Crohn''s disease in infancy: a case report

We present a patient with pure alexia following a hemorrhagic infarction in the left fusiform gyrus. The symptom began with alexia preferentially disturbed for kana, but during the course of recovery slight agraphia for kanji became pronounced. In the earlier phase, alexia was more severe than agraphia and he could write kanji that he could not read. Furthermore, kinesthetic reading was effective. These findings are consistent with the symptoms of pure alexia, although the fact that a writing disturbance for kanji persisted resembles the profile of alexia with agraphia for kanji due to a posterior inferior temporal lesion. Based on the fact that the posterior inferior temporal lesion showed more severe agraphic symptom and more frequent nonresponse writing errors of kanji, and that our patient's lesion was mainly located in the fusiform gyrus that is medial to the inferior temporal gyrus, we believe that alexia occurred when the inferior temporal gyrus was disconnected from the fusiform gyrus, as a result, visual information could not reach the inferior temporal gyrus in which the visual images of individual kanji are stored.     

Duodenal involvement of Crohn''s disease: three different clinicopathologic patterns

Autosomal dominant polycystic kidney disease (ADPKD) describes a group of at least three genetically distinct disorders with almost identical clinical features that collectively affects 1:1,000 of the population. Affected individuals typically develop large cystic kidneys and approximately one half develop end-stage renal disease by their seventh decade. It has been suggested that the diseases result from defects in interactive factors involved in a common pathway. The recent discovery of the genes for the two most common forms of ADPKD has provided an opportunity to test this hypothesis. We describe a previously unrecognized coiled-coil domain within the C terminus of the PKD1 gene product, polycystin, and demonstrate that it binds specifically to the C terminus of PKD2. Homotypic interactions involving the C terminus of each are also demonstrated. We show that naturally occurring pathogenic mutations of PKD1 and PKD2 disrupt their associations. We have characterized the structural basis of their heterotypic interactions by deletional and site-specific mutagenesis. Our data suggest that PKD1 and PKD2 associate physically in vivo and may be partners of a common signalling cascade involved in tubular morphogenesis.     

An unusual case of Crohn''s disease in a West Indian child

A method is described for the determination of the intracellular concentration of sodium in individual cells using the electron microprobe analyzer. This method gives an accuracy equal to that obtained by using flame photometry on tissues with large cell populations. Intracellular sodium was precipitated in the cell by a fixative containing pyroantimonate. Cartilaginous needles from shark fins which were equilibrated in saline solutions of differing concentrations were used as biological standards.     

Surgical treatment of Cronkhite-Canada syndrome associated with protein-losing enteropathy: report of a case

PURPOSE: The case of a patient with Cronkhite-Canada syndrome, who developed a protein-losing enteropathy, is reported. METHODS: After localization of the protein-losing region, a right colectomy was performed. RESULTS: Hypoproteinemia and ectodermal changes improved postoperatively. CONCLUSIONS: Surgery is an effective treatment for protein-losing enteropathy in Cronkhite-Canada syndrome. Ectodermal changes improve after correcting malnutrition.     

Surgical treatment of duodenal ulcer with chronic duodenal obstruction

Follow-up treatment results of 892 patients with duodenal ulcer (DU) are presented and the choice of surgical policy in its combination with chronic duodenal obstruction (CDO) in 307 (34.4%) patients is substantiated. Depending on peculiarities of DU, the kind and the stage of CDO, organ saving (140) or stomach resection (167) in combination with correction CDO interventions (287) were carried out. The course of the postoperative period was characterised by basic morphofunctional changes and by achievement of completeness of duodenal passage. Intermittent motor-evacuatory disorders were observed in 55 (17.9%) patients. Lethal outcome was in 1 case. Long-term functional results of the operations were studied in 267 patients. Excellent and good results were obtained in 66.3%, favorable-in 32.24% and unfavorable-in 1.44% of patients. The dependence of immediate and remote results of the operations on the effectiveness of the correction of associated CDO was established.     

Surgical treatment of spontaneous rupture of the esophagus: case report

OBJECTIVE: To explore the effect of the Xingbei Granule (XBG) in treating bronchial asthma and its mechanism. METHODS: Systematic clinical observation and animal experimental study on the XBG were carried out. RESULTS: The XBG not only could reduce the symptom of asthma, improve the ventilatory function effectively, but also has the effect of antagonizing the change of asthmatic inflammatory medium, alleviating the air-way's inflammatory reaction and lowering the air-way's hyperreactivity. CONCLUSION: XBG do attain the dual goal of controlling the asthmatic symptoms and reducing the asthmatic attack.     

Iatrogenic perforation of perivaterian duodenal diverticulum: report of a case

The author reports a case of iatrogenic perforation of a duodenal diverticulum, an extremely rare occurrence, during percutaneous radiologic extraction of a retained common-bile-duct-stone. Perforation was related to the perivaterian location of the duodenal diverticulum. Because an inflammatory reactions was present, tube duodenostomy was chosen over excision, closure and drainage to prevent the complication of lateral duodenal fistula and sepsis. Whenever iatrogenic duodenal perforation is suspected, prompt radiologic documentation and early surgical consultation should be sought.     

Tuberculous duodenal obstruction--a case report

Proximal duodenal obstruction due to tuberculosis can masquarade as duodenal ulcer. Although commonest cause of duodenal obstruction is ulcer, other causes must be considered, particularly tuberculosis which is common in tropics.     

Multiple organ malrotation syndrome with duodenal atresia: a case report

Allele frequency of D17S855 microsatellite locus, an intragenic polymorphic cytosine-adenine repeat located in the BRCA1 gene, was analyzed in 456 normal tissues of Japanese adults. The frequency of D17S855 microsatellite polymorphism was 77.9% (335/456). The size of D17S855 PCR fragments ranged from 137 to 157 bp. The most frequent allele was 151 bp (29.9%) followed by 149-bp (19.3%), 145-bp (17.5%), and 147-bp (17.0%) fragments. The observed allele distribution of this microsatellite was similar to that of Southern Swedes, while it was quite different from that of European Caucasian specimens deposited in the Genome Database.     

Total colectomy for Crohn''s disease

BACKGROUND: Hypertrophy of the internal anal sphincter may be apparent in some children, but its significance has not yet been determined. OBJECTIVE: To assess anal endosonographic findings in children with chronic constipation. Materials and methods. We performed anal endosonography in 46 children with chronic constipation and compared the results with values considered normal. RESULTS: We did not find a significant relationship between age and thickness of the internal or external anal sphincters. The clinical response to medical management did not differ between patients with or without sphincter hypertrophy. CONCLUSIONS: Although we did not find a significant correlation between sphincter hypertrophy and constipation or age, further studies may clarify its place amongst other techniques which are used in the investigation of anorectal pathology.     

Solitary bone cyst with epiphyseal involvement: a case report

A large solitary bone cyst involving the epiphysis produced 5.0 cm shortening of the proximal humerus in a 15-year-old girl. Biopsy revealed the cyst was filled with thin yellow fluid under pressure and lined by a fibrous membrane. X-rays 9 months after biopsy demonstrated progressive cyst obliteration. A review of literature revealed no similar case. It is postulated that solitary cysts rarely may involve the physeal and epiphyseal structures and thereby retard longitudinal bone growth.     

Muscular involvement in Beh?et's disease: case report and review of the literature

Acute necrotizing myositis is described in a 22-yr-old man with clinically diagnosed Beh?et's disease. Light microscopic examination revealed a prominently granulocytic-monocytic infiltration of the muscle with severe necrosis. An infectious (bacterial, fungal or parasitic) etiology could be excluded by specific staining techniques and by immunohistochemistry. Vascular deposition of immune complexes was detected by direct immunofluorescence. Electron microscopy revealed severe structural damage and phagocytosis of muscle fibers. In the endomysium, leukocytes and occasional erythrocytes were found. Virus-like particles were not seen. The relevant literature on muscular involvement in Beh?et's disease is reviewed. It is suggested that two different stages of inflammation occur in Beh?et's disease. In the acute stage it presents as a granulocytic-monocytic necrotizing reaction developing from a neutrophil-mediated vasculitis. In the later phase lymphocytic infiltrations predominate. Despite the rare involvement of muscles the diagnosis of Beh?et's disease should be considered particularly in younger patients presenting with muscular symptoms like pain and swelling pre-dominantly of the lower extremities.     

Surgical treatment of penile cancer: a follow-up report

One hundred and fifteen patients with penile cancer were treated at Roswell Park Memorial Institute from 1952 to 1975. A full follow-up is reported and factors involving the prognosis are analyzed. Although the clinical error of staging was near 30%, the lymphography results suggest that this study may result in added improvement in preoperative staging. Early diagnosis, adequate surgical resection, and lymph node dissection will improve the survival significantly. When dealing with an individual patient the prognosis is poor when any one or more of the follow factors are present: the lesion involves the shaft; there is a positive margin at the surgical resection; total penectomy is necessary to obtain tumor-free margin; lymphography is positive for tumor involvement of lymph nodes; lymph node dissection has not been performed; positive lymph nodes are found on surgical exploration. The good prognostic factors include: a lesion confined to the glans and partial penectomy is sufficient to obtain a tumor-free margin of resection; no clinical evidence (including lymphography) of lymph node invovlement; performance of lymph node dissection. The histological grading of the tumor should not influence the clinical decision for treatment in our opinion at this time, based on our current results. Further prospective studies of different factors involved in etiology, diagnosis, and treatment of penile cancer are suggested.     

Surgical treatment of Peyronie's disease: a new approach

We treated 12 patients with intractable Peyronie's disease with a new approach based on simple incision of the fibrotic plaque(s) and stenting of the corpora with penile implants. Infection in 1 case necessitated removal of the prostheses, while the remaining 11 patients had satisfactory functional and anatomical results. The procedure is indicated for patients with 1) Peyronie's disease and impotency, 2) normal potency and extensive disease and 3) normal potency and localized disease in selective cases. In this series insertion of the penile prostheses did not change sexual prowess in previously potent patients.     

Surgical treatment of the primary cardiac rhabdomyoma in the neonate--a case report

A successful surgical treatment of intracardiac rhabdomyoma in a 12-day-old female is reported. The neonate was first noted to have a cardiac murmur and she was diagnosed cardiac tumor by two dimensional echocardiography. It showed multiple cardiac tumors which occupied the right ventricular outflow tract and interventricular septum. Under the routine cardiopulmonary bypass the tumor of the right ventricular outflow tract was removed. Histological examination revealed a rhabdomyoma. Follow-up evaluation 7 years after operation showed normal growth and development of the patient. A repeated two dimensional echocardiogram revealed no sign of recurrence and the left ventricular cavity was not obstructed by the residual tumor in the interventricular septum. We reviewed 10 cases treated surgically in infancy including three neonate in Japan.     

Pathogenesis of Crohn''s disease

There remains a great deal to be accomplished in freeing many millions of gays and lesbian from the tyranny of fear of discovery, of actual and potential economic disenfranchisement, of the burden of ridicule, shame, and scorn, and of penalties for alleged criminal behavior. Nevertheless, the recognized status, for example, of openly gay and lesbian psychologists ... is light years away from their inferior and almost certainly closed status of 1954. They are not only free now of the criminal penalties and of the stigma of mental illness, but are in positions of trust, respect, and power.