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1.
Journal of Korean Medical Science ; : e324-2018.
Article in English | WPRIM | ID: wpr-718401

ABSTRACT

Oxysterol 7α-hydroxylase deficiency is a very rare liver disease categorized as inborn errors of bile acid synthesis, caused by CYP7B1 mutations. As it may cause rapid progression to end-stage liver disease even in early infancy, a high index of suspicion is required to prevent fatal outcomes. We describe the case of a 3-month-old boy with progressive cholestatic hepatitis and severe hepatic fibrosis. After excluding other etiologies for his early liver failure, we found that he had profuse urinary excretion of 3β-monohydroxy-Δ5-bile acid derivatives by gas chromatography/mass spectrometry analysis with dried urine spots on filter paper. He was confirmed to have a compound heterozygous mutation (p.Arg388Ter and p.Tyr469IlefsX5) of the CYP7B1 gene. After undergoing liver transplantation (LT) from his mother at 4 months of age, his deteriorated liver function completely normalized, and he had normal growth and development until the current follow-up at 33 months of age. We report the first Korean case of oxysterol 7α-hydroxylase deficiency in the youngest infant reported to undergo successful living donor LT to date.


Subject(s)
Humans , Infant , Male , Bile , Bile Acids and Salts , Fatal Outcome , Fibrosis , Follow-Up Studies , Growth and Development , Hepatitis , Liver , Liver Diseases , Liver Failure , Liver Transplantation , Living Donors , Mothers , Spectrum Analysis
2.
Journal of the Japan Society of Acupuncture and Moxibustion ; : 165-174, 1993.
Article in Japanese | WPRIM | ID: wpr-370786

ABSTRACT

The location of organs and tissues at the tip of acupuncture needle inserted in acupuncture points was studied on the human transverse sections.<br>A female, japanese, cadaver (139cm long, and 52kg in weight) was fixed with an aldehyde mixture and frozen for 3 weeks at -30°C. Then the cadaver was cut into blocks along the transverse planes perpendicular to the median plane at each point of Bladder meridian.<br>This report describes the depth of organs and tissues in 5 transverse planes from Hiyu (BL, 20 Bladder meridian) to KiKaiyu (BL, 24 Bladder meridian).<br>At Hiyu (BL, 20), acupuncture needle penetrates through 5mm thick skin, 4mm thick hypodermis, and 30mm thick deep back muscies in thick was respectively. The needle reches to the 12th rib at 39mm away from the surface.<br>At Iyu (BL, 21), the needle penetrates through the skin, hypodermis and deepback muscles of 5, 3, 28mm in thickness respectively. The needle reaches to the 1st lumber at 36mm away from the surface.<br>At Sanshoyu (BL, 22), needle pentrares through the skin hypodermis and deep back muscles of 5, 5, 26mm in thickness respectively. The needle reaches to the 2nd lumber at 36mm away from the surface.<br>At Jinyu (BL, 23), the needle penetrates through the skin, hypodermis and deep back muscles of 4, 5, 32mm in thickness respectively. The needle reaches to the 3rd lumber at 41mm away from the surface.<br>At Kikaiyu (BL, 24), the needle penetrates through the skin, hypodermis and deep back muscles of 4, 5, 30mm in thickness respectively. The needle reaches to the 4th lumber at 39mm away from the surface.<br>The depth of acupuncture points studies was discussed from the clinical points of view.

3.
Journal of the Japan Society of Acupuncture and Moxibustion ; : 125-134, 1993.
Article in Japanese | WPRIM | ID: wpr-370780

ABSTRACT

The location of organs and tissues at the tip of acupuncture needle inserted in acupuncture point was studied on the human transverse sections.<br>A female, Japanese, cadavar (135cm long and 52kg in weight) was fixed with aldehyde mixture and frozen from 3 weeks at -30°C. Then the cadaver was cut into blocks along the transverse planes perpendicular to the median plane at each point of Bladder meridian.<br>This report describes the depth of organs and tissues in 5 transverse planes from SHINYU BL 15 (Bladder meridian) to TANYU BL 19 (Bladder meridian).<br>At SHINYU BL 15, acupuncture needle penetrates through 5mm thick skin, 2mm thick hypodermis, 3mm thick trapezius, and 17mm thick deep back muscle in thick was respectively. The needle reaches to the 6th rib at 27mm away from the surface.<br>At TOKUYU BL 16, the needle penetrates through the skin, hypodermis, trapezius, and deep back muscles of 5, 3, 3, 17mm in thickness respectively.<br>The needle reaches to the 7th rib at 28mm away from the surface.<br>At KAKUYU BL 17, the needle penetrates through the skin, hypodermis, trapezius, latissimus dorsi, and deep back muscles of 4, 2, 3, 1, 20 in thickness respectively. The needle reaches to the 8th rib at 30mm away from the surface.<br>At KANYU BL 18, the needle penetrates through the skin, hypodermis and deep back muscles of 5, 6, 32mm in thickness respectively. The needle reaches to the 10th rib at 43mm away from the surface.<br>At TANYU BL 19, needle penetrates through the skin, hypodermis, and deep back muscles of 4, 7, 32mm in thickness respectively. The needle reaches to the 11th rib at 43mm away from the surface.<br>Depth of acupuncture points studies was discussed from the clinical points of views.

4.
Journal of the Japan Society of Acupuncture and Moxibustion ; : 271-280, 1991.
Article in Japanese | WPRIM | ID: wpr-370729

ABSTRACT

The location of organs and tissues at the tip of acupuncture needle inserted in acupuncture points was studied on the human transverse sections.<br>A female, japanese, cadaver (139cm long, and 52kg in weight) was fixed with an aldehyde mixture and frozen for 3 weeks at -30°C. Then the cadaver was cut into blocks along the transverse planes perpendicular to the median plane at each point of Bladder meridian.<br>This report describes the depth of organs and tissues in 5 transverse planes from DAITSUI (Governor vessel meridian) to KETSUINYU (Bladder meridian).<br>At DAITSUI, acupuncture needle penetrates through 7.5mm thick skin, 28.5mm thick hypodermis, and reaches to the transverse process of the 7th cervical vertebra at 36mm away from the surface. At DAIJO, the needle penetrates through the skin, hypodermis, trapezius, rhomboideus, and deep back muscles of 6, 8, 5.5, 10 and 14mm in thickness respectively. The needle reaches to the 2nd rib at 43.5mm away from the surface. At FUMON, the needle penetrates through the skin, hypodermis, trapezius rhomboideus, deep back muscles of 7, 5, 3.5, 4 and 18.5mm in thickness respectively. The needle reaches to the 3rd rib at 38mm away from the surface. At HAIYU, the needle penetrates the skin, hypodermis, trapezius, and deep back muscles of 6, 4, 3 and 22mm in thickness respectively, and reaches to the 4th rib at 35mm away from the surface. At KETSUINYU, the needle penetrates the skin, hypodermis, trapezius, and deep back muscles of 5, 3, 4 and 21mm in thickness respectively, and reaches to the 5th rib at 33mm away from the surface.<br>The depth of acupuncture points studies was discussed from the clinical points of view.

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