• 한국임상수의학회지
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• 제28권2호
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• pp.249-253
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• 2011

4살령의 아메리칸 코가 스파니엘이 식욕부진, 몸떨림 그리고 복부 팽만증세로 내원하였다. 혈청학적 검사, 방사선 검사, 초음파 검사 그리고 간생검을 통한 조직병리학적 검사를 통해 경화로 진행된 만성 간염으로 진단할 수 있었으며, 특수 염색법을 통해 대부분의 간세포내에 구리가 축적되어 있는 것을 확인할 수 있었으며, 구리 농도는 1460 ppm으로 매우 높음을 알 수 있었다. 이러한 검사 결과를 바탕으로 구리 축적으로 인한 간염이 간경화까지 진행된 말기 병변으로 진단하고 D-penicillamine, s-adenosymethionine, biphenyl-dimethyl-dicarboxylate과 대증 치료로 본 환자를 치료 하였으며, 본 환자는 진단 후 35개월을 생존하는 것에 성공하였다.

• Bulletin of the Korean Chemical Society
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• 제24권3호
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• pp.350-356
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• 2003

The mechanism of the decomposition of a bronchodilator, S-nitroso-N-acetyl-D,L-penicillamine (SNAP) by a bronchoconstrictor, aqueous sulfite, has been investigated in detail. The decomposition was studied using a conventional spectrophotometer at 336 nm over the ranges: 0.010 ≤ $[S^{IV}]_T$ ≤ 0.045 mol $dm^{-3}$, 3.96 ≤ pH ≤ 6.80 and 15.0 ≤ θ≤ 30.0 ℃, 0.60 ≤ I ≤ 1.00 mol $dm^{-3}$, and at ionic strength 1.00 mol $dm^{-3}$ (NaCl). The rate of reaction is dependent on the total sulfite concentration and pH in a complex manner, i.e., $k_{obs}\;=\;k_1K_2[S^{IV}]_T/ ([H^+]\;+\;K_2)$. At 25.0 ℃, the second order rate constant, $k_1$, was determined as $12.5\;{\pm}\;0.15\;mol^{-1}\;dm^3\;s^{-1}$. ${\Delta}H^{neq}\;=\;+32\;{\pm}\;3 kJ\;mol^{-1}\;and\;{\Delta}S^{\neq}\;=\;-138\;{\pm}\;13\;J\;mol^{-1}K^{-1}$. The N-nitrosohydroxylamine-N-sulfonate ion was detected as an intermediate before the formation of any of the by-products, namely, N-acetyl-D,L-penicillamine. The effect of concentration of aqueous copper(Ⅱ) ions on this reaction was also examined at pH 4.75, but there was no dependence on $[Cu^{2+}]$. In addition, the $pK_a$ of SNAP was determined as 3.51 ± 0.06 at 25.4 ℃ [I = 1.0 mol $dm^{-3}$ (NaCl)].

• 한국환경보건학회지
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• 제17권2호
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• pp.95-101
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• 1991

Lead acetate solution were intraperitoneally injected to rats of average body weight 220g to cause lead poisoning. Antidotes were orally administered using catheter to the rats and the urine was collected after 24 hours. ALA in the urine were determined by U. V. spectrophotometer. Antidotes used in this experiment were the water extracts of scutellaria radix and D-penicillamine solution. ALA in the urine was determind by Wada method. It was found that ALA content in the urine was decreased after oral administration of water extracts of scutellaria radix and D-penicillamine solution. In this study it is believed that water extract of suctellaria radix could be used as an excellent antidate in lead poisoning.

• Journal of Preventive Medicine and Public Health
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• 제11권1호
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• pp.76-82
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• 1978

In order to study the change of laboratory parameters of lead poisoning, 8 persona who had not been treated previously for lead poisoning (Group 1 and 6 persons who had been inadequately treated for few months for chronic lead poisoning at local clinic (Group 2) were examined. They had occupational exposure to lead for 3 to 18 years (mean, 7.6). In group 1 blood lead, urine lead, urine coproporphyrin and ${\delta}$-aminolevulinic acid levels before our treatment exceeded the critical levels of lead poisoning. In group 2 urine lead level exceeded but blood lead, urine coproporphyrin and ${\delta}$-aminolevulinic acid levels were within normal limits. All of them were treated with D-penicillamine for 4 months as inpatients at Industrial Accident Hospital. The dose of D-penicillamine was the same in all patients; 600 mg per day p.o. and the chelating agent was administer every other week. For laboratory analysis, 24 hour urine and 10 gm of whole blood were collected every 1 month on last day of non-administration period. The results were as follows: 1. It was found that urine lead level was decreased below the cirtical level of lead poisoning after 4 month's treatment with D-penicillamine and blood lead level was decreased more progressively below the critical level after 1 month treatment. 2. Urine coproporphyrin and ${\delta}$-aminolevulinic acid levels were decreased progressively to normal range after 1 month treatment. 3. Two months after treatment, blood lead, urine lead, urine coproporphyrin and ${\delta}$-aminolevulinic acid levels showed some increasing trends. 4. Urine lead level should be checked in a person who had been inadequately treated with chelating agents because blood lead, coproporphyrin and ${\delta}$-aminolevulinic acid might be in normal range.

• Bulletin of the Korean Chemical Society
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• 제11권4호
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• pp.281-286
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• 1990

The kinetics and mechanism of reduction of Cu(II) with an excess D-penicillamine have been examined at pH = 6.2 and 0.60M in ionic strength. The reaction at the initial stage is biphasic with a rapid complexation process to give "red" transient complex of $[Cu(II)(pen)_2]^2$- that is partially reduced to another transient "brown" intermediate. The "brown" intermediate is finally reduced to diamagnetic "yellow" complex, $[Cu(I)(Hpen)]_n$. The final reduction process is pseudo-first order in ["brown" transient] disappearance $with {\kappa} = {{\kappa}_{3a} + {\kappa}_{3b}[pen]^{2-}},$ where ${\kappa}_{3a} = (5.0{\pm}0.8){\times}10^{-3}sec^{-1}$ and ${\kappa} = (0.14{\pm}0.02) M^{-1}sec^{-1}$ at $25^{\circ}C$. The activation parameters for the $[H_2pen]$-independent and $[H_2pen]$-dependent paths are ${\Delta}H^{\neq} = (52{\pm}5)kJmol^{-1},$ and ${\Delta}S^{\neq} = ( - 27{\pm}3)JK^{-1}mo^{l-1},$ and ${\Delta}H^{\neq} = (56{\pm}2)kJmol^{-1}$ and ${\Delta} S^{\neq} = ( - 18{\pm}0.7)JK^{-1}mol^{-1}$ respectively. The nature of "brown" intermediate is not clearly identified, but this intermediate seems to be in the mixed-valence state, judging from the kinetic and spectroscopic informations.

• Archives of Pharmacal Research
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• 제26권1호
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• pp.24-27
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• 2003

A furanofuranoid lignan glycoside, with radical scavenging on peroxynitrite, total reactive oxygen species (ROS) and 1, 1-diphenyl-2-picrylhydrazyl (DPPH) radical, was isolated from the rhizome of Salvia miltiorrhiza and characterized as (+)-1-hydroxypinoresinol-1-Ο-$\beta$-D-glucoside based on spectroscopic evidence. The compound exhibited peroxynitrite, total ROS and DPPH radical scavenging activities with $IC_{50}$ values of 3.23$\pm$0.04, 2.26$\pm$0.07 and 32.3$\pm$0.13 $\mu$M, respectively. Penicillamine, Trolox (6-hydroxy-2,5,7,8-tetramethylchroman-2-carboxylic acid) and L-ascorbic acid, acting as positive controls, showed radical scavenging activities with $IC_{50}$ values of 6.72$\pm$0.25, 1.43$\pm$0.04 and 11.4$\pm$0.07 $\mu$M, respectively.

• Journal of Yeungnam Medical Science
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• 제40권3호
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• pp.311-314
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• 2023

Thallium poisoning is usually accidental. We present a case of a 51-year-old woman who was evaluated in June 2018 for myalgia, vertigo, asthenia, and abdominal pain. Physical examination revealed temporal-spatial disorientation, jaundice, and asterixis. The laboratory reported the following: bilirubin, 10.3 mg/dL; aspartate transaminase, 78 U/L; alanine transaminase, 194 U/L; albumin, 2.3 g/dL; prothrombin time, 40%; and platelet count, 60,000/mm³. Serology performed for hepatitis A, B, and C; Epstein-Barr virus; cytomegalovirus; and human immunodeficiency virus was negative, and a collagenogram was negative. Physical reevaluation revealed alopecia on the scalp, armpits, and eyebrows; macules on the face; plantar hyperkeratosis; and ulcers on the lower limbs. Tests for lead, arsenic, copper, and mercury were carried out, which were normal; however, elevated urinary thallium (540 ㎍/g; range, 0.4-10 ㎍/g) was observed. The patient was treated with D-penicillamine 1,000 mg/day and recovered her urinary thallium levels were within normal range at annual follow-up. Thallium poisoning is extremely rare and can be fatal in small doses. An adequate clinical approach can facilitate early diagnosis.

• 동의생리병리학회지
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• 제16권5호
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• pp.1066-1069
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• 2002

Wilson's disease is a rare inborn error of metabolism inherited as a autosomal recessive trait. The disease has varied mode of manifestations. It is characterized by different neurologic disorder and hepatic disease. I experienced a case of Wilson's disease in 40 year old woman who was suffered from liver cirrhosis, severe anorexia, and classical neurologic symptoms such as tremour, dysarthria and ataxia. The symptoms was not relieved by D-penicillamine, Youngyanggaksan and Samchulgunbi-tang but anorexia was improved significantly by same medication.

• Childhood Kidney Diseases
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• 제27권2호
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• pp.127-132
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• 2023

Cystinuria, a genetically inherited disorder, is a rare cause of kidney stones. It is characterized by impaired transport of cystine and amino acids in the proximal renal tubule and the small intestine. Most patients develop cystine stones throughout their lifetime. Recurrent renal stones need to be extracted by repeated urologic interventions. Treatment options of cystinuria for preventing stone recurrence are limited and poorly tolerated. In this study, we report a pediatric case of cystinuria with a heterozygous SLC3A1 mutation diagnosed by stone analysis, measurement of urine cystine excretion, and genetic analysis. There were recurrent renal stones despite repetitive shock wave lithotripsy and retrograde intrarenal surgery. However, the rate of stone formation seemed to be slower after D-penicillamine was added into adequate hydration and urinary alkalinization.

• 대한소아치과학회지
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• 제32권2호
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• pp.256-261
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• 2005

피부 경화증(scleroderma)은 만성 결체조직 질환으로 정확한 원인은 밝혀지지 않았으나 자가 면역 질환으로 알려져 있다. 대개 $30{\sim}50$세의 여성에서 호발하며 소아에서는 매우 드물다. 장기의 이환여부에 따라 국소성과 전신성 피부 경화증으로 분류할 수 있다. 국소적 형태는 예후가 양호하고 피부에만, 또는 피하 근육까지 이환되며, 내부 장기에는 이환되지 않는다. 전신적인 형태는 피부와 구강점막 및 위장계, 호흡계, 심혈관계 등을 포함하늘 전신 다발적 발생 (multisystemic involvement)이 특징적이다. 안면피부 이환시 작고 예리한 코, 무표정한 응시 (expressionless stare), 좁아진 입모양(narrow oral aperture) 등이 특징적이다. 대개 레이노 현상(Raynaud's phenomenon)이 존재하며 질환이 진행되면서 개구장애와 혀, 치은의 경화가 나타나게 된다. 피부 석회증(calcinosis cutis), 레이노현상(Raynaud's phenomenon). 식도 기능부전(esophageal dysfunction), 공지증(sclerodactyly), 모세혈관 확장증(telangioectasia)이 복합된 질환을 크레스트 증후군(CREST syndrome)이라 부른다. 피부 경화증의 치료는 국소적 및 전신적 스테로이드 치료, collagen cross-link inhibitor (D-penicillamine), 면역억제제 등을 사용한다. 개구제한의 치료는 설압자를 이용한 mouth stretching exercise를 통해 개선될 수 있다. 본 증례에서는 국소성 피부 경화증으로 진단되고 개구제한이 있는 6세 남아에게 아산화질소와 enflurane을 이용한 깊은 진정요법을 이용하여 상하악 제 1, 2 유구치의 우식치료 치험례를 보고한다.