Jarcho-Levin Syndrome Associated with Atrial Septal Defect and Partial Anomalous Pulmonary Venous Return: A Case Report

A bstract A 61-year-old woman suffering from Jarcho-Levin syndrome (JLS) was associated with atrial septal defect and partial anomalous pulmonary venous return and underwent corrective surgery. Pressure controlled postoperative ventilator therapy is preferred in patients with JLS.     

Experimental autoimmune uveoretinitis (EAU) in rats: isolation of S-antigen, EAU susceptibility of rat strains, genetic control of EAU induction, and effects of cyclophosphamide and irradiation on EAU

Highly purified S-antigen was isolated from bovine retinas by high performance liquid chromatography (HPLC), and was used to induce experimental autoimmune uveoretinitis (EAU) in various rat strains. Studies were then made of the genetic control of EAU, the effects of cyclophosphamide or irradiation on EAU, and the correlation between the EAU incidence and the serum levels of antibody to S-antigen. Lewis rats were the most susceptible to EAU followed by Wistar rats. F344 rats and BN rats were resistant to EAU. (Lewis X BN)F1 rats and (LBNF1 X Lewis) rats were susceptible to EAU, while (LBNF1 X BN) rats were resistant. These results indicate that susceptibility to EAU was inherited as an autosomal dominant trait. Treatment of rats with cyclophosphamide or irradiation (200 rad/rat) on the day before immunization markedly suppressed EAU development. On the other hand, the same dose of irradiation 7 days after the immunization did not affect the disease induction, yet the antibody levels to S-antigen were very high in the rats. In addition, BN rats resistant to EAU exhibited very high levels of antibody to S-antigen. Therefore, the antibody to S-antigen seems to play a minor role, if any, in the immunopathogenic mechanisms of EAU.     

Preferential inhibitions of hepatic P450IA2 expression and induction by lead nitrate in the rat

Male F344 rats were treated with lead nitrate and changes inthe expression and induction of P450IA subfamily enzymes anda placental form of glutathione-S-transferase (GST-P) in theliver were assessed by means of a bacterial mutation test, immunoblottingwith a monoclonal antibody reactive to P450IA1/IA2 and anti-GST-Psera and Northern blotting with P450IA2 cDNA as a probe. Treatmentof rats with lead nitrate (20, 50 or 100 µmol/kg bodywt) decreased P450IA2 mRNA and protein in the liver in the dose-dependentfashion and also decreased the microsomal activity for P450IA2-dependentmutagenization of aromatic amines. Pretreatment of rats withlead nitrate suppressed the inductions of both P450IA2 mRNAand protein by an inducer of P450IA subfamily enzymes in theliver. In addition, amount of the induced P450IA2 was decreasedalong with increase in that of the induced GST-P.     

Urinary dysfunction and autonomic control in amyloid neuropathy

Abstract Uro-neurological assessment was performed in four patients with small-fiber neuropathy due to amyloidosis (2 transthyretin-type/2 immunoglobulin light-chain-type). Voiding difficulties were due to detrusor weakness and impaired bladder sensation. In two patients cholinesterase inhibition treatment caused urge incontinence, indicating detrusor denervation supersensitivity. The underlying mechanisms of urinary dysfunction seem to involve postganglionic cholinergic and afferent somatic nerves.     

Pathogenesis of Mucocutaneous Lesions in Behçet's Disease

Behcet's disease (BD) is characterized by recurrent oral aphthae, skin lesions, eye lesions, and genital ulceration. To determine the pathogenesis of BD, we performed histological and immunohistochemical studies of these mucocutaneous lesions, an assay of neutrophil activity, and HLA typing. Dense dermal or subcutaneous infiltrations of polymorphonuclear cells (PMN) without leukocytoclastic vasculitis were found in 28 of 57 lesions. Immunohistochemically, deposits of C3 on the vessels were found in 12 of 31 lesions. Deposits of immunoglobulin were not found except for one of IgM. C3 deposits and PMN infiltrations were significantly related (p<0.05). PMN activity by polarization was enhanced; however, the results did not show a significant relationship with the PMN infiltrations or the C3 deposits. The incidence of HLA-B51 was significantly high in BD, but no significant relationship was found between HLA-B51 and the results of other examinations. These results suggest that the pathogenesis of BD lesions differs from that of collagen diseases and that C3 deposits on the vessels may play an important role in the development of mucocutaneous lesions where PMN have mainly infiltrated.     

Experimental study of single and combined coaxial exposure by high-peaked pulse wave CO2 and Nd:YAG laser on the brain: effect on the brain microvasculature in the subacute stage

The effect of single or combined coaxial exposure by Nd: YAG and CO2 (pulse wave) laser on the brain at the subacute stage was examined in experimental animals. Soft X-ray microangiography and histological examination of the brain were performed 48 hours after laser exposure. The lasers in this study were pulse wave form CO2 of 2, 4 and 8 watts and YAG lasers of 10, 20 and 40 watts, employed separately or simultaneously using 130 YZ of Nihon Infrared Industries Company. Japanese white rabbits were anesthetized with pentobarbital. Bilateral fronto-parietal craniectomy were made, and the dura was removed. After intravenous injection of Evans blue, the lasers were employed to the cerebral cortex using a micromanipulator attached to the operation microscope. The spot size was 0.7 mm in diameter for CO2 laser and 1.2 mm for Nd: YAG laser. Forty-eight hours after exposure, microangiography was performed and brains were prepared for the histological examination. Histological examination and microangiogram of the brain after CO2 laser exposure revealed semilunar avascular area in the edematous layer surrounded with dilated vessels. Histological examination and microangiogram of the brain after Nd: YAG laser exposure revealed broad avascular or oligovascular zones in the surrounding edematous tissue, in which the surviving vessels were narrowed. Edematous zones were also shown in the subcortical portion. The histological examination and microangiogram after combined coaxial exposure of CO2 and Nd: YAG lasers revealed triangular avascular or oligovascular zones in the edematous tissue, in which the surviving vessels were narrowed.(ABSTRACT TRUNCATED AT 250 WORDS)     

Effects of pulsatile cardiopulmonary bypass on the renin-angiotensin-aldosterone system following open heart surgery

The effects of pulsatile cardiopulmonary bypass on the renin-angiotensin-aldosterone system and tissue metabolism, especially those which occur soon after surgery, were studied in 26 patients who required total cardiopulmonary bypass for longer than 60 minutes. These patients comprised 11 who underwent open heart surgery utilizing nonpulsatile cardiopulmonary bypass (Group I) and 15 who underwent open heart surgery utilizing pulsatile cardiopulmonary bypass (Group II). Plasma angiotensin II and serum aldosterene levels were significantly increased one and 5 hours postoperatively in Group I when compared with the preoperative values, whereas no significant elevations were observed in Group II. Plasma angiotensin II and serum aldosterone levels one hour postoperatively in Group II were significantly lower than those in Group I. Lactate levels in the arterial blood were significantly elevated, one and 5 hours postoperatively in both Groups I and II. Moreover, no significant difference was observed in the lactate levels between Groups I and II, one hour postoperatively. In the nonpulsatile group (Group I), plasma angiotensin II levels one hour postoperatively were correlated significantly with the duration of total cardiopulmonary bypass. In conclusion, pulsatile cardiopulmonary bypass offers significant advantages in terms of lower plasma angiotensin II and serum aldosterone levels, when compared with nonpulsatile cardiopulmonary bypass soon after open heart surgery requiring total cardiopulmonary bypass for longer than 60 minutes, however, it does not offer a definite advantage for tissue metabolism.     

An experience with augmentation sigmoid cystoplasty for urinary incontinence caused by sacral agenesis: a case report]

Sacral agenesis is an uncommon disease. About 50 cases have been reported in Japan since 1929. Neurogenic bladder is often accompanied with the disease. The patient was a 26-year-old man who had suffered from persistent urinary incontinence since his childhood. Kidney-ureter-bladder (KUB) revealed Type IV sacral agenesis according to the classification by Renshaw. The upper urinary tract remained normal. Urodynamics study showed a low compliance bladder with low urethral pressure. Pharmacotherapy failed to improve his continence. Augmentation sigmoid-cystoplasty was undertaken to enlarge vesical capacity and it has successfully overcome his urinary incontinence. Clinical aspects of sacral agenesis are discussed focusing on urological problems.