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Gly-48 is in the conserved DGDQC sequence (residues 47-51 of human factor IX) of the first EGF (EGF-1)-like domain of factor IX. The importance of the Gly-48 is manifested by two hemophilia B patients; factor IXTainan and factor IXMalmo27, with Gly-48 replaced by arginine (designated IXG48R) and valine (IXG48V), respectively. Both patients were CRM+ exhibiting mild hemophilic episodes with 25% (former) and 19% (latter) normal clotting activities. We characterize both factor IX variants to show the roles of Gly-48 and the conservation of the DGDQC sequence in factor IX. Purified plasma and recombinant factor IX variants exhibited approximately 26%-27% normal factor IX's clotting activities with G48R or G48V mutation. Both variants depicted normal quenching of the intrinsic fluorescence by increasing concentrations of calcium ions and Tb3+, indicating that arginine and valine substitution for Gly-48 did not perturb the calcium site in the EGF-1 domain. Activation of both mutants by factor XIa appeared normal. The reduced clotting activity of factors IXG48R and IXG48V was attributed to the failure of both mutants to cleavage factor X: in the presence of only phospholipids and calcium ions, both mutants showed a 4 to approximately 7-fold elevation in Km, and by adding factor VIIIa to the system, although factor VIIIa potentiated the activation of factor X by the mutants factor IXaG48R and factor IXaG48V, a 2 to approximately 3-fold decrease in the catalytic function was observed with the mutant factor IXa's, despite that they bound factor VIIIa on the phospholipid vesicles with only slightly reduced affinity when compared to wild-type factor IXa. The apparent Kd for factor VIIIa binding was 0.83 nM for normal factor IXa, 1.74 nM for IXaG48R and 1.4 nM for IXaG48V. Strikingly, when interaction with the factor VIIa-TF complex was examined, both mutations were barely activated by the VIIa-TF complex and they also showed abnormal interaction with VIIa-TF in bovine thromboplastin-based PT assays. Taken together, our results suggest that mutations at Gly-48 altered the interaction of factor IX with its extrinsic pathway activator (VIIa-TF complex), its macromolecular substrate (factor X), and its cofactor (factor VIIIa).

Background/aims: A longitudinal study of premature infants was conducted to examine changes in refractive status and their relation with age and factors influencing the occurrence and degree of myopia. Identification of which of the various refractive factors play important parts in relation to myopia in premature infants was attempted., Methods: Under observation were 125 eyes in 65 patients who were found to demonstrate no signs of retinopathy of prematurity (ROP) or who had grade I or II ROP without or after cryotherapy. Cycloplegic refractions were conducted at 6 months, 3 years, and 6 years of age; at 6 years of age keratometric values, lens thicknesses, and axial lengths were recorded, and anterior chamber depths also were measured., Results: Myopia begins to appear at 6 months of age and its severity increases between the ages of 6 months and 3 years. The condition showed no further progress in subjects older than 3 years. Of the 104 eyes with ROP, those eyes with cicatricial retinopathy tended towards myopia and high myopia while there was no difference in the degree of myopia related to whether or not cryotherapy was conducted. At 6 years of age, the premature infants exhibited shallower anterior chambers, thicker lenses, and higher axial lengths when the degree of the myopia was higher. The keratometric values, however, appeared to bear no relation to the degree of the myopia., Conclusion: These results suggest that the occurrence of myopia is related more strongly to whether or not there is cicatricial retinopathy than whether or not there is cryotherapy. Also, the degree of the myopia was found to be related to the depth of the anterior chamber, the thickness of the lens, and the change in axial length but not to keratometric value.

Purpose: The purpose of this work was to characterize the MR features of post-traumatic osteolysis of the distal clavicle in patients who have sustained a previous separation of the ipsilateral acromioclavicular (AC) joint., Method: We studied eight male patients (mean age 25 years) with intractable pain in the AC joint after sustaining a traumatic joint separation. With use of the Rockwood classification, the separations were classified as Type 1 in one patient, Type 2 in two patients, and Type 3 in five patients. The MR studies were evaluated for periarticular soft tissue swelling, cortical irregularity defined as thinning or absence of portions of the cortex in the acromial and clavicular articular surfaces, hypertrophic osseous changes, periostitis, bone marrow edema, periarticular cyst-like changes, and joint space widening exceeding 6 mm. Radiographs were evaluated independently of the MR studies. Osteolysis of the distal clavicle was confirmed pathologically in seven patients and with surgery in one patient., Results: The incidence of osteolysis in patients who have had a previous AC joint separation was estimated to be approximately 6%. Observations on MRI included soft tissue swelling, bone marrow edema in the distal clavicle, and cortical irregularity associated with periarticular cyst-like erosions in eight patients, joint space widening in six patients, clavicular periostitis in three patients, and marrow edema in the cromion in five patients. Only one patient had osteophyte formation. Radiographic observations of periarticular soft tissue swelling, osteopenia of the distal clavicle, articular erosions, and joint space widening allowed diagnosis in only four patients prospectively., Conclusion: The MR features of posttraumatic osteolysis are characteristic of this process. We advocate the use of MRI in patients with chronic AC joint pain who have had a prior AC joint dislocation, particularly if follow-up radiographs are nonspecific, equivocal, or do not indicate the presence of secondary osteoarthritis.

Primordial germ cells (PGCs), as precursors of mammalian germ lineage, have been gaining more attention as a new resource of pluripotent stem cells, which bring a great possibility to study developmental events of germ cell in vitro and at animal level. EG4 cells derived from 10.5 days post coitum (dpc) PGCs of 129/svJ strain mouse were established and maintained in an undifferentiated state. With an attempt to study the differentiation capability of EG4 cells with a reporter protein: green fluorescence protein, and the possible application of EG4 cells in the research of germ cell development, we have generated several EG4-GFP cell lines expressing enhanced green fluorescence protein (EGFP) and still maintaining typical characteristics of pluripotent stem cells. Then, the differentiation of EG4-GFP cells in vitro as well as their developmental fate in chimeric embryos which were produced by aggregating EG4-GFP cells to 8-cell stage embryos were studied. The results showed that EG4 cells carrying green fluorescence have a potential use in the research of germ cell development and other related studies.

Scleral perforation occurred as a result of using a silicone band during scleral buckling surgery for subtotal retinal detachment in retinopathy of prematurity (ROP). The patient was initially treated by cryotherapy and scleral buckling surgery for ROP, and was later referred due to a dark bluish mass in the superotemporal quadrant of the eyeball. After removing the overlying whitish membrane, uveal tissue prolapsed through the melted scleral wound (5 mm x 5 mm). A silicone encircling band had passed through the wound and was exposed subconjunctivally around the temporal and the inferior limbus. The band was removed and a scleral allograft was performed. After three years, follow up revealed the eyeball was slightly microphthalmic. Though scleral bucking surgery is helpful for the treatment of advanced ROP, a scleral perforation may develop as a disastrous complication.

The visual results of laser photocoagulation for subfoveal choroidal neovascular membrane (CNVM) has not always been satisfactory. The surgical removal of the neovascular membrane may be another treatment option. To investigate the prognosis and risk factors of this surgery, we analyzed the results of surgical removal of subfoveal CNVM (23 eyes), subfoveal hemorrhage with CNVM (6 eyes), and subfoveal hemorrhage alone (6 eyes). The mean follow-up period was 17.7 months (range 2 to 47 months). The mean preoperative membrane size was 0.89 disc diameter and the mean postoperative retinal pigment epithelial (RPE) defect size was 1.33 disc diameter. Visual improvement was observed in 13 out of the 23 eyes (56.5%) with sufoveal CNVM, four out of the six eyes (66.6%) with subretinal hemorrhage and CNVM, and five out of the six eyes (83.3%) with subretinal hemorrhage only. The visual outcome of subfoveal CNVM surgery was related to the presence of a subfoveal RPE defect (p = 0.005) rather than to the size of the RPE defect. No recurrence of neovascular membrane was observed during the follow up period. In conclusion, surgical removal may be a good alternative treatment for subfoveal CNVM.

Objective: The purpose of this study was to find the best material for valvular wrapping operation of deep vein of lower limb and to study the diagnostic value of colored Ultrasonic-Doppler for valvular incompetency of the deep vein and the function of the popliteal valve., Methods: Strips of autogenous saphenous vein, autogenous fascia lata and pieces of polytetrafluroethylene artificial vessel were used respectively as the wrapping material for narrowing the valve in 30 dogs. The results of three different wrapping material were obtained by colored Ultrasonic-Doppler and transpopliteal venography in 78 patients. The hemokinetics of the popliteal valve was examined in 20 normal persons., Results: In the saphenous vein and fascia lata groups, diffuse fibrosis and marked narrowing of the femoral vein were found, while in the group of artificial vein graft, the graft was intact without prominent fibrosis and narrowing of the vein. In comparing with the result of venography, the accuracy of diagnosis by colored Ultrasonic-Doppler was 91.86%. The femoral and popliteal venous valves closed at the same time when holding the breath, but the popliteal venous valve opened more widely than the femoral venous valve when the calf muscles of the leg contracted., Conclusion: It was suggested that the graft was the best material for valvular wrapping operation, and colored Ultrasonic-Doppler was an important and non-invasive method for the diagnosis of incompetency of deep vein. The popliteal venous valve was the important barrier for protection of the function of deep vein.

We have developed a method that enables us to isolate cDNAs of putative membrane proteins. The system is designed to isolate a cDNA which can provide the transmembrane domain to the extracellular part of the IL-2 receptor alpha chain. We constructed a p18Mac vector by putting part of the IL-2 receptor alpha chain cDNA that encoded its signal sequence and extracellular domain, a cDNA cloning site and a poly(A) additional signal after a strong promoter SRalpha. If a cloned cDNA provides a transmembrane domain in-frame, the extracellular domain of the IL-2 receptor alpha chain will be expressed on the surface of the transfected cells. Otherwise, the chimeric protein will be either secreted or retained inside the transfected cells. We made a cDNA library using p18Mac and screened for cDNA clones which allowed the expression of the extracellular domain of the IL-2 receptor alpha chain on the cell surface. Of the 2000 clones screened, 5 clones were scored as positive. Partial sequence analysis revealed that one clone encoded the amyloid precursor protein, two others encoded mitochondrial proteins and the rest were new. These results suggest the system is effective in isolating cDNAs encoding putative membrane proteins.

Background and Objective: To report the surgical results of limbal lensectomy using an automated vitrectomy instrument for the treatment of subluxated lenses in children., Patients and Methods: The authors reviewed the charts of children who underwent surgery for subluxated lenses. Limbal lensectomies with an "in the bag" technique were performed on 18 eyes of 10 patients. The indications for surgery were a best-corrected visual acuity of less than 20/60, bisection of pupil by the lens, or both., Results: Best-corrected visual acuity improved in all 18 eyes (paired t test, P < .01). In 1 eye, transient anterior chamber hyphema occurred during the early postoperative period. No other significant complications were found during an average follow-up of 19.6 months., Conclusion: Limbal lensectomy for subluxated lenses in children improved visual acuity without severe complications.

Henoch-Schönlein purpura nephritis (HSPN) is a multi-organ systemic vasculitis, which shares many clinical, histological and immunological features with IgA nephropathy (IgAN). To address whether these two diseases have a common genetic background, the polymorphism of the variable number tandem repeat (VNTR) of IL-1 receptor antagonist (IL-1ra) gene has been analyzed using PCR in patients diagnosed with HSPN (N = 43) and IgAN (N = 97), together with normal controls (N = 98) and patients with acute post-infectious glomerulonephritis (APGN), under the concept that IL-1 might play an important role in mediating pathogenesis of vasculitis and glomerulonephritis. It was found that the allele frequency and carriage rate of the interleukin-1 receptor antagonist allele (IL1RN*2) of the IL-1ra gene increased significantly in HSPN patients as compared to IgAN (P < 0.01), APGN (P < 0.05) and normal subjects (P < 0.01). Interestingly, varied carriage rates of IL1RN*2 were found among various groups of IgAN patients presenting with different clinical manifestations. The carriage rate of IL1RN*2 was significantly higher in patients with recurrent gross hematuria than other groups of IgAN patients (P < 0.01). Furthermore, although the carriage rate of IL1RN*2 was higher in HSPN (46.5%) than average IgAN patients (26.8%; P < 0.01), there was no significant difference in the carriage rate of IL1RN*2 between HSPN and those IgAN patients with recurrent gross hematuria (42.8%l P > 0.05). It suggested that the IL1RN*2 allele might be a genetic marker shared by HSPN and a special group of IgAN patients with recurrent gross hematuria. Our preliminary observation provided a genetic evidence to support the hypothesis that HSPN and certain subgroup of IgAN are closely related diseases. Such an association of the gene polymorphism of IL-1ra between HSPN and IgAN with recurrent gross hematuria might serve as a key to explore their pathogenesis and eventually a specific intervention.

Cryotherapy has been shown to be an effective treatment for retinopathy of prematurity (ROP) stage 3+. However, the outcome of cryotherapy is less favorable in zone 1 ROP than in zone 2 ROP. We suspected whether there may be differences in the outcomes of cryotherapy if the zone of ROP is further divided. So we reviewed the records of 85 premature infants (145 eyes) who had undergone cryotherapy for ROP. The frequencies of favorable outcome were 42.9% of 14 eyes (zone 1), 78.9% of 38 eyes (posterior zone 2), 92.9% of 70 eyes (mid zone 2), and 100.0% of 23 eyes (anterior zone 2), respectively (p < 0.001). These results suggest that the more posteriorly the ROP is located, the less favorable the outcome of cryotherapy.

Persistent pupillary membranes are common congenital disorders. To reduce the surgical complications, we used vitreous scissors instead of conventionally used Vannas scissors to remove the membrane and reviewed the surgical results. Five eyes of 4 children with persistent pupillary membrane were treated by surgery. Through the 1.5 mm limbal incision, the membrane was removed by using vitreous scissors after intracameral sodium hyaluronate. After 22.6 months follow-up, all pupils of operated eyes were round and no complications associated with the surgery were found. Three eyes showed improved visual acuities after the operation. We suggest that the surgery with vitreous scissors is better than with Vannas scissors.

The promoter of TGF-beta type II receptor lacks TATA box or CAAT box. In order to define the exact transcriptional start site(s), we used "oligo-capping" method developed by ourselves. The major transcripts were started from GAA located between -35 and -33 relative to 5' end of cDNA. Other minor transcripts started from -4, +11 and +17. Deletion analysis of this promoter region revealed that it contained two adjacently located promoters (P1 and P2) capable of acting by itself. The P1 is located between -22 and +55 which covers the minor transcriptional start sites described above. The P2 is located just upstream of the P1 between -137 and -22. The results showed all of the transcripts were started either from P1 or P2.

To investigate the chronological change of refraction in premature infants after cryotherapy for retinopathy of prematurity (ROP), cycloplegic refractions had been performed at 6 months and 3 years after term in premature infants who underwent cryotherapy for ROP. The changes of refractions between the two study ages were evaluated not only in the total cryo-treated eyes, but also in the subdivided groups according to the posterior pole appearances. In the total 61 eyes of 32 premature infants, mean spherical equivalents were -4.05D vs. -5.94D (6 months vs. 3 years) (p = 0.0001). In the normal posterior pole group (48 eyes), mean spherical equivalents were -3.45D vs. -5.68D (6 months vs. 3 years) (p = 0.0000), and in the abnormal posterior pole group (13 eyes), -6.28D vs. -6.86D (6 months vs. 3 years) (p = 0.6496). These results mean that there is a myopic progressive change between 6 months and 3 years after term in the cryo-treated eyes for acute ROP and it is more evident in the eyes with normal posterior pole.

To determine the prognosis of advanced retinopathy of prematurity (ROP) after surgery, we reviewed retrospectively the results of vitrectomy performed between November 1987 and December 1993 in 38 eyes with stage 5 ROP. The patients' mean age at surgery was 11 months, mean follow-up period was 24.1 months. Anatomical success, defined as reattachment of posterior pole retina, was achieved in 11 eyes (29.0%) and functional success, defined as presence of fixation and following was achieved in 7 eyes (18.4%). Anatomical success was well correlated with preoperative configuration of retina, i.e. open-funnel type showed better results than closed-funnel type (44.4% compared to 15.0%). There were no differences between anatomical success and failure group according to gestational age, birth weight, age at the time of diagnosis of ROP, chronological and postconceptional age, and body weight at the time of surgery. For good prognosis, it is necessary to operate early in stage 5 ROP when retinal funnel is still open.

Purpose: Photosensitization is a mechanism by which oxygen and light may interact to generate free radicals capable of tissue injury. It has been proposed as a possible etiologic mechanism in the development of retinopathy of prematurity. The authors report the effects of light exposure and a photosensitizer, rose bengal (RB), on the developing retina of the beagle puppy., Methods: Seven purebred beagle puppies (2 or 7 days old) were given RB (7.5 mg/ml) intravenously (0.9 ml bolus followed by a 74 microliters/min constant infusion), and one eye was exposed to filtered light delivered by a modified slit lamp at 30 mW/cm2 for 5, 15, 25, 35, and 45 minutes. The fellow eye was not irradiated and served as an RB-only control. Three beagles were exposed to light (one eye only) for 15 to 120 minutes in the absence of RB to provide light-only and no-treatment control eyes. Animals were followed clinically by indirect ophthalmoscopy and fluorescein angiography, and eyes were processed for light microscopy., Results: Clinical and/or histologic abnormalities were found in all seven eyes exposed to light in the presence of rose bengal, as follows: vitreous hemorrhage in four eyes, incomplete peripheral retinal vascularization in four eyes, fibrovascular/fibrocellular proliferation with traction on the retina in two eyes (including preretinal neovascularization in one eye), total or partial retinal detachment in two eyes, dilated peripheral vessels compatible with shunt vessels in one eye, retinal dysplasia with loss of normal architecture and formation of rosettes in five eyes. All 13 control eyes showed normal, complete retinal vascularization., Conclusions: The failure to progress to the end-stage tractional retinal detachment seen in human infants is a shortcoming of existing animal models. Photosensitization injury to the developing retina in this animal model can produce a spectrum of retinal pathology that includes extraretinal vascularization and subsequent retinal detachment.

The authors studied the effect of tonsillectomy for IgA nephropathy and the relationship between chronic tonsillitis and IgA nephropathy. Forty-five patients with IgA nephropathy were treated by tonsillectomy in our department. The effective rate of hematuria type IgA nephropathy was 86.2% and that of non-hematuria type 62.5%. We also determined the immunoglobulin composition of the tonsilla tissue in 18 IgA nephropathy patients and compared it with that of 18 chronic tonsillitis patients. The immunoglobin level of the IgA nephropathy group was higher than that of the contrast group, indicating that the disorder of tonsilla immunity may be one of the pathogenic factors for IgA nephropathy. Since no specific method has been available for the treatment of IgA nephropathy, we use tonsillectomy as an effective procedure.

Since 1983, we have used free dorsal pedal flaps with m. extensor hallucis brevis for two patients with serious thenar defects to restore their thumb function. The results were satisfactory, as most of oponens function was restored, however the power of the thumbs was inadequate. Functional restoration of the thumb after the loss of thenar muscles involves many aspects of microsurgery. This article discusses only the dissection of the muscle, the orientation of transferred muscle, etc.

The authors performed cycloplegic refractions in 180 eyes of 99 premature infants at the age of 6 months to evaluate the incidence and the degree of myopia according to the development and disease course of retinopathy of prematurity (ROP) and to investigate the effect of cryotherapy on the refractive error. The incidences of myopia were not different between premature infants without ROP and premature infants with spontaneously and totally regressed ROP (36.3%, 25.5%), and the degrees of myopia were low in both groups (-1.76 D, -2.25 D). In premature infants with totally regressed ROP after cryotherapy, the incidence of myopia was high (75.5%) but the degree of myopia was low (-3.03 D). In premature infants with cicatricial ROP, cryotreated or not, both the incidence and the degree of myopia were high (93.9%, -5.50 D). It is suggested that cryotherapy increases the incidence of myopia but the degree of myopia induced by cryotherapy is low.

Electron microscopic examination of proliferative membranes in retinopathy of prematurity (ROP) was performed in order to evaluate the components of the membranes. The proliferative membranes were obtained from nine patients with ROP stage 5 during pars plicata lensectomy, vitrectomy, and delamination of membrane. Fibrous astrocytes, myofibroblasts, lymphocytes, macrophages, and calcification were found respectively in two cases, and fibroblast-like cells were found in one case. Varying amounts of collagen tissues were found in eight cases and vascular tissues in four cases. Most of membranes were hypocellular and composed mainly of collagen matrix. It is considered that fibrous astrocytes, myofibroblasts, fibroblasts, and vascular structures are involved in the formation of proliferative membranes of ROP, and that later these cells degenerate and disappear, and that finally only collagen matrix remains in the membranes.

When the oculomotor nerve is completely paralyzed, the affected eye shows severe outward displacement and poor cosmetic appearance. Past results of many surgical procedures for oculomotor palsy have been generally unsatisfactory. We tried a new surgical approach experimentally, in which the disinserted lateral rectus muscle was used as an adductor by medial transposition of the muscle. Five adult cats underwent disinsertion of the medial rectus muscle of both eyes to induce iatrogenic medial rectus paralysis. The disinserted medial rectus was removed as far back as possible to prevent reattachment. Then, the right lateral rectus muscle was disinserted and passed beneath the superior rectus muscle and resutured to the sclera 4mm superoposterior to the medial rectus insertion site. After excision of the bilateral medial rectus, a large exotropia of an average 47.6 delta (42.0-55.5 delta) was induced. The medial transposition of the right lateral rectus produced an average 36.6 delta (24.8-45.8 delta) correction of the exotropia. A satisfactory cosmetic result was achieved by this procedure.

A case with typical skin lesions of incontinentia pigmeti showed progressing retinal vascular abnormalities. So cryotherapy was done on avascular peripheral retina, and retinal vascular changes ceased. We propose that cryotherapy may be a good treatment method for progressing retinal vascular lesions of incontinentia pigmenti.

To elucidate the mechanism of increased intraocular pressure in primary open-angle glaucoma (POAG), the protein profiles of aqueous humor obtained from POAG patients were compared with those of cataract patients as a control group. Aqueous humor proteins were analyzed by sodium dodecylsulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and detected by the ultrasensitive silver staining technique. In 79% of the samples taken from POAG patients, protein bands of 140,000 or 160,000 daltons were stained, but none were stained from cataract patients. The presence of these protein bands revealed statistically significant differences between the two groups. Protein bands of 140,000 or 160,000 daltons were evenly visible at all ages in POAG patients, and the positivity of bands had no correlation with sex or initial intraocular pressure level. It is possible that the ultrastructural changes of the aqueous outflow pathway in POAG may be related to the changes in the aqueous protein, presence of 140,000 or 160,000 daltons protein bands.

The intraocular pressure and the anteroposterior length of the eye are of great clinical importance for the diagnosis and management, before and after surgery, of congenital glaucoma. It is well-known that normal intraocular pressure in children is different from the normal levels in adults. We performed measurements of intraocular pressure and axial length in 141 children who had been admitted for eye problems other than glaucoma. The intraocular pressures were measured with the Perkins hand-held applanation tonometer at the beginning of general anesthesia. Simultaneously, A-scan ultra-sound measurements of the axial lengths of the eyes were made. In 10 children under the age of two years, the intraocular pressure was 11.85 +/- 1.35 mmHg. In 79 children from two to seven years, the intraocular pressure was 12.80 +/- 1.73 mmHg. In 52 children from seven to 15 years, the intraocular pressure was 13.31 +/- 1.79 mmHg. The axial lengths of the eyes in children under the age of two years, from two to seven years, and from seven to 15 years, were 21.31 +/- 0.97 mm, 22.04 +/- 0.92 mm, and 23.22 +/- 1.00 mm, respectively. These results were considered to be guidelines for measuring intraocular pressure and axial length in children suspected of having congenital glaucoma. The differences of intraocular pressures stated by other authors are due to early measurement of the intraocular pressure at the beginning of general anesthesia.

Separation of a variety of purine bases, which include 7-methyl derivatives, was studied by using polyethyleneimine-coated silicagel which bound hypoxanthine, cytosine or guanine moieties. The separation behavior seems to be related to the interaction of imidazole part of purine derivatives with the resins through hydrogen bonding.

Eleven patients with Korean (epidemic) hemorrhagic fever were each studied three times with high-resolution CT in order to demonstrate necrosis of the pituitary gland and to correlate the CT findings with the patients' pituitary function and visual fields. Seven of the 11 patients showed varying degrees of progressive decrease in the height of the pituitary gland: one severe, two moderate, and four mild. The visual fields of all the patients were checked at the time of the third (last) follow-up CT. Six of the 11 patients had bitemporal superior quadrantanopsia. In five patients, the decreased height (atrophic change) of the pituitary gland and the visual-field defect were coincidental. The visual-field defects in those patients were not improved on follow-up examination 5 weeks later. Two patients in whom a 1-year follow-up examination was performed showed no interval changes in the defects. Pituitary function tests were performed in nine of the 11 patients (six with atrophic pituitary glands and three without atrophic changes) at the time of the third CT. Five of the six patients with atrophy showed decreased pituitary reserve function for follicle-stimulating hormone, cortisol, or human growth hormone, while only one patient showed decreased reserve function for cortisol among the three patients without atrophic change. The pituitary atrophic changes observed on follow-up sellar CT are thought to be the result of the ischemic necrosis of the gland. The high probability (five of seven) of visual-field defects in those patients with atrophic glands suggests optochiasmatic and pituitary ischemia as the basic pathogenesis.

An electrophoretic variant of red cell carbonic anhydrase I, designated CA I Hiroshima-1, has been observed in 12 apparently unrelated individuals during a survey of 13,019 individuals from the cities of Hiroshima and Nagasaki, Japan. Analyses of tryptic and chymotryptic peptide patterns of this CA I variant purified from 8 of the 12 individuals revealed the same altered peptides in each case. Examination of the amino acid sequence of an altered tryptic peptide purified from one of the variants showed that the aspartic acid residue at position 86 was replaced by a glycine residue. Thermostability studies demonstrated that all samples of CA I Hiroshima-1 were less stable than normal CA I. The specific esterase (p-nitrophenyl acetate) activities of the normal and variant CA I isozymes were essentially the same. The difference spectra of the normal and variant enzymes were essentially the same. The isoelectric focusing patterns of CA I Hiroshima-1 showed a different pattern of minor bands to those produced by normal CA I. The relative amounts of the normal and variant enzymes purified from single heterozygous individuals were similar.

The morphologic classification and clinical spectrum of 1,001 cases of primary glomerulonephritis (GN) in Jinling Hospital during 1980-1988 were analysed. It was found that the incidence of mesangial type lesion including mesangial proliferative GN, IgA nephropathy and IgM nephropathy ranks first (21.5%, 32.1% and 10.1% respectively), which is not only the commonest cause of patients clinically manifesting nephrotic syndrome and gross hematuria but also the predominant type of nephritis causing uremia and hypertension.

A new inherited variant of carbonic anhydrase I (CA I), designated CA INagasaki 1 (CA INGS 1), was discovered during a survey of hemolysates from 5852 individuals from the cities of Hiroshima and Nagasaki in Japan. Analysis of the amino acid composition of a tryptic peptide from the CA INGS 1 variant indicated that a glutaminyl residue was substituted for an arginyl residue at position 76. Heat degradation studies showed that the CA INGS 1 variant was less stable than normal CA I. The CO2 hydrase and esterase activities of the normal and variant carbonic anhydrases I, as well as the relative amounts of the two enzymes in heterozygotes, were similar.

Carbonic anhydrase II (CA II) generates the H+ required for osteoclast-mediated bone resorption in humans. We have developed the human promyelocytic cell line HL-60 as a model system with which to study the osteoclast-specific expression of the CA II gene. Treatment of the cell line with 1,25-dihydroxyvitamin D3 resulted in a dramatic de novo induction of CA II at both the protein and mRNA levels. CA II mRNA was also induced to a lesser extent by 12-O-tetradecanoyl phorbol 13-acetate. Treatment with dimethyl sulfoxide did not increase CA II mRNA. These findings indicate that the HL-60 cell line will be a useful model system to study the osteoclast-specific expression of the CA II gene.