Clinical Usefulness of Blenderized Food for Selenium Deficiency in Pediatric Patients with Severe Motor and Intellectual Disabilities.

INTRODUCTION: This study aimed to assess the preventive or therapeutic effects of blenderized food (BF) on selenium deficiency in pediatric patients with severe motor and intellectual disabilities (SMID). METHODS: The medical records of all 40 consecutive pediatric patients with SMID who underwent nutritional assessment were retrospectively reviewed and compared between two groups: the enteral formula (EF) group and the BF group fed with BF providing more than 10% of total caloric intake. Next, for the selenium-deficient patients who were newly started on blenderized tube feeds after the first nutritional assessment, improvement of selenium deficiency and change of dietary contents were assessed. RESULTS: The BF group patients had a significantly lower prevalence of selenium deficiency and higher serum selenium levels than the EF group patients. In all 7 selenium-deficient patients who started blenderized tube feeds after the first nutritional assessment, serum selenium levels were significantly increased at the second nutritional assessment, even though total selenium intake, selenium intake by EF, and total caloric intake did not differ significantly, and, in fact, caloric intake was significantly decreased by EF. CONCLUSION: Combined feeding of BFs can be useful for prevention and therapy of selenium deficiency in pediatric SMID patients.

Kampo medicines Rikkunshito and Hangeshashinto prevent cisplatin-induced intestinal mucosal injury in rats.

BACKGROUND/PURPOSE: We examined the effects and mechanisms of rikkunshito (RKT) and hangeshashinto (HST) on cisplatin-induced mucosal injuries in the rat small bowel. METHODS: Juvenile rats were divided into 6 groups: sham control, cisplatin injection without kampo medicines, and cisplatin injection with oral administration of low and high doses of RKT (1000 mg/kg and 2000 mg/kg) and HST (500 mg/kg and 1000 mg/kg). Fecal condition, intestinal morphological changes, enterocyte proliferation, and enterocyte apoptosis were assessed. RESULTS: Diarrhea and atrophy of ileal villi observed in the cisplatin group were significantly improved in all kampo groups. Injury scores of the jejunum were significantly lower with RKT (2000 mg/kg) and HST (500 and 1000 mg/kg) than with cisplatin, and those of the ileum were significantly lower with HST (500 and 1000 mg/kg) than with cisplatin. Enterocyte proliferation of the jejunum was significantly increased with RKT (2000 mg/kg) and HST (500 mg/kg) compared with cisplatin, and those of the ileum were significantly increased in all kampo groups compared with the cisplatin group. Jejunal and ileal apoptosis following cisplatin administration was significantly inhibited by HST. CONCLUSIONS: RKT and HST prevented cisplatin-induced intestinal mucosal injury with increasing proliferation of intestinal epithelial cells. HST also attenuated cisplatin-induced crypt cell apoptosis.

Problems during the long-term follow-up after surgery for pediatric solid malignancies.

INTRODUCTION: With the recent improvements in the prognosis of pediatric malignancies, the number of patients surviving long-term after surgery has been increasing. Therefore, the late effects of cancer treatments are important issues. In this study, we analyzed the problems associated with the treatment of pediatric patients during the long-term follow-up after surgery. PATIENTS AND METHODS: A total of 64 patients with pediatric malignancies who underwent surgical treatment and were followed up for more than 5 years and who were older than 13 years of age were included in this study. The average age was 20.8 (13-33) years, and the follow-up ranged from 5 to 31 years (mean, 17.7 years). Twenty-one patients (32.3%) received high-dose chemotherapy (HDC) and nine (14.1%) received radiotherapy. RESULTS: In this study, 46 patients (71.9%) developed at least one problem during the follow-up period. With regard to the surgical problems, 14 patients underwent nephrectomy, and 1 of them developed renal failure. One patient received cystectomy with urinary tract reconstruction. One patient received a partial vaginectomy. Two cases with ovarian tumors received oophorectomy, one of whom also received partial hysterectomy. Other complications such as ileus, scoliosis, and leg length discrepancies were seen in some patients. In terms of the medical problems, 15 patients showed growth retardation and 2 were treated with growth hormone therapy. Gonadal dysfunction was observed in 23 patients, and 8 of them were treated with hormone replacement therapy. Six patients developed hypothyroidism, two of whom were treated with thyroid hormone replacement therapy. Other medial issues, such as hearing impairment, low bone mineral density, and hepatitis, were seen in some patients. The rate of growth retardation, gonadal dysfunction, and hypothyroidism were significantly higher in the patients who received HDC (p<0.05). There was one case of second malignancy of the parotid gland. CONCLUSION: Various treatment-related complications may occur even many years after treatment, especially in patients who receive HDC. Medical problems, especially endocrine disorders, appear to be more serious than surgery-related problems. Lifetime medical surveillance and continuous follow-up by not only pediatric surgeons but also by various specialists, such as pediatric oncologists, pediatric endocrinologists, urologists, and gynecologists, are necessary.

Outpatient administration of radioactive iodine after total thyroidectomy for pediatric thyroid cancer: a report of three cases.

Radioactive iodine is used as an anti-cancer reagent for papillary and follicular thyroid carcinomas. Patients that are administered a large dose of radioactive iodine are required to stay alone in an isolated room for several days. Some young children with thyroid carcinoma who cannot take care of themselves are not able to undergo this therapy. We tried outpatient administration of (131)I for these patients. In Japan, administration for outpatients is restricted to 500 MBq (13.5 mCi). We administrated 13 mCi (131)I to three patients suffering from pediatric thyroid carcinomas. One patient with recurrent follicular carcinoma in the neck showed complete loss of the thyroid bed and an undetectable level of serum thyroglobulin 7 months after the first administration of (131)I, and no further recurrence was observed after 5 years. Another patient with multiple lung metastases showed weakened uptake of (131)I in the lung and an evident decrease in serum thyroglobulin after administration. However, the last patient with lung metastases with a low (131)I uptake showed no evident change in either (131)I uptake in the lung nor the serum thyroglobulin level. Administration of 13 mCi (131)I was effective in two of three patients. Thus, it is worth considering this therapy as a practical option for pediatric thyroid carcinoma in the countries in which outpatient administration of a large dose of (131)I is restricted.

Pediatric thyroid cancer arising after treatment for pleuropulmonary blastoma.

A 3-year-old female presented with a large tumor occupying the right thoracic space. Open biopsy revealed the pathological diagnosis of pleuropulmonary blastoma. After the first-line chemotherapy, the patient underwent surgical resection, then two courses of high-dose chemotherapy. Three years later, follicular carcinoma of the right thyroid lobe was found, so a right hemithyroidectomy was performed. Five months later, the thyroid tumor recurred. The remaining thyroid lobe was completely excised and radioiodine therapy was administered. The patient has remained tumor-free for 3 years. The etiology and treatment of the uncommon combination of pleuropulmonary blastoma and thyroid carcinoma is discussed.

Silencing of MYCN by RNA interference induces growth inhibition, apoptotic activity and cell differentiation in a neuroblastoma cell line with MYCN amplification.

Although it has been suggested that the MYCN oncoprotein functions may influence tumorigenesis and patient survival in neuroblastoma, the mechanism of these functions remains unclear. To elucidate such molecular and biological mechanisms, we performed knock-down of MYCN expression using RNA interference (RNAi) method. MYCN-siRNAs (MYCN-siRNA) were transfected into the MYCN-amplified cell line NB-1. To verify the sequence specificity of the siRNA, we prepared three control groups (siRNA control group: siRNAs with no significant homology to any known sequences in human genome, mock control group: reagent and PBS, and the untransfected control group). The cells were analyzed by real-time RT-PCR, Western blotting, immunocytochemistry for gene expression. Cell proliferation activity was measured by WST-1 assay. TUNEL staining was performed to evaluate apoptosis. After the MYCN-siRNA transfection, the expression level of the MYCN mRNA was significantly reduced to 30% of those of the three control groups (p<0.05). Western blotting revealed an obvious reduction in MYCN protein level in the MYCN-siRNA group. On immunocytochemistry, intensity of nuclear staining of MYCN was weaker in the MYCN-siRNA group than in the three control groups. On WST-1 viability assay, cell proliferation after the MYCN-siRNA transfection was significantly suppressed compared to the three control groups (p<0.05). The TUNEL positive cells were frequently observed in the MYCN-siRNA group. Additionally, after the MYCN-siRNA transfection, the morphologic change which was suggestive of neuronal cell differentiation was observed and TrkA and TrkC expressions were also significantly up-regulated. Using RNAi method, the knock-down of MYCN expression induced growth-inhibition, apoptotic activity and cell differentiation in MYCN-amplified NB-1 cell line.

Laparoscopically assisted anorectoplasty using perineal ultrasonographic guide: a preliminary report.

BACKGROUND/PURPOSE: As minimal invasive surgery, laparoscopically assisted anorectal pull-through has been reported with new devices. However, it is not easy to create an accurate pull-through canal (PTC) because of the narrow space between the urethra and puborectal sling. The authors describe a new method using perineal ultrasonography. METHODS: The rectourethral prostatic fistula was dissected laparoscopically. Externally, electrostimulation identified the center of the muscle contraction, over which a 1.2-cm skin incision was made, and the lower part of PTC was created by hemostat forceps guided by electrostimulation. An ultrasonographic probe applied to the perineum demonstrated the urethra, and the forceps was advanced behind the urethra into the pelvic cavity using the ultrasonographic guide. Anorectal pull-through was performed after dilatation of the PTC with dilators. RESULTS: The authors applied this procedure in 5 cases of male high and intermediate anomalies. Surgical damages to the urethra and the levator and vertical muscles were not encountered. Postoperative fluoroscopic study demonstrated good anterior angulation and intact contraction and relaxation of those muscles. CONCLUSION: The combination of laparoscopic dissection, pinpointing the center of anal sphincter by electrostimulation, and identification of the urethra by ultrasonographic images from the perineum facilitated creation of appropriate PTC in the muscle complex.

Cow's milk allergy presenting Hirschsprung's disease-mimicking symptoms.

The authors present a neonatal case of allergic colitis, which manifested the difficulty of spontaneous defecation and irregular narrowing of distal rectum in contrast enema. Rectal suction biopsy showed positive acetylcholinesterase activity. These clinical, radiological and histological findings were indistinguishable from Hirschsprung's disease. Gastrointestinal symptoms were improved by the cessation of cow's milk formula. The present findings may impact on the less recognizable gastrointestinal manifestation of allergic colitis.