• Title/Summary/Keyword: 구루병

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A Case of Proximal Renal Tubular Acidosis Accompanied by Vitamin D Deficient Rickets (비타민 D 결핍성 구루병에 동반된 근위 신 세뇨관성 산증 1례)

  • Kwon Seung-Yeon;Choi Youn-Jung;Kim Kee-Hyuck
    • Childhood Kidney Diseases
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    • v.10 no.1
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    • pp.58-64
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    • 2006
  • In the clinical state of vitamin D deficiency, it is possible that associated phosphate depletion, parathyroid hormone excess, and hypocalcemia may all depress the proximal tubular reabsorption of bicarbonate, in addition to abnormal skeletal modeling or remodeling, Although nutritional rickets is considered a rare disease in developed countries nowadays, cases of vitamin D deficient rickets caused by various unhealthy lifestyles such as insufficient exposure to sunlight, breast feeding infants without giving vitamin D supplements, unbalanced vegetarian diets of breast feeding mothers, low-birth weight, and maternal deficiency of vitamin D or calcium are increasing. Here, we present the case of an 8 month old girl, who was completely breastfed without any weaning diet or infant vitamin supplements. She visited our emergency room with hypocalcemic seizure and subsequently was diagnosed with vitamin D deficient rickets accompanied by overt bone changes and proximal renal lobular acidosis. After intravenous(IV) and oral calcium replacement therapy(IV calcium gluconate injection 1 mEq/kg/day for 6 days, 2 mEq/kg/day for 4 days followed by oral calcium gluconate administration 4 g/day for 3 days) with vitamin D supplement(Alfacalcidol 0.5 mcg/day) during admission, serum calcium level was normalized with clinical improvement. Oral sodium bicarbonate(0.6 g/day) was administered from the $2^{nd}$ hospital day for 2 weeks, which normalized the serum bicarbonate(measured by $tCO_2$) level. Calcium and vitamin D replacement were continued for 2 weeks and 3 months each. After discontinuing medications, follow up laboratory findings showed good maintenance of serum calcium, alkaline phosphate and bicarbonate levels with complete improvement of bone X-ray findings.

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A Case of Hypophosphatemic Rickets associated with Epidermal Nevus Syndrome (저인산혈증성 구루병을 동반한 표피모반증후군 1례)

  • Lee Yong Joo;Kang Ju Hyung;Lee Soo Jin;Park Ho Jin;Shin Choong Ho;Cheong Hae Il
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.263-268
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    • 2005
  • Epidermal nevus syndrome is a rare disease consisting of epidermal nevus and multisystem pathologic conditions associated with anomalies in the central nervous system, bone, eye, heart, vasculature and genito-urinary system. Hypophosphatemic rickets has been rarely observed in association with this syndrome. We report a case of hypophosphatemic rickets as sociated with epidermal nevus syndrome with review of the literature. (J Korean Soc Pediatr Nephrol 2005;9:263-268)

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Clinical Study of Hypophosphatemic Rickets (저인산혈성 구루병에 대한 임상적 고찰)

  • Lee Chang-Jin;Cho Hee-Yeon;Kang Ju- Hyung;Shin Choong-Ho;Ha Il-Soo;Cheong Hae-Il;Yang Sei-Won;Choe Yong
    • Childhood Kidney Diseases
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    • v.8 no.2
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    • pp.195-204
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    • 2004
  • Purpose: Hypophosphatemic rickets is a hereditary disease, characterized by hypophosphatemia due to renal phosphate wasting, impaired renal production of 1,25-dihydroxyvitamin $D_3$, rachitic bone deformities and impaired growth. The purpose of this study is to provide clinical profiles of patients with hypophosphatemic rickets in our hospital. Methods: Between July 1983 and February 2004, 56 patients were diagnosed as having hypophosphatemic rickets. The medical records of these patients were reviewed retrospectively. Clinical manifestations, family histories, laboratory data, treatment outcomes were described. Results: Fifty six patients were enrolled in this study. The average age at symptom onset and diagnosis were 20 months and 5 years respectively. Fourteen patients had family histories. The main clinical manifestations were bow legs and short stature. There was a significant negative correlation between the ages and the height z-scores at the time of diagnosis(r=-0.47, P=0.005). Initial laboratory data showed normocalcemia, hypophosphatemia, elevated serum alkaline phosphatase, decreased tubular reabsorption of phosphate and a normal range of 1,25-dihydroxyvitamin $D_3$ Radiographic examinations of bone revealed fraying, widening and cupping of the metaphyseal ends. Treatment consisted of Joulie solution and vitamin D metabolites, and resulted in improved biochemical and radiographic findings. However, height z-scores remained essentially unchanged(P=0.224). Complications of treatment were frequently observed, including hyperparathyroidism, nephrocalcinosis, and hypercalciuria. Sixteen patients had corrective osteotomy and 4 of them underwent leg lengthening together. Conclusion: There was a gap of several years between the onset of symptoms and the diagnosis. Early treatment seems to be essential to growth. For the earlier treatment, the offsprings of affected parents should be followed up closely.

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Subclinical rickets in breastfed infants (모유 수유아에서의 무증상적 구루병)

  • Park, Sin Young;Park, Sung Woo;Kang, Sung Kil;Jun, Yong Hoon;Kim, Soon Ki;Son, Byong Kwan;Lee, Jee Eun
    • Clinical and Experimental Pediatrics
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    • v.50 no.12
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    • pp.1188-1193
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    • 2007
  • Purpose : The prevalence of rickets in the world is on the rise not only in developing but also in developed countries. In Korea, breastfeeding has increased. There have been few studies on the possible association of rickets with breastfeeding. The purpose of this study was to identify the development and the clinical presentation of subclinical rickets in breastfed infants. Methods : We investigated patients who were breastfed and had hypovitaminosis D in the blood from May 2006 to April 2007, and who were diagnosed with vitamin D deficient rickets from May 2003 to April 2006. We evaluated the results of blood tests, x-rays and other relevant information in the medical record. A questionnaire that included questions on the diet of patients, the mothers activity during pregnancy and place of residence was administered. Results : Twelve patients (66%: male, 34%: female) were enrolled in this study. There were eight in the asymptomatic and four in the symptomatic group. The median age for each group was 8 months (range 4-11 month) and 5.5 months (5-8 month). All patients in the symptomatic group were breastfed until diagnosed. In the asymptomatic group, they were breastfed for four to six months, and then weaned with only thin rice soup and vegetables. Nine patients had a vitamin D concentration below 20 ng/mL and three patients had levels between 20 and 29 ng/mL. Alkaline phosphatase (ALP) and parathyroid hormone (PTH) were elevated in both groups. There were statistically significant $25-OHD_3$ levels in the blood in both groups (P=0.008). Ten of the patients (83%) also had iron deficient anemia. Conclusion : Vitamin D deficiency and subclinical rickets has been identified in Korea. However, the prevalence of this disease has not been determined. The main limitation of this study was the small number of patients and the absence of a control group.

흡수불량증후군

  • 윤현중
    • Monthly Korean Chicken
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    • v.63 no.57 s.123
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    • pp.118-121
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    • 2005
  • 육계농장을 방문할 때마다 자주 듣는 이야기가 병아리가 층아리가 많다, 이번 파스는 적다라는 이야기다. 육계에서 층아리를 발생시키는 대표적인 질병이 흡수불량증후군이다. 흡수불량증후군은 빈혈이 도는 닭, 황색 분변, 장내용물고 분변내에 소화가 되지 않은 사료가 보이며 비정상적인 날개(헬리콥터병). 위축, 구루병, 대퇴골두괴사증, 근위염 등의 질병이 한가지 이상 또는 복합적으로 나타나는 질병으로 주로 육계나 백세미에서 증체 지연과 사료효율 감소 등으로 인한 피해를 일으키는 질병이다. 흔히 말하는 층아리 발병이 많게 되는 질병이라 하겠다. 이 병은 레오바이러스, 곰팡이 독소, 아데노바이러스, 엔테로바이러스, 파보바이러스, 혐기성 미생물과 여러 밝혀지지 않은 유전적인 요인까지 복합적으로 원인체가 작용하는 질병이다. 따라서 그 원인을 밝히기가 어려우며, 명확한 대책의 수립도 쉽지 않은 질병이다. 그러나 이 중에서 개연성이 높은 질병인 레오바이러스 감염증을 위주로 해결책을 찾아보기로 하겠다.

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A Case of Rickets Developed after Feeding on Sunsik for Seven Months (7개월간 선식만 먹인 영아에서 발생한 구루병 1례)

  • Yang, Jeong-A;Jang, Kyung-A;Park, Hye-Won;Jang, Wook;Han, Man-Yong;Cho, Young-A;Lee, Eun
    • Clinical and Experimental Pediatrics
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    • v.46 no.11
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    • pp.1143-1146
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    • 2003
  • Rickets is a nutritional disorder which is caused either by deficiency of vitamin D or by a defective activation of vitamin D. In these days, even though the incidence of rickets has decreased through adequate nutritional support, we sometimes experience rickets in babies receiving a prolonged special diet as therapy for chronic diarrhea, or those subject to a in receiving the prolonged elimination of milk because of allergy. But there are no reports about rickets caused by absolute elimination of milk because of allergies in Korea. We report here a case of rickets developed after feeding on Sunsik( a mixture of several grain and fruits powder) during a seven months period in an 8-month-old male patient. This male infant manifested vomiting, poor feeding, decreased serum calcium and 25-hydroxycholecalciferol levels, and markedly increased serum alkaline phosphatase and parathyroid hormone levels. Skeletal X-rays showed cupping and fraying in distal metaphyses of radius and ulna, and generalized osteopenia. The patient improved with vitamin D and calcium therapy.

A Case of McCune-Albright Syndrome with Vitamin D Resistant Rickets (비타민 D 저항성 구루병을 동반한 McCune-Albright 증후군 1례)

  • Kang Hee;Choe Jeong-Hoon;Hong Young-Sook;Lee Joo-Won;Kim Soon-Kyum;Yoo Kee-Hwan
    • Childhood Kidney Diseases
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    • v.3 no.2
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    • pp.237-240
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    • 1999
  • The presence of polyostotic fibrous dysplasia of bone, hyperpigmented skin macules, and precocious sexual development in children is known as the McCune-Albright syndrome. In addition to the described in McCune-Albright syndrome, other endocrinopathies have been reported including hyperthyroidism, acromegaly, Cushing syndrome and vitamin D resistant rickets. The case describes a 6-year-old boy showing bony deformities due to polyostotic fibrous dysplasia, hyperpigmented skin macules, hyperthyroidism and vitamin D resistant rickets. The purpose of this report is to describe a patient of McCune-Albright syndrome with vitamin D resistant rickets which is very rare.

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HISTOPATHOLOGIC STUDY ON TEETH AND TIBIAL BONE OF THE EXPERIMENTAL RICHATIC RATS (실험적(實驗的) 구루병(病) 백서(白鼠)의 치아(齒牙) 급(及) 경골(脛骨)에 관(關)한 병리(病理) 조직학적(組織學的) 연구(硏究))

  • Oh, Chang-Min;Cho, Young-Pill
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.15 no.2
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    • pp.137-145
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    • 1993
  • The purpose of this study was to observe the effect on teeth and tibia bone histopathologically in rats by the richitogenic diet. For this purpose, 48 sprague-dewley rats, weighing 80g or more, divided into 6 groups, and sacrificed on the 1st, 2nd, 3rd, 4th, 5th, 6th weeks after experiments respectively. The tissues contain tooth, and tibial bone were fixed in 10% normal formalin solution, decalcified in Plank-Rychlo solution, embedded in paraffin, sectioned in $6-8{\mu}$ as usual manner, the tissues were stained in hematoxylin eosin, and examined hisopathologically. Follow results were attained 1. Pyknotic appearance of odontoblastic layer was noticed on the 1st week, and increased on the 2nd weeks, and it is appeared that degeneration and dearrangements of odontoblasts on the 4 weeks. But a little recovered on the 6th weeks. 2. The thickness of predentin layer was increased on the 2nd weeks, and increased remarkedly on the 3rd weeks and reached the maximum on the 5th weeks. 3. The interglobular dentin was appeared in spotty shape on the 2nd weeks, and increased on the 4th, 5th, weeks, and large amount of inter-globular dentin was appearanced on the 6th weeks. 4. It is appeared that enamel hypoplasia on the 4th weeks. 5. Epiphyseal and metaphyseal plate of tibia was increased from/on the 2nd weeks, increased maximumly on the 5th weeks. And pyknotic, dearrangements, and hyperchromatic appearances of chondrocytes on the plate were increased on the 1st week.

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Effect of irradiation on wound healing after tooth extraction in the rachitic rats (방사선조사가 구루병 백서의 발치창 치유에 미치는 영향)

  • Kim Mee-Kyung;Hwang Eui-Hwan;Lee Sang-Rae
    • Imaging Science in Dentistry
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    • v.32 no.1
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    • pp.1-10
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    • 2002
  • Purpose: To observe the histopathological changes following irradiation on the wound healing after tooth extraction in the rachitic rats. Materials and Methods: In order to carry out this study, the rats were divided into four groups: Group 1 (normal diet/non-irradiation group), Group 2 (normal diet/irradiation group), Group 3 (rachitogenic diet/non-irradiation group), and Group 4 (rachitogenic diet/irradiation group). Rachitic changes were induced with rachitogenic diet No. 2 (high calcium, low phosphorus, and Vitamin D deficient diet) for 5 weeks. After the extraction of both maxillary first molars of the rats in Group 2 and 4, the head and neck of the rats were irradiated with single absorbed dose of 10 Gy. The rats were sacrificed at the 1st, 5th, 10th, and 15th day after tooth extraction. The specimens including the extraction wound were sectioned, stained with the hematoxylin-eosin and Masson's trichrome method and examined under the light microscope. Results: In the Group 2, the amount of newly formed bone trabeculae on the periphery of extraction socket and osteoblastic activity were reduced. In the Group 3, epithelial fusion was not revealed on the 5th day after toothe extraction and growth rate of osteoid formation was reduced. In the Group 4, necrotized tissue at the outer surface of extraction socket and destructive changes on the alveolar bones were noted on the 10th day. Epithelial fusion was not revealed and large amounts of osteoclast were noted on alveolar bone on the 15th day. Conclusion: The healing process of wound after tooth extraction was retarded by irradiation and especially in the rachitic rats.

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Rickets caused by vitamin D deficiency in calves (송아지에서 비타민 D 결핍에 의한 구루병 증례 보고)

  • Kim, Ji-Hyeon;Lee, Kyunghyun;Jung, Ji-Youl;Yang, Siyeon;Lee, Hyunkyoung;So, ByungJae;Choi, Eunjin
    • Korean Journal of Veterinary Service
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    • v.40 no.4
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    • pp.281-285
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    • 2017
  • Six to seven-month-old 10 beef calves in 140-head beef cattle farm in Gimpo-si, Gyeonggi-do were suffered from ataxia and astasia. Two of them were submitted to Animal and Plant Quarantine Agency for diagnosis in July 9, 2015. At necropsy, the radius and tibia of both calves showed irregular thickening and hemorrhage of the physis in addition to hemorrhage and disruption of trabeculae in metaphysis. Histopathologically the physis showed irregular tongues of cartilage remaining the metaphysis. Disorganization, hemorrhage and fibrosis were observed in metaphyseal and epiphyseal bones. The serum 25-hydroxyvitamin $D_3$ ($D_3$) and calcium levels were lower than normal range. In addition, the serum $D_3$ levels in the other 9 calves of the same farm were lower than normal levels. Based on the pathological examination and serum chemistry measurements, we diagnosed this case as rickets caused by vitamin D deficiency in calves.