• Annals of Pediatric Endocrinology and Metabolism
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• v.23 no.4
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• pp.176-181
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• 2018

Noonan syndrome (NS) is an autosomal dominant disorder that involves multiple organ systems, with short stature as the most common presentation (>70%). Possible mechanisms of short stature in NS include growth hormone (GH) deficiency, neurosecretory dysfunction, and GH resistance. Accordingly, GH therapy has been carried out for NS patients over the last three decades, and multiple studies have reported acceleration of growth velocity (GV) and increase of height standard deviation score (SDS) in both prepubertal and pubertal NS patients upon GH therapy. One year of GH therapy resulted in almost doubling of GV compared with baseline; afterwards, the increase in GV gradually decreased in the following years, showing that the effect of GH therapy wanes over time. After four years of GH therapy, ~70% of NS patients reached normal height considering their age and sex. Early initiation, long duration of GH therapy, and higher height SDS at the onset of puberty were associated with improved final height, whereas gender, dosage of GH, and the clinical severity did not show significant association with final height. Studies have reported no significant adverse events of GH therapy regarding progression of hypertrophic cardiomyopathy, alteration of metabolism, and tumor development. Therefore, GH therapy is effective for improving height and GV of NS patients; nevertheless, concerns on possible malignancy remains, which necessitates continuous monitoring of NS patients receiving GH therapy.

• Asian-Australasian Journal of Animal Sciences
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• v.14 no.5
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• pp.697-700
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• 2001

A prolonged farrowing interval is common on small farms in Nepal and may be attributable to the absence of boar contact at the time of weaning. To examine whether synthetic boar pheromone spray might be of value to aid the stimulation and detection of estrus, 30 village farms, each with a single sow, were recruited into a field study. All sows entered the study on final weaning from their first lactation at 56 days post partum. For 13 treatment sows, a boar pheromone spray (SOA, Intervet, Boxmeer, The Netherlands) was applied each day after weaning whilst 17 other sows did not receive any stimulus (negative control). When estrous was detected by the farmer, sows were taken to the nearest boar for service. There was a significantly shorter weaning to re-mating interval for treatment sows (42.9 V 82.2 day, p<0.05), with a significantly higher proportion of treatment sows rebred by day 60 after weaning (p=0.024, Fishers Test). It is concluded that use of boar pheromones can improve reproductive efficiency of sows kept under Nepalese village conditions.

• Archives of Plastic Surgery
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• v.33 no.2
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• pp.241-244
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• 2006

Idiopathic scrotal calcinosis is rare entity, characterized by the presence of multiple, firm nodules within the dermis of scrotal skin. The lesions are evident as asymptomatic, round, firm, yellowish papules, from several millimeters up to 1 cm in diameter, with a tendency to occur symmetrically. Because polypoid formation of the calcified cysts is unusual in scrotal calcinosis, we report our experience of a case of scrotal calcinosis with cysts having polypoid appearence. A 34-year-old man was admitted to our department with painless, firm, widespread nodules within the scrotum. Patient started to have these lesions at puberty and progressed to a polypoid form over time. There was neither surgical nor medical treatment had been attempted before our treatment. Physical examination revealed localized polypoid cysts in addition to multiple small, firm nodules within the scrotal skin. No underlying anomalies were detected in the physical examination and laboratory analysis. We experienced the treatment of polypoid idiopathic scrotal calcinosis and a case is presented with the review of literatures.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.4 no.1
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• pp.173-178
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• 1993

Narcolepsy's age at onset is reported to be relatively homogeneous, occuring usually after the onset of puberty, although most cases are diagnosed when the patients are in their late teens to late 20s. It is very unusual for a patient to develop narcolepsy before 15 years of age or after 30 years of age. A 11-year old boy who has developed excessive daytime sleepiness since age of 7 and has all the four major features of narcolepsy by the time of evaluation is presented. On polysomnographic examination, the patient showed two sleep onset REM periods in the three latency test of the multiple sleep latency test and the nocturnal polysomnogram. In addition, the findings of typing HLA class I and II of the patient's family are presented. Reports of pediatric narcolepsy previously reported are reviewed.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.29 no.5
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• pp.422-428
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• 2007

The columella, nasal tip, lip relationship in the secondary bilateral cleft deformity remains an enigma and a great challenge for the cleft surgeon. A subset of patients with bilateral cleft lip still require columellar lengthening and nasal correction, despite the advances in preoperative orthopedics and primary nasal corrections. An approach to correct this deformity is described. This consists of 1) lengthening the columella, 2) open rhinoplasty, allowing definitive repositioning of lower lateral cartilages, ear cartilage grafting to the tip and columella when necessary, 3) nasal mucosal advancement, 4) alar base narrowing and 5) reconstruction of the orbicularis oris as required. In surgical repair of the cleft lip nose, the timing of the operation(during lip closure, before or after the puberty growth sput), and the operative technique play a key role in the final result. In this study, 13 cleft lip patients who had undergone a secondary cheilorhinoplasty at the Department of Oral and Maxillofacial Surgery, Pusan National University Hospital were evaluated to check the proper time and method of the operation.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.9 no.2
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• pp.134-141
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• 1998

Mutational falsetto is a kind of voice disorders due to the failure to acquire proper low-pitched voice during the puberty. The patients with mutational falsetto can produce the normal low-pitched voice by the surgical treatment, like the type III-thyroplasty, or the voice therapy. The present study is, focusing on the latter treatment, to consider the efficiency of voice therapy for the mutational falsetto. The 7 patients who were diagnosed as mutational falsetto by the laryngologists, and treated by the voice therapist were selected as subjects. Their voices of pretherapy and posttherapy were analyzed on the aspects of acoustics and aerodynamics. Acoustic analysis was done by the MDVP(Multidimensional Voice Program) of CSL(Computerized Speech Lab, Kay Elemetrics, Co.), and aerodynamic analysis, by the Maximum Sustained Phonation of Aerophone II(Kay Elemetrics, Co.). By these measurements, we could find that fundamental frequency(F0) was significantly lowered, on the average, 65Hz. Maximum phonation time(MPT) was increased 4.57 second, and shimmer was decreased 1.644%, respectively, and each changes was statistically significant, too. On the average, jitter was decreased 0.499%, mean flow rate(MFR) was decreased 27.71ml/sec, and NHR was increased 0.023 which was the only parameter not showing improvement. But the changes of jitter, MFR and NHR were not statistically significant.

• BLOOD RESEARCH
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• v.53 no.4
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• pp.314-319
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• 2018

Background Iron overload is a risk factor affecting all patients with thalassemia intermedia (TI). We aimed to determine whether there is a relationship of serum ferritin (SF) and alanine aminotransferase (ALT) with liver iron concentration (LIC) determined by R2 magnetic resonance imaging (R2-MRI), to estimate the most relevant degree of iron overload and best time to chelate in patients with TI. Methods In this cross-sectional study, 119 patients with TI (mean age years) were randomly selected and compared with 120 patients who had a diagnosis of thalassemia major (TM). Correlations of LIC, as determined by R2-MRI, with SF and ALT levels, were assessed in all participants. A P-value <0.05 was considered statistically significant. Results SF and LIC levels were lower in patients with TI than in those with TM; only ferritin values were significant. We found a statistically significant relationship between SF and LIC, with cut-off estimates of SF in patients with TI who had splenectomy and those who entered puberty spontaneously (916 and 940 ng/mL, respectively) with LIC >5 mg Fe/g dry weight (P<0.0001). A significant relationship was also found for patients with TI who had elevated ALT level (63.5 U/L), of 3.15 times the upper normal laboratory limit, using a cut-off for LIC ${\geq}5mg\;Fe/g\;dry\;weight$. Conclusion We determined the cut-off values for ALT and SF indicating the best time to start iron chelation therapy in patients with TI, and found significant correlations among iron overload, SF, and ALT.

• Clinical and Experimental Reproductive Medicine
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• v.38 no.2
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• pp.115-118
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• 2011

A 58-year-old woman who presented with inguinal hernia for the first time was diagnosed as seminoma and complete androgen insensitivity syndrome (CAIS). The patient received a late diagnosis, and therefore she could not take a proper management. CAIS is a rare X-linked recessive disease with an XY karyotype that is caused by androgen receptor defects. It usually present with primary amenorrhea or inguinal hernia. The risk of malignant transformation of undescended testis increases with age, thus gonadectomy should be performed after puberty. We present a case of large advanced seminoma in a woman with CAIS who was neglected and diagnosed lately.

• Journal of Animal Science and Technology
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• v.55 no.4
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• pp.249-255
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• 2013

Intercellular interactions are important for the proper development and regulation of tissue function. This is especially necessary in the epididymis, a part of male reproductive tract where sperm become mature and acquire their fertilizing capacity. The caput region of the epididymis consists of several types of cells, including principal, basal, and apical cells. Direct intercellular communication is thus required to precisely regulate the functions of the caput epididymis. In this regard, connexin (Cx) is a molecule that forms channels, which allow the direct exchange of small molecules between cells, enabling intercellular communication. In this study, the expression of Cx isoforms in the caput epididymis at different postnatal ages was determined by using quantitative real-time polymerase chain reaction analysis. Nine of 13 Cx isoforms were detected. The transcript levels of Cx30.3, 31, 31.1, 32, and 40 were highest at 45 days of age, while the expression of Cx43 and 45 gradually decreased with age. A substantial fluctuation of Cx26 expression was detected, with significant decreases before and during puberty, followed by a transient increase at adult-hood and rapid decreases at an old age. A significant increase in Cx37 transcript was observed at 25 days of age, followed by gradual decreases at adult and old ages. These results indicate the significant differential expression of various Cx isoforms in the caput epididymis during postnatal development. It further suggests that the functional regulation and developmental maturation of the caput epididymis are highly related to the postnatal age-related differential expression of Cx isoforms.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.15
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• pp.6307-6313
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• 2014

The aim of this study was to examine the knowledge and awareness of women in Kuwait with regard to risk factors, symptoms and diagnostic procedures of breast cancer. A total of 521 questionnaires were distributed among women in Kuwait. Results showed that 72% of respondents linked breast cancer factors to family history, while 69.7% scored abnormal breast enlargement as the most detectable symptom of the disease. Some 84% of participants had heard about self-examination, but knowledge about mammograms was limited to 48.6% and only 22.2% were familiar with diagnostic procedures. Some 22.9% of respondents identified the age over 40 years as the reasonable age to start mammogram screening. Risk factor awareness was independent on age groups (p>0.05), but both high education and family history increased the likelihood of postivie answers; the majority knew about a few factors such as aging, pregnancy after age 30, breast feeding for short time, menopause after age of 50, early puberty, and poor personal hygiene. In conclusion, 43.1% of participants had an overall good knowledge of breast cancer with regards to symptoms, risk factors and breast examination. Very highly significant associations (p<0.005) were evident for all groups except for respondents distributed by nationality (p=0.444). Early campaigns for screening the breast should be recommended to eliminate the confusion of wrong perceptions about malignant mammary disease.