Efficacy of granulocyte apheresis in pediatric patients with ulcerative colitis: a pilot study.

OBJECTIVES: Granulocyte apheresis (GCAP), involving the removal of granulocytes from the blood, may improve clinical symptoms and facilitate a reduction in the dose of steroids in adult patients with ulcerative colitis. As a preliminary trial, GCAP was used to taper the dose of steroids in 4 pediatric patients with ulcerative colitis. METHODS: Three males and 1 female ranging from 11 to 17 years old were treated with GCAP once per week for 5 consecutive weeks/course. The ages of patients at clinical onset ranged from 8 to 12 years and the length of time from the clinical onset to GCAP treatment ranged from 28 to 58 months (median, 38.5 months). RESULTS: In 2 patients, symptoms and signs indicating disease activity improved after 2 courses of GCAP. Laboratory data and endoscopic findings also improved after treatment and the clinical efficacy was judged to be excellent in these patients. In 1 patient, GCAP improved laboratory and endoscopic hallmarks, but bloody stools persisted. Finally, the treatment was ineffective in the fourth patient who eventually underwent surgery. CONCLUSIONS: GCAP is effective in improving clinical symptoms and may play an important role in converting steroid therapy to other treatments in children with steroid-refractory or steroid-dependent ulcerative colitis.

Diffuse xanthogranulomatous pyelonephritis in a patient with myotonic dystrophy and cerebral palsy.

Xanthogranulomatous pyelonephritis (XGP), a morphological and clinical variant of chronic pyelonephritis, is an uncommon disease in children. It is characterized by the destruction of the renal parenchyma and replacement by granulomatous tissue containing foamy lipid-laden macrophages and is classified into diffuse and focal XGP. We present a case of diffuse XGP in a child with myotonic dystrophy complicated by cerebral palsy and discuss the importance of correct diagnosis and preoperative management to reduce inflammation and improve malnutrition associated with the disease.

Laparoscopic versus open appendectomy in children with uncomplicated and complicated appendicitis.

PURPOSE: To address whether laparoscopic appendectomy could be an alternative to conventional open appendectomy in children with complicated appendicitis as well as uncomplicated appendicitis, a retrospective study comparing laparoscopic and open appendectomies was performed. METHODS: One hundred patients who were treated by immediate appendectomy, either laparoscopically or by the open method, between May 2000 and August 2003 were included in the study. There were 53 patients in the laparoscopic appendectomy group and 47 patients in the open appendectomy group. RESULTS: The operating time was significantly longer for laparoscopic appendectomy than for open appendectomy (P < .001). The length of hospital stay was significantly shorter in laparoscopic appendectomy in patients with uncomplicated appendicitis (P = .001). Thirteen of the 100 patients (13.0%) had 15 postoperative complications including wound infection (n = 8), intraabdominal abscess (n = 4), stitch abscess (n = 2), and small bowel obstruction (n = 1). In both uncomplicated and complicated appendicitis, there was no significant difference between laparoscopic and open appendectomies in the complication rates, and the incidences of each complication did not differ between the procedures. Among the 14 patients with generalized peritonitis, postoperative complications were seen in 5 patients (35.7%). Although the presence of generalized peritonitis was associated significantly with postoperative complications (P = .017), there was no significant association between the procedure and complications. Overall treatment costs were increased by 26.0% in laparoscopic appendectomy. CONCLUSIONS: Laparoscopic appendectomy should remain an option in children with uncomplicated and complicated appendicitis, and when laparoscopy is selected, consideration of the advantages and disadvantages of the procedure is essential.

Infantile intestinal leiomyosarcoma is prognostically favorable despite histologic aggressiveness: case report and literature review.

Intestinal leiomyosarcomas in children are extremely rare. The authors present a case of infantile intestinal leiomyosarcoma of the colon that was resected and was classified histologically as grade 2 because of abundant mitoses. The tumor cells infiltrated the submucosa and the serosal surface. Immunohistochemically, these cells were positive for alpha-smooth muscle actin but not for CD34 or CD117, by which the tumor was differentiated from gastrointestinal stromal tumor (GIST). The patient underwent follow-up without adjuvant therapy and is now doing well with no evidence of the disease for 42 months. Based on the current case and a review of 18 reported cases, it is concluded that infantile intestinal leiomyosarcoma, which is differentiated from GIST, has a favorable prognosis even when the histologic appearance is suggestive of aggressive biological characteristics. Adjuvant therapy is unnecessary when tumors are completely resected.

D-lactic acidosis in short-bowel syndrome managed with antibiotics and probiotics.

D-lactic acidosis sometimes occurs in malabsorbed patients with short-bowel syndrome and is characterized by recurrent episodes of encephalopathy and metabolic acidosis. The characteristic neurologic abnormalities and the presence of metabolic acidosis raises a diagnostic suspicion, and the diagnosis is made when the serum level of D-lactic acid is greater than 3 mmol/L. Standard treatment consists of restricting oral carbohydrates or fasting, correction of metabolic acidosis, and a long-term suppression of pathogenic floras with antibiotics. The authors present a case of D-lactic acidosis in a 22-year-old patient with short-bowel syndrome, to whom intestinal bacterial agents (probiotics) were given in addition to oral kanamycin. Recolonization of the intestine with nonpathogenic floras should be a long-term treatment for D-lactic acidosis.

Umbilicoplasty for large protruding umbilicus accompanying umbilical hernia: a simple and effective technique.

A large umbilical protrusion with redundant skin accompanying an umbilical hernia sometimes needs umbilicoplasty. Although several different techniques for making umbilical depression have been used, the results of the plastic surgery are sometimes unsatisfactory due to postoperative flattening or disappearance of the umbilical depression. To make a permanent umbilical depression that is cosmetically acceptable, we have modified the techniques. Umbilicoplasty was performed in 14 children whose ages ranged from 6 months-6 years and 3 months (median, 1 year and 10 months) and who had umbilical hernia with a large umbilical protrusion. After the fascial defect was closed, the diameter of the umbilicus was reduced to half that before surgery by removing fan-shaped skin flaps and approximating skin edges, and then inverting the umbilicus and fixing it caudally to the fascia and skin. There were no postoperative complications, and no flattening or disappearance of umbilical depression was observed during the follow-up of 10-19 months. The authors' technique of umbilicoplasty for a large protruding umbilicus accompanying umbilical hernia is a simple method that produces acceptable cosmetic results.

Epidermoid cyst: rare testicular tumor in children.

Epidermoid cyst of the testis, extremely rare in children, is a non-teratomatous benign tumor, and testis-sparing surgery should be the treatment of choice. To prevent unnecessary orchiectomy, recognition of this rare tumor in children is essential.