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.

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.