Experience of ZHANG Boli in Staged Treating Very Early Onset Inflammatory Bowel Disease Based on the Theory of “Similar Diseases and Syndromes of Damp-Turbidity-Phlegm-Rheum” / 中医杂志

This article summarizes the experience of Professor ZHANG Boli in the staged treatment of very early onset inflammatory bowel disease （VEO-IBD）. Grounded in the theory of “similar diseases and syndromes of damp-turbidity-phlegm-rheum”， it is believed that dampness and turbidity are crucial pathogenic factors in VEO-IBD. During the acute phase， the core pathogenesis centers on the accumulation of turbid toxins in the intestines. The treatment focuses on dispelling dampness and clearing turbidity to eliminate turbid toxins， while also regulating the flow of qi and nourishing the spleen and kidney. During the remission phase， the core pathogenesis involves spleen and kidney deficiency， which is treated by invigorating the spleen and warming the kidney to strengthen the body resistance. Additionally， promoting blood circulation and eliminating stasis is integrated throughout the treatment process. Medications are chosen to be mild and gentle， emphasizing balance and harmony， and attention is given to the methods of administration and psychological well-being， ensuring comprehensive care for both body and mind.

A case of very early onset inflammatory bowel disease / 中国综合临床

The clinical data of a child with very early onset inflammatory bowel disease (VEO-IBD) treated in the digestive department of Guangzhou Women's and children's Medical Center in October 2018 were analyzed retrospectively. The patient was hospitalized because of "shortness of breath and abdominal distension" after birth. The gastrointestinal manifestations were diarrhea, mucus bloody stool, feeding intolerance and weight loss; the extraintestinal manifestations were liver function damage and joint damage. Endoscopic examination considered VEO-IBD. The patients were treated with infliximab and enteral nutrition. When the clinical symptoms were relieved and the gastrointestinal mucosa healed, the enteral nutrition regimen was adjusted. At present, she returned to daily diet, and the weight and height reached the level of normal children. VEO-IBD patients can be combined with a variety of extraintestinal manifestations, clinicians need to identify the coexistence of these diseases, effective follow-up.

Recent Advance in Very Early Onset Inflammatory Bowel Disease / 대한소아소화기영양학회지

Recent studies on pediatric inflammatory bowel disease (IBD) have revealed that early-onset IBD has distinct phenotypic differences compared with adult-onset IBD. In particular, very early-onset IBD (VEO-IBD) differs in many aspects, including the disease type, location of the lesions, disease behavior, and genetically attributable risks. Several genetic defects that disturb intestinal epithelial barrier function or affect immune function have been noted in these patients from the young age groups. In incidence of pediatric IBD in Korea has been increasing since the early 2000s. Neonatal or infantile-onset IBD develops in less than 1% of pediatric patients. Children with “neonatal IBD” or “infantile-onset IBD” have higher rates of affected first-degree relatives, severe disease course, and a high rate of resistance to immunosuppressive treatment. The suspicion of a monogenic cause of VEO-IBD was first confirmed by the discovery of mutations in the genes encoding the interleukin 10 (IL-10) receptors that cause impaired IL-10 signaling. Patients with such mutations typically presented with perianal fistulae, shows a poor response to medical management, and require early surgical interventions in the first year of life. To date, 60 monogenic defects have been identified in children with IBD-like phenotypes. The majority of monogenic defects presents before 6 years of age, and many present before 1 year of age. Next generation sequencing could become an important diagnostic tool in children with suspected genetic defects especially in children with VEO-IBD with severe disease phenotypes. VEO-IBD is a phenotypically and genetically distinct disease entity from adult-onset or older pediatric IBD.

Comparison of clinical features between very earl-onset inflammatory bowel diseases and late-onset inflammatory bowel diseases / 中华实用儿科临床杂志

Objective To summarize the clinical data of the children with inflammatory bowel diseases (IBD),including Crohn's disease (CD) and ulcerative colitis (UC),and to analyze and compare the clinical features of very early-onset IBD (VEO-IBD) and late-onset IBD (LO-IBD).Methods A retrospective analysis of the clinical data of 184 cases of IBD hospitalized children diagnosed at Beijing Children's Hospital,Capital Medical University from January 2000 to December 2014.According to their ages of onset,the patients were divided into VEO-IBD group (＜6 years old) and LO-IBD group (6-16 years old);the patients with CD were divided into VEO-CD group (＜ 6 years old) and LO-CD group (6-16 years old);UC were divided into VEO-UC group (＜ 6 years old) and LO-UC group (6-16 years old).The clinical features among each group were analyzed and compared.Results A total of 184 IBD patients were included in the study,77 cases(41.8％) were VEO-IBD and 107 cases(58.2％) were LO-IBD.Comparison between VEO-CD group and LO-CD group indicated that abdominal pain was more common in LO-CD group (P ＜ 0.05),while diarrhea and hematochezia were more common in VEO-CD group (all P ＜ 0.05).In addition,comparison between VEO-UC group and LO-UC group indicated that abdominal pain was more common in LO-UC group(P ＜ 0.001),while diarrhea,fever,and oral ulcers were more common in VEO-UC group (all P ＜0.05).Both VEO-CD and LO-CD group were mainly ileocolonic[15/27 cases (55.6％),20/47 cases (42.6％)],non-narrow,non-penetrating [20/27 cases (74.1％),30/47 cases (63.8％)] and moderate-to-severe activity[23/27 cases(85.2％),37/47 cases (78.7％)].The incidence of perianal lesions in the VEO-CD group was as high as 51.9％ (14/27 cases),which was significantly higher than that in the LO-CD group (9/47 cases,19.1％) (P ＜ 0.05).Left-sided UC and severe UC were more common in VEO-UC group(all P ＜ 0.05),while pancolitis and mild UC were more common in LO-UC group (all P ＜ 0.05).The incidence of intestinal perforation in the VEO-UC group was significantly higher than that in the LO-UC group (P ＜0.05).The incidence of surgical rate,intestinal obstruction,and intestinal perforation in the LO-CD group were significantly higher than those in the LO-UC group (all P ＜ 0.05).Conclusions Compared between VEO-IBD and LO-IBD,VEO-IBD patients are more severe,with perianal lesions more common,and the incidence of intestinal perforation is higher.

A Pediatric Case of Inflammatory Bowel Disease with Renal Amyloidosis

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of “very early onset inflammatory bowel disease”. Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

Higher Morbidity of Monogenic Inflammatory Bowel Disease Compared to the Adolescent Onset Inflammatory Bowel Disease / 대한소아소화기영양학회지

PURPOSE: Monogenic inflammatory bowel disease (IBD) patients do not respond to conventional therapy and are associated with a higher morbidity. We summarized the clinical characteristics of monogenic IBD patients and compared their clinical outcomes to that of non-monogenic IBD patients. METHODS: We performed a retrospective cohort study of all children <18 years old who were diagnosed with IBD between 2005 and 2016. A total of 230 children were enrolled. Monogenic IBD was defined as a presentation age less than 6 years old with confirmation of a genetic disorder. We subdivided the groups into monogenic IBD (n=18), non-monogenic very early-onset IBD (defined as patients with a presentation age <6 years old without a confirmed genetic disorder, n=12), non-monogenic IBD (defined as all patients under 18 years old excluding monogenic IBD, n=212), and severe IBD (defined as patients treated with an anti-tumor necrosis factor excluding monogenic IBD, n=92). We compared demographic data, initial pediatric Crohn disease activity index/pediatric ulcerative colitis activity index (PCDAI/PUCAI) score, frequency of hospitalizations, surgical experiences, and height and weight under 3rd percentile among the patients enrolled. RESULTS: The initial PCDAI/PUCAI score (p < 0.05), incidence of surgery per year (p < 0.05), and hospitalization per year (p < 0.05) were higher in the monogenic IBD group than in the other IBD groups. Additionally, the proportion of children whose weight and height were less than the 3rd percentile (p < 0.05 and p < 0.05, respectively) was also higher in the monogenic IBD group. CONCLUSION: Monogenic IBD showed more severe clinical manifestations than the other groups.

Demographic Characteristics, Medication Profile and Treatment Outcome of Patients with Very Early-Onset Schizophrenia in One Hospital

OBJECTIVES: Very early-onset schizophrenia (VEOS) is a type of psychosis having a low frequency, insidious onset, and devastating clinical outcome. In this study, the demographic features, information on medication, clinical outcomes, and intellectual capability of patients diagnosed with VEOS in a hospital were analyzed to provide therapeutic strategies for this type of schizophrenia. METHODS: Using the electronic medical records of the National Center for Mental Health, 69 patients with VEOS were identified based on the DSM-5 criteria of schizophrenia. The data were summarized and analyzed according to the demographic characteristics, medications used, intellectual strength measured by the full intelligence quotient (FIQ) score, and current clinical status measured by the Clinical Global Impression-Severity (CGI-S) and various combinations of these parameters. RESULTS: The screened study group contained similar numbers of males and females. The younger the onset of psychosis, the lower the frequency. The study population included a significantly higher proportion of births in the winter season than that of the general population. The 3 most frequently used antipsychotic medications were risperidone and its derivatives, clozapine and olanzapine. Valproic acid and divalproex sodium were the most commonly added drugs for outcome augmentation. 53.5% of the study population had received benzodiazepines and/or hypnotics. The average FIQ of the study population was 69.4, which is quite low compared to previous Korean studies with similar populations. There was a weak negative correlation between FIQ and CGI-S, but it was not statistically significant. The average CGI-S score was 4.2, which meant that the patients were moderately ill. CONCLUSION: This study demonstrated that patients with VEOS showed more frequent intellectual deficits at baseline and poorer outcomes than the control group. Risperidone, clozapine, valproic acid and their combinations were the most preferred medications for the treatment of psychosis. Benzodiazepines were quite commonly added for various reasons.

Very early-onset inflammatory bowel disease and immunodeficiency / 中华实用儿科临床杂志

The etiology of inflammatory bowel disease(IBD) is complex and multifactorial.Recent genetic association studies within large IBD cohorts have identified 163 genetic loci.But these 163 loci in total explain 13.6％ of Crohn's disease and 7.5％ of ulcerative colitis total disease variance.The classification defines very early-onset IBD as diagnosis before the age of 5 years old,and also before the age of 6 years old in some research.It is likely that genetic factors contribute more to the etiology of very early-onset IBD.Recently some cases have been reported,which have linked to very early-onset IBD,such as IL-10 and IL-10 receptor gene mutation,immune dysregulation,polyendocrinopathy,enteropathy,X-linked syndrome,X-linked lymphoproliferative syndrome,IL-21 deficiency,mevalonate kinase deficiency,and so on.And many of these diseases are classified as primary immunodeficiencies.This paper introduces these series of very early-onset IBD in brief.