Congenital Pseudarthrosis of Femur and Sciatic Nerve Palsy with Congenital Constriction Band Syndrome: Report of the First Case in Literature

Congenital constriction band syndrome has varied clinical presentations ranging from small, incomplete skin deep constriction band to in utero amputation. Pseudarthrosis of underlying bone most commonly tibia has been reported by many authors. We report the first case of congenital pseudarthrosis of the femur with congenital constriction band syndrome. Nine-day-old female presented with the constriction band in the left thigh with open pseudarthrosis of the femur. The left femur had gross recurvatum deformity and the posterior apex of the pseudarthrosis was exposed via skin ulceration. She had an ipsilateral paralytic clubfoot. She was treated with single-stage excision of constriction band and Z-plasty. Spontaneous union of the femur was achieved at 3 months. Procurvatum deformity of the femur improved gradually over 3 years. This happens to be the first and only reported case of congenital pseudarthrosis of the femur with sciatic nerve palsy due to congenital constriction band.     

Perspectives on rapid elimination and ultimate global eradication of paralytic poliomyelitis caused by polioviruses

Poliomyelitis caused by polioviruses has already been eradicated from industrialized countries of North America, Europe, Asia and Oceania, but the procedures by which this eradication was achieved are not adequate for the poor tropical and subtropical countries. The major challenge now is first to eliminate it rapidly from Asia and Africa where an estimated 250,000 cases and 25,000 deaths currently occur annually. The great progress toward eradication of wild polioviruses from poor tropical and subtropical countries in Latin America was achieved not by the procedures still recommended by the WHO Expanded Program on Immunization (EPI) but by the independently organized annual, national days of antipolio vaccination — all based on the use of large armies of well-trained non-professional, community volunteers — first used in Cuba (1962), Brazil (1980), Nicaragua (1981), Dominican Republic (1983), Paraguay (1985), and Mexico (1986). This novel approach, described in some detail in this communication, is recommended for the rapid elimination of wild polioviruses from Asia and Africa, and for ultimate global eradication with the help of a special cadre within the EPI of WHO. The extensive use by the Pan American Health Organization (PAHO) of highly sophisticated regional virus laboratories has led to the recognition that, in areas from which poliomyelitis caused by polioviruses has been largely eliminated, there are thousands of cases of acute flaccid paralysis, previously clinically diagnosed as probable poliomyelitis, that have no viral etiology, a phenomenon previously reported by Dr. Manuel Ramos Alvarez in Mexico City in 1967.     

Occurrence of paralytic shellfish poison (PSP) in the starfish Asterina pectinifera collected from the Kure Bay, Hiroshima Prefecture, Japan.

Assays were made for paralytic toxicity of marine invertebrates inhabiting at the coasts of Hiroshima Bay, where the infestation of bivalves such as cultured oysters with paralytic shellfish poison (PSP) has been occurred. The starfish Asterina pectinifera collected at the estuary of Nikoh River, Hiroshima Bay, was found to contain moderate levels of paralytic toxicity. Its highest toxicities as PSP found on July 30, 1999 were 12.5 MU/g for whole body, 11.0 MU/g for integument tissues and 3.9 MU/g for viscera, respectively. The toxicity of integument was changed from 3.6 to 11.0 MU/g in 1 year. Its paralytic toxin principles were identified as PSP toxins, composing mainly from saxitoxin (STX) group toxins such as carbamoyl-N-hydroxy neosaxitoxin (hyneoSTX), and STX, by HPLC and LC-MS, accounting for over 90 mol%. The PSP toxins contained in the starfish A. pectinifera considered to be transferred from bivalves or detritus living in the same area, which were contaminated with PSP. However, the involved pathway may be different from that of Asterias amurensis which was infested directly through food chain from its food bivalves, for its toxin pattern.     

Salinity effect on growth and toxin production of four tropical Alexandrium species (Dinophyceae).

Four tropical PSP toxins-producing dinoflagellates, Alexandrium minutum, Alexandrium tamiyavanichii, Alexandrium tamarense and Alexandrium peruvianum from Malaysian waters were studied to investigate the influences of salinity on growth and toxin production. Experiments were conducted on constant temperature 25 degrees C, 140 microE mol m(-2) s(-1) and under 14:10 light:dark photo-cycle with salinity ranged from 2 to 30 psu. The PSP-toxin congeners, GTX 1-6, STX, dcSTX, NEO and C1-C2 were analysed by high performance liquid chromatography. Salinity tolerance of the four species in decreasing order is A. minutum>A. peruvianum>A. tamarense>A. tamiyavanichii. Specific growth rates and maximum densities varied among these species with A. minutum recorded as the highest, 0.5 day(-1) and 6 x 10(4) cells L(-1). Toxin content decreased with elevated salinities in A. minutum, the highest toxin content was about 12 fmole cell(-1) at 5 psu. In A. tamiyavanichii, toxin content peaked at optimal growth salinity (20 and 25 psu). Toxin content of A. tamarense, somehow peaked at sub-optimal growth salinity (15 and 30 psu). Results of this study implied that salinity fluctuation not only influenced the growth physiology but also toxin production of these species.     

High affinity for the rat brain sodium channel of newly discovered hydroxybenzoate saxitoxin analogues from the dinoflagellate Gymnodinium catenatum.

The paralytic shellfish poison family has been recently extended by the discovery of several analogues possessing a hydoxybenzoate moiety instead of the carbamoyl group one finds in saxitoxin, the parent molecule of this toxin family. We have investigated the potency of these new analogues on a representative isoform of the pharmacological target of these toxins, the voltage gated sodium channel. These toxins were found to have K1's in the low nanomolar range, only slightly less potent than saxitoxin. The hydroxybenzoate group may increase the lipophilicity of these toxins and improve their ability to pass through epithelia and therefore its uptake and elimination in both intoxication victims and animals that bioaccumulate paralytic shellfish toxins.     

Occurrence of saxitoxins as a major toxin in the ovary of a marine puffer Arothron firmamentum.

Eleven male and 14 female specimens of a marine puffer Arothron firmamentum were collected from Oita and Iwate Prefectures, Japan. The toxicity assay using mouse showed that only ovary and skin of the female specimens were toxic, the toxicity scores being 5-740 as paralytic shellfish poison and <5-30 MU/g as tetrodotoxin (TTX), respectively. The toxin extracts from the both tissues were then treated with cartridge columns, and subjected to high performance liquid chromatography and liquid chromatography-mass spectral analyses. In the analyses, saxitoxin (STX) and decarbamoylSTX (dcSTX) were identified as the major toxins in the ovary, while the skin contained only TTX.     

直肌肌束部分转位移植矫正麻痹性斜视的临床观察

目的 探讨直肌肌束部分转位移植矫正麻痹性斜视手术方法和治疗效果.方法 总结27例麻痹性斜视行直肌肌束部分转位移植,并观察在术前、术后麻痹肌的运动情况、双眼视功能、眼球运动情况、眼位及复视消失情况等结果.结果 27例患者术后眼球运动功能有不同程度好转,除2例外全部过中线,转动1～2mm者12例、3mm者10例、4～5mm者2例、7mm者1例.术后被转位的肌肉运动正常.术后21例患者不同程度恢复了双眼视功能,其中具备Ⅰ度双眼单视功能13例,具备Ⅱ度双眼单视功能6例,2例有Ⅲ度双眼单视功能.6例同视机检测仍是单眼抑制.术后眼位正位22例,欠矫5～10度5例,23例复视消除,3例轻度改变注视方向也可消除复视.1例仍有复视不能耐受行二次手术.结论 直肌肌束部分转位移植可矫正麻痹性斜视眼位,消除复视,改善眼球运动,恢复部分双眼视功能,而且睫状肌血管分离保留可减少眼前段供血不足的危险,是目前安全、可行、有效地手术方法.     

Determination of paralytic shellfish poisoning toxins in Norwegian shellfish by liquid chromatography with fluorescence and tandem mass spectrometry detection.

A novel extraction and clean-up method has been developed for the determination of paralytic shellfish poisoning (PSP) toxins in shellfish samples. Raw shellfish material was extracted with an acidic acetonitrile/water (80:20, v/v) solution, whilst being homogenised. During the homogenisation the sample extraction solution was cooled with ice water. Subsequently, the extract was frozen at -20 degrees C for at least 4h. During freezing, two layers were formed, only the lower predominantly aqueous layer was used for the determination. The final extract solution was cleaned-up using a combination of Oasis HLB and Carbograph activated carbon SPE columns. The developed extraction and clean-up methods combined with gradient elution liquid chromatography (LC)-mass spectrometry/mass spectrometry (MS/MS) has resulted in a method which can determine the analogs GTX 1-5, C1-2, DcGTX 2-3, DcSTX, Neo, STX in a single analysis with an overall detection limit of 313mug STXdiHCL-eq./kg shellfish meat. The use of the developed extraction method with post-column high performance liquid chromatography (HPLC) with fluorescence detection (FLD) method provided an overall limit of detection of 89mug STXdiHCL-eq./kg shellfish meat for the same toxins. Both post-column HPLC-FLD and LC-MS/MS was used to investigate the Norwegian PSP toxin profile. It was found that the PSP toxins could be detected in shellfish samples from the Norwegian coastline for 10 months of the year, from March till December. The toxin profile consisted mainly of the carbamate toxins, GTX 1-4, Neo and STX, in terms of both concentrations and contribution to the overall toxicity. In addition, several of the n-sulfo-carbamoyl toxins were either detected in the samples at relatively low concentrations or their presence in the samples were indicated but could not be confirmed by the post-column HPLC-FLD and LC-MS/MS analyses.     

织纹螺麻痹性贝类毒素毒力测定

目的测定引起食物中毒的织纹螺麻痹性贝类毒素的毒力。方法根据出口贝类麻痹性贝类毒素检验方法,小白鼠下腹腔注射织纹螺麻痹性贝类肉质提取液,测定鼠单位(MU)。结果市场水产摊位采集的织纹螺毒力为100g肉中9300MU;食物中毒者家中食用剩余的织纹螺的毒力为100g肉中8370MU,而作为对照的血蛤螺的毒力<400 MU/100g肉。结论引起食物中毒的织纹螺的毒力是国际上规定的安全限值的20.9-23.3倍。     

Wernicke encephalopathy,dysautonomia and excessive soft drink consumption: An unusual triad

We report the clinical observation of a 46 years old woman developing Wernicke encephalopathy with an atypical presentation, which is complicated by severe dysautonomic syndrome with paralytic ileus, a rarely described complication. Moreover, the patient's Coca-Cola® addictive behaviour highlights the importance of considering the multifactorial origin of thiamine deficiency. Identifying the risk population and recognizing the symptoms in time is essential to avoid a diagnostic delay, potentially fatal, and can lead to a more efficient care management.