15:18,6,May,2007 | (1275/0/0) | Original

multiple gastrointestinal polyps syndrome

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Abstract principles of Cronkhite-Canada
Syndrome (CCS) of the clinical features, after advancing to the taste of Cronkhite-Canada syndrome. Cronkhite-Canada deposited Chen Style
Syndrome in 1 case, through the process of unity of history and endoscopic examination literature search of the disease to analyze the negotiations. Efficacy of endoscopic diagnosis of Cronkhite-Canada
Syndrome, endoscopic polypectomy coagulation, transurethral resection for symptomatic relief, but again broke out. Conclusion
The rare disease, of unknown etiology, clinical and ectoderm mainly gastrointestinal syndrome night shift for two years, and now there is no specific treatment style, the disease is pre-cancerous condition, requiring regular follow-up.
Words】 【minute cooling-off hair coloring gastrointestinal polyps (toe) A contraction syndrome
Case Report of Cronkhite Canada syndrome and Review of
Zhang Min, Xu Canxia, Xiao Dinghua

Department of Digestion, the 3rd Xiangya hospital, Central South
University, Changsha, Hunan, 410013

Abstract Objective To study
the clinical characters of Cronkhite-Canada
syndrome (CCS) and enhance our understanding of it.
Methods The history and endoscopy result of one
case with CCS was reported and discussed here with relevant
references. Results The case was diagnosed as CCS
by endoscopy and its symptoms were released after Polyps Endoscopic
electrocoagulation and Electricity Cut treatment, but relapses were
regular. Conclusion This unusual case was in
pre-cancerous state and without obvious etiology. Its mainly
clinical symptoms were Syndromes that have changes in
gastrointestinal tract and ectoderm. There is no particular
treatment for it and regular follow-up was

Key words
Cronkhite-Canada syndrome

Cronkhite-Canada syndrome, also known as the gastrointestinal tract polyps, cool colors, hair loss, a malnutrition syndrome, rare in the clinic, 1 patient admitted to our department,
Looking back memories of documents deposited unity Chen below.
A medical record
1.1 history
Patients, a chapter, male, aged 66, March 18, 2008 to December 25, 2008 because of diarrhea, anorexia, many stay in our department. Private prosecution in February 2008 compared with no incentive from diarrhea, anorexia, Nianye it was watery, yellow, 6 to 10 times / day, no pus and blood will, no heavier than the mitigation and capacity, each for 3 7 days symptoms self-improvement, 3 to 5 days after the symptoms and signs recurred, accompanied by diminished sense of taste, abdominal pain, retching, fatigue symptoms. No chills, fever, cold sweat. With previous history of chronic obstructive pulmonary disease. Physical examination: chronic disea
se tolerance, malnutrition, body weight loss, double superficial palmar skin, Queensland season (toe) Remote rough, hypertrophy distortion, no light, (toe) a proximal end department off.
Hand, foot, head and face and trunk skin pigment cool, dark brown, not Duan Fang shape, about 0.3cm ~ 2.0cm big and small. Strange body hair segment, yellow, armpit hair, pubic hair off Nianye sector. Swollen thyroid gland does not Nianye, Flying breath sounds low, does not smell of the mouth and more with the wet and dry rales, heart rate, Qi, no noise. Flat stomach, liver and spleen not palpable, shifting dullness negative. Since the onset, the spirit of poor patients, anorexia, weight loss, weight loss of about 10kg. No similar family history. Hospitalized patients with repeated several times, the care intestines, diarrhea, intestinal flora and swap down polyps colonoscopy transurethral electric coagulation in patients with symptoms after treatment can be improved. But restraint is not stable condition after discharge, the symptoms are still repeated outbreaks. Now the situation in patients with compact,
Still in follow-up.
Review the implementation of room search
Blood, red blood albumen said General Orders: 99g / L; liver effects shows white albumen 24.9g / L; immune C3 full display
0.6000g / l; big and small will be General Orders, kidney effects, C12, complete hepatitis, HIV antibody, syphilis antibody, a full set of hyperthyroidism, tuberculosis antibodies were negative; ESR, complete with lupus, rheumatoid connective bodies were no more with a full set of very; Nianye ratio of 4:6 will be the club flora; Nianye showed no fungal development will bring, no Vatican cassock coli and Shigella development. Nail fungus examination showed negative.
Imaging examination search
Full-thickness gastric barium meal reminded to consider hypertrophic gastritis or Menetrier's disease; colon and distal ileum abundance of small nodular defects, multiple polyps considered approximate. CT reminded the stomach lesser curvature of gastric body, gastric antrum, duodenal bulb and descending part of general thickening of the antrum compared with, reason reason unknown origin, consider the chronic inflammation; lymphoma?
1.4 endoscopic examination search
October 21, 2008 colonoscopy: into the mirror to the cecum, intestine scales of hundreds of pieces big and small set of diffuse divergent derivative, polyps of various shapes, endorsement congestive rough, since the beginning followed by ileocecal with 30W power down coagulation polyps more than 40 stars burning, wound white, surgery well, on the intestine 2.0 * 0.8cm long pedicle polyps Biopsy 2, authorities more brittle. (Figure a) Endoscopic diagnosis: multiple polyps in the colon after coagulation. Multiple colon polyps: Ca be deleted.
Figure I (colonoscopy): (omitted)
Figure I a before treatment (abbreviated)
Figure I b treatment (abbreviated)

October 23, 2008 endoscopy: gastric fundus, gastric body, the stomach and the gastric antrum, the gastric mucosa showed a bunch of grapes-like change, endorsed a high degree of edema, translucent, than the narrowed stomach, endoscopy can be well through the process of observation of gastric motility is not indecent freehand, no normal gastric mucosa, the gastric corpus biopsies 4, authorities crisp; H. Yuan, continuous open; bulb ball cavity were normal, the walls scattered diffuse diffraction big and small diameter of about 0.1cm ~ 0.2cm sessile polypoid bulge profiles congestion; descending descending seen scattered in the diffuse mucosal diameter of about 0.2cm ~ 0.3cm Yan big and small polyps, diffuse into the film department of Yan. (Figure b) endoscopic diagnosis: common gastric and duodenal mucosa see changes (CC syndrome)
Figure II (gastroscopy): (omitted)
1.5 Pathological examination search
Intestinal biopsy: Colonic tubular adenoma at 40cm from the incisors, mild epithelial dysplasia. Ileocecal appropriate inflammatory polyps, gonadal epithelial lesions by throwing reserved. Gastric biopsy: adenomatous polyps, Hp (-). Palm skin (pigment calm Office) mucosal biopsy: skin compound nevus, basal and cutting edge clean.
2 talks
2.1 Epidemiology
Cronkhite-Canada syndrome (CCS), also known as gastrointestinal polyposis - calm the skin pigment - hair loss - (toe) A contraction syndrome. Rarity of the disease, initially by Cronkhite and Canada [1] in 1955, deposited the first 2 cases of Chen,
1966 Jarnum and Jensen will be named the Cronkhite-intrinsic
Canada syndrome. According to statistics in 1985, Stone vine, the highest incidence rates in Japan, from 1955 to 1985 all the national total of CCS
154 deposited Chen, of which 110 cases in Japan [2], followed by North America and Europe, and its derivatives to diffuse public opinion and tooth density is proportional. Age 26 to 85 years, mean 61 years, about 80% of patients onset is over the age of 50 years. Europe no difference in incidence between men and women dressing Chen, but the literature in recent years, male to female ratio of 3:2 and Statistics [3]. Country for Yang Lisheng et al [4] deposited Chen 1 case. To 2008, 26 cases of domestic deposited Chen. Full-scale ban on the country reported a total of 387 cases in 2002,
One Nianyenianye all (about 75%) Man Yan in Japan [5-6].
2.2 Etiology
CCs of unknown etiology and pathogenesis, Nianye all of that is acquired, non-genetic diseases, broad and infection, lack of development factors, arsenic poisoning, immune disorder related to [7 -
8]. Spirit of the dying, fatigue is a common disease induced by the identity [3].
2.3 The emergence of clinical
CCS relatively acute onset, Nianye all patients from onset to diagnosis was 3 months to 1 year [9]. The clinical features of multiple polyps in the whole gastrointestinal tract, skin pigmentation associated with calm, hair loss, (toe) A contraction [1]. The cases were with the above characteristics. Clinical symptoms: about 92% of clinical patients with diarrhea, 80% of patients with abdominal pain, weight loss, almost all patients have [10]. The small intestine in patients with diarrhea and poor rustic disaccharidases, intestinal bacteria develop active immunity and malabsorption of fat and carbohydrate on. It has been reported many cases can be lax in the stool of patients with Clostridium difficile; mostly abdominal pain, severe abdominal pain often reminded continuously of gastrointestinal perforation, or intussusception [10]. Diminished sense of taste is also a common clinical symptom,
Nianye about 41% of patients endorsed this as the first [3]. The most characteristic signs of the disease changes for the ectoderm, such as (toe) A shrinking loss, hair loss and skin pigmentation calm
The incidence of the approximately 91%, 90% and 87% [10]. (Toe) A normal surface is rough, like atrophy, disappearance to light, deformation, softening, severe (toe) A fall off to reveal a bed. Nang past changes in the ectoderm that CCS is malabsorption secondary to gastrointestinal tract,
But the rhetoric found in patients with ectodermal changes much earlier in the gastrointestinal tract symptoms for weeks or even months,
It is not yet connected the two Nianye white. Some scholars think the broad changes in the ectoderm and the white of an egg lose quality and nutritional malabsorption related to the missing; However, some scholars feel that the ectoderm of the change is inherent in their emerging diseases, as in other diseases, much lower albumen and nutrient poor Patients did not show a similar surface ectoderm. Is,
Nianye scholars ectodermal lesions are the gastrointestinal tract polyps in parallel with the primary lesion [11]. Review the implementation of room search: low albumen hyperlipidemia is the emergence of a serious disease, Daniel deposited Chen 55 cases, 45 cases of lower plasma albumen, white of an egg white in 29 cases lower than 30g / L, 12   less than 20g / L. The sake of low-albumen hyperlipidemia reason more to do with the diarrhea is caused by the amount of albumen lost intestinal Nianye disappearance. Anemia, low albumen acidosis, hypokalemia, hypocalcemia, hypomagnesemia than common sector effect in patients with impaired immunity, blood albumen in the immunoglobulin IgG, IgM were lower than normal. Chen also apply the serum carcinoembryonic antigen (CEA) levels. Imaging change: search barium meal examination, the stomach with multiple nodular or polypoid abundant defects, big and small, ranging Nianye may to 3cm. Common mucosal fold thick Nianye film, and occasionally can be mistaken for Menetrier disease, that giant hypertrophic gastritis;; universality of the small intestine is emerging defects or polypoid mucosa, thick, rich, most of them within the duodenum. Barium double contrast barium enema or colon, rectum polyps common. Scales set by chance polyp profusion, so that the normal colorectal mucosa without visible cavity. Search endoscopic examination: polyps can diffuse from the esophagus to the rectum derivative of any part of the whole digestive tract, big and small, ranging up to Nianye by 2 ~ 3cm, pedunculated polyps can also sessile, profiles congestion erosion. Pathological examination search: polyps typical of a variety of pathological, to little children type (retention type) mainly hamartoma, adenoma is relatively rare, both can be muddy there. School change is a typical organ polyps epithelial cells and epithelial organs plague complete tortuous gland hyperplasia, cystic dilatation was some leakage of hyperthyroidism, containing white of an egg-like fluid or mucus. Lamina propria vascular congestion, and chronic inflammation; around the mucosal hyperemia, superficial erosion and infiltration of eosinophils. Little children adenomatous polyps may occur changes (mostly tubular adenoma), and even can be transformed into adenocarcinoma. Many case reports exist CCS combined with tumor, it was felt that CCS polyps roughly translates into the tumor [5]. Clinical classification: the Japanese scholar stone vine disease based on the intrinsic through, will be divided into 4. I type: the onset of diarrhea as a symptom; type: symptoms at all before the very first, taste; type: onset symptoms of hair loss, Zhao Jia atrophy; type: first, loss of appetite, general malaise, followed by emergence Zhao Jia atrophy, hair loss and taste it is, but no diarrhea. The type I patients should be grouped.
Diagnosis and differentiate diagnosis of CCS diagnosis based on the following: adult onset,
More common in men; no family history of polyps; the whole gastrointestinal tract of multiple polyps, emerged as diarrhea, abdominal pain with loss of appetite, anorexia, weight loss and other symptoms;
Ectodermal lesions such as skin pigmentation have a calm, hair loss, (toe) A shrinkage [12]. CCS differentiate between the main phase with the following diseases: 1) Peutz-Jeghers syndrome (
PJS), of course, have a cool skin pigmentation and gastrointestinal polyps, but the difference is between the two: PJS is an autosomal dominant genetic disease, most patients have family history;
PJS patients cool pigments appeared early after birth or in early childhood that occurs commonly in the lips, perioral, buccal mucosa, the accumulation of non-integration of the needles or mung bean brown spots on beating in the darkness;
Although PJS polyps for the whole gastrointestinal tract, but mainly the small intestine; PJS patients often intussusception,
Abdominal pain and intestinal obstruction [13]. 2) Menetrier's disease: changes in gastric mucosa with CCS is similar to have the white of an egg lost the characteristics of the missing enteropathy,
However, Menetrier's disease is limited to the stomach, not with ectodermal lesions.
In 26 cases, 2 cases were misdiagnosed as Menetrier's disease. Others, the disease to other family members need to distinguish between gastrointestinal polyps, such as children polyposis disease, family gonadal Liu Bing (FAP) and Gardner syndrome [10].
3 Treatment

CCS is no specific treatment style, the department by symptomatic and supportive treatment can ease the condition. Treatment approach is to correct the diarrhea and weight loss, improved ectoderm lesions. In the full nutritional support,
Maintain water and electrolyte balance have a foundation, according to the situation Lingding or solidarity glucocorticoid use (prednisone 40 mg / d), antibiotics (tetracycline, ampicillin, etc.)
Histamine H1 or H2 receptor antagonists, cromolyn sodium, etc.
Zinc supplementation for improving the effectiveness of good taste have diminished [14-15]. Ward et al [16] also proposed to accept H1 and H2 receptor antagonists, cromolyn sodium, prednisone and antibiotics in treatment of the disease solidarity,
Its efficacy needs further indecent observation. Not a relief for severe malabsorption complicated by gastrointestinal obstruction symptoms and overlay or thinking briefly suspected malignant polyps can be accepted surgery. Sulfasalazine in recent years, the line was applied anti-inflammatory treatment. Japanese scholars accept the high-energy therapy, efficacy must be achieved. Because of the small number of cases,
Now the style of various treatments are biographical in nature. Er Chen a case to apply the literature [13] alone can dispose of and nutrition support symptomatic relief of symptoms and reports of ectodermal lesions disappear, the mechanism of further study;
CCS also reminds us that the relief of clinical symptoms and intestinal polyps in parallel will not fade.
4 Prognosis

CCS progressive disease often develop a poor prognosis. 1982, Daniel et al [17] deposited Chen death rate of the disease pass away up to 55%. Patients often pass away in the CCS complications
Such as gastrointestinal bleeding, secondary infection, systemic failure, cachexia, congestive heart failure. Chadalavada [18] statistics were more than 300 cases, through treatment,
About 5% of the patient's condition can be fully mitigated. Ease on their own without treatment were only 5 cases reported in the literature. Course can be alternated with recurrence and remission. 50% to 60% of patients no matter what treatment to accept the style in the diagnosis of CCS
Death within 2 years pass away, as if a worse prognosis of women [19]. About 16.5% of colorectal cancer associated with CCS, Zugel so deposited in the diagnosis of CCS Chen
2 years after the development of colon cancer [20] the case, concluded in 1997 the country reported in the literature Nakatsubo CCS
280 cases, complicated by colorectal cancer accounted for 34 cases (12.4%), based on this disease as a pre-cancerous colon Nianye condition requiring regular follow-up.
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[3] Ward EM, Wolfsen HC. Review article: the non-inherited
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[4] Yang Lisheng, Wu Du, Yancy Hill, et al. Cronkhite2Canada syndrome. Journal of Internal Medicine, 1987, 27:
[5] Yashiro M, Kobayashi H, Kubo N, et al. Cronkhite-Canada
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[6] Ward EM, Wolfsen HC. Review article: the non-inherited
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[8] the money can be big. Cronkhite-Canada syndrome [J]. Chinese Journal of Internal Medicine, 2000, 20 (2): 80 -
[9] Goto A, Mimoto H, Shibuya C, et al.Cronkhite-Canada syndrome: an
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[10] Qiu Huizhong, Zhang Taiping, et al.
Cronkhite2Canada syndrome [J]. Journal of General Surgery, 2001,16 (7): 446
[11] Gan Ge, Xu Yan, et al. Polyps - pigmentation - hair loss - a malnutrition syndrome in 1 case [J]. Journal of Clinical Dermatology, 2006,
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[12] Wuxi Run, Wang Ling, Wang Qi, et al. Cronkhite-Canada syndrome - 1 case report and literature review of domestic [J].
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[16] Ward E, Wolfsen HC, Ng C. Medical management of
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multiple gastrointestinal polyps syndrome

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Cases of gastrointestinal polyposis syndrome cases outlined the reasons for the diagnosis and clinical diagnostic laboratory tests do not treat
This syndrome is mainly involved multiple colonic polyposis, most associated with intestinal manifestations. According to the degree of involvement of the gastrointestinal tract, with the extraintestinal manifestations, with or without genetic predisposition and polyps of different genetic approaches and the general performance of the histological classification. Can be divided into adenomatous and hamartomatous polyposis syndrome, two broad categories.
(A) of the adenomatous polyposis syndrome is characterized by multiple adenomas associated with high incidence of colon cancer. There are three:
1. Familial polyposis coli is an autosomal dominant genetic disease, occasionally with no family history. The whole colon and rectum may have multiple adenomas. Most pedunculated adenoma. Papillary rare. The number of polyps ranging from about 100 to thousands. Since the size of soybean to a few centimeters in diameter. Often arranged in dense, sometimes clusters, the same organizational structure and general adenoma. The disease is not limited to colon polyps, such as 22 cases of the disease in 15 cases with gastric polyps and were followed for 10 years found that 9 cases has been associated with duodenal polyps; also found that half of patients with skeletal abnormalities associated with , 13% had soft tissue tumors, suggesting that the disease and the relationship between Gardner syndrome. Onset usually in adolescence or young adulthood, the majority of 20 to 40 years of age to be diagnosed. A high degree of cancerous tendencies. It was reported that polyps occur in the first 5 years, cancer was 12% in 15 to 20 years is> 50%.
Most patients can be asymptomatic. The earliest symptoms of diarrhea, may also have abdominal cramps, anemia, weight loss and intestinal obstruction. The biopsy sigmoidoscopy general can be confirmed. Patients should be an early total colectomy and ileal - anal anastomosis or ileal - rectal anastomosis. Still need for regular colonoscopy after examination, such as the discovery of new polyps can be electrocautery treatment.
2. Gardner syndrome is a bone and soft tissue tumors associated with intestinal polyposis. Generally believed caused by the autosomal dominant inheritance, the nature and distribution of polyps and familial polyposis coli were similar, but fewer polyps (usually <100) larger. Tend to have a high degree of cancer, but the cancer later in some of the age. Osteoma mainly seen in the skull, mandible, sphenoid, and long bone. Soft tissue tumors can sebum cyst, lipoma, fibrosarcoma, leiomyoma and so on. In addition, these patients also had thyroid gland, adrenal gland, cancer of the tendency of duodenal ampulla.
The disease of bone and soft tissue tumors often occur before the intestinal polyps, it is found to have positive family history and physical examination of bone or soft tissue tumors, should be gut check.
Principle of treatment of this disease and familial polyposis same.
3. Turcot syndrome is a genetic disease, is rare. The characteristics of a patient's disease associated with familial adenomatous tumors of other organs, often accompanied by central nervous system tumors, such as the brain or spinal cord glioblastoma or medulloblastoma, glioma and therefore there polyposis syndrome, said. High rate of colorectal cancer adenomas have said often in the teen years has caused cancer deaths occurred.
(B) contains a set of hamartomatous polyposis syndrome disease, characterized by some organizations in some intestines without the involvement of a mixture of regular, but with non-neoplastic tumor-like proliferation of features.
1. Peutz-Jeghers syndrome (melanin spot - multiple gastrointestinal polyps syndrome) associated with the disease, Department of mucous membranes, skin pigmentation of the entire gastrointestinal tract of multiple polyposis. May be more than a single dominant gene inheritance. Explicit high rate of morbidity by the same family for many (50% of patients in the incidence of children), often before the age of onset of 10. Most polyps found in the small intestine, can cause bleeding and intussusception, may also have abdominal pain, diarrhea and protein losing enteropathy and so on. The nature of hamartoma polyps, intestinal polyps in patients with recent study found that 2% of the cancer rate. The age of these cancer often <35 years old, than the average age of onset of colorectal cancer as early as 10 years. Because hamartomas are often associated with adenomas or hamartomas coexist within the adenoma component, so cancer can not be sure from the hamartoma itself or adenoma. The incidence of intestinal cancer may be as high as 10% to 30%. Pigmentation more common in and around the lips, cheeks, face, finger skin, occasionally in the mucosa, but there are limitations of pigmentation in the trunk and limbs were. Pigment can be presented black, brown, gray, blue and other colors. Only a very small number of patients without intestinal polyps pigmentation. As extensive lesions of the disease, the general be symptomatic treatment only in serious complications such as bleeding or obstruction can not control before considering surgery. Polyps removed during surgery as far as possible.
2. Polyps in juvenile polyposis syndrome is most common in the rectum, but can occur throughout the colon, occasionally in the stomach and small intestine.
Polyposis may include the following three:
(1) juvenile polyposis coli (juvenile polyposis coli, JPC): The average age of onset is 6 years old. No family history. The main clinical manifestations are gastrointestinal bleeding, often accompanied by anemia, hypoproteinemia, malnutrition and growth retardation. Often accompanied by congenital malformations, such as intestinal malrotation, umbilical hernia, and cerebral edema. With adenomatous polyps exist.
(2) familial juvenile polyposis coli (familial juvenilepolyposis coli, FJPC): family history, the Department of autosomal dominant inheritance. Symptoms rectal bleeding, rectal prolapse, and growth retardation is common. Most patients with polyps showed the typical features of hamartoma, but a few in combination adenomatous polyps. There may be malignant.
(3) systemic familial juvenile polyposis (familialgeneralized juvenile polyposis, FGJP): there is genetic. In addition to colorectal polyps, there stomach or jejunal polyps, either alone or co-exist with colorectal polyps, some patients combined or alone stomach, duodenum, pancreas, or colon cancer. Some people think that the disease may be the same with the FJPC disease.
3. Cronkhite-Canada syndrome, previously considered to be adenomatous polyps are considered to be hamartomatous in nature. The main features are: â‘  the gastrointestinal tract have polyps; â‘¡ ectodermal changes, such as hair loss, nail dystrophy and pigmentation, etc.; â‘¢ no family history of polyps; â‘£ adult disease.
The most
common symptom was diarrhea, seen in more than 80% of cases, large bowel, and blood fat, or the naked eye, most patients have significant weight loss, followed by abdominal pain, anorexia, fatigue, vomiting, decreased sexual desire and taste. Almost always (toe) A change in hair loss, pigmentation. Laboratory tests have anemia, hypoalbuminemia, malabsorption and electrolyte disturbances. There may be malignant, although the survival of more than 10 years after treatment case report of severe illness, but in general the disease and poor prognosis.
Treatment is symptomatic treatment, rehydration, supplementary nutrients and maintain water and electrolyte balance, a small number of patients with corticosteroids, anabolic hormones, antibiotics and surgical removal of large bowel to ease the condition. But it is generally only apply to serious surgical complications, such as heavy bleeding, prolapse, intussusception, intestinal obstruction and bowel disease was malignant or shorter.
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