Unusual manifestations of henoch-schonlein purpura

Henoch schonlein syndrome (HSP) represents a diagnostic challenge when gastrointestinal symptoms or other atypical symptoms precede the cutaneous manifestations. We describe two patients whose abdominal symptoms antedated cutaneous manifestations. Six-year-old, black male patient, presented with 1 wk history of vomiting (without diarrhea or urinary symptoms), hypertension (140/90 mmHg), diffuse abdominal pain and hypoactive bowel sounds:. WBC’s 40,000/Cu mm³ with 80 percent hypoproteinemia, (albumin 1.6 G%), high plasma renin and stools positive for occult blood. Renal function and urinalysis was normal. Two weeks later patient developed urticarial purpuric rash over the ankles, gross hematuria and gross edema. Biopsy of the skin lesion was consistent with HSP vasculitis. Second patient 4 yr Brazilian male patient presented with abdominalpain, vomiting, blood in the stools, and pain in the right testis. Three days later, developed purpuric rash over his limbs and trunk. He also had hypoproteinemia, hypoalbuminemia, leukocytosis and normal urine analysis. Abdominal pain, bloody stools, painful testicular swelling and hypoproteinemia abated with steroid therapy. In HSP patients, atypical presentation of abdominal pain, hypertension, hypoproteinemia without proteinuria testicular swelling and leucocytosis in the absence of cutaneous and joint manifestations poses as diagnostic problem. Protein-losing enteropathy should be considered in HSP patients with hypoproteinemia.     

Effect of age on the refractive outcome of myopic photorefractive keratectomy

PURPOSE: To evaluate the effect of age on the outcome of myopic photorefractive keratectomy (PRK). SETTING: University-based refractive surgery practice. METHODS: A retrospective analysis of 197 eyes that had PRK for myopia was performed. Four groups of patients were compared: Group 1, patients < 30 years (n=35); Group 2, patients 31 to 40 years (n=56); Group 3, patients 41 to 50 year s (n=47); Group 4, patients > 50 years (n=59). The percentage of eyes with an uncorrected visual acuity of 20/20 or better and 20/40 or better and the percentage of eyes with spherical correction within +/-0.5 and +/-1.0 diopter (D) of the attempted correction were derived for each age group. The percentage of patients overcorrected and undercorrected by 1.0 D or more in each age group was also calculated.The difference between the percentage of patients who achieved a visual acuity of 20/20 or better in Group 4 and in the younger groups was statistically significant at 3 months (P =.02) but not at 6 months (P =.70) and 12 months (P =.55). The difference between the percentage of patients who achieved an acuity of 20/40 or better was not significant at any time. The difference between the percentage of patients who were within +/-0.5 D of intended correction in Group 4 and in the younger groups was statistically significant at 3 months (P =.001), 6 months (P =.006), and 12 months (P =.008); the difference between the percentage of patients who were within +/-1.0 D of intended correction was not significant at any time. The difference between the percentage of patients overcorrected and undercorrected by 1.0 D or more in Group 4 and in the younger groups was not significant at any time.In the 3 younger groups, age did not significantly affect visual outcome or predictability. However, there was a significant difference between Group 4 and the 3 younger groups in predictability of the refractive outcome at 3, 6, and 12 months. Age may play a role in the outcome of refractive surgery.     

Understanding corneal topography

With the increase in corneal laser refractive surgery, there is a greater need for precise evaluation of the corneal surface. Articles published in the past year have reported possible use of confocal scanning laser technology-based devices to image the cornea. Other studies have compared existing instruments and software in an effort to determine if data from different instruments are comparable. Topographic evaluation also has served to highlight long-term complications of procedures like radial keratotomy and the promise of newer surgical procedures like the intrastromal corneal ring segments. Studies of the corneal surface have helped refine surgical procedures like photorefractive keratectomy, laser in situ keratomileusis, penetrating keratoplasty, and cataract surgery. Topographic measures that serve as direct correlates of ocular visual performance, however, still remain elusive. Studies in the past year have confirmed that corneal topographic evaluation may be a powerful tool in the search for a genetic basis of keratoconus. Important areas for future research include precise determination of the power of the postrefractive surgery cornea to allow precise estimation of intraocular lens power in these eyes. Detecting the presence of past corneal refractive surgery in donor eyes also is likely to be a challenge. Despite the numerous advances in the field of corneal topography in the past year, there is still a need to present the data in a standardized format that is universal to all instruments and technologies.     

Corneal autografting: a systematic approach

OBJECTIVE: To describe a systematic approach to corneal autografting. PATIENTS AND METHODS: Retrospective review of case records of 3 patients who underwent this procedure. RESULTS: All patients achieved satisfactory visual recovery. Oversized grafts of 0.5 mm had less post-operative complications in the recipient eye. A phakic blind eye that received a 0.5 mm undersized graft had a temporary ocular hypertensive phase. CONCLUSIONS: Corneal autografting is a safe and effective technique in selected patients. The preferred surgical technique consists of surgery first on the blind eye, excision of a 0.5 mm oversized donor graft, cataract extraction if the eye is phakic, and use of an appropriate temporary keratoprosthesis to maintain donor eye integrity until autografting is completed in the eye with visual potential.     

Regulation of steroid hormone biosynthesis in R2C and MA-10 Leydig tumor cells: role of the cholesterol transfer proteins StAR and PBR

The MA-10 mouse Leydig tumor cell line produces large amounts of steroids only in response to hormonal stimulation while the R2C rat Leydig tumor cell line is constitutively steroidogenic in nature. In an effort to uncover the potential reasons for constitutive steroidogenesis in R2C cells, we have recently shown that compared to MA-10 cells, R2C cells express much higher levels of the Scavenger Receptor Class B type 1 which results in a higher capacity for cholesteryl ester uptake through the selective uptake pathway. We also found an enhanced expression of Hormone Sensitive Lipase and the Steroidogenic Acute Regulatory protein in these cells and reasoned that they may further facilitate the conversion of cholesteryl esters to free cholesterol and its mobilization to the inner mitochondrial membrane, thus rendering them constitutively steroidogenic. Given the proposed role of the peripheral-type benzodiazepine receptor (PBR) in conferring a constitutively steroidogenic phenotype to the R2C cells, the current study was conducted to investigate the relationship between its expression in MA-10 and R2C cells and correlate it with the constitutive nature of R2C cell steroidogenesis. Our studies show that PBR expression as measured by PK 11195 ligand binding and Western analysis is much higher in MA-10 cells than R2C cells. We also determined that the affinity of ligand binding to the PBR is comparable in the two cell lines, suggesting that PBR is unlikely to be solely responsible for the constitutive nature of R2C cell steroidogenesis.