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.     

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.     

Sonographic measurement of cervical length and fetal fibronectin testing in threatened preterm labor.

OBJECTIVE: In women presenting with threatened preterm labor, both fetal fibronectin and sonographic measurement of cervical length have been shown to distinguish between true and false labor. The aim of this study was to determine whether the combination of both tests provides a better prediction than the individual tests alone. METHODS: We examined 195 women with singleton pregnancies presenting at 24-36 (median 31) weeks of gestation with regular and painful uterine contractions, intact membranes and cervical dilatation of less than 3 cm. On admission to the hospital fetal fibronectin positivity in cervicovaginal secretions was determined and transvaginal sonographic measurement of cervical length was carried out. The results were not made available to the attending obstetrician. The primary outcome measure was delivery within 7 days of presentation. RESULTS: Delivery within 7 days occurred in 51.4% (18 of 35) of those with cervical length below 15 mm and 0.6% (1 of 160) of those with cervical length of 15 mm or more, in 21.2% (18 of 85) of the fibronectin positive group and in 0.9% (1 of 110) of the fibronectin negative group. There was a significant association between cervical length and the incidence of fibronectin positivity (r = -0.921, P = 0.003). Logistic regression analysis demonstrated that the only significant contributor to the prediction of delivery within 7 days was cervical length, with no significant contribution from fibronectin positivity, ethnic origin, maternal age, gestational age, body mass index, parity, previous history of preterm delivery, cigarette smoking, or use of tocolytics. CONCLUSIONS: In women with threatened preterm labor assessment of fetal fibronectin in cervicovaginal secretions does not improve the prediction of delivery within 7 days provided by the sonographic measurement of cervical length.     

An experience of renal replacement therapy in a combined neonatal and paediatric intensive care unit of Hong Kong

Intensive care services are expensive. The experience of developing a combined paediatric and neonatal intensive care unit (ICU) in a regional hospital is reported with reference to the provision of renal support for the critically ill patients. The combined unit is staffed by a team of paediatric intensivists, each of whom has special interest in a subspecialty, including cardiology, respiratory medicine, nephrology and neonatology. In the past 7 years, renal replacement therapy (peritoneal dialysis and haemofiltration) was provided to 40 patients, with comparable mortality and complication rates to other reports. This arrangement has been feasible and might be more efficient than running separate paediatric and neonatal ICUs or combining the paediatric ICU with the adult ICU.     

The prevalence of transsexualism in Singapore

The prevalence of transsexualism in Singapore was estimated by counting all the patients who sought sex-reassignment surgery and were subsequently diagnosed as transsexuals by psychiatrists. Up to 1986, there were a total of 458 Singapore-born transsexuals, of which 343 were males and 115 were females. This was a prevalence of 35.2 per 100,000 population age 15 and above (or 1/2900) for male transsexualism, and 12.0 per 100,000 (or 1/8300) for female transsexualism. The sex ratio was about 3 males to 1 female. The main reason for the high prevalence was the availability of sex-reassignment surgery.     

Intrapartum sonography to determine fetal occipital position: interobserver agreement.

OBJECTIVE: To investigate the interobserver agreement on the intrapartum ultrasonographic definition of the fetal occipital position. METHODS: In 60 singleton pregnancies in labor at term the fetal occipital position was determined by transabdominal ultrasound by two appropriately trained sonographers who were not aware of each other's findings. The Bland-Altman plot was performed and the 95% limits of agreement were calculated. Logistic regression analysis was used to investigate the association between complete agreement in the fetal occipital position between the two observers and maternal and labor characteristics. RESULTS: The two observers had complete agreement on the fetal occipital position in 22/60 (36.7%) cases and disagreement by 15 degrees and 30 degrees in 31 (51.7%) and seven (11.6%) cases, respectively. The mean of the differences between the two observers was 0.25 degrees and the 95% limits of agreement were -28.9 degrees (-32.2 degrees to -25.6 degrees) to 29.4 degrees (26.1 degrees to 32.7 degrees). There were no significant associations between complete agreement and maternal and labor characteristics. CONCLUSION: The interobserver agreement on sonographically determined fetal occipital position during labor is within 15 degrees in nearly 90% of cases and within 30 degrees in all cases.