Eissa Hanna

Correlation of filtration bleb morphology with histology.

Guarded filtration surgery (ie, trabeculectomy), introduced by Sugar and Cairns, remains the procedure of choice for the majority of patients with glaucoma. Antimetabolites, 5-fluorouracil (5-FU) and mitomycin C (MMC), allow wound healing modulation and an individualized approach to modify the inflammatory/fibrotic response to the surgical insult. Despite advances in operative technique guarded trabeculectomy augmented by antimetabolites can have significant complications such as hypotony, suprachoroidal hemorrhage, choroidal effusions, hypotony maculopathy, bleb leak, blebitis, bleb encapsulation, failure, and endophthalmitis. These complications are partially attributed to the contradictory concepts of traditional glaucoma surgery: prevention of wound healing around the surgical fistula and inhibition of fibrosis of Tenon capsule to sclera on one hand and normal tissue repair of the overlying delicate conjunctiva to maintain the functional and anatomic integrity of the bleb constituting an adequate barrier against infection on the other.

A case of autoimmune-related retinopathy and optic neuropathy syndrome treated by autologous nonmyeloablative hematopoietic stem cell transplantation.

Autoimmune-related retinopathy and optic neuropathy (ARRON) syndrome is characterized by visual loss and often the presence of antibodies against retinal or optic nerve antigens in the absence of cancer. Limited success has been reported in treatment of ARRON syndrome with medications that suppress the immune system. In many patients, current strategies are insufficient to control the disease. A 47-year-old woman with progressive visual and hearing loss attributed to ARRON syndrome that was resistant to conventional therapies underwent autologous hematopoietic stem cell transplantation (HSCT). Clinical manifestations appeared to stabilize. This report suggests that autologous HSCT may have a therapeutic role in ARRON syndrome.

Subepidermal calcified nodule of the eyelid

Subepidermal calcified nodule, a rare form of calcinosis cutis, is a benign lesion of the eyelid that occurs in children. We report a case of a 9-year-old boy with a nodule of the right upper eyelid simulating Molluscum contagiosum. The histopathologic examination of the excised nodule confirmed the diagnosis.

Induction of positive cooperativity by amino acid replacements within the C-terminal domain of Penicillium chrysogenum ATP sulfurylase

ATP sulfurylase from Penicillium chrysogenum is an allosteric enzyme in which Cys-509 is critical for maintaining the R state. Cys-509 is located in a C-terminal domain that is 42% identical to the conserved core of adenosine 5′-phosphosulfate (adenylylsulfate) (APS) kinase. This domain is believed to provide the binding site for the allosteric effector, 3′-phosphoadenosine 5′-phosphosulfate (PAPS). Replacement of Cys-509 with either Tyr or Ser destabilizes the R state, resulting in an enzyme that is intrinsically cooperative at pH 8 in the absence of PAPS. The kinetics of C509Y resemble those of the wild type enzyme in which Cys-509 has been covalently modified. The kinetics of C509S resemble those of the wild type enzyme in the presence of PAPS. It is likely that the negative charge on the Cys-509 side chain helps to stabilize the R state. Treatment of the enzyme with a low level of trypsin results in cleavage at Lys-527, a residue that lies in a region analogous to a PAPS motif-containing mobile loop of true APS kinase. Both mutant enzymes were cleaved more rapidly than the wild type enzyme, suggesting that movement of the mobile loop occurs during the R to T transition.

Congenital pupillary-iris-lens membrane with goniodysgenesis: a rare cause of glaucoma and vision loss.

Congenital pupillary-iris-lens-membrane with goniodysgenesis (CPILMG) is a rare congenital unilateral sporadic anterior segment malformation, whose key distinctive features are pupillary membrane that can be progressive and goniodysgenesis of the chamber angle. The condition was first defined by Cibis et al in 1986, and later elaborated upon by Lambert et al, Robb, and Cibis and Walton (although a similar case was described by Stephenson and another case can be found in German literature). We report a case of a 10-year-old boy with CPILMG briefly described earlier by David S. Walton along with a review of the literature.

Exaggerated postsurgical inflammation in a patient with insufficiently treated Addison disease.

A patient with Addison disease developed fever, pain, and marked orbital inflammation 3 days after evisceration in the setting of perforated corneal ulcer. He was treated for presumed orbital cellulitis without improvement. Increasing the corticosteroid dose for his Addison disease resulted in complete resolution of the inflammation.

Congenital Pupillary-Iris-Lens Membrane With Goniodysgenesis: Histopathologic Findings in an Enucleated Eye

A 10-year-old boy with clinically confirmed congenital pupillary-iris-lens membrane with goniodysgenesis underwent light microscopic examination of the enucleated eye. The anterior segment changes consisted of the absence of the iridial pupillary sphincter muscle and dilator muscle processes. Endothelialization and descemetization of the anterior chamber angle and the anterior surface of the iris covered the original eccentric pupillary opening (occlusio pupillae), but grew around the fibrotic edges of the surgically created, patent pseudopupil, probably secondary responses to surgery. The anterior chamber findings in this case establish a localized syndrome that includes absence of the pupillary sphincter and dilator muscle processes. Incomplete development of the iris may be partly attributable to an abnormality of stromal development and inductions by the anterior neuroectodermal layer, together with anomalies of the pupillo-iridial vasculature.