Fetal intracranial teratoma: US diagnosis of three cases and a review of the literature

We describe three cases of fetal intracranial teratoma diagnosed by ultrasound and review the literature. Sonographic features include cranial enlargement, gross distortion of normal cerebral architecture by a hyperechoic, multicystic mass, and polyhydramnios. Despite early diagnosis, the cesarean section rate is high and the overall prognosis is dismal.     

中药升麻的化学成分

从中药兴安升麻[Cimicifuga dahurica(Turcz.)Maxim.]的干燥根茎中分得五个化合物,其中四个为三萜木糖甙:升麻醇-3-O-β-D-吡喃木糖甙(23R,24S)(Ⅰ),25-O-乙酰升麻醇-3-O-β-D-吡喃木糖甙(23R,24S)(Ⅱ),12-羟基升麻醇-3-O-β-D-吡喃木糖甙(23R,24S)(Ⅲ),升麻醇-3-O-β-D-吡喃木糖基(3→1)-β-D-吡喃木糖甙(23R,24S)(Ⅳ),另一个为异阿魏酸(Ⅴ)。Ⅲ和Ⅳ为新化合物,通过光谱(IR,MS,¹H及¹³C NMR)解析确定了它们的结构,分别命名为升麻甙A(cimiside A)和升麻甙B(cimiside B)。     

Screening for congenital metabolic diseases in mentally retarded patients in 6 psychiatric institutions of Lower Saxony

The results of screening for inborn errors of metabolism in patients in six institutions for the mentally retarded in Lower Saxony are presented. A battery of standard chemical and chromatographic tests for amino acids, carbohydrates and mucopolysaccharides in urine were employed. Combined gas chromatography-mass spectrometry was used to detect organic aciduria. All together 975 patients - 347 females and 628 males - aged from 5 to 93 years were studied. 78.6% of the patients were over 20 years of age. There were positive findings in 56 cases: phenylketonuria (n = 8), alcaptonuria (1), pentosuria (5), galactosuria (2), tryptophanuria (1), xanthurenic aciduria (3), mucopolysaccharidosis type III A (1), hyperglycinemia with hyperglycinuria (1), renal hyperaminoaciduria (9, lactic aciduria (3), 4-hydroxyphenylaceticaciduria (2), organic aciduria (12) and mellituria (8) of unknown type. The organic acids most often detected in urine were lactic, 4-hydroxyphenylacetic and 3-hydroxybutyric acids. Only 20 of the patients (2.0%) excreted appreciable amounts of benzoic acid in urine. The results are discussed and compared with other investigations.     

Progression of X-linked adrenoleukodystrophy under interferon-β therapy

The cerebral phenotype of X-linked adrenoleukodystrophy (ALD) is a rapidly progressive neurodegenerative disorder characterized by a cerebral inflammatory response and elevated very long-chain fatty acids (VLCFA). Interferon- (INFB) is known to suppress the synthesis of tumour necrosis factor and interferon-, which have been reported to be elevated in the margin of the areas of demyelination in ALD brains. We report on treatment with interferon- in 8 patients with cerebral ALD, who additionally received glycerol trioleate/glycerol trierucate. INFB-1a (Rebif, Serono, Switzerland) was given subcutaneously once a week, 3 million units for the first 3 months and 6 million units for the next 9 months. All patients showed an unimpeded progression of neurological symptoms during INFB therapy. Therapy was stopped within 6 months in 4 patients because of the fast neurological deterioration with loss of walking. In all patients the MRI demonstrated a progression of demyelination with a qualitatively unchanged gadolinium enhancement. Further studies are needed to elucidate the pathomechanism of demyelination in ALD in order to find an effective therapy for cerebral ALD patients.     

The surface to volume ratio of mitochondria,a suitable parameter for evaluating mitochondrial swelling

Summary Cellular changes occuring in the left ventricular myocardium during ischaemia after different methods of cardiac arrest have been evaluated by morphological and morphometric parameters: volume densities of mitochondria (V_VMi), sarcoplasm (V_VSp), myofibrils (V_VMf), surface densities of mitochondria (S_VMi). The surface to volume ratio of mitochondria (S_Vratio_Mi) has been used as an independent parameter of mitochondrial swelling.Since ischaemic swelling of myocardial cells increases the volume of the reference space and ischaemic swelling of mitochondria decreases the free sarcoplasm, V_VMi and V_VSp cannot be considered as reliable indicators of the degree of oedema. S_VMi/V_VMf remains nearly constant after different forms of cardiac arrest, demonstrating the integrity of mitochondrial outer membranes. The inverse linear ratio between S_Vratio_Mi and the mean mitochondrial volume indicates that the increase in mitochondrial volume is achieved by surface smoothing.Loss of matrix structure and fragmentation of cristae occur at an S_Vratio_Mi of about 5.8, cristolysis at 5.5 to 5.6 and amorphous matrix densities at an S_Vratio_Mi of less than 5.5 m²/m³.The S_Vratio_Mi is a suitable parameter for evaluating mitochondrial swelling both at the onset and during global myocardial ischaemia, independent of the method of cardiac arrest used. It serves as an indicator of the state of structural preservation of mitochondria during ischaemia.Supported by the Deutsche Forschungsgemeinschaft, SFB 330-Organprotektion — Göttingen     

Effects of methylene blue-treated plasma on red cells and stored platelet concentrates

BACKGROUND: Photochemical methods can effectively inactivate extracellular viruses and bacteria found in blood components. Treatment of plasma with methylene blue (MB), a phenothiazine dye, and visible light inactivates enveloped viruses including HIV-1. The effects of MB- treated plasma on cellular components stored in vitro have not been well characterized. STUDY DESIGN AND METHODS: MB-treated plasma (83 microg MB/250 mL plasma) was added to single-donor platelets, stored AS- 1 red cells (RBCs), irradiated RBCs, and frozen-deglycerolized RBCs. In vitro platelet assays performed after 1 and 5 days of storage in MB- treated plasma included pH, pO2, pCO2, HCO3, platelet number, lactate dehydrogenase, glucose, osmotic recovery, and CD62 expression. RBC components were examined at specific intervals for leakage of potassium, plasma hemoglobin level, and percentage of hemolysis. Direct antiglobulin tests, osmotic fragilities, and RBC antigen stability tests were also performed on RBCs stored in MB-treated plasma. Components stored with autologous plasma or nontreated allogeneic plasma served as controls. RESULTS: Similar storage-induced changes in pH, glucose, and platelet numbers, as well as increases in lactate dehydrogenase, CD62 expression, and lactate were seen in single-donor platelets stored with MB-treated and control plasma. Platelet morphology scores and osmotic recoveries were not altered. Plasma hemoglobin and potassium and percentage of hemolysis increased equally in the various RBC components stored with MB-treated or nontreated plasma. Osmotic fragility and RBC antigen stability were not appreciably altered by MB-treated plasma. CONCLUSION: Plasma treated by MB photoinactivation can be used for in vitro resuspension and storage of platelets or RBCs, because of the lack of influence of MB-treated plasma on a variety of in vitro platelet and RBC assays.