Idiopathic fibrosing pancreatitis: a rare cause of obstructive jaundice in children

AIMS: To analyse the significance of antibodies to p53 protein as a serological marker for changes in p53 gene expression in patients with hepatocellular carcinoma. METHODS: Thirty eight patients with hepatocellular carcinoma, 19 showing accumulation of p53 protein by immunohistochemistry and 19 having no accumulation, were studied. The presence of anti-p53 was tested using a novel ELISA utilising a recombinant p53 protein as a capture system and verified by western blotting. p53 gene mutations were sought by single strand conformational polymorphism and DNA sequencing analyses. RESULTS: Of 19 patients with p53 protein accumulation in tumour tissue, 10 (52%) had antibodies to p53 in serum by ELISA. Four patients with p53 negative immunohistochemistry also had detectable anti-p53. Western blot analysis confirmed the specificity of the ELISA positive serum samples. The presence of anti-p53 was independent of serum alpha-fetoprotein and was detected in 50% of small tumours while only 8% were alpha-fetoprotein positive. Mutations affecting exons 5 and 6 seem to be more frequently associated with development of anti-p53, than mutations in exons 7 or 8. CONCLUSIONS: The ELISA for anti-p53 is a convenient and specific tet for the detection of humoral response to alterations in p53 gene expression and could be of value in the diagnosis and characterisation of patients with hepatocellular carcinoma.     

A rare cause of dysphonia: laryngeal rhabdomyoma

The authors report their finding concerning the frequency of postoperative pulmonary thromboembolism in an autopsy series of subjects who had died with 15 days of surgery. In all cases the relationship was evaluated between death and age, sex, the underlying pathology and the type of surgery.     

A rare case of severe acute pancreatitis complicated with pancreatic pseudocysts, obstructive jaundice and intraperitoneal hemorrhage

A 58-year-old man visited our hospital because of back pain. Blood examinations revealed the presence of acute inflammation and an increase of pancreatic enzymes. Abdominal computed tomography indicated pseudocysts in the pancreas. The patient was diagnosed as having acute pancreatitis with pseudocysts formation. During the course of the disease, a newly formed pseudocyst in the pancreatic head compressed the common bile duct, leading to the obstructive jaundice. In addition, the rupture of a pseudocyst in the pancreatic tail caused intraperitoneal hemorrhage. This is an interesting case of acute pancreatitis with pseudocysts in which two rare complications developed.     

A case of biliary cystadenoma with obstructive jaundice

Biliary cystadenoma is a rare cause of obstructive jaundice. We report a case of a 78-year-old Japanese man with biliary cystadenoma presenting repetitive abdominal pain and jaundice. Ultrasound sonography revealed a hyperechoic mass in the left lateral lobe of the liver. Histological examination revealed a biliary cystadenoma. Intracystic hemorrhage was assumed to be the cause of obstruction of the bile ducts.     

Surgical treatment of obstructive jaundice

Genetically engineered structural variants of human beta2-microglobulin (beta2m) were produced by sequence exchange with mouse beta2m for the purpose of examining species-specific antigenic determinant expression. For aggregate mapping, mouse and human beta2m, which differ by 30% in their primary sequence of 99 amino acids, were prepared as chimeric (human X mouse) molecules and expressed in the FO-1 beta2m-null human melanoma cell line. A chimera containing residues 1-69 from human beta2m (and residues 70-99 from mouse beta2m) induced expression of the epitopes defined by the anti-beta2m monoclonal antibodies (mAb) BBM.1, NAMB-1, and L368; the reverse chimera did not, although HLA class I heavy chain was evident on the cell surface as determined with the TP25.99 mAb. For fine dissection of the epitopes defined by these mAbs, site-directed mutants of beta2m were prepared by replacement of individual amino acids in human beta2m with the dimorphic residue from mouse beta2m. Substitutions were made at each divergent residue between positions 1 and 66 and, as controls for COOH-terminal modification, a series of residues between positions 75 and 94. Replacement of amino acids 38, 44, and 45, but not 16 other dimorphic residues in the linear stretch from residue 1 to residue 66, resulted in the loss of, or gross reduction in, binding by mAbs BBM.1 and NAMB-1. A reduction in binding was also observed for mAb L368. These data provide strong evidence that the antigenic epitopes defined by these mAb map to a region including S3 and its adjacent intra-beta-strand turn of the three-stranded beta-pleated sheet of beta2m. The mapping of these epitopes is consistent with their accessibility in the assembled major histocompatibility complex class I molecule and indicates that the region from amino acid 38 to 45 is an important structural feature in the "foreignness" of human and mouse beta2m.     

Sarcoidosis presenting as obstructive jaundice

Sarcoidosis is an uncommon disorder characterized by a multi-systemic granulomatous disease of undetermined etiology and pathogenesis. The diagnosis is established by the presence of a compatible clinical illness and by histologic demonstration of noncaseating epithelioid cell granulomas in the affected organs. Accurate diagnosis requires a thorough evaluation to exclude infectious and neoplastic diseases that can mimic sarcoidosis. Although all organs and systems can be affected, the lungs and intrathoracic lymph glands are the most common sites of involvement. We describe an unusual case of extrapulmonary sarcoidosis presenting as obstructive jaundice.     

A rare cause of urinary frequency]

Induction and maintenance of peripheral tolerance are important mechanisms to maintain the balance of the immune system. In addition to the deletion of T cells and their failure to respond in certain circumstances, active suppression mediated by T cells or T-cell factors has been proposed as a mechanism for maintaining peripheral tolerance. However, the inability to isolate and clone regulatory T cells involved in antigen-specific inhibition of immune responses has made it difficult to understand the mechanisms underlying such suppression. Here, we show that chronic activation of both human and murine CD4+ T cells in the presence of interleukin (IL)-10 gives rise to CD4+ T-cell clones with low proliferative capacity, producing high levels of IL-10, low levels of IL-2 and no IL-4. These antigen-specific T-cell clones suppress the proliferation of CD4+ T cells in response to antigen, and prevent colitis induced in SCID mice by pathogenic CD4+CD45RBhigh splenic T cells. Thus IL-10 drives the generation of a CD4+ T-cell subset, designated T regulatory cells 1 (Tr1), which suppresses antigen-specific immune responses and actively downregulates a pathological immune response in vivo.     

Neuralgic amyotrophy: A rare cause of bilateral diaphragmatic paralysis

In 1994, 171 (27%) of all positive blood cultures in our hospital were due to Acinetobacter species. Of these, 138 cultures were considered significant, 91 (66%) were community-acquired and 47 (34%) were nosocomial. Most acinetobacter bacteraemia in children < or = 1 year old was community-acquired, while nosocomial infection was more common in children > 1 year old (P = 0.01). Most children < or = 5 years old were severely malnourished. The incidence of bacteraemia was lowest during the post-monsoon to early winter months. Acinetobacter bacteraemia associated mortality was twice (16%) that of all other patients (7.7%, P < 0.0005) and accounted for 4.5% of all hospital deaths during the study period. Bacteraemia caused by Acinetobacter species is an important cause of morbidity and mortality among our patient population with diarrhoeal disease.     

A rare cause of respiratory failure: Echinococcus of the pulmonary artery

To combine the advantages of the standard technique and the bicaval technique of orthotopic heart transplantation, we use a muscular flap of recipient heart right atrium for connecting the superior vena cava with the donor heart right atrium. The results in respect to the maintenance of atrioventricular valve competence as well as atrial conduction are promising.     

A gonadotrophin dependent stromal luteoma: a rare cause of post-menopausal virilization

A stromal luteoma is an uncommon ovarian tumour in post-menopausal women, which presents rarely with hyperandrogenism and virilization. We present the case of a 64-year-old woman referred for evaluation of virilization which had developed over 5 years. Testosterone, FSH and LH were markedly inhibited following the administration of a GnRH analogue, suggesting a gonadotrophin dependent, testosterone secreting ovarian tumour, which could not be localized with imaging techniques. Surgery revealed normal sized ovaries with no apparent lesions, but microscopic examination showed a small stromal luteoma in the right ovary and severe stromal hyperthecosis in the adjacent and contralateral ovarian stroma. We conclude that stromal luteoma is an uncommon cause of virilization in post-menopausal women. This case illustrates, to our knowledge for the first time, that a stromal luteoma is not autonomous but is gonadotrophin dependent.     

Mesenteric cysts: rare cause of abdominal pain

For about two years a now 47-year-old woman had been suffering from heartburn, diarrhoea and constipation, at times also abdominal colic and a pressure sensation in the right lower abdomen. Physical examination and biochemical tests were according to age, except for an raised erythrocyte sedimentation rate of 25/50 mm. Ultrasound revealed an intraperitoneal echo-free multi-chambered structure, 11 x 5 x 2 cm, with dorsally increased echo density and smooth entry echo, apparently arising from the mesentery. Computed tomography confirmed these findings and at laparoscopy a cyst, 12 x 8 x 5 cm, was identified. It was filled with yellow clear fluid and arose from the mesentery of the ascending colon. The cyst was scooped out surgically. Histological examination showed a single-layered mesothelial coat. The postoperative course was unremarkable. At re-examination 8 months later the patient had remained symptom-free.     

Melanocanthoma: a rare cause of oral hyperpigmentation

The oral features of a black woman with melanocanthoma of the oral mucosa are detailed, and the current literature of melanocanthoma of the oral mucosa is briefly reviewed.     

Current therapy of obstructive jaundice due to cholelithiasis

On recent development of apparatus and techniques of ultrasonography and endoscopy we prefer to try the following procedures for removing jaundice instead of surgical operation: 1) Percutaneous Transhepatic Biliary Drainage (PTBD) 2) Endoscopic Retrograde Biliary Drainage (ERBD) 3) Endoscopic Naso-Biliary Drainage (ENBD) 4) Endoscopic Sphincterotomy (EST). After removing the jaundice and inflammation of bile duct, we try following procedures for elimination of gallstone: 1) endoscopic mechanical lithotripter (EML) 2) (Extracorporeal shock wave lithotripsy (ESWL) And we have got the excellent results.     

Shy-Drager syndrome: a rare cause of orthostatic hypotension

HISTORY AND ADMISSION FINDINGS: A 71-year-old man was admitted because of treatment-resistant orthostatic hypotension of unknown aetiology. When aged 64 years he developed some impotence and later urinary incontinence and urinary frequency. At 68 years he noted vertigo on physical activity, and a year later he had signs of reversible cerebral ischaemia. At this point the Schellong test demonstrated vasovagal circulatory dysfunction. After his 70th birthday the unsteadiness on walking and standing got worse and he had recurrent syncopes. He was in a wheel-chair when hospitalized and even the unsteady walk he could maintain for only a few seconds. INVESTIGATIONS: Plasma and urinary concentrations of catecholamines were at the lower limit of normal but failed to increase during orthostasis. Hormonal, cardiological and infectious causes of the orthostatic hypotension were excluded. Orthostatic tests after Schellong and with the tilting table showed orthostatic hypotension without increased sympathetic activity but hypertensive blood pressure levels during the recumbent period. Intravenous infusion of norepinephrine produced an excess rise in blood pressure (raised norepinephrine sensitivity). The recurrent urinary infection was shown to be due to a hypotonic bladder detrusor muscle. Neurological examination revealed cerebellar dysfunction, signs of pyramidal tract abnormality and sensory polyneuropathy. A Shy-Drager syndrome was diagnosed on the basis of the history, absent blood pressure rise and lack of catecholamine release during orthostasis with increased epinephrine sensitivity and characteristic neurological signs. TREATMENT AND COURSE: Physiotherapy and elastic stockings with administration of mineralocorticoids as well as of one direct (norfenefrine) and one indirect (amezinium) sympathomimetic drug failed to improve adequately the abnormal orthostatic response. But on additional administration of an alpha 2-receptor antagonist (yohimbine) the patient was able to stand and walk for a few minutes, but the urinary incontinence and the other neurological signs remained treatment-resistant. CONCLUSION: If orthostatic hypotension occurs together with neurological symptoms, a Shy-Drager syndrome should be taken into account.     

Primary pancreatic lymphoma. A rare cause of pain-free icterus

Toxocariosis is a zoonosis which has been widely studied in dogs. However, not much is known about this parasitosis in cats. The aim of the present work was to determine the frequency of Toxocara cati in domestic cats in Mexico City and the State of Mexico. Feces of 660 domestic cats were studied by the Faust concentration-floating test; 401 samples were from cats living in Mexico City of which 308 lived in houses and 93 in apartments; 231 were from urban areas of the State of Mexico and 28 from a rural area of the same state. The total frequency of T. cati eggs obtained from domestic cats in Mexico City was 42.9%; in cats living in apartments it was 18.3% and in cats living in houses it was 50.3%. In domestic cats from the State of Mexico, T. cati frequency was 36.4% in the urban and 21.4% in the rural areas. We consider that toxocariosis frequency observed at the two studied sites is high and that the need to prevent dissemination of the infectious forms of T. cati is urgent, as is the necessity of informing the population of the risk of living with T. cati parasited animals and of the anatomopathological alterations caused by T. cati in man.