A Chinese adolescent girl with Fechtner-like syndrome

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients.     

Occurrence of the t(2;5)(p23;q35) in non-Hodgkin's lymphoma

Primary CD30(Ki-1)-positive anaplastic large-cell lymphoma (ALCL) is considered by some to be a distinct clinicopathologic entity associated with the t(2;5) (p23;q35). However, the specificity of t(2;5) for ALCL has not been carefully studied. Therefore, we performed a detailed analysis of all cases of ALCL with abnormal cytogenetics results in the Nebraska Lymphoma Study Group registry, as well as all other cases of non-Hodgkin's lymphoma with t(2;5) in the registry. We found the t(2;5) in only five of 10 cases of ALCL, four of whom were young patients. However, we also found the t(2;5) in 11 other cases of nonanaplastic lymphoma, including eight children with typical peripheral T-cell lymphomas of various types. The t(2;5) was also found in three older adults with B-cell lymphomas of various types. Thus, the t(2;5) was not specific for CD30+ ALCL. However, t(2;5) may define a clinicopathologic entity in children and young adults characterized by variable morphologies with a T-cell or indeterminate phenotype, CD30-positivity, nodal disease with frequent extranodal involvement, advanced stage, and an excellent response to therapy, including bone marrow transplantation for relapsed disease. The clinical relevance of the t(2;5) in older patients requires further study.     

Stability of famotidine 20 and 40 mg/L and amino acids in total parenteral nutrient solutions

The stability of famotidine in total parenteral nutrient (TPN) solutions and the concentrations of amino acids in the presence of famotidine were determined. Two famotidine concentrations (20 mg/L and 40 mg/L) and two amino acid concentrations (20 g/L and 42.5 g/L) were studied under the following storage conditions: refrigerated for 24 hours and then kept at room temperature (20-22 degrees C) for 24 hours, at room temperature for 48 hours, or refrigerated for seven days. Control TPN solutions were studied under the same storage conditions. TPN solutions also contained dextrose 25%, electrolytes, trace elements, and vitamins. Famotidine concentration was determined at 0, 24, and 48 hours and at seven days by high-performance liquid chromatography. Amino acid concentration was determined in the TPN solutions containing 42.5 g/L of amino acids without famotidine and with famotidine 40 mg/L under both 48-hour storage conditions. At 24 hours, all solutions retained at least 95% of the initial famotidine concentration. Seven of the eight famotidine solutions retained more than 95% of the initial famotidine concentration at 48 hours. All samples refrigerated for seven days retained more than 95% of the initial famotidine concentration. The concentration of amino acids in TPN solutions containing 42.5 g/L of amino acids was not affected by the addition of famotidine 40 mg/L under either 48-hour storage condition. Famotidine in concentrations of 20 mg/L and 40 mg/L is stable under the studied 48-hour storage conditions in TPN solutions containing amino acid concentrations of either 20 g/L or 42.5 g/L.(ABSTRACT TRUNCATED AT 250 WORDS)     

INVOLVEMENT OF NON-NMDA AND NMDA RECEPTORS IN GLUTAMATE-INDUCED PRESSOR OR DEPRESSOR RESPONSES OF THE PONS AND MEDULLA

1. Fifty-five intact and six baroreceptor denervated and vagotomized cats of either sex were anaesthetized intraperito-neally with urethane (400 mg/kg) and a-chloralose (40 mg/kg). Responses of the systemic arterial pressure (SAP), mean SAP (MSAP) and sympathetic vertebral nerve (VNA) and renal nerve activities (RNA) were recorded. 2. In intact animals, monosodium L-glutamate (Glu, 0.1 mol/L, 50 nL) was microinjected into pressor areas of the locus coeruleus (LC), gigantocellular tegmental field (GTF), rostral ventrolateral medulla (RVLM) and dorsomedial medulla (DM), and the depressor areas of caudal ventrolateral medulla (CVLM). The induced actions were compared before and after microinjection of either glutamate antagonists, glutamate diethylester (GDEE, 0.5 mol/L, 50–100nL), a competitive AMPA receptor blocker, or 2-amino-5-phosphonovaleric acid (D-AP5, 0.025 mol/L, 50–100 nL), a competitive N-methyl-D-aspartate (NMDA) receptor blocker. GDEE completely blocked the increases of SAP and VNA elicited from all pressor areas. D-AP5 only partially blocked the pressor but slightly blocked VNA and RNA responses from LC, GTF and DM, particularly those from RVLM. Neither GDEE nor D-AP5 blocked the depressor responses of SAP and two nerve activities elicited from CVLM. 3. In baroreceptor denervated animals, NMDA (2 mmol/L, 50–100 nL) and AMPA (0.2 mmol/L, 50–100 nL) were micro-injected into the same pressor areas of GTF, RVLM and DM and the depressor area of CVLM responsive to Glu activation (0.1 mol/L, 30 nL). In RVLM, DM and CVLM, the results of either NMDA or AMPA were similar to those induced by Glu. However, in GTF, microinjection of either NMDA or AMPA did not induce similar responses to Glu. This suggests that the nature of GTF may differ from RVLM and DM. 4. The above results suggest that the Glu-induced pressor responses from LC, GTF, DM and especially RVLM, are primarily mediated through AMPA receptors. The Glu-induced depressor responses from CVLM may not be predominantly mediated by either AMPA or NMDA receptors. 5. In both baroreceptor-intact and -denervated cats stimulation of the pressor areas often produced an increase of VNA and a decrease of RNA, while in the depressor CVLM decreased both VNA and RNA. The VNA, but not RNA were positively correlated with the pressor responses, while both VNA and RNA were positively correlated with the depressor responses. This may suggest that neurons of the sympathetic vertebral and renal nerves are topographically organized in the brain.     

Buspirone treatment of aggression and anxiety in mentally retarded patients: a multiple-baseline, placebo lead-in study

Buspirone, a new anxiolytic agent, was used in a multiple-baseline, placebo lead-in study for the treatment of aggression and anxiety in six mentally retarded adult patients. The findings demonstrate buspirone's effectiveness in reducing aggression and anxiety in a mentally retarded group of subjects without causing deleterious cognitive side effects. The authors review a body of literature on animal and human subjects where buspirone was used in the treatment of aggression and other mental disorders. Unlike neuroleptics and benzodiazepines, buspirone does not cause sedation which can compromise adaptive and intellectual capacities and thus reduce the patient's potential to benefit from training programs.