Human histamine N-methyltransferase pharmacogenetics: cloning and expression of kidney cDNA.

Histamine N-methyltransferase (HNMT) catalyzes the NT-methylation of histamine. The level of HNMT activity in human red blood cells is controlled by a common genetic polymorphism. We set out to clone and express a cDNA for HNMT from human tissue as a first step toward a determination of the molecular basis for this genetic polymorphism.

Long-term results of radical esophagogastrectomy for bleeding varices due to unshuntable extrahepatic portal hypertension.

This report describes the long-term results of one-stage total gastrectomy and distal two-thirds esophagectomy, with reconstruction by esophagojejunostomy (16 Roux-en-Y; 2 interposition), in 18 adult patients with recurrent variceal hemorrhage due to unshuntable extrahepatic portal hypertension (EHPH) from occlusion of all major tributaries of the portal venous system. The etiology of portal venous occlusion was unknown in 11 patients, abdominal trauma in 3, peritonitis in 3, and thrombotic coagulopathy in 1. Almost half of the patients had their first episode of bleeding in childhood, and 83% experienced bleeding before 40 years of age.

[Mucopolysaccharidosis type I, Hurler-Scheie phenotype with ocular involvement. Clinical and ultrastructural study].

The authors report the case of a 37 year-old male who presented with type I Hurler-Scheie (H-S) mucopolysaccharidosis revealed by ocular complications including bilateral corneal opacification and glaucoma. These complications are identical to those seen in Scheie's mucopolysaccharidosis. The patient underwent a trabeculectomy and a penetrating keratoplasty in both eyes.

Is portal-systemic shunt worthwhile in Child's class C cirrhosis? Long-term results of emergency shunt in 94 patients with bleeding varices.

A prospective evaluation was conducted of 94 unselected patients ("all comers") with biopsy-proven Child's class C cirrhosis (93% alcoholic) and endoscopically proven acutely bleeding esophageal varices who underwent emergency portacaval shunt (EPCS) (85% side-to-side, 15% end-to-side) within 8 hours of initial contact (mean, 6.1 hours) during the past 12 years. Follow-up has been 100% and includes all patients for at least 1 year, and 61 patients (65%) for 5 to 12 years.

Visual improvement after transethmoid-sphenoid decompression in optic nerve injuries.

Transethmoid-sphenoid decompression has been performed on 11 patients with indirect optic nerve injury. Visual improvement occurred in 8 patients, including 4 patients with initial total blindness. Optic neuropathy improved even when there was a long interval, up to 92 days, between trauma and decompression.

Ocular toxoplasmosis in human immunodeficiency virus-infected patients.

The files of 45 human immunodeficiency virus-infected patients with ocular toxoplasmosis were reviewed, with a median follow-up of eight months. The condition was unilateral in 37 of the 45 patients (82%) and was bilateral in eight (18%). Inflammation of the anterior chamber and the vitreous was present in 32 of 53 eyes (60%) and 38 of 53 eyes (72%), respectively.

Treatment of Budd-Chiari syndrome due to inferior vena cava occlusion by combined portal and vena caval decompression.

This study concerns Budd-Chiari syndrome (BCS) caused by occlusion of the subdiaphragmatic inferior vena cava (IVC). It describes the experimental and clinical evaluation of the treatment of this disorder by one-stage combined portal and vena caval decompression with a direct side-to-side portacaval shunt (PCS) and a caval-atrial shunt (CAS) graft. BCS was produced in rats by gradual occlusion of the suprahepatic IVC with an ameroid constrictor.

[X-ray computed tomography in surgical indication of physiological section of the optic nerve. Apropos of 15 cases].

Optic nerve trauma induces loss of vision and absence of direct pupillary light reflex. Persistence of consensual pupillary light reflex excludes anatomical section. Trans ethmoidal-sphenoidal decompression relieves the optic nerve and allows an improvement of visual function.

[Surgical decompression of the optic nerve in intracanal injuries. Indications and results].

Fifteen patients with traumatic optic neuropathy (anatomical section excluded) underwent surgical anterior decompression of the optic nerve, via the trans ethmoid-sphenoid approach. Improvement of visual function assessed by visual acuity and visual field measurements, was obtained in 10 patients. Results remained stable for a follow-up period of at least 6 months.

[Ocular prognosis in Behçet's disease].

The ocular prognosis of Behçet's disease was assessed in 31 French native patients with ocular involvement. The mean follow-up time was 6 years. Ophthalmologic manifestations revealed the disease in 61% of patients, and it was the first symptom in 29%.

Reversal of diabetic somatic neuropathy by whole-pancreas transplantation.

To answer the crucial question regarding reversibility of diabetic somatic neuropathy by whole-pancreas transplantation, metabolic studies and electron microscopic morphometry of the sciatic and testicular nerves were performed monthly for 2 years in three groups of highly inbred rats: (1) NC, 47 nondiabetic controls; (2) DC, 90 untreated alloxan-induced diabetic controls; and (3) DT, 230 diabetic rats given syngeneic pancreaticoduodenal transplants 6, 9, 12, 15, 18, and 21 months after induction of diabetes mellitus (DM). Six diabetic nerve lesions were quantitated by a "blind" protocol: (1) loss of myelinated axons, (2) intraaxonal glycogen deposits, (3) axons with glycogen deposits, (4) demyelinated axons, (5) degenerating axons, and (6) loss of intact axoglial junctions in paranodal terminal myelin loops. In the DT group, testicular nerve specimens were obtained just before transplantation and at death so that each animal served as its own control.

[Corticosteroids and ophthalmology].

Corticosteroids are widely used in the treatment of inflammatory and immunological diseases of the eye. They are given by three routes: topical application, periocular injection and systemic administration. Topical application, periocular injection and systemic administration.

Cotton-wool spots and AIDS related complex.

Twenty-nine patients with the AIDS Related Complex (ARC) were studied retrospectively. Cotton-wool spots (CWSs) were the main ophthalmological finding, present in 9 patients (31%) at this stage of the disease. The CWSs were asymptomatic and were therefore discovered during a routine examination.

[Ophthalmologic manifestations of the Pierre Robin syndrome. Report of a case of microphthalmia].

The classical description of the Pierre Robin syndrome includes micrognathia, glossoptosis, airway obstruction, and usual presence of a cleft palate. The Pierre Robin syndrome is currently defined as the combination of retrognathia, cleft palate, and respiratory distress. This last is mixed, with a peripheral component due to glossoptosis and a central component due to brain stem immaturity.

[Bouchut's tubercles. A clinical and angiographic study].

Four patients (3 young adults and 1 child) with disseminated tuberculosis had choroidal tubercles; none of them was immunodepressed. The clinical and angiographic aspects of these nodules and their evolution are described. Their clinical interest is discussed: fundus examination is always indicated in cases of disseminated tuberculosis and, more generally, in cases of systemic granulomatosis.

[Post-traumatic decompression of the optic nerve. Ophthalmologic and x-ray computed tomographic evaluation. Results in a series of 23 cases].

Post traumatic optic nerve compression gives a Clinic picture of physiological section. CT Scan is consistent with optic canal lesion when considering direct signs (bony fragments compressing the nerve) or indirect (fractures and hematoma of posterior orbital wall, of posterior ethmoid, and sphenoid). 23 patients had a surgical decompression through transethmoidosphenoidal approach.

[Microphthalmos in Pierre Robin syndrome. Clinical and x-ray computed tomographic study].

The Pierre Robin Syndrome is characterized by three defects (8,9): micrognathia, cleft palate and glossoptosis responsible for respiratory failure. The new definition of this syndrome associates retrognathia, cleft palate and respiratory distress. This respiratory distress is mixed: obstructive due to glossoptosis, and central, secondary to brainstem immaturity (1,2).

[Homonymous lateral quadranopsia disclosing cerebral toxoplasmosis in a patient with AIDS].

A case of Homonymous lateral quadranopsia revealing cerebral toxoplasmosis is reported in AIDS patient. Neuro-ophthalmologic manifestations must be explored by neuro-radiologic examination. Nuclear magnetic resonance would rather be performed than CT scan.

Foscarnet in the treatment of cytomegalovirus retinitis in acquired immune deficiency syndrome.

Cytomegalovirus (CMV) retinitis is the major cause of visual loss in acquired immune deficiency syndrome (AIDS). Thirty-one patients with active CMV retinitis were treated with the new antiviral drug, Foscarnet (trisodium phosphonoformate). After a 3-week course of induction therapy, the retinitis improved in 29 of 31 patients (93.

Long term results of treatment of Budd-Chiari syndrome by side to side portacaval shunt.

This is a report of a long term prospective study of 13 seriously ill patients with Budd-Chiari syndrome as a result of occlusion of the hepatic veins who were treated by side to side portacaval shunt from four to 78 weeks after the onset of symptoms and who were under observation for three to 16 years. The patient population was young, ranging in age from 19 to 45 years; seven were men and six were women. The presumed cause was the use of oral contraceptives in three, polycythemia rubra vera in two, Behcet disease in one patient and unknown in seven patients.

[Diffuse infiltrating retinoblastoma].

We report 3 new cases of diffuse infiltrating retinoblastoma, and we review the 21 cases of the literature. Clinical features are typical but must not be confused with uveitis. The average age of onset is about 7 years, later than the usual retinoblastoma.

[Pathologic study of the eye and central nervous system in 25 cases of AIDS].

We have reviewed the clinical and pathologic findings of 25 patients with the acquired immunodeficiency syndrome (AIDS) who had a complete post-mortem examination including a study of the nervous system and of one or both eyes. Cytomegalovirus (CMV) retinitis was the most frequent type of ocular infection observed fundoscopically (9 of 18 cases examined clinically). There were only 3 cases of CMV encephalitis amongst the 8 cases of CMV retinitis documented pathologically.