伴有LADD综合征的角膜缘干细胞缺乏

泪管-耳-牙-指（趾）（LADD）综合征是一种具有多种表现的常染色体显性遗传疾病。最初在1967年由Levy报道。1例双侧泪液系统缺失、杯状耳、干嘴，以及牙、手臂和指（趾）异常的单发病例。之后一些新发现的临床表现，如肾脏异常、唾液腺缺乏、先天性髋脱位、先天性裂孔疝和横膈疝、感觉性耳聋和传导性耳聋、牙发育不全、四肢异常、口腔干燥和眼干都被报道与此综合征有关。     

Effects of topical pre-treatment with dexamethasone on the immediate and late phases of topically induced ocular anaphylaxis in the rat

We tested the effects of pre-treatment with dexamethasone on topically induced ocular anaphylaxis in the rat. Rats were immunized with dinitrophenylated Ascaris suum extract and challenged with di-DNP-lysine. Dexamethasone was administered topically once (24, 6, or 1 h before challenge) or three times (6, 4, and 2 h before challenge). A single pre-treatment given at 24 or 6 h had no significant effect. A single pre-treatment 1 h before challenge reduced the extent of edema assessed histologically but not clinically, and had no significant effect on the eosinophil count in conjunctival tissue examined 6 h after challenge. Eyes pre-treated with dexamethasone 6, 4, and 2 h before challenge showed a significant reduction in conjunctival edema assessed histologically and clinically 1 h after challenge. In addition, 6 h after challenge the number of eosinophils was significantly reduced. We conclude that repeated pre-treatment with dexamethasone can suppress both the immediate phase and the cellular late phase of topically induced ocular anaphylaxis.     

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.     

Detection of cryoglobulins by Coulter Counter model S-Plus IV/D

Cryoglobulins may produce interference in results from electronic particle counters. Depending on their size, cold-precipitable globulin crystals can be counted as leucocytes and/or platelets. The Coulter Counter model S-Plus IV/D permits detection of this interference and its associated spurious counts by producing a characteristic WBC histogram accompanied by a leucocyte 'flag'. Using this instrument over a 1-year period, 10 cases of cryoglobulinaemia were detected, four from patients with lymphoproliferative disease and one from a patient with autoimmune disease. The remaining five were classified as essential mixed cryoglobulinaemia. The reproducibility of this instrument anomaly permits identification of the presence of cryoglobulins.     

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.     

Anatomic basis of the liver for the development of a perihepatic prosthesis

We studied specimens from 50 cadavers (27 men and 23 women) to obtain anatomical data concerning the liver and its attachments. The results allowed us to develop a polyglactin perihepatic prosthesis for compression of the injured liver. The falciform ligament and a narrowing of the hepatic parenchyma at this level allow attachment of the prosthesis. Separate prostheses were designed for each lobe. Clinical use has demonstrated that our prosthesis can be used to achieve effective control of haemorrhage and bile leakage.

---

Etude anatomique du foie. Développement d'une prothèse péri-hépatique

Résumé Sur une série de 50 pièces anatomiques d'adultes (27 hommes, 23 femmes), l'étude morphométrique du foie, de ses attaches dorsales et leurs variations a permis la confection d'une prothèse périhépatique en polyglactine adaptable à chaque lobe et fixée grâce à des artifices anatomiques. Celle-ci assure une compression efficace du parenchyme lésé et donc une hémostase et une bilistase correctes. Le ligament falciforme et le rétrecissement du parenchyme hépatique à son niveau assurent le maintien de la prothèse. Un exemplaire a été réalisé pour chaque lobe.