Multi-center analysis of calcinosis in children with juvenile dermatomyositis]

OBJECTIVES: To reveal the frequency and the clinical characteristics of dystrophic calcification that occurs in children with juvenile dermatomyositis, multi-center analysis was constructed. METHOD: Fifty children with JDM were enrolled, and 14 of them (28.0%) were complicated with calcinosis. Clinical symptoms and laboratory tests at onset, initial therapy and disease course were compared in children with and without calcinosis. RESULTS: The mean age of the onset of calcinosis was 4.78 +/- 3.33 years, and it was younger than those of children without calcinosis (8.66 +/- 3.85 years) (P = 0.0017). No differences of clinical manifestation except Gower's sign were observed. The frequency of positive anti-nuclear antibody was 7.1% in children with calcinosis and 52.9% without calcinosis (P = 0.0112). The initial therapy of methylprednisolon pulses gave no effects on prognosis of calcium deposition. The calcinosis appeared in 1.56 +/- 1.91 year after the onset of the disease. The various types of calcium deposition including large tumorous clumps, subcutaneous plaques or nodules, sheet-type calcification were deserved. They appeared over knee joints (64.3%), elbow joint (64.3%), and hip processes (50.0%). Calcinosis affecting the subcutaneous tissues frequently resulted in painful superficial ulceration of the overlying skin (42.9%), local infection (50.0%), and limitation of joint movement (14.3%). Although aluminum phosphate was effective in 2 children among 7, no other effective treatment was recommended. In 5 cases, surgical removal of tumorous clumps was operated. Thus, juvenile dermatomyositis is frequently complicated with calcinosis. This type of calcinosis was found to be unlikely to resolve completely, and resulted in severe disability in children.     

INTRA- AND INTERINDIVIDUAL VARIATION IN THE PHARMACOKINETICS OF TACROLIMUS (FK506) IN KIDNEY TRANSPLANT RECIPIENTS—IMPORTANCE OF TROUGH LEVEL AS A PRACTICAL INDICATOR

Background: Tacrolimus (FK506) is currently used as the primary immunosuppressant in clinical kidney transplantation in some centers. The purpose of this study was to evaluate the pharmacokinetics of this drug and to see if trough level, which has been used widely in therapeutic drug monitoring, can be used as an appropriate substitute for other pharmacokinetic measurement tests. Methods: The blood concentration-time curve was studied in 10 kidney transplant recipients on 26 Occasions after oral dosage of 2 to 18 mg every 12 hours. Whole blood concentration was measured by two-step irnmunoabsorption assay. Methylprednisolone was used as a concomitant immuno-suppressive drug. Results: The blood concentration-time curves showed remarkable interindividual variation. lntraindividual variation was also found, but the degree of variation was slight compared with interindividual variation. On 17 occasions of measurement in one patient, the dose was significantly correlated with trough (r = 0.684). C_max (r = 0.838) and AUC_0–12 (r = 0.817). In nine patients on nine occasions, however, the dose was not significantly correlated with trough (r = 0.351), C_max (r = 0.270) or AUC_0–12 (r = 0.355). t_max ranged from one to four hours (mean + SD; 2.8 + 1.3) and fluctuated in both intra- and interindividual measurements. In spite of a wide variation in the blood concentration-time-curve patterns, a highly significant linear relationship between trough and C_max or AUC_0–12 was observed in both intraindividual (C_max, r = 0.876; AUC_0–12, r = 0.926) and interindividual (C_max, f = 0.943; AUC_0–12, r = 984) measurements. Concluslons: We conclude that trough level is a practical acceptable indicator of the blood levels of tacrolimus, and can be used to monitor blood concentration.     

NECROTIZING MYOPATHY AS A REMOTE EFFECT OF GASTRIC CANCER ACCOMPANIED WITH HASHIMOTO'S THYROIDITIS

An autopsy case of a 74-year-old male who had shown clinically hypothyroidism due to chronic atrophic thyroiditis (Hashimoto's thyroiditis), and pathologically necrotizing myopathy as a remote effect of gastric cancer was reported.
Morphological features of this necrotizing myopathy was those of carcinomatous myopathy rather than those of hypothyroid or diabetic myopathy.
As for the pathogenesis of the necrotizing myopathy (as a Group IV of polymyositis of Walton and Adams), the malignancy might have played an important role as a trigger of the secondary immunological abnormality upon a pre-exizting longstanding immune disorder of Hashimoto's thyroiditis. Pseudomembranous colitis, which was thought to be related to antibiotics (Lincomycin), was also briefly discussed.     

Prognosis of hepatocellular carcinoma associated with Child class B and C cirrhosis in relation to treatment: a multivariate analysis of 411 patients at a single center

Background/Purpose: Given that the prognosis of patients with hepatocellular carcinoma (HCC) complicating severe cirrhosis remains uncertain, particularly with regard to various therapeutic strategies, we have evaluated the prognosis in a series of patients with homogeneous diagnostic and therapeutic histories. Methods: From 1990 to 1998, 411 consecutive HCC patients associated with Child class B and class C cirrhosis who did not have lymph node or distant metastasis were treated by partial hepatectomy (PH; n = 48), percutaneous ethanol injection (PEI; n = 105), transcatheter arterial chemoembolization (TACE; n = 189), chemotherapy, or supportive care (chemo/supportive; n = 69). Univariate survival curves were estimated. The Cox model, stratified by the treatment groups, was used for multivariate analysis. Results: As of January 1999, 305 patients (74.2%) had died. Overall median survival was 23.4 months. There were statistically significant differences between the survival times of patients receiving PH or PEI and TACE, as compared with those receiving chemo/supportive care. According to multivariate analysis, the independent predictive survival factors were: albumin level (≥3.0 g/dl), esophageal varices (i.e., absence), tumor size (≤3.0 cm), tumor number (solitary), and α-fetoprotein (AFP) level (<400 ng/ml). According to the total number of risk factors and the median survival, all patients were divided into four subgroups. For the score 0 group (no risk factor group), 3- and 5-year survival rates were 83.1% and 68.0% for PH, and 87.5% and 62.3% for PEI, respectively. In the score 1–2 group (one or two risk factors), survival rates at 3 and 5 years were 53.1% and 40.3% for PH, 54.8% and 33.2% for PEI, and 35.4% and 22.8% for TACE, respectively. For patients with a score of 3 or more, there were no differences among the treatment groups, excluding those with chemo/supportive care. Conclusions: These findings indicate that, in HCC patients with complicating Child B and C cirrhosis, PEI and PH should be considered first for subgroups of patients with scores (risk factors) of 0–2, as an acceptable survival rate was obtained in such patients. Therefore, the advantages and disadvantages of these therapies regarding tumor size and location should be counterbalanced. In patients with a score of 3 or more, TACE, when possible, could be a first choice because of its applicability and its adjuvant nature with respect to other therapies such as liver transplantation. Received: February 6, 2002 / Accepted: May 22, 2002 Offprint requests to: S. Ueno     

Lymphoepithelial Cyst of the Pancreas: Report of a Case

A lymphoepithelial cyst (LEC) is an extremely rare benign lesion of the pancreas. During a medical check-up, a 77-year-old man without any symptoms was found to have a cyst in the body of the pancreas. His serum carbohydrate antigen 19-9 level was slightly elevated. Computed tomography showed a multilocular, low-attenuating cyst on the superior surface of the pancreatic body. Thus, we performed distal pancreatectomy with splenectomy. Histological examination revealed that the cyst wall was lined with squamous epithelium and surrounded by abundant mature lymphoid tissue. Keratinous substances were present in the cyst. An LEC of the pancreas is associated with a good prognosis and, although unusual, it should be considered in the differential diagnosis of pancreatic cystic lesions. Minimal resection of the cyst should be performed whenever possible, and extensive surgery avoided. For patients with a high surgical risk, fine-needle aspiration biopsy may be considered.     

Spontaneous rupture of common iliac artery associated with fibromuscular dysplasia presenting with colic pain suggestive of ureteral stone

We report a case of spontaneous rupture of the common iliac artery associated with fibromuscular dysplasia (FMD). A 21-year-old previously healthy male presented with acute onset of colic pain, suspected to be caused by a ureteral stone. Abdominal computed tomography and angiography revealed a retroperitoneal hematoma caused by rupture of the common iliac artery. In spite of an emergency operation initiated quickly, the patient died. A pathological examination demonstrated FMD of the common iliac artery. Although very rare, it is important to bear in mind that the possibility of retroperitoneal hemorrhage exists in patient with sudden lumbago.     

Intraoperative blood salvage during liver resection: a randomized controlled trial

OBJECTIVE: A randomized controlled trial was conducted to clarify the effectiveness of intraoperative blood salvage in reducing blood loss. BACKGROUND: Although reduction of central venous pressure (CVP) is thought to decrease blood loss during liver resection, no consistently effective and safe method for obtaining the desired reduction of CVP has been established. METHODS: Living liver donors scheduled to undergo liver graft procurement were randomly assigned to a blood salvage group, in which a blood volume equal to approximately 0.7% of the patient's body weight was collected before the liver transection, or a control group. The surgeons were blinded to the randomization results. The primary outcome measure was blood loss during liver parenchymal division. A multivariate analysis was also performed. RESULTS: Seventy-nine donors were allocated intraoperatively to the blood salvage group (n = 40) or the control group (n = 39). The amount of blood loss during liver transection was significantly smaller in the blood salvage group than in the control group (median loss during transection, 140 mL vs. 230 mL, P = 0.034). The CVP at the beginning of the liver parenchymal division was significantly lower in the blood salvage group than in the control group (median, 5 cm H2O vs. 6 cm H2O, P = 0.005). The results of a multivariate analysis revealed that intraoperative blood salvage offered the advantage of reduced blood loss during liver parenchymal division (adjusted OR, 0.31; 95% CI, 0.11-0.85, P = 0.025). CONCLUSION: Modest intraoperative blood salvage significantly and safely reduced blood loss during hepatic parenchymal transection.     

A nonadhesive liquid embolic agent composed of ethylene vinyl alcohol copolymer and ethanol mixture for the treatment of cerebral arteriovenous malformations: experimental study

OBJECT: The authors have developed a mixture of ethylene vinyl alcohol copolymer (EVAL) and iopamidol, which is dissolved in ethanol, as an alternative solvent to provide a safe means of embolizing arteriovenous malformations (AVMs). METHODS: A two-stage delivery technique is required to prevent premature precipitation in the catheter when using this material: the catheter is first infused with 30% ethanol and this is followed by the delivery of the EVAL-ethanol mixture. Acute angiographic changes were analyzed after superselective delivery of dimethyl sulfoxide (DMSO) and 30% ethanol into the renal artery of rabbits. Histological changes following the embolization of the renal artery achieved using the EVAL-ethanol mixture were recorded at 1 hour and at 2 and 16 weeks after the procedure. Although DMSO always produced severe, rapidly progressive vasospasm in the renal artery during a 1- to 60-minute postinfusion, 30% ethanol did not. Microscopically, the lumens of embolized vessels examined 1 hour after embolization with EVAL-ethanol appeared to be filled with EVAL sponges, leaving almost no open spaces. The space between the EVAL sponges and the inner surface of the vessels was filled with fresh thrombus. In the vessel walls of specimens examined 2 weeks after embolization there was no or a slight inflammatory reaction. Scattered in the EVAL sponges were almost equal numbers of neutrophilic granulocytes and mononuclear cells, indicative of a mild inflammatory response. In specimens examined 16 weeks postembolization, the changes noted at 2 weeks were intensified. There was no definite histopathological evidence of mural hemorrhage, perivascular extravasation of the mixture, or perivascular hemorrhage in any specimen that was examined. CONCLUSIONS: Although the degree of permanence of this embolization material is yet unknown, the mixture was easy to handle, and appeared safe and effective for AVM embolization. Its nonadhesive characteristic and its ability to be infused by repeated injections make it an attractive alternative to currently available materials. The good results obtained in this study led us to undertake a clinical trial, the results of which are contained in a companion article in this issue of the Journal of Neurosurgery.     

Differential diagnosis of kidney transplant rejection and cyclosporin/tacrolimus nephropathy using urine cytology

Abstract: A total of 9000 urine samples from 69 kidney transplant recipients were studied for differential diagnoses of transplant rejection and cyclosporin/tacrolimus toxicity. New–Sternheimer and Papanicolaou staining were used to differentiate cells in urine. We also employed an immunocytochemical technique for further identification of exfoliated cells. With New–Sternheimer and Papanicolaou staining, the predominance of proximal tubular cells was useful to differentiate cyclosporin/tacrolimus toxicity from acute rejection in cases of increased serum creatinine level. During rejection episodes, an increased number of mononuclear cells and renal epithelial cells were found. Immunocytochemical analysis showed a significant increase of CD2-, CD4- CD8-, CD25- and HLA-DR-positive cells with rejection. However, there was no relationship between Banff criteria rejection grade and the increase of mononuclear cells.     

Airway compression by the ascending aorta due to a thin thoracic cage

An 89-year-old woman with dyspnea and disturbed consciousness due to hypoventilation was admitted to our hospital. Chest radiography showed no abnormal shadow, but she was intubated for deteriorated hypoventilation. Bronchoscopy demonstrated obstruction of the left main bronchus at the carina. Computed tomography (CT) showed neither a mass lesion in the mediastinum nor an aortic aneurysm, but compression of the airway by the ascending aorta was observed. It was thought that the patient’s thin thoracic cage was unable to support the weight of the ascending aorta, which consequently compressed the left main bronchus. After inserting stents into both main bronchi, the patient’s consciousness improved, and respirator support was withdrawn. In aged, bedridden, thin patients with hypopnea or recurrent airway infection, CT and bronchoscopy should be performed to investigate airway patency.