1.Clinical characteristics and related factors analysis of adrenal crisis occurred in children with primary nephrotic syndrome.
Na GUAN ; Hui Jie XIAO ; Bai Ge SU ; Xu Hui ZHONG ; Fang WANG ; Sai Nan ZHU
Chinese Journal of Pediatrics 2023;61(9):805-810
Objective: To investigate the clinical characteristics and related factors of corticosteroid induced adrenal crisis (AC) in children with primary nephrotic syndrome (NS). Methods: Case control study. The case group included 7 children aged 1 to 18 years with NS combined with AC hospitalized in Peking University First Hospital from January 2016 to May 2021 (AC group). According to the ratio of case group: control group 1: 4, 28 children aged 1 to 18 years who were diagnosed with NS without AC during the same period were matched as controls (non-AC group). Clinical data were collected. The clinical characteristics of AC were described. The clinical parameters were compared between the 2 groups by t test, Mann-Whitney U test or Fisher's test. Receiver operating characteristic (ROC) curve was used to analyze the cutoff values of clinical parameters for prediction of AC. Results: The AC group included 4 boys and 3 girls aged 6.9 (4.6, 10.8) years. The non-AC group included 20 boys and 8 girls aged 5.2 (3.3, 8.4) years. All AC events occurred during the relapse of NS with infection. Seven children had gastrointestinal symptoms such as nausea, vomiting and abdominal pain. Six children had poor mental state or impaired consciousness. No significant differences in NS course, corticosteroid treatment course, corticosteroid type, steroid dosage, steroid medication interval, the proportion of gastroenteritis and fever existed between the two groups (all P>0.05). Compared with the non-AC group, the duration from the onset of the relapse of NS until hospitalization in the AC group was significantly shorter (0.2 (0.1, 0.6) vs. 1.0 (0.4, 5.0) month,U=25.50, P=0.005). The 24 h urinary total protein (UTP) level was significantly higher in the AC group (193 (135, 429) vs. 81 (17, 200) mg/kg, U=27.00,P=0.036) than the non-AC group. The serum albumin level in the AC group was significantly lower((13.1±2.1) vs. (24.5±8.7) g/L,t=-6.22,P<0.001) than the non-AC group. There were significantly higher total white blood cell counts ((26±9)×109 vs. (11±5)×109/L,t=4.26,P=0.004), percentage of neutrophils (0.71±0.08 vs. 0.60±0.19,t=2.56,P=0.017) and the proportion of children with C reactive protein level≥8 mg/L (3/7 vs. 0,P=0.005) in the AC group than in the non-AC group. ROC curve analysis showed that the cutoff value of 24 h UTP was 122 mg/(kg·d) with a sensitivity of 100.0% and specificity of 70.4%. The cutoff value of serum albumin was 17.0 g/L with a sensitivity of 100.0% and specificity of 82.1%. Conclusions: Gastrointestinal symptoms and poor mental state were prominent manifestations of AC in children with NS. High 24 h UTP level, low serum albumin level, high peripheral white blood cell counts, high neutrophils percentage, and high C-reactive protein level during the early stage of NS relapse may be related to the occurrence of AC in children with NS.
Nephrotic Syndrome/drug therapy*
;
Humans
;
Child
;
Adolescent
;
Male
;
Female
;
Gastrointestinal Diseases/diagnosis*
;
Adrenal Cortex Hormones/therapeutic use*
;
Nausea/chemically induced*
;
Vomiting/chemically induced*
;
Abdominal Pain/chemically induced*
;
Mental Processes/drug effects*
;
China
3.Clinicopathological characteristics and prognosis of patients with small bowel tumors: A single center analysis of 220 cases.
Xu Liang LIAO ; Yun Feng ZHU ; Wei Han ZHANG ; Xiao Long CHEN ; Kai LIU ; Lin Yong ZHAO ; Kun YANG ; Jian Kun HU
Chinese Journal of Gastrointestinal Surgery 2023;26(5):467-474
Objective: To analyze the clinicopathological characteristics and prognosis of patients with small bowel tumors. Methods: This was a retrospective, observational study. We collected clinicopathological data of patients with primary jejunal or ileal tumors who had undergone small bowel resection in the Department of Gastrointestinal Surgery, West China Hospital, Sichuan University between January 2012 and September 2017. The inclusion criteria included: (1) older than 18 years; (2) had undergone small bowel resection; (3) primary location at jejunum or ileum; (4) postoperative pathological examination confirmed malignancy or malignant potential; and (5) complete clinicopathological and follow-up data. Patients with a history of previous or other concomitant malignancies and those who had undergone exploratory laparotomy with biopsy but no resection were excluded. The clinicopathological characteristics and prognoses of included patients were analyzed. Results: The study cohort comprised 220 patients with small bowel tumors, 136 of which were classified as gastrointestinal stromal tumors (GISTs), 47 as adenocarcinomas, and 35 as lymphomas. The median follow-up for all patient was 81.0 months (75.9-86.1). GISTs frequently manifested as gastrointestinal bleeding (61.0%, 83/136) and abdominal pain (38.2%, 52/136). In the patients with GISTs, the rates of lymph node and distant metastasis were 0.7% (1/136) and 11.8% (16/136), respectively. The median follow-up time was 81.0 (75.9-86.1) months. The 3-year overall survival (OS) rate was 96.3%. Multivariate Cox regression-analysis results showed that distant metastasis was the only factor associated with OS of patients with GISTs (HR=23.639, 95% CI: 4.564-122.430, P<0.001). The main clinical manifestations of small bowel adenocarcinoma were abdominal pain (85.1%, 40/47), constipation/diarrhea (61.7%, 29/47), and weight loss (61.7%, 29/47). Rates of lymph node and distant metastasis in patients with small bowel adenocarcinoma were 53.2% (25/47) and 23.4% (11/47), respectively. The 3-year OS rate of patients with small bowel adenocarcinoma was 44.7%. Multivariate Cox regression-analysis results showed that distant metastasis (HR=4.018, 95%CI: 2.108-10.331, P<0.001) and adjuvant chemotherapy (HR=0.291, 95% CI: 0.140-0.609, P=0.001) were independently associated with OS of patients with small bowel adenocarcinoma. Small bowel lymphoma frequently manifested as abdominal pain (68.6%, 24/35) and constipation/diarrhea (31.4%, 11/35); 77.1% (27/35) of small bowel lymphomas were of B-cell origin. The 3-year OS rate of patients with small bowel lymphomas was 60.0%. T/NK cell lymphomas (HR= 6.598, 95% CI: 2.172-20.041, P<0.001) and adjuvant chemotherapy (HR=0.119, 95% CI: 0.015-0.925, P=0.042) were independently associated with OS of patients with small bowel lymphoma. Small bowel GISTs have a better prognosis than small intestinal adenocarcinomas (P<0.001) or lymphomas (P<0.001), and small bowel lymphomas have a better prognosis than small bowel adenocarcinomas (P=0.035). Conclusions: The clinical manifestations of small intestinal tumor are non-specific. Small bowel GISTs are relatively indolent and have a good prognosis, whereas adenocarcinomas and lymphomas (especially T/NK-cell lymphomas) are highly malignant and have a poor prognosis. Adjuvant chemotherapy would likely improve the prognosis of patients with small bowel adenocarcinomas or lymphomas.
Humans
;
Prognosis
;
Intestinal Neoplasms/diagnosis*
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Duodenal Neoplasms
;
Gastrointestinal Stromal Tumors
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Lymphoma
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Adenocarcinoma/surgery*
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Constipation
;
Abdominal Pain
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Retrospective Studies
4.A case of duodenal ulcer as prominent manifestation of IgG4-related disease.
Min FENG ; Zhe CHEN ; Yong Jing CHENG
Journal of Peking University(Health Sciences) 2023;55(6):1125-1129
A case of IgG4-related disease presented with a duodenal ulcer to improve the understan-ding of IgG4-related diseases was reported. A 70-year-old male presented with cutaneous pruritus and abdominal pain for four years and blackened stools for two months. Four years ago, the patient went to hospital for cutaneous pruritus and abdominal pain. Serum IgG4 was 3.09 g/L (reference value 0-1.35 g/L), alanine aminotransferase 554 U/L (reference value 9-40 U/L), aspartate aminotransferase 288 U/L (reference value 5-40 U/L), total bilirubin 54.16 μmol/L (reference value 2-21 μmol/L), and direct bilirubin 29.64 μmol/L (reference value 1.7-8.1 μmol/L) were all elevated. The abdominal CT scan and magnetic resonance cholangiopancreatography indicated pancreatic swelling, common bile duct stenosis, and secondary obstructive dilation of the biliary system. The patient was diagnosed with IgG4-related disease and treated with prednisone at 40 mg daily. As jaundice and abdominal pain improved, prednisone was gradually reduced to medication discontinuation. Two months ago, the patient developed melena, whose blood routine test showed severe anemia, and gastrointestinal bleeding was diagnosed. The patient came to the emergency department of Beijing Hospital with no improvement after treatment in other hospitals. Gastroscopy revealed a 1.5 cm firm duodenal bulb ulcer. After treatment with omeprazole, the fecal occult blood was still positive. The PET-CT examination was performed, and it revealed no abnormality in the metabolic activity of the duodenal wall, and no neoplastic lesions were found. IgG4-related disease was considered, and the patient was admitted to the Department of Rheumatology and Immunology of Beijing Hospital for further diagnosis and treatment. The patient had a right submandibular gland mass resection history and diabetes mellitus. After the patient was admitted to the hospital, the blood test was reevaluated. The serum IgG4 was elevated at 5.44 g/L (reference value 0.03-2.01 g/L). Enhanced CT of the abdomen showed that the pancreas was mild swelling and was abnormally strengthened, with intrahepatic and extrahepatic bile duct dilation and soft tissue around the superior mesenteric vessels. We pathologically reevaluated and stained biopsy specimens of duodenal bulbs for IgG and IgG4. Immunohistochemical staining revealed remarkable infiltration of IgG4-positive plasma cells into duodenal tissue, the number of IgG4-positive cells was 20-30 cells per high-powered field, and the ratio of IgG4/IgG-positive plasma cells was more than 40%. The patient was treated with intravenous methylprednisolone at 40 mg daily dosage and cyclophosphamide, and then the duodenal ulcer was healed. IgG4 related disease is an immune-medicated rare disease characterized by chronic inflammation and fibrosis. It is a systemic disease that affects nearly every anatomic site of the body, usually involving multiple organs and diverse clinical manifestations. The digestive system manifestations of IgG4-related disease are mostly acute pancreatitis and cholangitis and rarely manifest as gastrointestinal ulcers. This case confirms that IgG4-related disease can present as a duodenal ulcer and is one of the rare causes of duodenal ulcers.
Aged
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Humans
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Male
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Abdominal Pain/drug therapy*
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Acute Disease
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Bilirubin
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Duodenal Ulcer/etiology*
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Immunoglobulin G
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Immunoglobulin G4-Related Disease/diagnosis*
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Pancreatitis/drug therapy*
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Positron Emission Tomography Computed Tomography
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Prednisone/therapeutic use*
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Pruritus/drug therapy*
5.Upper gastrointestinal ulcer in children: a clinical analysis of 173 cases.
Chinese Journal of Contemporary Pediatrics 2022;24(4):372-376
OBJECTIVES:
To study the clinical manifestations and gastroscopic characteristics of upper gastrointestinal ulcer in children.
METHODS:
A retrospective analysis was performed for the children who underwent gastroscopy and were found to have upper gastrointestinal ulcer for the first time at the Endoscopy Center of Shengjing Hospital, China Medical University, from January 2011 to May 2021. According to the cause of the disease, they were divided into primary ulcer group (primary group; n=148) and secondary ulcer group (secondary group; n=25). The clinical data were compared between the two groups.
RESULTS:
A total of 173 children with upper gastrointestinal ulcer were enrolled, with a male/female ratio of 3.9:1. Compared with girls, boys had significantly higher proportions of duodenal ulcer and primary ulcer (P<0.05). Compared with the children aged below 6 years, the children aged 6-14 years had higher proportions of duodenal ulcer and primary ulcer and lower proportions of giant ulcer and multiple ulcers. Of the 148 children in the primary group, 95 (64.2%) had Helicobacter pylori infection. Abdominal pain was the most common clinical symptom and was observed in 101 children (68.2%). Duodenal ulcer was common and was observed in 115 children (77.7%), followed by gastric ulcer in 25 children (16.9%) and esophageal ulcer in 7 children (4.7%). Multiple ulcers were observed in 32 children (21.6%). Seventy children (47.3%) experienced complications, among which bleeding was the most common complication and was observed in 63 children (43.6%). Of the 25 children in the secondary group, abdominal pain was the most common clinical symptom and was observed in 9 children (36.0%), with a significantly lower incidence rate than the primary group (P<0.05); foreign body in the digestive tract was the most common cause of ulcer and was observed in 17 children (68%), followed by abdominal Henoch-Schönlein purpura in 5 children (20.0%) and Crohn's disease in 3 children (12.0%). The secondary group had a significantly higher proportion of multiple ulcer or giant ulcer than the primary group (P<0.05).
CONCLUSIONS
Upper gastrointestinal ulcer is more common in boys than girls, and duodenal ulcer and primary ulcer are more common in boys. Children aged 6-14 years often have duodenal ulcer and primary ulcer, and giant ulcer and multiple ulcers are relatively uncommon. Primary ulcer in children has a variety of clinical manifestations, mainly abdominal pain, and duodenal ulcer is relatively common, with bleeding as the main complication. The clinical symptoms and endoscopic manifestations of secondary ulcer are closely associated with the primary causes, and it is more likely to induce huge ulcers and multiple ulcers.
Abdominal Pain
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Child
;
Duodenal Ulcer/epidemiology*
;
Endoscopy, Gastrointestinal/adverse effects*
;
Female
;
Helicobacter Infections/diagnosis*
;
Helicobacter pylori
;
Humans
;
Male
;
Retrospective Studies
;
Ulcer
6.Association between functional dyspepsia and serum levels of brain-gut peptides in children.
Dong-Wei WANG ; Xiao-Lin YE ; Jie WU
Chinese Journal of Contemporary Pediatrics 2022;24(4):387-391
OBJECTIVES:
To study the association between functional dyspepsia (FD) and serum levels of brain-gut peptides including calcitonin gene-related peptide (CGRP), nesfatin-1, and ghrelin in children.
METHODS:
A total of 38 children with FD who attended Shengjing Hospital of China Medical University from November 2019 to December 2020 were enrolled as the FD group. Thirty-four healthy children were enrolled as the control group. Serum samples were collected from all of the children. Enzyme-linked immunosorbent assay was used to measure serum levels of CGRP, ghrelin, and nesfatin-1 for comparison between the two groups. The scores of clinical symptoms were determined for the children with FD. Spearman rank correlation analysis was used to investigate the correlation of symptom scores with the serum levels of brain-gut peptides.
RESULTS:
The FD group had significantly higher serum levels of nesfatin-1 and CGRP than the control group (P<0.05), while there was no significant difference in the serum level of ghrelin between the two groups (P>0.05). The serum level of nesfatin-1 was positively correlated with the symptom score of early satiety (rs=0.553, P<0.001), but was not significantly correlated with the total score of FD (rs=0.191, P=0.250). The serum level of CGRP was positively correlated with the scores of abdominal pain (rs=0.479, P=0.002) and belching (rs=0.619, P<0.001) and the total score of FD (rs=0.541, P<0.001).
CONCLUSIONS
CGRP and nesfatin-1 may play an important role in the pathophysiological process of FD.
Abdominal Pain
;
Brain
;
Calcitonin Gene-Related Peptide
;
Child
;
Dyspepsia/diagnosis*
;
Ghrelin
;
Humans
9.Gastric Xanthoma in the Pediatric Population: A Possible Herald for Malignancy?
Joseph D RUSSELL ; Jacquelin PECK ; Claudia PHEN ; Janna L LINEHAN ; Sara KARJOO ; Johnny NGUYEN ; Michael J WILSEY
Pediatric Gastroenterology, Hepatology & Nutrition 2020;23(1):110-114
diagnosis. The underlying etiology is not fully understood; however, it has been linked to Helicobacter pylori gastritis and gastric cancer. GX in the pediatric population is largely unreported in the literature. Because of the relative rarity, documentation with case reports are essential to provide as much data as possible to see if there is a correlation between GX and malignant potential in the pediatric population. Our group is reporting two cases, a 10-year-old male and a 7-year-old male, both who presented with chronic dysphagia, upper abdominal pain, nausea, vomiting, and loss of appetite. Upper endoscopies for both patients revealed small polypoid lesions located in the antrum with foamy histiocytes on histology, leading to the diagnosis of gastric xanthoma.]]>
Abdominal Pain
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Adult
;
Appetite
;
Child
;
Deglutition Disorders
;
Diagnosis
;
Endoscopy
;
Gastritis
;
Helicobacter pylori
;
Histiocytes
;
Humans
;
Incidental Findings
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Male
;
Nausea
;
Pediatrics
;
Stomach Neoplasms
;
Vomiting
;
Xanthomatosis
10.A rare case of ovarian vein thrombosis in a gestational trophoblastic neoplasia patient
In Young KIM ; Seung Hyun KIM ; In Taek HWANG ; Joong Gyu HA ; Jae Ho CHA
Obstetrics & Gynecology Science 2019;62(3):190-193
Ovarian vein thrombosis (OVT) is a rare disease with complications that can be life-threatening. An ovarian vein thrombus in a gestational trophoblastic neoplasia (GTN) is an extremely rare condition that has not been previously reported in the literature. We report the case of a 23-year-old woman who presented with symptoms of amenorrhea for 15 weeks and 6 days along with intermittent lower abdominal pain. She was diagnosed with a hydatidiform mole, and a metastatic workup was scheduled. Abdominal computed tomography showed a right ovarian vein thrombus. She received methotrexate chemotherapy combined with oral anticoagulants. Complete radiological remission was obtained. During the 12-month follow-up period, no disease progression or recurrence was noted. Early recognition and detection of the condition are of the utmost importance. The differential diagnosis of OVT must be considered when there is unexplained abdominal pain, fever, and leukocytosis during the diagnosis and treatment of GTN. A high level of suspicion is required for prompt diagnosis of OVT.
Abdominal Pain
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Amenorrhea
;
Anticoagulants
;
Diagnosis
;
Diagnosis, Differential
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Disease Progression
;
Drug Therapy
;
Female
;
Fever
;
Follow-Up Studies
;
Gestational Trophoblastic Disease
;
Humans
;
Hydatidiform Mole
;
Leukocytosis
;
Methotrexate
;
Pregnancy
;
Rare Diseases
;
Recurrence
;
Thrombosis
;
Veins
;
Venous Thrombosis
;
Young Adult

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