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The Arizona Department of Health Services performed an investigation to determine the health effects associated with the use of a mercury-containing beauty cream. A urine test for mercury was offered to cream users who contacted the Arizona Department of Health Services. Those with urine mercury levels > 20 micrograms/L were offered clinical evaluation. Eighty-nine urine specimens were submitted for testing. Of these, 66 showed an elevated urine mercury level (> 20 micrograms/L), and 55 people were evaluated in clinic. There were no major abnormalities found through physical examination or laboratory testing. Urine mercury levels declined from an initial mean of 170 micrograms/L to 32 micrograms/L at the final test (mean, 139 days later). The high urine mercury levels indicate that the use of this cosmetic cream constitutes a significant exposure. Neuropsychiatric symptoms were frequently reported, but few objective signs were noted.

Purpose: To report a patient who developed photic maculopathy after pterygium removal., Methods: A 27-year-old woman underwent pterygium removal with a conjunctival autograft after administration of retrobulbar anesthesia. A coaxial operating microscope was used, with an estimated retinal exposure of approximately 40 minutes., Results: On the first postoperative day, the patient noted a paracentral scotoma. A fluorescein angiogram on the fourth postoperative day documented a phototoxic lesion in the macula., Conclusions: Phototoxic injury to the macula may occur after pterygium removal. Ophthalmologists should take precautions to minimize prolonged intense coaxial illumination of the retina while performing any ocular microsurgery.

Objective: To find out if giving steroids reduces postoperative swelling in orbital surgery., Design: Prospective, randomized, double-blind trial., Setting: Regional unit, teaching hospital, UK., Subjects and Interventions: 20 patients who require orbital floor exploration after injuries. Patients were given methylprednisolone 250 mg (or placebo identically packaged) at induction of anaesthesia, with a further three doses at 6-hourly intervals postoperatively., Main Outcome Measure: Differences in interpalpebral width before and after operation., Results: Those given steroids (n=11) had a significantly increased interpalpebral width compared with placebo (n=9) postoperatively (P < 0.01, 95% confidence intervals of the difference = 1 mm-6 mm)., Conclusion: Short courses of steroids therapy should be considered in orbital surgery for appropriate patients. There is an advantage in being able to assess the globe postoperatively.

Objective: To report the first case of a serious hypoglycemic episode associated with the misuse of repaglinide., Methods: The history, clinical findings, laboratory features, and clinical course of this case are described., Results: In a 48-year-old man with autoimmune hepatitis, diabetes developed in association with corticosteroid therapy. Postprandial plasma glucose levels remained in the range of 150 to 200 mg/dL after a 6-week trial of diet-alone therapy, and treatment was begun with repaglinide, 0.5 mg before each meal. On the 11th day of therapy, the patient had a severe episode of hypoglycemia (plasma glucose level of 33 mg/dL) and seizures during the night, after forgetting his dose of repaglinide before the evening meal and subsequently taking the medication 3 hours after that meal., Conclusion: Serious hypoglycemia is always a potential consequence of agents that stimulate endogenous release of insulin. In this case report, serious hypoglycemia occurred in a patient at higher risk by virtue of hepatic insufficiency and was clearly associated with the misuse (late postprandial administration) of repaglinide. Continued emphasis on the need to administer repaglinide before meals (and to omit the drug if a meal has been missed or the dose has not been taken preprandially) is indicated if hypoglycemic episodes are to be avoided.

Neurological complications of malignant infantile osteopetrosis are well recognised; successful bone marrow transplantation, when performed early in life, can prevent or halt some of them. In a subgroup of infants osteopetrosis is associated with primary retinal degeneration and/or generalised neurodegeneration. Bone marrow transplantation, in spite of being successful in correcting the osseous and haematological abnormalities, does not influence the progressive course of the neurodegenerative disorder. Thus, the recognition of this subgroup of infants with a very poor prognosis is essential before deciding on bone marrow transplantation.

Predictable biomechanical retention of nasal prostheses can now be achieved using Brånemark osseointegrated implants. These prostheses are tissue compatible, simple in design and easy to place, can be worn independently of spectacles and can in the majority of cases be provided soon after surgical excision. We have treated 14 patients (30 implants) 8 of whom had the implants placed at the time of surgical excision of the tumour. The longest survival so far has been 62 months (mean 38 months). Craniofacial osseointegration changes patients' perceptions of a nasal prosthesis and improves quality of life. Initial reconstructive surgery after radical nasal excision should be limited to reconstruction of the upper lip and preparation of the surgical defect for an implant-retained prosthesis.

Respiratory syncytial virus and parainfluenza virus infection carry a poor prognosis in severe combined immunodeficiency (SCID), particularly if the viral load is high. Patients with high viral load develop severe pneumonitis at engraftment which may possibly be modulated by immunotherapy, including high dose nebulised corticosteroids. Further work is required to develop effective treatment for this severe condition.

Objective: To compare the effect of the addition of regular insulin as a premixed 70/30 insulin to the treatment regimen of patients with type 2 diabetes who had used NPH insulin alone relative to overall glycemic control (postprandial blood glucose), patient satisfaction, and health-related quality of life., Methods: We studied 90 patients with type 2 diabetes in a 10-week, randomized, double-blind, crossover trial involving 9 clinical investigators. Patients previously treated with NPH insulin alone were transferred to 30% regular insulin added to 70% NPH as a premixed insulin (70/30) administered twice daily. Patients in one sequence group received NPH insulin twice daily for 4 weeks followed by 70/30 insulin for 4 weeks; in the second sequence group, the order was reversed., Results: The magnitude of the 1.5- and 2-hour postprandial glucose excursion was reduced with 70/30 insulin in comparison with NPH insulin, and patients treated with 70/30 insulin experienced fewer hypoglycemic events than with NPH insulin. With regard to health-related quality of life, patients treated with 70/30 insulin rated their physical functioning as better; rated their ability to be spontaneous, follow the meal plan, and interact socially to be less difficult; and had less fear of hypoglycemia and perceived their diabetes to be better controlled than when treated with NPH insulin alone., Conclusion: In patients with type 2 diabetes mellitus, premixed 70/30 insulin improved postprandial glycemic control and health-related quality of life without increasing the frequency of hypoglycemic events and without any additional cost.

Several but not all studies indicate that chorionic villus sampling (CVS) is associated with an increased risk for transverse limb deficiencies, including digital deficiencies. It has been suggested that variations in results regarding the transverse digital deficiencies (TDDs) may be due to the use of different classification criteria. We present the combined analysis of two case-control studies, the U.S. Multistate CVS (US) study and the Italian Multicentric Birth Defects (IP-IMC) study, using two different definitions of TDDs. We compared the frequency of CVS exposure in control infants with that among those infants with any number of affected digits (any TDD), and those with all five digits of at least one limb affected (extensive TDDs). The estimated relative risk (RR) for any TDD following CVS was 10.6 (IPIMC) and 6.6 (US). For the extensive TDDs, the RR was 30.5 (IPIMC) and 10.7 (US). In both studies, extensive TDDs were less than 25% of all TDDs. Compared to all TDDs, extensive TDDs were more likely to occur after CVS performed earlier in the first trimester (before 10-11 weeks' gestation). These findings suggest a relationship between the timing of CVS and the severity of TDDs; indicate that using a restrictive definition of TDDs (all five digits affected) may limit the ability to evaluate the association between CVS and TDDs in populations in whom CVS is usually performed at or after 10 weeks' gestation; and highlight the necessity to consider gestational age in any evaluation of the relative risk for limb deficiencies associated with CVS.

Although numerous infants have been reported with transverse limb deficiencies after their mothers had undergone chorionic villus sampling (CVS), it has been unclear whether the procedure caused these defects. We report the results of the first multistate case-control study to assess and quantify the risk for specific limb deficiencies associated with CVS. Case subjects were 131 infants with nonsyndromic limb deficiency ascertained from 7 population-based birth defect surveillance programs, and born from 1988-1992 to mothers 34 years of age or older. Control subjects were 131 infants with other birth defects. We ascertained exposure to CVS from medical records and maternal and physician questionnaires. We assessed rates and timing of exposure to CVS, and estimated relative and absolute risks for anatomic subtypes of limb deficiency. The odds ratio for all types of limb deficiency after CVS from 8-12 weeks' gestation was 1.7 (95% confidence interval, 0.4-6.3). For specific anatomic subtypes, the strongest association was for transverse digital deficiency (odds ratio = 6.4; 95% confidence interval, 1.1-38.6). The risk for transverse digital deficiency increased with earlier gestational exposure (P < 0.01 for trend). We estimated that the absolute risk for transverse digital deficiency in infants after CVS was 1 per 2,900 births (0.03%). Exposure to CVS was associated with a sixfold increase in risk for transverse digital deficiency. The causality of this association is supported by its strength, specificity, biologic plausibility, and consistency with the results of previous studies. Although some centers already inform patients about risk for limb deficiency, this study quantifies the magnitude of risk associated with CVS from 8-12 weeks' gestation.

A new software system designed for use in high-throughput DNA sequencing laboratories is described. The Genome Reconstruction Manager (GRM) was developed from requirements derived from ongoing large-scale DNA sequencing projects. Object-oriented principles were followed in designing the system, and tools supporting object-oriented system development were employed for its implementation. GRM provides several advances in software support for high-throughput DNA sequencing: support for random, directed, and mixed sequencing strategies; a novel system for fragment assembly; a commercial object data-base management system for data storage; a client/server architecture for using network computational servers; and an underlying data model that can evolve to support fully automatic sequence reconstruction. GRM is currently being deployed for production use in high-throughput DNA sequencing projects.

A case is reported of a papillary cystadenocarcinoma of the sublingual gland which presented as a ranula. This is both rare in site and mode of presentation.

The authors report 2-year follow-up information on 179 of 198 eyes (90%) enrolled in a previously published multicenter, randomized, controlled clinical trial comparing pneumatic retinopexy (PR) with scleral buckling (SB) for the management of selected retinal detachments. Scleral buckling was compared with PR with regard to redetachment after the initial 6-month follow-up period (1% versus 1%), overall attachment (98% versus 99%), subsequent cataract surgery (18% versus 4%; P less than 0.05), preoperative visual acuity (no significant difference), and final visual acuity of 20/50 or better in eyes with macular detachment for a period of 14 days or less (67% versus 89%; P less than or equal to 0.05). Reoperations after a failed PR attempt did not adversely affect visual outcome. After 2 years, PR continues to compare favorably with SB.

Removal of tumours from the parapharyngeal space is often difficult because of limited surgical access. Mandibulotomy has proved useful for improving access but may cause damage to the inferior dental and lingual nerves. Use of the inverted 'L' osteotomy provides excellent access avoiding damage to the nerves and the technique is illustrated with three surgical cases.

A case is reported of a central acinic cell carcinoma of the mandible. This is an unusual site for this rare tumour and to our knowledge only three cases have been previously reported in the literature.

A retrospective audit was undertaken of the value of routine cross-matching of blood for patients undergoing orthognathic surgery at Canniesburn Hospital, Glasgow. Overall, routine cross-matching of blood was wasteful of resources for all but the more major types of osteotomy procedure: Le Fort III, Le Fort II, malar osteotomies requiring a bicoronal flap and bimaxillary osteotomies. The study has financial implications regarding the proper use of cross-matching in selected patients undergoing elective orthognathic surgery. A blood ordering tariff has been suggested for the various procedures studied.

Osteosarcoma is a highly malignant tumour which despite modern surgery and chemotherapy still retains a relatively poor prognosis. This prognosis is, however, much better in the rare variant known as juxtacortical osteosarcoma. Both types of osteosarcoma mainly affect the long bones but the juxtacortical variant may be treated less aggressively and has a much better survival rate. The authors report four cases of juxtacortical osteosarcoma affecting the jaws and discuss these unusual tumours and their management.

Facial expressions and body language constitute a major component of communication in the human population. There are no studies in the dental literature that have examined children's ability to recognize facial expressions. The purpose of this study was to determine children's accuracy and latency to respond to facial expressions (photographs) as a function of age and sex. Sixty children were recruited randomly and placed into three groups according to their age (3, 6, and 9 years) with 20 children per group. They were shown photographs of three different emotions (happiness, anger, and sadness) expressed by actors at three different exposure times (0.2, 0.5, and 1.0 sec), tachistoscopically. The children's responses (accuracy and latency) were recorded. The results indicated that three year olds were significantly less accurate and took longer to respond than the older children. Factors that may have influenced the results and clinical implications are discussed.

There have been 26 published series with a total of 1274 detachments operated with pneumatic retinopexy. Eighty percent were reattached with a single procedure and 98% with reoperations. New breaks occurred in 13% and PVR in 4%. The complications published in 101 papers on pneumatic retinopexy in the last 5 years are analyzed as to frequency, prevention, management, and results.

Two large angiomas (greater than 3 disc diameters) in two patients with von Hippel disease were removed successfully by eye wall resection. The resection of angioma in the first case resulted in improved visual acuity from counting fingers to 20/140, decreased exudates, and cessation of preoperative diffuse retinal capillary leakage observed by fluorescein angiography. The resection in the second case with pre-existing local tractional retinal detachment was tolerated well. The patient retained her preoperative visual acuity of 20/25, and retinal traction was released. Although a complicated surgical procedure, resection of large angiomas offers a good alternative therapy to tumors resistant to cryocoagulation or photocoagulation.

Three patients developed vitreous hemorrhage secondary to breakthrough bleeding from presumed extramacular subretinal neovascularization. In one patient, the vitreous hemorrhage cleared spontaneously. In two other patients, trans-pars plana vitrectomy was performed. All patients regained 20/30 vision or better. Residual peripheral retinal pigment epithelial atrophy, organized subretinal hemorrhage, and/or subretinal fibrous membranes were present in all patients. A definite extramacular subretinal neovascular membrane was identified in one patient. Ocular diseases associated with subretinal neovascularization are tabulated. Indications for trans-pars plana vitrectomy in patients with breakthrough vitreous hemorrhage secondary to presumed extramacular subretinal neovascularization are proposed.

The Macular Photocoagulation Study (MPS) Fundus Photograph Reading Center has developed a standard set of methods for assessing color photographs and fluorescein angiograms on study patients. For pretreatment angiograms, these methods are used to determine the location and extent of the choroidal neovascularization. For posttreatment color fundus photographs, these methods are used to assess the extent and intensity of treatment. Although these methods were developed to judge eligibility and treatment of patients enrolled in the MPS, they provide an excellent way for all treating ophthalmologists to evaluate their patients' angiograms and to assess immediately the intensity and extent of laser photocoagulation. The technique requires a microfilm reader or slide projection device to determine the completeness of treatment. The authors superimpose independent drawings made from pre- and posttreatment photographs. The techniques described can be applied readily in clinical practice. Since persistent neovascularization is highly correlated with incomplete and/or inadequate photocoagulation treatment, clinicians may adopt these Reading Center techniques to minimize the frequency of persistent neovascularization and, possibly, to reduce the frequency of visual loss in treated eyes.

A case of a man with an otherwise simple facial fracture which was complicated by mediastinal emphysema is reported. A review of the literature indicating the pathogenesis, clinical and radiographic features and management of this condition is given.

Four patients with macular hamartomas of the retinal pigment epithelium and retina are described. Associated choroidal neovascularization was noted in one patient. The clinical, fluorescein angiographic, and histopathologic findings of this entity are reviewed.

The efficacy of daily intramuscular injection of mixed gangliosides (Cronassial) for highly symptomatic diabetic neuropathy was investigated in a randomized double-blind trial. There were 37 patients treated with 40 mg of gangliosides or placebo for 12 weeks and studied with physical examination, symptom scales, and electrophysiologic parameters. There was no demonstrated benefit of therapy. Given the spontaneous variation in the measurements employed, larger numbers of patients would have been needed to identify a significant change of 10% owing to therapy.

A simple technique for the fixation of maxillary fractures using premaxillary pins is described. Three case reports have been used to illustrate the technique which is recommended when maxillary fractures occur in conjunction with craniofacial or multiple injuries and when conventional methods of fixation are difficult to apply.

Sixty-eight consecutive patients with retinitis pigmentosa were studied to determine the frequency of the nonpigmented form of the disease. There was an overall incidence of 22%. Fifty percent of all cases had no characteristic pigmentation, if the duration of night vision difficulty was three years or less. The nonpigmented form of retinitis pigmentosa was frequently an early stage of the disease and not an unusual or atypical variant. Patients without the pigmentary changes characteristic of the disorder also showed less functional impairment: the electroretinographic b wave was more apt to be recordable, although impaired, and the rod threshold, as determined by dark adaptation measurement, was less elevated. The clinician should suspect retinitis pigmentosa, even in the absence of pigmentary changes, if there is a family history of the disorder, night blindness, peripheral field loss, and an impaired or nonrecordable electroretinographic response.

A major cause for failure of krypton red laser photocoagulation in patients with exudative age-related macular degeneration has been the development of recurrent choroidal neovascularization adjacent to the previously treated areas. After reviewing the possible causes of recurrences, it is apparent that a certain number are iatrogenic, ie, induced by krypton red laser causing disruption and damage to the pigment epithelium-Bruch's membrane-choroidal complex. We describe three separate episodes in two patients of iatrogenic recurrent choroidal neovascularization after krypton red laser photocoagulation.

Optimal control of any stage of diabetes is possible only with optimal dietary management. Furthermore, in 90% of stable patients who comply with regimens like those described, this dietary approach alone will suffice for control.

Sixty-eight consecutive patients with retinitis pigmentosa were studied to determine the frequency of the non-pigmented form of the disease. There was an overall incidence of 22%. Fifty percent of all cases had no characteristic pigmentation, if the duration of night vision difficulty was three years or less. The study lends statistical support to the concept that the non-pigmented form of retinitis pigmentosa is frequently an early stage of the disease and not an unusual or atypical variant. Patient without the pigmentary changes characteristic of the disorder also showed less functional impairment: the ERG b-wave was more apt to be recordable, although impaired; and the rod threshold, as determined by dark adaptation measurement, was less elevated. The clinician should suspect retinitis pigmentosa, even in the absence of pigmentary changes, if there is a family history of the disorder, night blindness, peripheral field loss, and an impaired or non-recordable electroretinographic response.

Double blind placebo controlled trials of dialy intramuscular injections of 40 mg of mixed gangliosides were carried out in ALS and diabetic neuropathy. Forty patients with ALS were treated for six months and monitored with tests of strength and pulmonary function. No effect of gangliosides was found. Forty patients with symptomatic diabetic neuropathy will be treated for three months. Some of the data from the first 25 patients to complete the study were analyzed in a preliminary fashion. While no difference in nerve conduction studies was found in the treated group compared to the control group, there was greater symptomatic improvement in the patients treated with gangliosides.

Increasing use of penile prostheses to treat impotence in diabetic men warrants a detailed assessment of functional and subjective outcomes within this group. This study uses record review and patient-partner questionnaire techniques to examine this population and evaluate postoperative satisfaction in terms of changes in patterns of sexual activity, health status, and complication rates. The satisfaction rates among patients and partners were 81% and 83%, respectively. Frequency of sexual activity was increased, and physical enjoyment was improved among satisfied recipients. Most postoperative complications were treatable and led to dissatisfaction only if they were irreparable. Most prostheses are therefore being used successfully. We recommend that impotent diabetic men be informed of the availability of prosthetic implants.

An unusual case of a large rhinolith, which initially presented as an oronasal fistula, is reported. The surgical removal of the rhinolith through a maxillary osteotomy at the Le Fort I level with immediate direct fixation using 'Champy' plates is described. This approach provided excellent access for removal of the mass and repair of the oronasal fistula.

A case is reported of a foreign body in the left parotid gland, which presented in an unusual manner following a trivial injury and posed a difficult diagnostic problem, trismus being the most prominent clinical feature. The importance of having a high index of suspicion for retained foreign bodies is stressed.

Osteomyelitis in adults due to Haemophilus influenzae is exceedingly rare. We report a case of H. influenzae type b vertebral osteomyelitis in an adult. Review of the English literature reveals one other adult patient with H. influenzae osteomyelitis, and sporadic pediatric cases. Although a variety of predisposing host resistance factors have been postulated to account for this infection, the deficiency responsible for adult infection remains undelineated. Bone biopsy is mandatory for diagnosis when blood cultures are negative. The isolation and identification of H. influenzae may be delayed because of its fastidious growth requirements. Treatment with ampicillin or chloramphenicol appear to be most efficacious considering the antibiotic sensitivities of the organism.

To determine the modes of transmission in a cholera epidemic in a newly infected population on an atoll, two studies were conducted, in which persons with cholera were individually matched, by age and sex, with neighbourhood controls. Among the first recognised cases, cholera was associated with frequent (daily or weekly) consumption of raw lagoon fish that had been salted and partially dried. Vibrio cholerae was isolated from the lagoon, which was contaminated by human wastes. During the declining phase of the epidemic, cholera was associated with consumption of raw clams, salt-fish, sardines, and other fish from the lagoon. When seafood from enclosed bodies of faecally contaminated water is frequently eaten raw, imported V. cholerae can become established and can be extremely difficult to eradicate.