Use of an appendicitis medical information sheet in the pediatric primary care system.

BACKGROUND: Accurate and prompt diagnosis is required for the primary evaluation of pediatric appendicitis. Among pediatricians and surgeons working in Yamanashi Prefecture, the pediatric appendicitis medical information (PAMI) sheet was edited in April 2011 to reflect the diagnostic results of the pediatric primary and emergency medical service and used as a referral document for surgical consultation to secondary hospitals. METHODS: The PAMI sheet consisted of sections for history taking, symptoms, physical signs and laboratory findings without a scoring system. For 32 consecutive months starting in April 2011, 59 patients hospitalized for suspected appendicitis were retrospectively reviewed. In particular, a total of 17 referral patients evaluated with the PAMI sheet were assessed in order to evaluate the utility of the form. RESULTS: The pediatric surgeons were able to easily determine patient condition from the PAMI sheet. In total, 13 of 17 patients had appendicitis. According to the physical findings of the 17 studied patients, the judgment of right lower quadrant tenderness (κ = 0.63) and guarding (κ = 1.00) was consistent between the pediatric surgeons and primary attending pediatricians. CONCLUSIONS: The PAMI sheet aids in the collection of detailed history and objective data with a high level of accuracy, and provides useful referral diagnostic information to the secondary-level hospitals.

[Thoracoscopic diaphragmatic plication for eventration of diaphragm in children using no-knife automatic suturing device].

UNLABELLED: Diaphragm eventration could inhibit the lung development due to compression. Thus diaphragm plication is required for the diaphragm eventration to prevent lung compression causing lung immaturity. However, we sometimes encounter the difficulty in endoscopic plication for fragile diaphragm without damaging it in narrow thoracic space in children. We demonstrate the plication using no-knife automatic suturing device. METHOD: Two linear ridges are made using stapler on the flaccid diaphragm without cutting the tissue. Then the created 2 ridges are sutured so that the diaphragm is plicated. BENEFITS: Once the stapler was applied to make 2 linear ridges, we easily sutured and gathered them without checking the damage of the intra-abdominal organs. Furthermore, reinforced ridges could be plicated without damaging the fragile diaphragm. We conclude that above described method is preferable for the diaphragm eventration in pediatric patients with fragile diaphragm and limited thoracic space.

Costal BCG osteomyelitis developing 1 year after BCG vaccination.

Reported here is the case of a 15-month-old boy with costal osteomyelitis due to the bacillus Calmette-Guérin (BCG) vaccine against tuberculosis. Mild complications of this vaccine, such as localized abscess and regional lymphadenitis, are occasionally recognized, but its association with osteomyelitis is extremely rare.

ERAP140/Nbla10993 is a novel favorable prognostic indicator for neuroblastoma induced in response to retinoic acid.

In the present study, we identified a gene termed Nbla10993 whose expression levels are higher in favorable neuroblastomas versus unfavorable ones. Structural analysis showed that Nbla10993 is a novel splicing variant of the ER-associated protein of 140 kDa (ERAP140), which lacks the central acidic as well as the COOH-terminal Cys/His-rich domain. Similarly, ERAP140 was preferentially expressed in favorable neuroblastomas relative to unfavorable ones. During the all-trans-retinoic acid (ATRA)-mediated neuronal differentiation in neuroblastoma-derived RTBM1 cells, the expression levels of ERAP140/Nbla10993 increased at the mRNA level. Consistent with these observations, the luciferase reporter analysis demonstrated that the ERAP140/Nbla10993 promoter responds to ATRA. In addition, the immunoprecipitation/immunoblotting experiments showed that ERAP140 forms a stable complex with RARalpha but not with RXRalpha in cells, suggesting that ERAP140 is involved in RAR-mediated transcriptional regulation. Furthermore, the quantitative real-time PCR analysis using 109 primary neuroblastoma samples demonstrated that the expression levels of ERAP140/Nbla10993 significantly correlate with a better clinical outcome of neuroblastomas. Taken together, our present findings indicate that ERAP140/Nbla10993 plays an important role in the regulation of ATRA-mediated neuronal differentiation, and is a novel member of prognostic indicators for neuroblastoma.

Perforation of Meckel's diverticulum manifesting as aseptic peritonitis in a neonate: report of a case.

We report a case of perforated Meckel's diverticulum with aseptic peritonitis in a 17-day-old neonate. The baby had been brought to the hospital with fever and abdominal distention. Abdominal computed tomography showed a 5-cm abscess in the lower abdomen, and emergency laparotomy was performed for suspected perforated appendicitis. However, we found a perforated Meckel's diverticulum. No bacteria were detected in the purulent ascites from the peritoneal cavity. We speculate that the narrow lumen between the small intestine and the diverticulum, accompanied by poor self-emptying had caused acute inflammation resulting in perforation of Meckel's diverticulum. The anatomic limitations in "walling off" the perforated Meckel's diverticulum by the surrounding loops of small intestine prevented the bowel contents from spreading within the peritoneal cavity.

Stabilization of p73 by nuclear IkappaB kinase-alpha mediates cisplatin-induced apoptosis.

In response to DNA damage, p53 and its homolog p73 have a function antagonistic to NF-kappaB in deciding cell fate. Here, we show for the first time that p73, but not p53, is stabilized by physical interaction with nuclear IkappaB kinase (IKK)-alpha to enhance cisplatin (CDDP)-induced apoptosis. CDDP caused a significant increase in the amounts of nuclear IKK-alpha and p73alpha in human osteosarcoma-derived U2OS cells. Ectopic expression of IKK-alpha prolonged the half-life of p73 by inhibiting its ubiquitination and thereby enhancing its transactivation and pro-apoptotic activities. Consistent with these results, small interfering RNA-mediated knockdown of endogenous IKK-alpha inhibited the CDDP-mediated accumulation of p73alpha. The kinase-deficient mutant form of IKK-alpha interacted with p73alpha, but failed to stabilize it. Furthermore, CDDP-mediated accumulation of endogenous p73alpha was not detected in mouse embryonic fibroblasts (MEFs) prepared from IKK-alpha-deficient mice, and CDDP sensitivity was significantly decreased in IKK-alpha-deficient MEFs compared with wild-type MEFs. Thus, our results strongly suggest that the nuclear IKK-alpha-mediated accumulation of p73alpha is one of the novel molecular mechanisms to induce apoptotic cell death in response to CDDP, which may be particularly important in killing tumor cells with p53 mutation.

Spontaneous disappearance of focal ground-glass opacity in the lung detected on the high-resolution computed tomography.

We report a case of a 52-year-old female with spontaneous disappearance of focal ground-glass opacity (GGO) detected on high-resolution computed tomography (HRCT). She initially was found to have a focal GGO lesion on follow-up computed tomography five years after left lower lobectomy for primary lung adenocarcinoma. Two months later, this lesion spontaneously disappeared in spite of the suspicion of subsequent lung cancer. A GGO appearance on HRCT can be found in a variety of diseases with partial air-space filling or interstitial thickening. Lung cancer, especially bronchioloalveolar carcinoma, is also included in the differential diagnosis for GGO lesions. However, there is little definite evidence about the management of lesions showing a GGO appearance. When we detect a GGO lesion, initial monitoring for several months may be useful for excluding the presence of lung neoplasms.

Bronchioloalveolar carcinoma of the lung 3 centimeters or less in diameter: a prognostic assessment.

BACKGROUND: Bronchioloalveolar carcinoma (BAC) of the lung is a subtype of adenocarcinoma for which the incidence is actually rising, but the histologic definition of BAC has been recently changed by the revised World Health Organization (WHO) classification in 1999. The clinical features of patients with BAC diagnosed according to the recently revised WHO classification have not yet been clarified. In this retrospective study, we investigated the pattern of recurrence and survival outcome for patients with resected BAC by pathology review, compared with those in patients who had adenocarcinoma other than BAC. METHODS: From 1985 through 2002, 108 patients underwent surgical resection for pulmonary adenocarcinoma 3 cm or less in diameter at the University of Yamanashi, Japan. All of the resected specimens of these 108 patients were pathologically reviewed again to confirm the diagnosis as BAC or adenocarcinoma other than BAC. The tumor was defined as BAC when the adenocarcinoma lesion had a pure bronchioloalveolar growth pattern and no evidence of stromal, vascular, or pleural invasion according to the WHO classification (third edition). RESULTS: Twenty-five patients (23%) had a diagnosis of BAC, and 83 (77%) had a diagnosis of other adenocarcinoma. There was a female predominance among both patients with BAC and those with other adenocarcinoma. Lymph node involvement was seen for 30 lesions (36%) of adenocarcinoma other than BAC, but not for any BAC lesions. The median duration of follow-up after surgery was 5.1 years. There was no recurrence in the postoperative course in patients with BAC for a 5-year disease-free survival rate of 100%, whereas the 5-year disease-free survival rate for other adenocarcinoma was 63.5%. CONCLUSIONS: The patients with resected BAC, which is defined as a noninvasive adenocarcinoma by the revised WHO classification, had an excellent prognosis. However, these results may depend on a strictly accurate pathology diagnosis as BAC. Limited resection might be curative in patients with focal BAC based on evidence of pathologic noninvasive features.

[Tamoxifen-induced severe hypertriglyceridemia--report of 3 cases].

Tamoxifen, an anti-estrogen, has been used for a long time as an adjuvant therapy in cases of estrogen receptor positive breast cancer. Tamoxifen also demonstrates some weak estrogenic activity. A small increase in serum triglycerides is commonly found after tamoxifen administration. Herein we report 3 cases of sever hypertriglyceridemia due to tamoxifen. Case 1 recovered with tamoxifen withdrawal. Tamoxifen was replaced with toremifene in case 2. The level of triglyceride decreased significantly after the change of agent. Tamoxifen was discontinued and anastrozole administration was started in the third patient. Her triglyceride levels improved. Tamoxifen-induced severe hypertriglyceridemia seen in these patients was an effect of its estrogen action. Anastrozole has been used to treat postmenopausal metastatic breast cancer, and several clinical trials in the adjuvant setting are ongoing. Anastrozole does not affect lipid metabolism. Therefore, anstrozole might be safe for patiens with abnormal triglyceride profiles during tamoxifen treatment. We recommended that a periodic serum triglyceride check is needed for patients treated with tamoxifen.

[Development of severe aplastic anemia in a girl with Hashimoto's thyroiditis and papillary thyroid carcinoma].

A 14-year-old girl was admitted because of general fatigue and cervical lymphadenopathy. She showed bilateral struma (IInd degree) and enlargement of her left cervical lymph nodes. Laboratory data revealed neutropenia (219/microliter) and thrombocytopenia (Plt 5.1 x 10(4)/microliter) with mild anemia (Hb 11.1 g/dl), and the bone marrow aspirate and biopsy specimens showed hypocellularity. In addition, auto-antibodies against thyroid peroxidase (TPO) and thyroglobulin (TG) were highly elevated. Computed tomography of the neck showed a nodule in the left thyroid lobe with marked lymphadenopathy, and fine needle aspiration biopsy demonstrated papillary thyroid carcinoma with Hashimoto's thyroiditis and metastasis to the lymph nodes. One month after left thyroid lobectomy and cervical lymphadenectomy, the patient's condition progressed to very severe aplastic anemia, and she received immunosuppressive therapy consisting of cyclosporin A and anti-thymocyte globulin. Hematologically, partial and complete responses were obtained three and six months later, respectively. Of interest, anti-TPO and TG antibody titers remarkably decreased after immunosuppressive therapy. The patient had HLA-DR 2(DRB 1*1501) and DR 8(DRB 1*0802). The former is frequently found in patients with cyclosporin A-dependent aplastic anemia, and the latter is frequently found in Asian patients with Hashimoto's thyroiditis, suggesting an underlying autoimmune background for the simultaneous outbreak of aplastic anemia and Hashimoto's thyroiditis complicated by thyroid carcinoma.

[Delivery of twins after undergoing adjuvant LHRH analogue and CEF therapy].

Chemotherapy is an important part of adjuvant therapy for younger patients with breast cancer, but prevention of ovarian dysfunction due to chemotherapy is important. There are some reports that pretreatment with LHRH analogue can preserve ovarian function. The present patient, who was treated in this way, delivered twins 6 years after surgery. A 33-year-old woman underwent breast conserving surgery for 1 cm carcinoma in the left A region, and pathologic findings were papillotubular carcinoma. t2n1 alpha (2/18) ER (+) PgR (+). LHRH analogue was administrated together with radiation therapy for the preserved breast. Six subsequent courses of CEF therapy were given, and then LHRH analogue was administered for 2 years and tamoxifen for 2 years and six months. Menstruation was recovered 6 months later and twins were delivered 3 years after the last administration of hormone therapy. This case should be referred in the treatment of young patients.