Surgical repair of the isolated incomplete median cleft lip.

Median cleft lip refers to a vertical cleft on the midline of the upper lip. It is a rare congenital craniofacial anomaly brought about by a fusion failure in the medial nasal prominence. A novel surgical approach to median cleft lips and their repair is reported herein, with reference to a clinical case. The patient had a cleft in the lower half of the upper lip. There were no other craniofacial anomalies in this patient other than the cleft. Within the framework of the patient's surgical treatment, a functional and cosmetically satisfactory result was achieved by performing a V-Y advancement flap on the columella base, Z-plasty in the vermillion zone, and Z pattern muscular tissue repair, without having to resort to any tissue excisions.

Effects of tamoxifen on survival of cutaneous and myocutaneous flap (experimental study).

OBJECTIVES: Breast cancer is the most common non-skin malignancy in women. In breast cancer, the basic principle of endocrine therapy is to deprive tumor cell from the growth-stimulating effect of estrogens. The oldest, best-known and most widely used endocrine therapy medicine is tamoxifen, which is a selective estrogen receptor blocker. All factors that are known to have adverse effects on flap and pre-reconstruction flap survive should be eliminated 3 weeks before the surgery and started at the end of the 3rd week after the surgery. When the literature is reviewed, there are limited studies about that tamoxifen increases the risk of deep vein thrombosis and pulmonary embolism. The aim of this study was to research whether tamoxifen had adverse effects on the skin flap and muscle-skin flap survive. MATERIALS AND METHODS: In this study, 32 female Sprague-Davley rats were used. Their weights ranged from 220 to 250 g. 4 groups, each consisting of 8 rats, were formed. In this experimental study, in which rat models were used caudal based rat dorsal skin flap and superior pedicle rectus abdominis musculocutaneous flaps were applied. Control groups were formed for both flap models. Study groups were treated with tamoxifen citrat and control groups were treated with placebo. RESULTS: As a result of the statistical evaluation done by measuring the surviving flap areas, it was found out that tamoxifen had no negative effect on flap survive. CONCLUTIONS: Based on these findings, it was concluded that there was no need to stop tamoxifen as long as 6-7 weeks in patients undergoing breast reconstruction with pedicle flap techniques.

Trabeculectomy combined with deep sclerectomy and scleral flap suture tension adjustment under an anterior chamber maintainer: a new modification of trabeculectomy.

The objective of this study was to investigate the efficacy and safety of trabeculectomy combined with deep sclerectomy in patients with primary open angled glaucoma (POAG) and to compare the results with those from conventional trabeculectomy. In the study 12 eyes of 10 patients operated with trabeculectomy combined with deep sclerectomy (Group I) and 16 eyes of 16 patients operated with conventional trabeculectomy surgery (Group II) were included. In the described form of combined deep sclerectomy with the trabeculectomy technique the superficial scleral flap was sutured with 10/0 monofilament, the number and tension of sutures were adjusted according to the outflow dynamics, under the scleral flap, of balanced salt solution (BSS) provided by an anterior chamber maintainer. In groups I and II all the patients were male POAG cases with mean ages of 60.0 +/- 19.4 and 67.0 +/- 7.1 years, respectively. The mean follow-up periods were 8.3 +/- 3.5 months for group I and 16.6 +/- 7.0 months for group II. Preoperative mean IOP were 29.7 +/- 8.3 and 29.1 +/- 12.8 mmHg mmHg, and average topical antiglaucomatous medications were 2.9 +/- 0.7 and 2.7 +/- 1.1 for groups I and II, respectively. Postoperatively mean IOP measurements were reduced in groups I and II to 10.5 +/- 2.9 vs. 9.6 +/- 4.8 mmHg at 1 week, 13.7 +/- 5.6 vs. 16.0 +/- 9.8 at 1st month, 12.3 +/- 6.4 vs. 17.3 +/- 8.0 at 3rd month, 11.0 +/- 4.1 vs. 15.3 +/- 5.8 at 6th month, 10.8 +/- 1.6 vs. 16.1 +/- 4.4 at 12th month, and 11.0 +/- 1.0 vs. 16.7 +/- 5.0 at 18th month. Statistical analysis revealed that mean postoperative IOP measurements for group I were significantly lower than for group II for all measurements except the first week (P < 0.05). At 12th month, the complete (IOP <22 mmHg without medication) and qualified (IOP <22 mmHg with medication) success rates were 83.3 and 100% for group I and 63.6 and 90.9% for group II. Postoperatively at 12th month, the mean number of antiglaucoma medications had fallen to 0.0 +/- 0.0 in group I and 0.81 +/- 1.1 in group II. Trabeculectomy surgery combined with deep sclerectomy and suture adjustment under an anterior chamber maintainer provided sufficient IOP decrease and diffuse bleb morphology. This technique could be used as a safe method for management of glaucoma.

The effect of recombinant human hyaluronidase on dexamethasone penetration into the posterior segment of the eye after sub-Tenon's injection.

PURPOSE: The aim of this study was to investigate the extent if recombinant human hyaluronidase (rhuPH20) can enhance trans-scleral penetration of sub-Tenon's dexamethasone (DM) into the posterior segment of the eye. METHODS: rhuPH20 was purified from conditioned media through a series of ion exchange, hydrophobic interaction, aminophenylboronate, and hydroxyapatite chromatography to greater than 90% purity based upon specific activity. Only the right eye of each rabbit was injected. The first group (n = 16) received an injection of DM and rhuPH20, whereas the second group (n = 16) received DM only. The eyes were enucleated 1, 2, 3, and 6 h after the injection, and the choroid, retina, vitreous, aqueous, and serum were harvested. DM concentration was assessed by mass spectrometry. Histology (n = 2) and immunohistochemistry (n = 2) was performed to detect toxicity and the presence of the rHuPH20, respectively. RESULTS: We observed no histopathologic damage to ocular tissues after sub-Tenon's injection. This enzyme significantly increased DM level in the choroid and the retina 3 h after administration. The rise in levels was transient returning to normal levels by 6 h. CONCLUSIONS: Sub-Tenon's coinjection of rHuPH20 with DM resulted in a general increase in DM levels in ocular tissues and the serum, with significant increase in the choroid and the retina, 3 h after administration.

Therapy with ribozyme to the proliferating cell nuclear antigen-ribozyme and 5-fluorouracil of experimental choroidal neovascularization in rats.

PURPOSE: The aim of this study was to evaluate the efficacy of hammerhead ribozyme to the proliferating cell nuclear antigen (PCNA-Rz) and 5-fluorouracil (5-FU) in experimental choroidal neovascularization (CNV) model in rats. METHODS: Laser was used to induce CNV in each eye of 44 rats. For angiography studies, injections of either a mixture of PCNA-Rz 10 microg/microL and 5-FU 1.5 microg/microL, versus the same dose of either drug alone versus a control injection of Hanks' Balanced Salt Solution (HBSS) were performed. We also studied this regimen to evaluate scar size and volume. RESULTS: There was significantly less angiographic leakage for the treated eyes compared to the controls by 3.53 grading points (P = 0.0005); CNV leakage was reduced in the combination group compared to 5-FU alone by 1.75 grading units (P = 0.04) and compared to PCNARz by 2.22 grading units (P = 0.07). The scar size and volume were smaller (diameter 354.6 +/- 174.2 microm vs 477.3 +/- 157.0 microm), (thickness 52.7 +/- 43.0 microm versus 79.6 +/- 46.2 microm) with a reduction in scar volume of 44.8%. CONCLUSIONS: Subretinal injection of PCNA-Rz and 5-FU mixture is more effective as treatment of laser-induced CNV, than either drug alone. The majority of the antiangiogenic effect is a result of 5-FU activity with a contribution by the PCNA ribozyme.

Characteristic features of the genotype and phenotype of hereditary spherocytosis in the Japanese population.

Hereditary spherocytosis (HS) is the most common hemolytic anemia of congenital origin in the Japanese population. Among 844 cases of 520 kindred with congenital red cell membrane disorders studied at the Kawasaki Medical School in the last 25 years (1975-1999), 407 cases (48.2%) of 215 kindred had HS. Among the recent 60 kindred with HS, autosomal dominant (AD) transmission was proven in 19. The remaining 41 non-AD HS included 1) homozygous patients with autosomal recessive inheritance, 2) HS patients with de novo gene mutations, and 3) mild HS with AD inheritance. The extent of clinical severity in the non-AD HS cases was nearly identical to that in the AD cases. The incidence of membrane protein abnormalities in our 60 Japanese HS kindred was unique: there were lower ankyrin deficiencies (7%), moderate band 3 deficiencies (20%), and much higher protein 4.2 deficiencies (45%), with 28% of unknown etiology. The incidence of membrane protein deficiencies corresponded to that determined by gene analyses; i.e., mutations mostly in band 3 and/or in protein 4.2 genes and fewer ankyrin gene mutations. In the band 3 gene, 11 mutations pathognomonic for HS were identified (3 frameshift and 8 missense mutations). There were 5 mutations of the protein 4.2 gene (3 missense mutations, 1 nonsense mutation, and 1 splicing mutation) pathognomonic for HS. On the other hand, 2 missense mutations were detected in the ankyrin gene in this study. The genetic abnormalities in our HS patients correlated well with the phenotypic ultrastructural abnormalities of red cell membranes in situ. Ankyrin mutations (ankyrin Marburg and ankyrin Stuttgart with frameshift mutations) were associated mostly with a disrupted cytoskeletal network, and band 3 mutations (band 3 Kagoshima with frameshift mutation) typically demonstrated anomalies of intramembrane particles (IMPs). Protein 4.2 mutations (homozygotes of protein 4.2 Nippon) with complete protein 4.2 deficiency showed abnormalities of both the cytoskeletal network and IMPs.

Ankyrin-1 mutations are a major cause of dominant and recessive hereditary spherocytosis.

Hereditary spherocytosis (HS) is the most common inherited haemolytic anaemia in Northern Europeans. The primary molecular defects reside in the red blood cell (RBC) membrane, particularly in proteins that link the membrane skeleton to the overlying lipid bilayer and its integral membrane constituents. Ankyrin-1 is the predominant linker molecule. It attaches spectrin, the major skeletal protein, to the cytoplasmic domain of band 3, the RBC anion exchanger. Two-thirds of patients with HS have combined spectrin and ankyrin-1 deficiency; deficiency of band 3 occurs in about 15 to 20% (ref.1). These data suggest that ankyrin-1 or band 3 defects may be common in HS. To test this we screened all 42 coding exons plus the 5' untranslated/promoter region of ankyrin-1 and the 19 coding exons of band 3 in 46 HS families. Twelve ankyrin-1 mutations and five band 3 mutations were identified. Missense mutations and a mutation in the putative ankyrin-1 promoter were common in recessive HS. In contrast, ankyrin-1 and band 3 frameshift and nonsense null mutations prevailed in dominant HS. Increased accumulation of the normal protein product partially compensated for the ankyrin-1 or band 3 defects in some of these null mutations. Our findings indicate that ankyrin-1 mutations are a major cause of dominant and recessive HS (approximately 35 to 65%), that band 3 mutations are less common (approximately 15 to 25%), and that the severity of HS is modified by factors other than the primary gene defect.

Acute systolic hypertension after acute myocardial infarction: prognostic and therapeutic significance.

In this study, the incidence of acute systolic hypertension (ASH) after acute myocardial infarction and the effects of this complication on the clinical course and prognosis of the disease were studied retrospectively in 950 cases. ASH was characterized as an elevation of systolic blood pressure over 170 mmHg for at least 30 minutes in a previously normotensive subject. There were 50 cases with ASH (5.3%) and 370 (38.9%) with normal blood pressures among the studied 950. The incidences of acute left ventricular failure and of arrhythmias such as sinus tachycardia, atrial and ventricular premature beats and ventricular tachycardia were significantly higher in the group with ASH in comparison with those with normal blood pressures. The group with ASH had also a greater average number of recurrent ventricular fibrillation attacks. The peak SGOT levels were found to be significantly increased in cases in whom the ASH sustained for more than 12 hours. The results suggested that the ASH which occurred predominantly in the early period of acute myocardial infarction was associated with more extensive myocardial damage resulting in a poor clinical course complicated by frequent ventricular arrhythmias and left ventricular failure. It is our conclusion that ASH after myocardial infraction is associated with relatively poor prognosis.

Prognostic significance of isolated left anterior hemiblock and left axis deviation in the course of acute myocardial infarction.

In 700 patients with acute myocardial infarction admitted to the intensive coronary care unit of our hospital, the incidence and significance of left anterior hemiblock and left axis deviation has been studied in the acute phase of disease. In 102 (14.6%) of the 700 patients, isolated left axis deviation (mean QRS axis-45 degrees) was found and 69 of them (9.9%) met the criteria of left anterior hemiblock. Of the 69 patients with left anterior hemiblock, 61 had acute anterior myocardial infarction, 5 had inferior infarction, and 3 had subendocardial infarction. The anterior hemiblock was transient in 5 patients, but persisted in 64. All patients with and without isolated left anterior hemiblock and left axis deviation were compared statistically with reference to mortality rate and the incidence of arrythmias; no significant difference was noted. However, in patients over the age of 65 and also in those with hypertension, the incidence of left axis deviation was significantly higher (P less than 0.05 and P less than 0.001, respectively). It was concluded that isolated left anterior hemiblock and left axis deviation occurring in the course of acute myocardial infarction no influence on the prognosis of acute myocardial infarction.