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Immunotherapy withdrawal by step‐down to mesalamine in pediatric patients with ulcerative colitis 儿科溃疡性结肠炎患者逐步停用美沙拉嗪后的免疫疗法
Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12048
R. Szigeti, R. Kellermayer
Parents and pediatric patients with ulcerative colitis (UC) who progressed to systemic immunotherapy are concerned about lifelong risks from such treatments. There is limited knowledge about withdrawal of such agents and step‐down (SD) to enteral 5‐aminosalicylic acid (mesalamine) before transitioning to adult care.We studied nine pediatric cases with moderate to severe UC who after a median of 2.18 years of clinical remission on systemic immunotherapy stepped down to oral mesalamine treatment.Average follow‐up time from SD was 3.49 years. Five patients (55.5%) had sustained remission (without any flare noted) after SD during follow‐up. Sustained clinical remission was 88.9% (8/9) at 1 year, 87.5% (7/8) at 2 years, and 66.7% (4/6) at 3 years after SD. Out of those tested (one patient was not tested), 62.5% (5/8) had fecal calprotectin <50 μg/g. Four out of six patients examined (66.6%) had mucosal healing on post‐SD colonoscopy.We propose that SD to mesalamine can be a reasonable therapeutic consideration for pediatric patients with UC before transitioning to adult gastroenterology care. Shared decision‐making is important before such treatment changes.
家长和接受全身免疫疗法的溃疡性结肠炎(UC)儿科患者担心这种治疗会带来终身风险。我们研究了 9 例中度至重度 UC 儿童患者,他们在接受全身免疫疗法临床缓解中位数为 2.18 年后,转而接受口服美沙拉明治疗。从SD开始的平均随访时间为3.49年。随访期间,有5名患者(55.5%)在SD后获得了持续缓解(无任何复发迹象)。SD后1年的持续临床缓解率为88.9%(8/9),2年为87.5%(7/8),3年为66.7%(4/6)。在接受检查的患者中(一名患者未接受检查),62.5%(5/8)的粪便钙蛋白<50 μg/g。在接受 SD 后结肠镜检查的六名患者中,有四名患者(66.6%)的粘膜已经愈合。我们建议,对于患有 UC 的儿科患者,在过渡到成人胃肠病治疗之前,将 SD 转为美沙拉明是一种合理的治疗考虑。在改变治疗方案之前,共同决策非常重要。
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引用次数: 1
Invited commentary to immunotherapy withdrawal by step‐down to mesalamine in pediatrics patients with ulcerative colitis 应邀评论儿科溃疡性结肠炎患者逐步停用美沙拉嗪后的免疫疗法问题
Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12047
Daniel M. O'Connell, Jonathan Moses
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引用次数: 0
Characteristics, clinical laboratory, histopathology, and outcomes of glycogenic hepatopathy in children 儿童糖源性肝病的特征、临床实验室、组织病理学和预后
Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12046
Chaowapong Jarasvaraparn, Iván A. González, Kyla Tolliver, Nadine G. Haddad, Jean P. Molleston
Glycogenic hepatopathy (GH) is a rare complication of type I diabetes mellitus (DM1), resulting in abnormal deposition of glycogen in the liver due to poor glycemic control. Clinical characteristics and natural history of GH are not completely understood in children. In this study, we investigated clinical, biochemical, histologic parameters and outcomes in children with GH.This was a retrospective review of patients less than 18 years old diagnosed with GH and DM. GH was confirmed on liver biopsy. Medical records were reviewed for clinical presentation, laboratory tests, and clinical outcomes. Liver biopsy findings were reviewed by a pediatric pathologist (I. A. G.).Nine children were diagnosed with GH and type 1 DM. The median age at diagnosis of GH was 16 (IQR 14.5−17) years. Duration of diagnosis of DM until GH diagnosis was 7 (IQR 5−11) years. The median frequency of diabetic ketoacidosis before GH diagnosis was three times (IQR 2−5.25). Peak Aspartate transaminase (AST) and Alanine transaminase (ALT) ranged from 115 to 797, and 83−389 units/L, respectively. Only two children had mild fibrosis. Seven of nine had steatosis without steatohepatitis. There was no correlation between glycosylated hemoglobin (HbA1c), or other laboratory tests and liver fibrosis on biopsy. HbA1c was 11.2 (IQR 10.2−12.8) at GH diagnosis and 9.8 (IQR 9.5−10.8) with normalization of liver enzymes.GH appears to be related to poor glycemic control in teenagers with long‐term diabetes. GH presents with high to very high aminotransferase especially AST > ALT and resolves with modestly improved glycemic control. Diffuse hepatocyte swelling, steatosis, minimal fibrosis without hepatocyte ballooning or lobular inflammation are most common histological features.
糖原性肝炎(GH)是 I 型糖尿病(DM1)的一种罕见并发症,由于血糖控制不佳而导致肝脏中糖原异常沉积。儿童 GH 的临床特征和自然病史尚不完全清楚。在这项研究中,我们调查了 GH 儿童的临床、生化、组织学参数和预后。肝活检证实了 GH。对病历中的临床表现、实验室检查和临床结果进行了审查。九名儿童被诊断为 GH 和 1 型 DM。9名儿童被确诊为GH和1型DM,确诊时的中位年龄为16岁(IQR为14.5-17岁)。从诊断出 DM 到确诊 GH 的时间为 7(IQR 5-11)年。确诊 GH 前发生糖尿病酮症酸中毒的频率中位数为 3 次(IQR 2-5.25)。天冬氨酸转氨酶(AST)和丙氨酸转氨酶(ALT)的峰值分别为115至797和83至389单位/升。只有两名患儿有轻度纤维化。九名儿童中有七名患有脂肪变性,但没有脂肪性肝炎。糖化血红蛋白(HbA1c)或其他实验室检查与活组织切片检查的肝纤维化之间没有关联。确诊 GH 时的 HbA1c 为 11.2(IQR 10.2-12.8),肝酶正常后为 9.8(IQR 9.5-10.8)。GH 表现为高到极高的转氨酶,尤其是 AST > ALT,随着血糖控制的适度改善而缓解。最常见的组织学特征是弥漫性肝细胞肿胀、脂肪变性、轻微纤维化,但无肝细胞气球或小叶炎症。
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引用次数: 0
Characteristics, clinical laboratory, histopathology, and outcomes of glycogenic hepatopathy in children 儿童糖源性肝病的特征、临床实验室、组织病理学和预后
Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12046
Chaowapong Jarasvaraparn, Iván A. González, Kyla Tolliver, Nadine G. Haddad, Jean P. Molleston
Glycogenic hepatopathy (GH) is a rare complication of type I diabetes mellitus (DM1), resulting in abnormal deposition of glycogen in the liver due to poor glycemic control. Clinical characteristics and natural history of GH are not completely understood in children. In this study, we investigated clinical, biochemical, histologic parameters and outcomes in children with GH.This was a retrospective review of patients less than 18 years old diagnosed with GH and DM. GH was confirmed on liver biopsy. Medical records were reviewed for clinical presentation, laboratory tests, and clinical outcomes. Liver biopsy findings were reviewed by a pediatric pathologist (I. A. G.).Nine children were diagnosed with GH and type 1 DM. The median age at diagnosis of GH was 16 (IQR 14.5−17) years. Duration of diagnosis of DM until GH diagnosis was 7 (IQR 5−11) years. The median frequency of diabetic ketoacidosis before GH diagnosis was three times (IQR 2−5.25). Peak Aspartate transaminase (AST) and Alanine transaminase (ALT) ranged from 115 to 797, and 83−389 units/L, respectively. Only two children had mild fibrosis. Seven of nine had steatosis without steatohepatitis. There was no correlation between glycosylated hemoglobin (HbA1c), or other laboratory tests and liver fibrosis on biopsy. HbA1c was 11.2 (IQR 10.2−12.8) at GH diagnosis and 9.8 (IQR 9.5−10.8) with normalization of liver enzymes.GH appears to be related to poor glycemic control in teenagers with long‐term diabetes. GH presents with high to very high aminotransferase especially AST > ALT and resolves with modestly improved glycemic control. Diffuse hepatocyte swelling, steatosis, minimal fibrosis without hepatocyte ballooning or lobular inflammation are most common histological features.
糖原性肝炎(GH)是 I 型糖尿病(DM1)的一种罕见并发症,由于血糖控制不佳而导致肝脏中糖原异常沉积。儿童 GH 的临床特征和自然病史尚不完全清楚。在这项研究中,我们调查了 GH 儿童的临床、生化、组织学参数和预后。肝活检证实了 GH。对病历中的临床表现、实验室检查和临床结果进行了审查。九名儿童被诊断为 GH 和 1 型 DM。9名儿童被确诊为GH和1型DM,确诊时的中位年龄为16岁(IQR为14.5-17岁)。从诊断出 DM 到确诊 GH 的时间为 7(IQR 5-11)年。确诊 GH 前发生糖尿病酮症酸中毒的频率中位数为 3 次(IQR 2-5.25)。天冬氨酸转氨酶(AST)和丙氨酸转氨酶(ALT)的峰值分别为115至797和83至389单位/升。只有两名患儿有轻度纤维化。九名儿童中有七名患有脂肪变性,但没有脂肪性肝炎。糖化血红蛋白(HbA1c)或其他实验室检查与活组织切片检查的肝纤维化之间没有关联。确诊 GH 时的 HbA1c 为 11.2(IQR 10.2-12.8),肝酶正常后为 9.8(IQR 9.5-10.8)。GH 表现为高到极高的转氨酶,尤其是 AST > ALT,随着血糖控制的适度改善而缓解。最常见的组织学特征是弥漫性肝细胞肿胀、脂肪变性、轻微纤维化,但无肝细胞气球或小叶炎症。
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引用次数: 0
Immunotherapy withdrawal by step‐down to mesalamine in pediatric patients with ulcerative colitis 儿科溃疡性结肠炎患者逐步停用美沙拉嗪后的免疫疗法
Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12048
R. Szigeti, R. Kellermayer
Parents and pediatric patients with ulcerative colitis (UC) who progressed to systemic immunotherapy are concerned about lifelong risks from such treatments. There is limited knowledge about withdrawal of such agents and step‐down (SD) to enteral 5‐aminosalicylic acid (mesalamine) before transitioning to adult care.We studied nine pediatric cases with moderate to severe UC who after a median of 2.18 years of clinical remission on systemic immunotherapy stepped down to oral mesalamine treatment.Average follow‐up time from SD was 3.49 years. Five patients (55.5%) had sustained remission (without any flare noted) after SD during follow‐up. Sustained clinical remission was 88.9% (8/9) at 1 year, 87.5% (7/8) at 2 years, and 66.7% (4/6) at 3 years after SD. Out of those tested (one patient was not tested), 62.5% (5/8) had fecal calprotectin <50 μg/g. Four out of six patients examined (66.6%) had mucosal healing on post‐SD colonoscopy.We propose that SD to mesalamine can be a reasonable therapeutic consideration for pediatric patients with UC before transitioning to adult gastroenterology care. Shared decision‐making is important before such treatment changes.
家长和接受全身免疫疗法的溃疡性结肠炎(UC)儿科患者担心这种治疗会带来终身风险。我们研究了 9 例中度至重度 UC 儿童患者,他们在接受全身免疫疗法临床缓解中位数为 2.18 年后,转而接受口服美沙拉明治疗。从SD开始的平均随访时间为3.49年。随访期间,有5名患者(55.5%)在SD后获得了持续缓解(无任何复发迹象)。SD后1年的持续临床缓解率为88.9%(8/9),2年为87.5%(7/8),3年为66.7%(4/6)。在接受检查的患者中(一名患者未接受检查),62.5%(5/8)的粪便钙蛋白<50 μg/g。在接受 SD 后结肠镜检查的六名患者中,有四名患者(66.6%)的粘膜已经愈合。我们建议,对于患有 UC 的儿科患者,在过渡到成人胃肠病治疗之前,将 SD 转为美沙拉明是一种合理的治疗考虑。在改变治疗方案之前,共同决策非常重要。
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引用次数: 1
Letter to the editor: A response to Hildreth and Schwimmer. 致编辑的信回应 Hildreth 和 Schwimmer。
Pub Date : 2024-02-05 eCollection Date: 2024-05-01 DOI: 10.1002/jpr3.12027
Rachel B Schenker, Brian Kim, George Yanni
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引用次数: 0
Invited commentary to immunotherapy withdrawal by step‐down to mesalamine in pediatrics patients with ulcerative colitis 应邀评论儿科溃疡性结肠炎患者逐步停用美沙拉嗪后的免疫疗法问题
Pub Date : 2024-02-05 DOI: 10.1002/jpr3.12047
Daniel M. O'Connell, Jonathan Moses
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引用次数: 0
Idiopathic fibrosing pancreatitis: A rare cause of obstructive jaundice 特发性纤维性胰腺炎:阻塞性黄疸的罕见病因
Pub Date : 2024-01-30 DOI: 10.1002/jpr3.12018
Mindy Huynh, Rodolfo Silva, Nikhil Thiruvengadam, K. Parashette
Jaundice is an important physiologic manifestation of both benign and insidious diseases. We report on the case of an 11‐year‐old male who presented with diffuse pruritis, jaundice, and later abdominal pain. Initial work‐up revealed an obstructive cholestatic pattern, warranting investigation for structural anomalies. Extensive imaging revealed a lesion on the pancreatic head, and biopsy of the lesion confirmed the diagnosis of idiopathic fibrosing pancreatitis (IFP). Temporary stenting of the common bile duct successfully treated our patient's symptomatic IFP.
黄疸是良性和隐匿性疾病的重要生理表现。我们报告了一例 11 岁男性的病例,他出现弥漫性瘙痒、黄疸,随后出现腹痛。初步检查发现他患有梗阻性胆汁淤积症,需要进行结构异常检查。广泛的影像学检查发现胰腺头部有病变,病变部位的活检证实了特发性纤维化胰腺炎(IFP)的诊断。胆总管临时支架置入术成功治疗了患者无症状的特发性纤维性胰腺炎。
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引用次数: 0
Mucosal prolapse syndrome mimicking Peutz–Jeghers syndrome in a pediatric patient 一名儿科患者模仿 Peutz-Jeghers 综合征的粘膜脱垂综合征
Pub Date : 2024-01-29 DOI: 10.1002/jpr3.12022
Phinga Do, Claudia Phen, Michele Alkalay, Vivekanand Singh, Isabel Rojas
Mucosal prolapse syndrome (MPS) is a rare group of benign conditions characterized by a set of unifying histologic findings thought to be the result of repeated mucosal shearing and submucosal vascular congestion caused by straining. This set of conditions is often misdiagnosed as other polyposis syndromes, inflammatory bowel disease, or malignancy due to its clinical presentation, appearance, and rarity. We report a case of a 15‐year‐old male who presented with painless rectal bleeding. He was found to have four rectal polyps thought to be due to Peutz–Jeghers syndrome. A repeat colonoscopy with biopsies a year later revealed a diagnosis of MPS. Our case highlights the morphologic similarity between hamartomatous polyp and mucosal prolapse histology. Since MPS is a rare diagnosis even among the adult population, it has not been well described in pediatrics. This syndrome should be on the differential diagnosis for pediatric rectal polyps to prevent unnecessary invasive testing and a delay in treatment.
粘膜脱垂综合征(MPS)是一组罕见的良性疾病,其特点是有一系列统一的组织学发现,被认为是反复粘膜剪切和粘膜下血管充血造成的。由于其临床表现、外观和罕见性,这组病症经常被误诊为其他息肉病综合征、炎症性肠病或恶性肿瘤。我们报告了一例 15 岁男性无痛直肠出血病例。他被发现患有四个直肠息肉,被认为是由 Peutz-Jeghers 综合征引起的。一年后,他再次接受结肠镜检查并进行活检,结果确诊为 MPS。我们的病例凸显了火腿肠息肉和粘膜脱垂组织学在形态上的相似性。由于 MPS 即使在成人中也是一种罕见的诊断,因此在儿科中还没有很好的描述。该综合征应作为小儿直肠息肉的鉴别诊断,以避免不必要的侵入性检查和延误治疗。
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引用次数: 0
Gallbladder remnant: A potential source for biliary stones postcholecystectomy; a case report in a child with sickle cell disease 胆囊残余:胆囊切除术后胆道结石的潜在来源;镰状细胞病患儿的病例报告
Pub Date : 2024-01-25 DOI: 10.1002/jpr3.12039
Ahmad Miri, Shahab Abdessalam, Andria M. Powers, Ruben E. Quiros‐Tejeira, Chinenye R Dike
Stone formation in a gallbladder remnant is a rare postcholecystectomy complication. This report describes the case of gallstones in a gallbladder remnant of an adolescent with sickle cell disease (SCD) years after laparoscopic cholecystectomy. A 15‐year‐old female with SCD presented to our gastroenterology clinic with concerns of recurrent choledocholithiasis despite cholecystectomy 2 years before presentation. About 4 months before presentation to our clinic, she was evaluated at the referring physician's emergency department for recurrent severe abdominal pain of 1 month duration. After admission to the hospital, common bile duct stones were seen on magnetic resonance cholangiopancreatography (MCRP) imaging and subsequently removed via endoscopic retrograde cholangiopancreatography (ERCP). On review of her MRCP and ERCP at our hospital, a remnant of gallbladder containing multiple stones was identified. She subsequently underwent a laparoscopic resection of the gallbladder remnant. Clinicians should consider biliary duct imaging in children with biliary colic following cholecystectomy, especially those with history of chronic hemolysis.
胆囊切除术后胆囊残余部位形成结石是一种罕见的并发症。本报告描述了一例患有镰状细胞病(SCD)的青少年在腹腔镜胆囊切除术后数年在胆囊残余部位形成胆结石的病例。一名患有镰状细胞病的 15 岁女性患者因反复发作胆总管结石而到我院消化内科就诊,尽管她在就诊前两年接受了胆囊切除术。在来我院就诊前约 4 个月,她因持续 1 个月的反复剧烈腹痛到转诊医生所在的急诊科就诊。入院后,磁共振胰胆管造影(MCRP)检查发现了胆总管结石,随后通过内镜逆行胰胆管造影(ERCP)取出了结石。在我院复查 MRCP 和 ERCP 时,发现胆囊残余物中含有多颗结石。随后,她接受了腹腔镜胆囊残余切除术。对于胆囊切除术后出现胆绞痛的患儿,尤其是有慢性溶血病史的患儿,临床医生应考虑进行胆道造影检查。
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引用次数: 0
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