Recurrent Heerfordt-Waldenström Syndrome with thyroid and meningeal involvement in a Chinese woman.

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology, manifesting with bilateral hilar adenopathy, pulmonary reticular opacities, skin, joint or eye lesions. Heerfordt-Waldenström Syndrome - a constellation of facial palsy, fever, uveitis and parotitis - is a rare presentation of this disorder. A 47-year-old Chinese woman presented with unintentional weight loss, lethargy with mediastinal and hilar lymphadenopathy. Biopsy of the right paratracheal lymph node via mediastinoscopy showed mycobacterial granulomatous lymphadenitis consistent with tuberculosis with several acid-fast bacilli identified. Lymphoproliferative disorder was ruled out. She was started on treatment for tuberculosis. Eleven weeks into treatment, she developed a right facial palsy accompanied with fever, uveitis and occipital headache. At this juncture, further history revealed a background of recurrent alternating facial palsy and parotid gland enlargement which was treated for Bell's palsy by three different doctors. New nodules appeared in the left lobe of the thyroid gland. Biopsy of a palpable thyroid nodule and a right supraclavicular lymph nodule showed histological features suggestive of sarcoidosis. Fungal and mycobacterial infections were ruled out. In addition, examination of her cerebral spinal fluid showed lymphocytic inflammation. The serum ACE level was not raised. A diagnosis of sarcoidosis with incomplete features of Heerfordt-Waldenström Syndrome along with thyroid and meningeal involvement was made. The patient was commenced on prednisolone and azathioprine and her symptoms responded shortly after. We present a rare case of Heerfordt-Waldenström Syndrome with thyroid and meningeal involvement in a Chinese woman.

Reducing the Time to Blood Administration after Pediatric Injury: A Quality Improvement Initiative.

Introduction: Hemorrhage is the leading cause of preventable death in pediatric trauma patients. Timely blood administration is associated with improved outcomes in children and adults. This study aimed to identify delays to transfusion and improve the time to blood administration among injured children. Methods: A multidisciplinary team identified three activities associated with blood transfusion delays during the acute resuscitation of injured children. To address delays related to these activities, we relocated the storage of un-crossmatched blood to the emergency department (ED), created and disseminated an intravenous access algorithm, and established a nursing educator role for resuscitations. We performed comparative and regression analyses to identify the impact of these factors on the timeliness and likelihood of blood administration. Results: From January 2017 to June 2021, we treated 2159 injured children and adolescents in the resuscitation area, 54 (2.5%) of whom received blood products in the ED. After placing a blood storage refrigerator in the ED, we observed a centerline change that lowered the adjusted time-to-blood administration to 17 minutes (SD 11), reducing the time-to-blood administration by 11 minutes (ß = -11.0, 95% CI = -22.0 to -0.9). The likelihood of blood administration was not changed after placement of the blood refrigerator. We observed no reduction in time following the implementation of the intravenous access algorithm or a nursing educator. Conclusions: Relocation of un-crossmatched blood storage to the ED decreased the time to blood transfusion. This system-based intervention should be considered a strategy for reducing delays in transfusion in time-critical settings.

The natural history of polypoidal choroidal vasculopathy: a multi-center series of untreated Asian patients.

PURPOSE: We aimed to evaluate the long-term natural history of polypoidal choroidal vasculopathy (PCV) in untreated patients. METHODS: This is a retrospective observational case series. Patients with symptomatic PCV who did not receive any treatment for at least 12 months were included from the records of three ophthalmic clinics in Asia. The medical records and imaging data were reviewed. Visual outcomes at month 12 and at last follow-up were analyzed. The influence of demographics and presenting features on visual outcome was analyzed. RESULTS: A total of 32 eyes (32 patients) were included in this analysis. The mean follow-up was 59.9 months (range, 18-119 months), the mean age was 65.7 years and 21 (65.6 %) patients were male. The mean presenting logMAR visual acuity was 0.79 (Standard deviation [SD] 0.49). The center of the fovea was involved by the PCV complex in 25 eyes (78.1 %). The mean greatest linear dimension (GLD) of the PCV complex was 2584 µm (SD 880). Twenty-three eyes (71.9 %) had a cluster-of-grapes configuration on indocyanine green angiography. Leakage of fluorescein angiography was present in 29 eyes (90.6 %). The mean logMAR vision deteriorated from 0.79 at baseline to 0.88 at month 12 (p = 0.11), and further to 1.14 (p = 0.003) at the last follow-up. The proportion of eyes that improved, remained unchanged and worsened was 21.9 %, 31.3 % and 46.9 %, respectively, at month 12; and 28.1 %, 9.4 % and 62.5 %, respectively, at last follow-up. The proportion of eyes with logMAR vision worse than 1.0 was 28.1 % at presentation, and increased to 31.3 % at month 12 and further to 53.1 % at last follow-up. Reasons for poor vision were due to retinal, subretinal or vitreous hemorrhage, and retinal pigment epithelium (RPE) atrophy and scarring. None of the presenting features were found to significantly influence visual outcome. CONCLUSIONS: Half of eyes presenting with symptomatic PCV had a relatively benign course without treatment and some even had vision improvement. However, in the remaining eyes, vision deteriorated significantly, mainly due to hemorrhage and scarring. There may be subtypes of PCV with divergent natural history.

National hospital quality measures data: supporting nurses' key roles in organizational performance improvement.

With the emergence of so many methodologies for generating comparison data and with the growing accountability demands from so many sectors (each, seemingly, with its own preferred comparative methodology), nurses and quality improvement professionals may feel as if they have many masters to serve. This article outlines the Maryland Hospital Association's Quality Indicator Project's approach to working with quality improvement professionals to build their understanding of comparative data and help them determine which data analysis tools best fit their reporting needs.

The outer and inner retinal function in patients with multiple evanescent white dot syndrome.

BACKGROUND: To investigate the outer and inner retinal function in patients with multiple evanescent white dot syndrome (MEWDS). METHODS: The retinal function of three subjects with MEWDS was investigated using one or a combination of full-field electroretinography (ERG), multifocal electroretinography (mfERG) or recording of multifocal oscillatory potentials (mfOP). RESULTS: In case 1, the scotopic, maximal, photopic and flicker ERG responses of the two eyes were similar but the amplitudes of the dark- and light-adapted OPs were markedly reduced in the affected eye. In cases 2 and 3, the ERG responses were grossly reduced in amplitude and as expected the OPs were also diminished. However, using the mfERG a residual area of 'normal' retinal function in the affected eye was identified. The local OP, assessed by the mfOP, within the residual 'normal' retinal area was reduced as compared with the corresponding retinal area of the fellow unaffected eye. Subsequently, the mfERG responses of the 'normal' retinal area were also reduced. CONCLUSION: The OPs were reduced throughout the retina in patients with MEWDS, even in the area with a normal mfERG. The electrophysiological findings suggest that functional abnormality in MEWDS may occur initially in the inner retina and subsequently involves the outer retina.

Photodynamic therapy for choroidal neovascularization in stargardt disease and retinitis pigmentosa.

PURPOSE: To report the successful use of photodynamic therapy (PDT) in three patients with choroidal neovascularization (CNV) secondary to retinal dystrophies. METHODS: Three patients who presented for blurring of vision were found to have CNV on fluorescein angiography. Two patients had retinitis pigmentosa and one patient had Stargardt disease. They underwent standard regime of PDT. RESULTS: Resolution of hemorrhage with improvement of visual acuity was seen in all three patients after one round of PDT. One patient required a second round of PDT because of the recurrence of CNV. Two patients were subsequently followed up for at least 2 years and 1 patient who defaulted after the 3 month follow-up showed no recurrence. CONCLUSION: Stargardt disease and retinitis pigmentosa are common retinal dystrophies. We report our successful treatment of CNV with PDT in two patients with retinitis pigmentosa and one patient with Stargardt disease. Despite the increasing use of antivascular endothelial growth factors drugs, standard treatments can still be applied to certain forms of CNV.

Outcomes of femtosecond laser-assisted penetrating keratoplasty.

PURPOSE: To evaluate outcomes from the use of a femtosecond laser to trephine both donor and recipient corneas during penetrating keratoplasty (PK). DESIGN: Prospective interventional case series. METHODS: Patients were recruited from the cornea clinic of the Singapore National Eye Centre. We used a 10-kHz Femtec (20/10 Perfect Vision, Heidelberg, Germany) femtosecond laser to perform trephination of the donor cornea on an artificial anterior chamber, followed by trephination of the recipient cornea. Trephination cuts were straight and performed 90 degrees to the corneal surface. Tissue bridges were bluntly separated with a Barrett phaco chopper. The donor button was then sutured to the recipient with double continuous sutures, or interrupted sutures if significant host corneal vascularization was present. Postoperatively, visual acuity, refraction, intraocular pressures, and optical coherence tomography (Visante; Carl Zeiss, Jena, Germany) were evaluated. RESULTS: Eight eyes of eight patients underwent PK for conditions ranging from bullous keratopathy to corneal scarring from herpetic stromal keratitis. Patients were followed up for a mean of 9.5 months. Best-corrected visual acuities of patients with no ocular comorbidity ranged from 20/20 to 20/80. Mean cylindrical refractive error at last review was 2.56 diopters [D] (range, 0.50 to 4.00 D). Tissue bridges were bluntly dissected except for one case that required scissors completion of trephination. No complications were encountered related to use of the Femtec laser. CONCLUSION: The Femtec laser reliably trephines both donor and recipient corneas for PK, with good visual outcomes and relatively low degrees of astigmatism.

Intraocular avastin (bevacizumab) for neovascularisation of the iris and neovascular glaucoma.

INTRODUCTION: The aim of this study was to determine the effectiveness of intraocular injections of bevacizumab for neovascularisation of the iris and neovascular glaucoma. CLINICAL PICTURE: Three patients with neovascularisation of the iris due to various causes were recruited. TREATMENT: Patients were treated with intraocular bevacizumab. OUTCOME: Neovascularisation of the iris was noted to have completely regressed as early as 3 days after the injection and in all the patients (100%) within 8 days after injection. They were followed up for at least 1 month with no clinical evidence of recurrence. Visual acuity remained stable or improved, and the intraocular pressure was controlled in all the 3 patients' eyes. Vitreous haemorrhage also cleared. No signs of inflammation or complications were observed. CONCLUSION: Intraocular injection of bevacizumab is effective and safe for patients with neovascularisation of the iris and neovascular glaucoma with or without vitreous haemorrhage.

Empirical vs Bayesian approach for estimating haplotypes from genotypes of unrelated individuals.

BACKGROUND: The completion of the HapMap project has stimulated further development of haplotype-based methodologies for disease associations. A key aspect of such development is the statistical inference of individual diplotypes from unphased genotypes. Several methodologies for inferring haplotypes have been developed, but they have not been evaluated extensively to determine which method not only performs well, but also can be easily incorporated in downstream haplotype-based association analyses. In this paper, we attempt to do so. Our evaluation was carried out by comparing the two leading Bayesian methods, implemented in PHASE and HAPLOTYPER, and the two leading empirical methods, implemented in PL-EM and HPlus. We used these methods to analyze real data, namely the dense genotypes on X-chromosome of 30 European and 30 African trios provided by the International HapMap Project, and simulated genotype data. Our conclusions are based on these analyses. RESULTS: All programs performed very well on X-chromosome data, with an average similarity index of 0.99 and an average prediction rate of 0.99 for both European and African trios. On simulated data with approximation of coalescence, PHASE implementing the Bayesian method based on the coalescence approximation outperformed other programs on small sample sizes. When the sample size increased, other programs performed as well as PHASE. PL-EM and HPlus implementing empirical methods required much less running time than the programs implementing the Bayesian methods. They required only one hundredth or thousandth of the running time required by PHASE, particularly when analyzing large sample sizes and large umber of SNPs. CONCLUSION: For large sample sizes (hundreds or more), which most association studies require, the two empirical methods might be used since they infer the haplotypes as accurately as any Bayesian methods and can be incorporated easily into downstream haplotype-based analyses such as haplotype-association analyses.

Lacrimal gland amyloidosis.

Primary localized lacrimal gland amyloidosis is an extremely rare occurrence. The typical clinical and radiologic appearances have been suggested but not clearly established. This report describes two patients with amyloid tumor of the lacrimal gland. Both patients were middle-aged women of Asian origin, one a Chinese with unilateral lacrimal gland swelling and the other, a Malay with bilateral lacrimal gland swelling. Neither patient had associated systemic disease. They were otherwise asymptomatic except for prominence over the lacrimal gland region. Neither visual impairment nor evidence of optic nerve dysfunction was noted in either patient. CT findings mimic that of an inflammatory or lymphoproliferative disorder of the lacrimal gland. The diagnosis of amyloidosis was only proved on histology. Further investigations revealed that the disease process was truly localized and not part of a systemic process. Subsequent observation proved no recurrence.

Extensive upper-extremity venous thrombosis after fluorescein angiography.

PURPOSE: To report a case of extensive cephalic and basilic venous thrombosis after fluorescein angiography. DESIGN: Interventional case report. METHODS: A 38-year-old Chinese male presented with blurring of vision in his left eye. Clinical examination revealed central serous retinopathy, and a routine fluorescein angiography was performed. RESULTS: He developed inflammation over the right arm over 4 days. Duplex scan disclosed extensive superficial venous thrombosis of the right upper limb with occlusion of the basilic vein and cephalic vein in the proximal right upper arm to the level of the midarm. He was treated medically but was subsequently lost to follow-up. CONCLUSIONS: Extensive upper-extremity venous thrombosis is infrequent after single-shot intravenous cannulation. Venous thrombosis occurring after fluorescein angiography has not been reported up to this time. It is possible that proper arm positioning during the procedure may reduce this risk, and ophthalmologists should be vigilant of this potentially unusual complication.