavender and tea tree essence oil in the same way. This study was carried out from March 20 to June 13, 1998. Pruritus score, skin pH and stratum corneum hydration were measured before and after each treatment. But, biochemical parameters were measured before the treatment of the mineral oil massage, after the treatment of the mineral oil massage and the aromatherapy. Data of this study were analyzed by paired t-test, repeated measures ANOVA, Bonferroni multiple comparisons and Pearson correlation coefficient. The results were as follows ; 1. Pruritus score was significantly decreased after the aromatherapy, while no significant change after the treatment of the mineral oil massage. Therefore, there was a significant difference in the priritus score between the two treatments. 2. Stratum corneum hydration was significantly increased after the aromatherapy, while no significant change after the treatment of the mineral oil massage. Therefore, there was a significant difference in the stratum corneum hydration between the two treatments. 3. Skin pH was significantly increased after the treatment of the mineral oil massage, while no significant change after the aromatherapy. Therefore, there was no significant difference in the skin pH between the two treatments.4. After the aromatherapy, the serum calcium was significantly increased. Whereas the serum parathyroid hormone intact was significantly decreased compared with the treatment of the mineral oil massage. But the level of the serum Ca and PTH-intact were within the normal range. 5. Stratum corneum hydration was decreased corresponding to the duration of hemodialysis, while pruritus score and skin pH showed no change corresponding to the duration of hemodialysis and the age of the subjects. The correlation of pruritus score on skin pH, stratum corneum hydration and biochemical parameters was not significant. In conclusion, this findings indicate that aromatherapy may be effective in decreasing skin xerosis and pruritus score in uremic pruritus patients undergoing maintenance hemodialysis.
Osteoporosis is defined as a pathologic condition in which there occurs no change in the chemical composition of the bone, while bone resorption is abnormally increased. This compares with osteogenesis, which leads to a decrease in the amount of bone. Though many varieties of therapies have been tried, no fully effective method has been found. Korean red ginseng is an important variety of Korean ginsengs and many studies have been performed to investigate its effectiveness. But there has been no report on red ginseng's effects on osteoporosis. The purpose of this study was to determine the clinical effects of Korean red ginseng on postmenopausal osteoporosis patients. The study was designed as a double-blind study. Group I consisted of 30 postmenopausal osteoporosis patients who were administered red ginseng extracts and 15 patients who were administered maltose capsules, were included in group II. The following results were obtained after one-year administration to both groups. 1. There were no significant differences in the degree of wedging, number of compression fractures in the vertebral body, trabeculations, and Cobb's angles between the experimental and control groups. 2. Even though bone mineral density increased in the lumbar spine, and decreased in the femur, there was no significant statistical difference. 3. The serum levels of parathyroid hormone (PTH), calcium, phosphate, and alkaline phosphates showed no difference between the two groups regardless of treatment. 4. Urine Deoxy-pyridinoline (DPYD) decreased in the red ginseng group while it increased in the maltose group, but statistically there was no significant difference between the two groups. 5. The clinical presentations showed no significant differences using Visual Analog Scale. 6. The specific complications related with the long-term use of red ginseng did not occur in all patients. In conclusion, there were no statistically significant differences in clinical presentations, biochemical, and radiological studies between the red gins eng and maltose groups. No definite effectiveness of red ginseng on patients with osteoporosis was found.
It was demonstrated that Transactivating transcriptional activator(TAT) protein from HIV-1 shown to enter cells when added to the surrounding media. TAT peptide chemically attached to various proteins was able to deliver these proteins to various cell and even in tissues in mice with high levels in heart and spleen. In this study, the tripeptide GKH(Glycine-Lysine-Histidine) derived from Parathyroid hormone (PTH), which was known as lipolytic peptide, is attached to 9-poly Lysine(TAT) to be used as a cosmetic ingredient for slimming products. When Glycerol release, expressed as extracellular glycerol concentration, is lipolysis index, TAT-GKH at $10^{-5}$mo1/L induces approximately 41.5% maximal lipolytic effects in epididymal adipocytes isolated from rats, compared with basal lipolysis. Epididymal adipose tissues of male rats is assessed ex vivo by microdialysis. Probes are perfused with Ringer solution in which increasing concentrations of TAT-GKH. The perfusion of TAT-GKH induces lipolytic effect. Penetration study showed that TAT-GKH efficiently elevates 36 times higher penetration into the excised hairless mice skin than GKH. in vivo study showed that TAT-GKH had a better effect upon the relative volume of eye bag after 28 days of application on twenty(+2) healthy female volunteers. It was identified that TAT-GKH increases penetration enhancement and lipolytic effects in both in vitro, ex vivo and in vivo.
Bak, Na Ry;Song, Eun Song;Yang, Eun Mi;Kim, Chan Jong
Childhood Kidney Diseases
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v.23
no.2
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pp.111-115
/
2019
Vitamin D dependent rickets type 1A (VDDR1A) is an autosomal recessive disorder caused by mutations in CYP27B1. Clinical findings are growth retardation, hypotonia, muscle weakness, hypocalcemic seizures, and radiological features of rickets. We aimed to present the VDDR1A case with a genetic study of CYP27B1. The 14-month-old boy was admitted to the hospital due to a seizure. Serum calcium, phosphorus, alkaline phosphatase, parathyroid hormone (PTH), 25(OH) vitamin D, and 1,25(OH)2 vitamin D values were 5.1 mg/dL, 3.7 mg/dL, 705 IU/L, 429 pg/mL, 24.9 ng/mL, and 8.8 pg/mL, respectively. Radiological study showed cupping and fraying of the distal ulna and radius. The molecular genetic study revealed that the patient had a compound heterozygous mutation, $Phe443Profs^*24$ and c.589+1G>A, in CYP27B1. Genetic analysis of the family members presented that the mother was heterozygous for the mutation c.589+1G>A, and that the father was heterozygous for $Phe443Profs^*24$. The patient was treated with calcium lactate and calcitriol. Until now, six Korean patients with VDDR1A have been studied. Including this case, Korean patients with VDDR1A were found to have only three different mutations in 14 alleles, indicating that the mutation in the CYP27B1 gene is homogeneous in the Korean population.
Osteoporosis is a systemic skeletal disease caused by low bone mass and the decrease of bone density in the microstructure of trabecular bone. Drug therapy(PTH Parathyroid hormone) may increase the trabecular thickness and thus bone strength. Vertebroplasty is a minimally invasive surgery foy the treatment of osteoporotic vertebral compression fracture. This Procedure includes Puncturing vertebrae and filling with Polymethylmethacrylate(PMMA). Although altering recommended monomer-to-Powder ratio affects material properties of bone cement, clinicians commonly alter the mixture ratio to decrease viscosity and increase the working time. The Purposes of this study were to analyze the effect of 4he monomer-to-powder ratio on the mechanical characteristics of trabecular. In this paper, the finite element model of human vertebral trabecualr bone was developed by modified Voronoi diagram, to analyze the relative effect of hormone therapy and vertebroplasty at the treatment of osteoporotic vertebrae. Trabeuclar bone models for vertebroplasty with varied monomer-to-Powder ratio(0.40∼1.07 ㎖/g) were analyzed. Effective modulus and strength of bone cement-treated models were approximately 60% of those of intact models and these are almost twice the values of hormone-treated models. The bone cement models with the ratio of 0.53㎖/g have the maximum modulus and strength. For the ratio of 1.07㎖/g, the modulus and strength were minimum(42% and 49% respectively) but these were greater than those for drug therapy. This study shows that bone cement treatment is more effective than drug therapy. It is found that in vertebroplasty, using a monomer-to-powder ratio different from that recommended by manufacturer nay significantly not only reduce the cement's material Properties but also deteriorate the mechanical characteristics of osteoporotic vertebrae.
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