Neuroblastoma metastatic to the liver in infants

Four infants are described who presented with rapid enlargement of the liver. This was found to be due to neuroblastoma which had metastasized to the liver; the condition was associated with high levels of urinary vanillylmandelic acid (VMA). In 3 infants the primary tumour was in the adrenal gland and in one it was not identified. One infant died after laparotomy and 2 infants survive disease free with normal VMA levels, one after adrenalectomy and hepatic irradiation and one after a short course of chemotherapy. The fourth patient responded initially to hepatic irradiation and chemotherapy, but relapsed 2 years later with recurrent disease; at the same time the VMA level which had been normal, again rose. The importance of screening for an abnormal VMA level in any infant with a rapidly enlarging liver in order to obtain an early diagnosis is stressed. Careful follow-up, with serial VMA estimations, is essential to detect recurrent disease. The prognosis for some infants with this distribution of neuroblastoma which has metastasized to the liver, but not to the bones or oribt, is good.     

Uncooked cornstarch--efficacy in type I glycogenosis

Uncooked cornstarch (UCCS) loads in 14 patients with type 1 glycogenosis revealed that satisfactory glycaemia was achieved for a median of 4.25 hours (range 2.5 to 6). Length of glycaemia was related weakly to UCCS dose, but not to patient age or measures of metabolic control. Careful monitoring is required during UCCS treatment.     

Temporomandibular joint arthrography: comparison of morbidity with ionic and low osmolality contrast media

We compared patient morbidity associated with temporomandibular joint (TMJ) arthrography using both meglumine/sodium diatrizoate (60%) and the new monoacidic dimer, Hexabrix, in a double-blind randomized clinical trial in 31 patients. Patients experienced maximal discomfort from TMJ arthrography with the initial joint filling and joint distension; this rapidly resolved over 10 minutes. Delayed exacerbation of pain is less than described for shoulder arthrography. The newer contrast media promise to decrease patient morbidity with arthrography.     

A pharmacological approach to gastric acid inhibition

Proton pump inhibitors have changed our approach to gastric acid-related diseases. They are much more potent acid inhibitors than H2-antagonists and exhibit a sophisticated mechanism of action. The present review analyses the general pharmacology of proton pump inhibitors and differentiates the specific characteristics of the various compounds that belong to this family. Special emphasis has been placed in describing the clinical implications of such differences and the potential importance for adverse effects.     

锌缺乏对神经病理性疼痛模型小鼠行为学的影响

目的研究饮食锌缺乏对神经病理性疼痛模型小鼠痛域的影响。方法制备饮食锌缺乏小鼠,采用小鼠坐骨神经分支选择损伤模型,金属自显影和原子吸收光谱检测小鼠脊髓后角锌稳态,检测小鼠痛域。结果 AMG结果证实,与假手术组相比,模型组小鼠和锌缺乏组小鼠脊髓后角游离锌离子减少,与模型组小鼠相比,锌缺乏小鼠脊髓后角游离锌离子减少。原子吸收光谱结果表明,锌缺乏组小鼠总锌含量较假手术组减少。痛域检测结果表明,锌缺乏小鼠痛域下降。结论锌离子可能参与小鼠脊髓后角痛觉专递。     

巨大下颌骨囊性病变分期治疗效果评价

目的:探讨对巨大下颌骨囊性病变进行分期治疗的效果。方法:选择2005—2009年在本院收治的下颌骨范围大于5cm的囊性病变18例,其中伴牙列不齐者6例,一期局麻下行减压术,待范围减小至小于原来的1/2后,行二期全麻下囊性病变摘除术,即刻Bio-oss骨粉植入,术后每月定期拍摄口腔全景片观察骨密度变化,伴牙列不齐的6例患者手术后2个月开始以常规正畸加力牵引。结果:一期术后4~6个月,病变减小至原来的1/2左右,二期术后6~12个月,骨粉与周围正常骨密度基本一致,2~3a后无一例复发。6例牙列不齐的患者,经1~2a正畸治疗后,错畸形得以矫正。结论:分期治疗既彻底摘除了病变,又保存了下颌骨的连续性及自体牙,还可同时结合正畸治疗,改善错畸形,提高美学效果。     

Ambiguous phenotypes and genotypes in 16 children with acute leukemia as characterized by multiparameter analysis

Ambiguous phenotypes and genotypes were observed in 16 children with acute leukemia. Surface marker, cytogenetic, molecular genetic, and DNA flow cytometric analyses as well as standard morphologic and cytochemical studies were used to divide the patients into three groups. The first group comprised five children with acute leukemia whose blast cells were morphologically lymphoid, while immunophenotyping disclosed simultaneous expression of early pre-B cell and myeloid features. Molecular genetic studies showed evidence of heavy-chain immunoglobulin (Ig) gene rearrangements in all patients. Cytogenetic data, available in three of these children, revealed t(4;11). In five of the 16 patients, morphologic and surface marker analyses indicated the coexistence of two separate cell populations, one with myeloid and the other with early pre-B cell features. Further evidence of B cell commitment in these patients was provided by demonstration of Ig heavy-chain gene rearrangements in all five patients. Surprisingly, one of the five patients showed oligoclonal Ig heavy-chain as well as monoclonal gene rearrangement for the beta chain of the T cell receptor (beta-TCR). The last group consisted of four cases with otherwise typical acute lymphoblastic leukemia (ALL), early pre-B cell phenotype, and coexpression of myeloid or T cell-associated antigens, and two children with unequivocal acute myeloid leukemia (AML) and coexpression of T cell antigens. Gene rearrangement of Ig heavy-chain could be demonstrated in five of six patients, additional Ig light-chain gene rearrangement in two children with ALL, and bigenotypic features (Ig heavy-chain and beta-TCR gene rearrangement) in one patient. In none of the 16 patients did flow cytometry disclose clonal abnormalities of leukemic cell DNA content. Based on these findings, we suggest that malignant transformation in the first and second group of patients took place at a stage ontogenetically close to the pluripotent stem cell, whereas ambiguous phenotypes in the third group resulted from aberrant gene expression or insufficient reagent specificity.