Cerebrospinal fluid beta-endorphin in congenital insensitivity to pain

Spontaneously elevated nociceptive threshold levels were markedly diminished after Naloxone injections in 4 patients with congenital insensitivity to pain. This finding suggested the hypothesis of a relation between congenital insensitivity to pain and permanent hyperfunction of an endomorphinic system. Radio-immunoassay of CSF beta-endorphin was performed in all 4 cases. The normal or only slightly elevated levels cannot explain electrophysiologic findings, but as a function of the multiplicity of endogenous opioid systems, hyperactivity of another endomorphinic system cannot be excluded. Other hypotheses may also be proposed.     

Benign intracranial hypertension and recombinant growth hormone therapy in Australia and New Zealand

Benign intracranial hypertension (BIH) is reported in three children from Australia and one from New Zealand, who were being treated with recombinant human growth hormone (rhGH). Three males and one female, aged between 10.5 and 14.2 y, developed intracranial hypertension within 2 weeks to 3 months of starting treatment. A national database, OZGROW, has been prospectively collecting data on all 3332 children treated with rhGH in Australia and New Zealand from January 1986 to 1996. The incidence of BIH in children treated with growth hormone (GH) is small, 1.2 per 1000 cases overall, but appears to be greater with biochemical GHD (<10IUml ^-1), i.e. 6.5/1000 (3 in 465 cases), relative risk 18.4, 95% confidence interval 1.9-176.1, than in all other children on the database. The incidence in patients with Turner's syndrome was 2.3/1000 (1 in 428 cases). No cases in patients with partial GHD (10–20 IUml ^-1) or chronic renal failure were identified. Possible causative mechanisms are discussed. The authors'practice is now to start GH replacement at less than the usual recommended dose of 14IUm-² week^-1 in those children considered to be at high risk of developing BIH. Ophthalmological evaluation is recommended for children before and during the first few months following commencement of rhGH therapy and is mandatory in the event of peripheral or facial oedema, persistent headaches, vomiting or visual symptoms. The absence of papilledema does not exclude the diagnosis.     

Iotrol versus metrizamide in lumbar myelography: a double-blind study

In a prospective, randomized, double-blind study, 49 patients underwent lumbar myelography using iotrol (24 patients) or metrizamide (25 patients). The diagnostic imaging adequacy of iotrol was comparable with that of metrizamide. After iotrol myelography, adverse reactions were fewer, less severe, and of shorter duration than were those following metrizamide myelography. Thirteen of 24 patients (54%) receiving iotrol reported some adverse reactions compared with 24 of 25 patients (96%) receiving metrizamide. Five moderate and one severe adverse reaction occurred in the group receiving iotrol. Fourteen moderate and eight severe adverse reactions occurred in the group receiving metrizamide. Thirty-eight patients underwent electroencephalography both before and after myelography (19 iotrol and 19 metrizamide). None of the EEGs obtained after iotrol myelography changed from baseline, while seven of the EEGs obtained after metrizamide myelography showed changes from baseline. Iotrol was judged superior to metrizamide as a contrast medium in this patient population.     

Chronic myelomonocytic leukemia: from biology to therapy

Chronic myelomonocytic leukemia represents a distinct myelodysplastic syndrome in which an excess of monocytes is observed both in the blood and bone marrow of the patients. Whereas diagnosis is relatively easy, therapeutic design and efficacy is difficult and no treatment has to date provided complete or significant partial response. In vitro data suggest that the growth and differentiation of myelomonocytic progenitors may be altered inasmuch as monocytic or granulo-macrophagic colonies show spontaneous growth. Different entities may be observed: the childhood form, Juvenile Chronic Myelomonocytic Leukemia (JCML) shows in vitro a typical pattern with constitutive growth of only macrophagic colonies and hypersensitivity to GM-CSF; in the adult form at least two patterns may be observed one close to the JCML form and one more heterogeneous with absence of GM-CSF sensitivity and spontaneous growth of both CFU-GM and CFU-M colonies. Chemotherapy reduces all myeloid colonies in vitro whereas retinoic acid has a selective effect on monocytic colonies with a concomitant increase of CFU-G colonies forwarding an explanation for the correction of pancytopenia observed in some patients. Recent analysis of altered molecular pathways in this disease suggest a common disruption of intracelleular signalling pathways namely the Ras pathway and targetting for drugs with may selectively control or inhibit a constitutive activation may forward novel therapeutic perspectives.     

A stratified intraoperative surgical strategy is mandatory during laparoscopic common bile duct exploration for common bile duct stones

Background: Open exploration and endoscopic sphincterotomy (ES) remain the preferred treatment of common bile duct stones (CBDS). The recent spread of laparoscopy has worsened the dilemna of choosing between surgical and endoscopic treatment of CBDS. The aim of this study was to critically evaluate the results of our preliminary experience with laparoscopic common bile duct exploration (CBDE) for CBDS. Methods: Ninety-two consecutive patients were prospectively submitted to laparoscopic CBDE. Surgical strategy included an initial transcystic approach or laparoscopic choledochotomy. Failure of stone clearance was managed by conversion to open CBDE or by postoperative ES. Electrohydraulic lithotripsy and papillary balloon dilatation were selectively used. Stone clearance was assessed by choledochoscopy and control cholangiography. Results: The overall laparoscopic stone clearance in this series was 84% (transcystic route 63% and choledochotomy 93%). Conversion to laparotomy was mandatory in 12% of the patients because of incomplete stone clearance and in 5% because of intraoperative complications. Postoperative ES was required in 4% of the patients, giving an overall surgical success rate of 96%. When indicated (small and limited number of stones located below the cysticocholedochal junction, with a dilated and patent cystic duct) the transcystic route had the lower success rate, the higher complication rate, and the shorter operative time and postoperative hospital stay. When indicated (accessible and dilated common bile duct over 7 mm), laparoscopic choledochotomy had the higher success rate, the lower complication rate, the longer operative time, and the longer postoperative hospital stay, which is related to associated external biliary drainage. The hospital mortality included two high-risk patients (2%) and the complications rate was 15%. Conclusions: Laparoscopic CBDE is safe in selected patients. A stratified intraoperative surgical strategy is mandatory in deciding between a transcystic route and choledochotomy with specific indications for each approach. When feasible, laparoscopic choledochotomy is more efficient and safe than the transcystic route, but it is associated with a longer postoperative hospital stay, which is due to external biliary drainage. Received: 7 May 1996/Accepted: 19 November 1996     

Hot-water epilepsy in an adult: ictal EEG, MRI and SPECT features.

Reflex epilepsy constitutes a rare form of epileptic seizures. We observed a 20-year-old man who presented with seizures induced by immersion in hot water. The trigger stimulus was specific. Contrast CT scan and MRI were all normal, not revealing any structural lesion. Ictal EEG recorded during a hot bath showed focal epileptic discharges in the left temporo-occipital area. Interictal SPECT showed a hypometabolism in the same cerebral region. Neuroimaging studies were rarely performed in this uncommon type of epilepsy. Nevertheless, in our case the result of the SPECT suggests a localized functional disturbance in the emergence of the disorder.     

臂丛神经根性损伤膈神经移位术对青壮年患者早期呼吸功能的影响

目的研究臂丛神经损伤膈神经移位术对青壮年患者早期呼吸功能的影响.方法对16例接受膈神经移位治疗的患者,在术前、术后（10 d）进行肺功能指标的比较,同时定期进行门诊随访,观察呼吸系统自觉症状程度.结果13例术后出现了不同程度的供氧不足症状,16例全部出现一侧膈肌抬高,术后第10天肺活量（VC）、肺活量预计值百分数（VC%）分别比术前减少37.98%和26.88%,两者差异有统计学意义（tvc=11.532、tvc%=0,P＜0.01）.其它项目如残气量（RV）较术前轻度下降,肺总量（TLC）下降值达到术前肺总量的36.49%,残气量/肺总量比值（RV/TLC%）较术前上升了4.75%,上述各指标的差值均有统计学意义.1 s用力呼气量/用力肺活量比值（FEV1/FVC）和术前比基本无改变,但其差值有统计学意义.膈神经移位右侧（10例）与左侧（6例）术前、术后肺活量比较差异有统计学意义.术后随访8个月～2年,所有患者均无明显呼吸困难和胸闷等症状.结论膈神经移位术后对青壮年患者肺容量有较大的丧失,肺通气功能减弱和小气道阻力增加,但其丧失程度在机体自身代偿耐受范围内,不会导致急剧发生的严重呼吸功能障碍.建议对右侧臂丛神经根性损伤的患者,术前进行严格的肺、心功能检查,避免发生较为严重的并发症.