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.     

先天性麻痹性斜视眼肌的形态学改变及临床意义

目的研究先天性麻痹性斜视眼肌的形态学改变。方法将先天性麻痹性斜视12例18眼按年龄不同分为三组，将术中切下的眼外肌制成光镜和电镜标本进行比较。结果三组先天性麻痹性斜视的眼外肌均有病理性改变。年龄越大、病程越长，其病理改变越明显。结论先天性麻痹性斜视应早期手术治疗，以免眼外肌病理改变加重，影响双眼视功能恢复。     

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。     

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.     

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.     

Reintroducing OPV in Israel on the journey to global polio eradication – Estimation at a low rate of contraindicated population

IntroductionThe 2013 reemergence of wild poliovirus in Israel led to the reinstatement of a routine OPV vaccination. Fearing VAPP in immunocompromised, the MOH regulated contraindications for vaccination candidates and household contacts. In this study we estimate the size of the contraindicated population to OPV vaccination.MethodWe studied vaccination candidates aged 2–9 and 14–23 months and probable household contacts. Using the rate of contraindications extracted for each study group from a medical records database, a statistical model was built to estimate the probability of contraindications in candidates.Results3.9% of the 2–9-month-old study group and 4% of the 14–23-month-old group had contraindications by either self or household contacts.ConclusionA statistical model can provide an estimation of the contraindicated population and can be used in the future when devising vaccination campaigns. In contrast to concerns raised by the MOH, our findings show a smaller than anticipated contraindicated population.