• Asian-Australasian Journal of Animal Sciences
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• v.15 no.2
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• pp.274-278
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• 2002

A culture system for lactating rat mammary acini was evaluated, where the primary indicator of performance was lactose secretion, measured by a sensitive bioluminescence assay. Lactose secretion was reduced by half (p<0.01) over the first 6 h of culture by overnight feed withdrawal (FW) from tissue donors but was sensitive to increased glucose concentration in the culture media (p<0.001) up to 30 mM. Lactose production of cells from fed donors over the first 6 h in culture in 30 mM glucose was 8.9 fmol/cell/h - a rate calculated to be about half that in vivo. No significant difference was shown in lactose secretion by cells from fed or FW rats over 6-24 h. Lactose secretion was 3.6 fmol/cell/h by cells from fed animals in 40 mM glucose concentration media over the 6-24 h culture period. Addition of insulin to the culture media had no effect on rates of lactose secretion while addition of prolactin and hydrocortisone, with or without insulin, significantly (p<0.001) decreased lactose production over both 0-6 h and 6-24 h culture periods. Lactose synthesis in vitro was significantly enhanced by aeration of the media during collagenase digestion of mammary tissue (p<0.05). No improvement in lactose secretion was effected by shaking of cells during culture, Matrigel coating of culture dishes or change in cell density over a range up to 2.5 million cells per ml.

• Journal of Genetic Medicine
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• v.13 no.1
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• pp.31-35
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• 2016

Antley-Bixler syndrome (ABS) is a rare form of syndromic craniosynostosis with additional systemic synostosis, including radiohumeral or radioulnar synostosis. Another characteristic feature of ABS is mid-facial hypoplasia that leads to airway narrowing after birth. ABS is associated with mutations in the FGFR2 and POR genes. Patients with POR mutations present with either skeletal manifestations or congenital adrenal hyperplasia with ambiguous genitalia. We report here two cases of ABS caused by mutations in FGFR2 and POR. Although the patients had craniosynostosis and radiohumeral synostosis in common and cranioplasty was performed in both cases, the male with POR mutations showed an elevated level of $17{\alpha}$-hydroxyprogesterone during newborn screening and was diagnosed with congenital adrenal hyperplasia by adrenocorticotropic hormone stimulation. This patient has been treated with hydrocortisone and fludrocortisone. He had no ambiguous genitalia but had bilateral cryptorchidism. On the other hand, the female with the FGFR2 mutation showed severe clinical manifestations: upper airway narrowing leading to tracheostomy, kyphosis of the cervical spine, and coccyx deformity. ABS shows locus heterogeneity, and mutations in two different genes can cause similar craniofacial and skeletal phenotypes. Because the long-term outcomes and inheritance patterns of the disease differ markedly, depending on the causative mutation, early molecular genetic testing is helpful.

• The Journal of Internal Korean Medicine
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• v.10 no.1
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• pp.137-155
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• 1989

In order investigate the effect of Boyanghwanotang (BT) on thrombosis, Sprague-Dawley rats injected endotoxin into the caudal vein were oral administrated solid extract of BT-182. 4mg/200g (Sample A) and 364.8mg/200g (Sample B), and then observed Platelet, fibrinogen, prothrombin time and FDP. Another group injected hydrocortisone acetate into the muscular rump were adopted to observe the effect of BT on elevated blood Viscosity, administrated Sample A and Sample B, thereafter measured body weight, body temperature, whole blood viscosity, plasma viscosity, hematocrit, RBC, WBC, hemoglobin, glucose, albumin, total protein, triglyceride, cholesterol and prothrombin time. 1. Platelet was increased significantly in Sample B compared with the control group. 2. Fibrinogen of the trial groups compared with the control group was increased, and revealed a significance in Sample B. 3. Prothrombin time was decreased significantly in the trial groups compared with the control group. 4. FDP was decreased in the trial groups compared with the control group. 5. Body weight was increased significantly in Sample B after 7 days, body temperature revealed a significance in the trial groups after 5 days. 6. Whole blood viscosity and plasma viscosity of lower shear rates, both were highly decreased in the trial groupn than the control group. 7. The increase of WBC and decrease of hematocrit, RBC and hemoglobin was showed in the trial groups, and Sample B revealed a significance on the increase of WBC and decrease of RBC. 8. The increase of prothrombin time and decrease of glucose, albumin, total protein, triglyceride and cholesterol was showed in the trial group, and Sample B revealed a Significance on the increase of prothrombin time and decrease of albumin, total protein and triglyceride. 9. As compare with Sample A and Sample B, generally Sample B was highly Significant. According to the above results, it was considered that Boyanghwanotang may be the curative effects for thrombosis and elevated blood viscosity.

• Journal of Life Science
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• v.10 no.2
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• pp.150-156
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• 2000

This experiment was performed to evaluate the effect of TSH (thyroid-stimulating) on the ERp29 (endoplasmic reticulum resident 29 kDa protein) gene expression in the rat thyrocytes of FRTL-5 cells. Although ERp29 mRNA was constantly expressed, its expression began to increase remarkably from 10-9 M TSH. and its maximum expression was at 5×10-9 M TSH (about 3.5 fold). On the other hand, the effect of TSH on the abundance of ERp29 mRNA started within 6 h, and peaked at 8 h (about 2.5 fold). Actinomycin D (transcription inhibitor) strongly blocked this effect while cycloheximide (translation inhibitor) did not. The half-life of ERp29 mRNA was about 4.5 h in the presence or absence of TSH that was not affected by the stability of ERp29 mRNA. The effect of TSH on the ERp29 gene expression was specific, while other growth factors (transfferin, insulin, and hydrocortisone) did not alter its expression. Our data indicate for the first time that the expression of ERp29 is regulated transcriptionally by TSH in the thyrocytes.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.199-205
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• 2002

Congenital hypopituitarism is a possible cause of neonatal cholestasis, but the mechanism is still unknown. The pathogenesis of cholestasis may be due to hormone deficiency, which has effects on the physiological maturation of bile acid synthesis and transport. We experienced a case presenting with cholestasis and recurrent hypoglycemia associated with congenital hypopituitarism. Cholestasis resolved with thyroxine and hydrocortisone replacement therapy.

• Endocrinology and Metabolism
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• v.32 no.3
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• pp.353-359
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• 2017

Background: Cushing syndrome is characterized by glucose intolerance, cardiovascular disease, and an enhanced systemic inflammatory response caused by chronic exposure to excess cortisol. Eosinopenia is frequently observed in patients with adrenal Cushing syndrome, but the relationship between the eosinophil count in peripheral blood and indicators of glucose level in patients with adrenal Cushing syndrome has not been determined. Methods: A retrospective study was undertaken of the clinical and laboratory findings of 40 patients diagnosed with adrenal Cushing syndrome at Chungnam National University Hospital from January 2006 to December 2016. Clinical characteristics, complete blood cell counts with white blood cell differential, measures of their endocrine function, description of imaging studies, and pathologic findings were obtained from their medical records. Results: Eosinophil composition and count were restored by surgical treatment of all of the patients with adrenal Cushing disease. The eosinophil count was inversely correlated with serum and urine cortisol, glycated hemoglobin, and inflammatory markers in the patients with adrenal Cushing syndrome. Conclusion: Smaller eosinophil populations in patients with adrenal Cushing syndrome tend to be correlated with higher levels of blood sugar and glycated hemoglobin. This study suggests that peripheral blood eosinophil composition or count may be associated with serum glucose levels in patients with adrenal Cushing syndrome.

• Archives of Pharmacal Research
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• v.15 no.2
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• pp.169-175
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• 1992

Anti-inflammatory glucocorticoid (GC) derivatives have been clinically used in immune-malfunctional diseases for their immunosuppressive activity. However, there is still a lack of knowledge on the relationship between anti-inflammatory and immunosuppressive activities. In order to compare immunosuppressive activities with the known anti-inflammatory activities of the GC derivatives, eight clinically used GC derivatives including hydrocortisone, prednislone, 6$\alpha$-methyl prednisolone, triamcinolone, dexamethasone, betamethasone, triamcinolone acetonide and fluocinolone acetonide were selected, and lymphoblastosis inhibition and plaque-forming cell (PFC) response in mice were studied as immunological parameters. In Con A-induced lymphoblatosis inhibition invitro, all derivatives showed potent inhibition $IC_{50}$ values of the derivaties except methyl prednisolone and triamcinolone were less than $10^{-7}$M and good dose dependency was obtained. This result was well correlated with that of their anti-inflammatory potencies obtained. This result was well correlated with that of their anti-inflammatory potencies and their receptor binding affinities. However, in PFC response, consistent result were not obtained. Total numbers of PFCs per spleen were decreased by some derivatives, but numbers of PFCs per $10^6$ cells were not decreased by systemic administration of but numbers of PFCs per $10^6$ cells were not decreased by systemic administration of GC at the dose of 0.05 mg/mouse. Furthermore, at the dose of 0.1 mg/mouse, numbers of PFCs per $10^6$ cells were found to be increased, although total PFCs per spleen were decreased.

• Journal of Genetic Medicine
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• v.18 no.1
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• pp.64-69
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• 2021

Primary cilium has a signal transduction function that is essential for brain development, and also determines cell polarity and acts as a mediator for important signaling systems, especially the Sonic Hedgehog (SHH) pathway. TBC1D32 is a ciliary protein, implicated in SHH signaling. Biallelic mutations in the TBC1D32 gene causes a kind of ciliopathy, heterogeneous developmental or degenerative disorders that affect multiple organs, including the brain. Here we report a boy who carried compound heterozygous variants in TBC1D32. The patient showed hypotonia, respiratory difficulty, and multiple anomalies at his birth. He was diagnosed with congenital hypopituitarism and treated with T4, hydrocortisone, and growth hormone. Despite the hormonal replacement, the patient needed long-term respiratory support with tracheostomy and nutritional support with a feeding tube. His developmental milestones were severely retarded. Hydrocephalus and strabismus developed and both required surgery, during the outpatient follow-up. Whole-exome sequencing indicated compound heterozygous variants, c.2200C>T (p.Arg734^*) and c.156-1G>T, in TBC1D32 gene. This is the first Korean case of TBC1D32-related ciliopathy and we reported detailed and sequential clinical features. This case demonstrated the utility of whole-exome sequencing and provided valuable clinical data on ultra-rare disease.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.207-214
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• 2009

Purpose : To evaluate the cause of asymptomatic bacteriuria (AB) or aseptic pyuria (AP) on physiologic phimosis and to evaluate the effect of topical steroid therapy and preputial hygiene on the resolution of AB and AP. Methods : Ninety uncircumcised boys (age 1-72 month, median 16 month) with AB or AP were examined for physiologic phimosis and allocated by the preputial retractibility into the non-retractile group (n=59) or the retractile group (n=31). Topical steroid therapy [topical application of hydrocortisone (0.1%) cream with physiotherapy] were prescribed (three times a day) and the method of preputial hygiene (gentle retraction of prepuce and water cleansing) was instructed to the non-retractile group. After 2-4 weeks, the preputial retractibility was reevaluated and urine examination was repeated. To the retractile group, only the method of preputial hygiene was instructed and urine examination was repeated two weeks later. Results : Among 90 boys with AB and AP, 65.6% (59/90) had the nonretractile prepuces and nonperformed preputial hygiene. In the nonretractile group, the prepuces became retractile in 81.4% (48/59) after topical steroid therapy. Among boys (n=48) whose prepuces became retractile after topical steroid therapy, AB or AP resolved in 77.1%, decreased in 18.7% and persisted in 4.2%, which were significantly different to 18.2%, 2.37%, 54.5% in boys (n=11) whose prepuces were persistently nonretractile (P=0.0114). In the retractile group (n=31), 65.2% was compliant to preputial hygiene. In boys (n=23) who were compliant to preputial hygiene, AB or AP resolved in 65.2%, decreased in 26.0% and persisted in 8.2%, which were significantly different to 12.5%, 50%, 37.5% in boys (n=8) who were not compliant (P=0.0457). Conclusion : Physiologic phimosis was an important cause of AB or AP. Simple topical steroid therapy on the nonretractile prepuces and good preputial hygiene could improve AB or AP.

• Journal of Veterinary Clinics
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• v.29 no.1
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• pp.1-7
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• 2012

Fifty-four dogs with otitis externa were enrolled in the study for the Evaluation of efficacy of a Hydrocortisone aceponate - Gentamicin - Miconazole otic combination ($Easotic^{(R)}$, Virbac, Carros, France). Otitis externa patients were treated by $Easotic^{(R)}$ once daily for 5 days and 2 days off treatment and evaluated on $7^{th}$ day. If otitis externa persisted, additional $Easotic^{(R)}$ treatment was administered once daily for 5 days and rested 2 days and reevaluated on $14^{th}$ day. For the evaluation of efficacy of $Easotic^{(R)}$, eight clinical signs were scored on a severity scale and infectious agents from ear sample were also graded using semi-quantitative scale at each visit. Sum of clinical scores and cytological scores was defined as Global Clinical Score. When $Easotic^{(R)}$ was applied once daily for 5 days, global clinical score was reduced 76.0%. When $Easotic^{(R)}$ was administered for 10 days, during first 5 days administration, 46.6% reduction of global clinical score was detected. During additional 5 days administration, 82.2% reduction of global clinical score was observed compared with Day 0. Any relevant adverse effect was not reported during the study in all cases. Thus, $Easotic^{(R)}$ treatment once daily for 5 days and 10 days appears to be effective and safe treatment for canine otitis externa.