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.     

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.     

A rare cause of obstructive jaundice: peripancreatic pseudoaneurysm

We report the case of a 54-year-old-man with alcoholic calcified chronic pancreatitis complicated by jaundice and abdominal pain. Investigations (Doppler ultrasonography examination and computed tomography scan) showed peripancreatic pseudoaneurysm of the posterior and inferior pancreatico-duodenal artery and a dilatation of the common bile duct. Selective embolization of the pseudoaneurysm resulted in rapid regression of both jaundice and abdominal pain. Common bile diet compression is a rare complication of peripancreatic pseudoaneurysm. Selective embolization seems to be the first line treatment in this indication.     

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.     

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 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.     

Endobronchial lipomatous hamartoma: a rare cause of bronchial occlusion

A case of branch retinal artery occlusion due to an embolus from a retained left atrial catheter is presented. Removal was accomplished by reoperation. Prompt removal of any retained intracardiac catheter is recommended.     

Pseudoxanthoma elasticum: a rare cause of hypertension in children

Urge incontinence and frequency, persisting despite oral anticholinergics and antibiotics for four months, brought a 72-year-old woman to our hospital. Plain abdominal X-ray followed by cystoscopy demonstrated a large stone (43 x 37 mm) in the bladder, and the patient underwent suprapubic cystotomy to remove the stone. The stone contained a surgical sponge, which was apparently left in situ at transvaginal hysterectomy two years previously. The sponge had most likely eroded the bladder wall and migrated into the cavity. We found a total of 17 cases reported in Japan of a surgical sponge migrating into the bladder. In particular, our case was associated with transvaginal surgery, while the other 16 cases involved transabdominal surgery.     

Postradiation colospinal fistula: a rare cause of spinal epidural abscess

We describe a rare case of spinal epidural abscess, which resulted from a colospinal fistula that developed as a complication of previous surgical resection and radiotherapy for rectal carcinoma. The patient's clinical course and the imaging features of colospinal fistula are described, along with a brief review of the literature.     

Mondini's dysplasia: a rare cause of recurrent meningitis in childhood

Recurrent meningitis in children may be due to immune deficiency or abnormal communication of CSF spaces with mucosal spaces (basal skull fractures, encephalocele, etc.). We report a 6-year-old boy, suffering from recurrent meningitis and sensorineural hearing loss. CT scans of the temporal bones demonstrated a bilateral Mondini dysplasia. In patients with this congenital inner ear anomaly, recurrent meningitis may be due to a CSF-middle ear fistula. We think that it is necessary to investigate all patients with recurrent meningitis and sensorineural hearing loss with CT scans of the temporal bones.     

Abdominal cocoon in a male: rare cause of intestinal obstruction

The present paper describes the possible clastogenic activity of the following synthetic sugar substitutes, such as cyclamate in daily doses of 11 and 110 mg/kg, saccharin, 5 and 50 mg/kg, acesulfam, 15 and 150 mg/kg, sucralose, 15 and 150 mg/kg, aspartame, 40 and 400 mg/kg, orally given to C57Bl/6 mice during 5 days. No clastogenic activity was found in the compounds tested.     

Cystic rectal duplication: a rare cause of neonatal intestinal obstruction

A case of cystic rectal duplication revealed on day 2 of life by a low intestinal occluding syndrome is reported. Radiologic imaging (ultrasonography, cystography, rectography) showed a large, retrorectal liquid formation in the pelvis and abdomen, with pelvic compression of the terminal alimentary canal and lower urinary tract. Magnetic resonance imaging demonstrated a liquid formation with clearly defined edges and no medullary involvement, thus ruling out the possibility of a previous meningeal hernia. Biological markers were within normal limits. On day 4, a 9 x 6-cm cystic rectal duplication was removed, followed by a temporary colostomy. Pathologic examination demonstrated typical rectal architecture with ciliated cells. Radiologic and clinical findings at 2-month follow-up were reassuring. This case report is exceptional for the following reasons: (1) As a rule, rectal duplications are relatively rare (70 cases reported in the literature); (2) The means of disclosing a neonatal rectal duplication is unusual (4 cases reported in the literature); (3) The volume of the malformation was considerable; and (4) Heterotopic ciliated epithelium was present.     

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.     

Self-inflicted intramyocardial injury with a sewing needle: a rare cause of pneumothorax

Ganciclovir susceptibilities and UL97 sequences were analyzed in 20 cytomegalovirus (CMV) isolates recovered from 15 bone marrow transplant recipients with active CMV infection after prophylaxis with acyclovir (group I; 12 isolates) or after acyclovir prophylaxis followed by ganciclovir therapy (group II; 8 isolates). All group I isolates were susceptible to ganciclovir. Five group II isolates were susceptible to ganciclovir, and 3 isolates (all from the same person) were resistant to ganciclovir (IC50 > 12 microM). Ganciclovir resistance UL97 mutations were found in 4 group II isolates, including a ganciclovir-susceptible isolate obtained from 1 patient after 41 days of therapy with ganciclovir and 3 ganciclovir-resistant isolates obtained from another patient after 73, 116, and 132 days of treatment with ganciclovir. Ganciclovir-resistant CMV isolates may emerge rapidly in bone marrow transplant recipients who are treated with ganciclovir after receiving prophylaxis with acyclovir.