Immunoglobulin A vasculitis (IgAV) primarily affects childhood and can be categorized as immune complex vasculitis. It typically presents with purpura, abdominal pain, arthritis, and nephritis. IgAV can be diagnosed without hesitation when the characteristic skin lesions appear at onset; however, in cases where the abdominal symptoms precede the skin rash or there is no purpura at all, diagnosis can be challenging. Delayed diagnosis of IgAV may be associated with serious abdominal complications, such as gastrointestinal perforation. Here, we describe a girl with IgAV complicated with severe abdominal symptoms and lacking purpura. Despite this lack, the patient's elevated levels of D-dimer and C-reactive protein (CRP), suggestive of vasculitis, and localized small bowel intestinal wall thickening suggested IgAV. After administration of steroids relieved the abdominal symptoms and hypoalbuminemia, treatment was discontinued. Given the limited reports of patients with IgAV complicated with severe abdominal symptoms and no skin symptoms, the diagnosis and management process remains unclear. Therefore, it is imperative to consider IgAV as a differential diagnosis in patients with severe abdominal symptoms. Furthermore, we suggest checking D-dimer, CRP, and coagulation factor XIII activity levels in these patients.
Periodic fever syndromes are heterogeneous diseases. Familial Mediterranean fever (FMF) is one of the hereditary periodic fever diseases caused by a Mediterranean fever (MEFV) gene abnormality. FMF can be categorized as typical or atypical, based on clinical findings and genetic screening. Atypical FMF has a wide variation of clinical findings and disease-causing mutations of MEFV. Therefore, it is sometimes difficult to diagnose an unknown fever as FMF. To date, a large number of various typical and atypical FMF cases have been reported in Japan. Here, we describe a Japanese boy with heterozygous MEFV p.Ser503Cys exon 5 variant who developed periodic fever. He was treated with colchicine; a complete eradication of his fever and various accompanying symptoms have been subsequently achieved for more than a year. Given that there have been a few reports about patients with this variant, little is known about the genetic and phenotypic role of heterozygous MEFV p.Ser503Cys exon 5 variant. It is therefore imperative to consider atypical FMF as a differential diagnosis when a periodic fever is encountered. Furthermore, we suggest that it is worthwhile to integrate MEFV gene analysis with the potential effects of colchicine treatment in patients with periodic fever.
A case of Jejunogastric intussusception (JGI) after gastrectomy is presented. A 72-year-old woman was admitted to the hospital because of sudden epigastric pain and vomiting followed by hematemesis. There was a history of undergoing a gastric resection (Billroth II) for a gastric cancer 17 years before. Endoscopic examination revealed that a large reddish-blue intesitne loop filled the lumen of the residual stomach. Abdominal CT also revealed a mass with ringed structure in the gastric remnant and jejunum. Based on the diagnosis of JGI, an emergency laparotomy was performed 24 hours after the onset of the symptoms. At operation, the efferent jejunal loop was found to be intussuscepted retrogradely through the entero-enterostomy and then via the afferent loop into the gastric remnant. After manual disinvagination, a part of gangrenous jejunal intussuscepiens was resected and gastro-jejunostomy was performed with Roux-en Y-type reconstruction. The postoperative course was uneventful and the patient was discharged on 26th hospital day. In a review of the Japanese literature, anather 28 cases of JGI are seen. These 29 cases including our case are characterized by: 1) that Billroth II method was employed in the initial operation in all cases; 2) most cases had a retrograde intussusception of the efferent jejunal loop; 3) the disease suddenly developed after a long-term interval after the initial operation; 4) the diagnosis was easily made by endoscopy, X-ray examination of the stomach, abdominal CT and abdominal ultrasonography; and 5) the prognosis was relatively good by appropriate treatment.
Angiogenesis inhibitors have attracted considerable interest. The anti-tumor and anti-metastatic effects of TNP-470, an angiogenesis inhibitor, and mitomycin C (MMC), a representative anti-neoplastic agent, were investigated using a xenotransplanted human colon cancer, TK-4. Suturing of small pieces of TK-4 tumors to the cecal wall in nude mice (orthotopic transplantation) induced liver metastasis. Mice were randomly divided into 3 groups; a control group given saline solution, a group receiving TNP-470 and a group receiving MMC. TNP-470 was given s.c. on alternate days for 5 weeks from day 10 after cecal transplantation and MMC was administered intraperitoneally (i.p.) once a week from day 10 after cecal transplantation. MMC significantly inhibited cecal tumor growth. In the control group, liver metastases developed in 9 out of 10 mice, including 3 with more than 20 metastatic foci. Liver metastasis also developed in 8 out of 10 mice receiving MMC, 2 of which had many metastases. In contrast, liver metastasis developed in only 2 out of 8 mice in the TNP-470 group and neither of these animals had numerous metastases.
Interleukin-2 was entrapped in liposomes (Lip-IL-2) and injected into rats. The intraperitoneal injection of Lip-IL-2 into rats bearing an ascites-forming rat hepatoma (AH-66) significantly increased the survival time when compared with rats administered free IL-2 or saline-containing liposomes. The number of peritoneal exudate cells (PEC) increased markedly after intraperitoneal injection of Lip-IL-2 and consisted mainly of macrophages. The level of tumor necrosis factor alpha (TNF-alpha) and the intensity of free radicals increased in the ascites at 48 hrs after Lip-IL-2 administration, whereas TNF-alpha was not detected and the intensity of free radicals did not increase after free IL-2 administration. Our findings suggested that entrapment of IL-2 into liposomes enhanced its potential for cancer therapy, presumably by activating macrophages to produce TNF-alpha and free radicals.
