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Role of Tetrahydrobiopterin (BH<sub>4</sub>) Therapy in PKU
Shintaku, Haruo The Korea Society of Inherited Metabolic Disease 2015 대한유전성대사질환학회지 Vol.15 No.2
Tetrahydrobiopterin ($BH_4$) can normalize blood phenylalanine (Phe) levels in $BH_4$ deficiency, but typically not in phenylketonuria (PKU). In 1999, Kure et al. reported that some PKU patients showed decreased blood Phe levels after $BH_4$ loading, and thereafter, those PKU patients were identified by neonatal PKU screening. A natural cofactor for phenylalanine hydroxylase (PAH) is a 6R-isomer of $BH_4$, which is first synthesized in Japan as Sapropterin dihydrochloride (Biopten$^{(R)}$) in 1982. In Japan, Biopten$^{(R)}$ is first approved for the treatment of $BH_4$ deficiency in 1992, and then for $BH_4$-responsive PAH deficiency (BPKU) in 2008. The discovery of BPKU has vast clinical implications. After Biopten$^{(R)}$ (Kuvan$^{(R)}$) is available for the treatment of BPKU, the QOL of both patients and their families were improved very much, since the serum phenylalanine levels were controlled within 4 mg/dL by $BH_4$ mono-therapy with a normal diet or $BH_4$ combined use of mild phenylalanine-restricted diet. Biopten$^{(R)}$ therapy in patients with BPKU is highly efficacious (70%) at maintaining serum Phe levels within recommended control range and provides excellent safety at least average use period of 10 years (range, 1-17 years) with no unwarranted side effects in Japan. In addition it has been confirmed that sapropterin therapy initiated before 4 years of age was very effective to maintain plasma Phe levels within the favorable range and was safe in Japanese patients with BPKU.
Invasion of the canalis sinuosus by dental implants: A report of 3 cases
Shintaku, Werner Harumiti,Ferreira, Cimara Fortes,Venturin, Jaqueline de Souza Korean Academy of Oral and Maxillofacial Radiology 2020 Imaging Science in Dentistry Vol.50 No.4
The canalis sinuosus (CS) and its accessory canals (ACs) are anatomical structures in the anterior maxilla. These structures are often neglected when planning implant surgery because their clinical significance is still not well-defined. After a retrospective evaluation of 194 patients rehabilitated with dental implants in the anterior maxilla, 3 patients were identified who presented unexpected chronic neurosensory disturbances without any clinical signs supportive of implant failure. Tomographic assessment using cone-beam computed tomography (CBCT) revealed the invasion of the CS and ACs by dental implants, which appeared to explain the patients' symptoms. The purpose of this report was to familiarize practicing dentists and specialists with the CS and its ACs. Unanticipated neurosensory symptoms after implant placement in the anterior maxilla justify the use of CBCT to rule out an injury to this neurovascular bundle.
Sultana, Halima,Ishida, Takeshi,Shintaku, Toshihiro,Kanda, Shuhei,Itabashi, Hisao Asian Australasian Association of Animal Productio 2008 Animal Bioscience Vol.21 No.9
The objective of this study was to investigate the effect of dietary supplementation with calcium salts of soybean oil fatty acids (CaSO) and linseed oil fatty acids (CaLO) on c9,t11-CLA production in ruminal fluid and milk fat from Holstein dairy cows. Rumen fermentation, lactational performances and fatty acid profiles in ruminal fluid and milk fat were also investigated. Twenty multiparous Holstein dairy cows were allotted randomly into two groups consisting of ten cows in each group according to calving date and average milk yield. The first group of cows was fed a control (without calcium salts) diet and a treatment as 1.0% of CaSO (on DM basis) for 30 days in each period. In the second group, cows were fed the same control diet and 1.0% of CaLO as a treatment in the same manner. The forage: concentrate ratio was 52:48, and diets were formulated to contain 17% crude protein (DM basis) for both groups. Ruminal pH, protozoal numbers and the concentration of total volatile fatty acids were unchanged, however, the ruminal ammonia-N decreased by feeding CaSO or CaLO treatment compared to the control diet. The vaccenic acid (trans-11 C18:1; VA) in rumen fluid increased (p<0.01) by 169% and 153%, and the c9,t11-CLA content of rumen fluid increased (p<0.01) by 214% and 210% in the CaSO and CaLO treatments, respectively, compared to the control diet. In milk fatty acids, the VA content increased by 130% and 132% in the evening and morning milking times, respectively, and the c9,t11-CLA content increased by 125% in both milking times for the CaSO supplementation than that of control diet. In the case of CaLO supplementation, the VA increased by 117% and 114%, and the c9,t11-CLA increased by 96% and 94% in the evening and morning milking times, respectively, compared to the control diet. The contents of VA and c9,t11-CLA of milk fatty acids were numerically higher in the evening milking time compared to the morning milking time for control and both treatments. Finally, these results indicated that the supplementation of CaSO or CaLO treatment increased the VA and the c9,t11-CLA in both ruminal fluid and milk fat of Holstein dairy cows.
Mastoid osteoma: A rare incidental finding in an orthodontic patient
Borissova, Ioanna B.,Venturin, Jaqueline S.,Claro-Woodruff, Wanda I.,Shintaku, Werner H. Korean Academy of Oral and Maxillofacial Radiology 2020 Imaging Science in Dentistry Vol.50 No.4
Mastoid osteomas of the temporal bone are rare, benign, and usually asymptomatic tumors. However, depending on their size and extension, mastoid osteomas may cause facial palsy, a sensation of ear fullness, pressure-related pain, hearing loss, recurrent external ear infections, and chronic discharge. The etiology of mastoid osteomas is still unknown, but congenital, infectious, and traumatic factors have been proposed. Surgical treatment may be performed with minimal postoperative morbidity. In this article, the authors report a case of a 48-year-old woman seeking orthodontic treatment with an unusual retroauricular protruding mass, including the diagnostic process and differential diagnosis. This case supports the essential role of cone-beam computed tomography to analyze and identify the lesion as a mastoid osteoma.