The usefulness of intra-operative endoscopy in Peutz-Jeghers syndrome: a case report

The Peutz-Jeghers syndrome is an autosomal dominant inherited disease manifested by a combination of mucocutaneous pigmentation and gastrointestinal hamartomatous polyps that usually cause intussusception and intestinal hemorrhage. We report a case in which the patient has been followed-up on for 14 years and who underwent surgical and endoscopic polyp removal several times as well as one intestinal resection. This time, with the use of combined surgery and perioperative endoscopy, 27 polyps were removed, performing only 3 enterotomies. This is the highest number in one session to be reported in the literature. The usefulness of this technique is providing a "clean small intestine" that allows the patient a longer time interval between laparotomies and reduces the complications associated with multiple laparotomies and resections.     

Small trocar perforation of the small bowel: a case report

Mood disturbances have been identified in association with changes in levels of reproductive hormones. The use of hormonal measures in the diagnosis of reproductive endocrine-related mood disorders is highly variable, ranging from necessary in perimenopausal depression to irrelevant in premenstrual syndrome. This article describes our view of the use of hormonal measures for diagnostic and research purposes in perimenopausal depression and premenstrual syndrome. We suggest that the understanding of these disorders lies in as yet unidentified contextual factors rather than in hormonal excesses or deficiencies.     

Hemolytic-uremic syndrome due to deoxycoformycin: a report of the second case

A hypercalcemic patient with adult T-cell leukemia was treated with deoxycoformycin (DCF) in a dose of 5 mg/m2 daily for three days. Several days later, severe thrombocytopenia appeared abruptly and then microangiopathic hemolytic anemia ensued. Subsequently, renal failure and hypertension developed, and the patient died seven weeks after DCF therapy. Needle necropsy of the kidney showed glomerular damage including swelling of endothelial cells, mesangiolysis and segmental collapse. This is the second reported case of hemolytic-uremic syndrome due to DCF.     

Traumatic incarceration of the small bowel: case report

While bowel injuries associated with blunt abdominal trauma are a well recognized entity, entrapment of bowel between vertebral bodies has seldom been described. The unusual case of traumatic jejunal incarceration between two lumbar vertebrae is presented.     

The Peutz-Jeghers syndrome: a report of 3 cases

Three cases of the Peutz-Jeghers syndrome are reported and the literature is reviewed. The difficulties in the management of these patients are described and the importance of radiology and endoscopy is emphasised.     

Biliary obstruction due to duodenojejunal intussusception in Peutz-Jeghers syndrome

We reported an operative case of bilateral coronary artery fistulae to pulmonary artery associated with a giant saccular aneurysm, the largest of which measured 30 x 30 mm. The patient was a 75 year old female who had anginal pain due to coronary steal phenomenon. A continuous murmur was detected. After establishing total cardiopulmonary bypass, two distal orifices of the fistula connected to the main pulmonary artery were closed with 5-0 polypropylene plegeted sutures. Aneurysmorrhaphy was then performed for giant saccular aneurysm. Postoperative course was uneventful.     

Polyposis: the Peutz-Jeghers syndrome

The classical pigmentation of Peutz-Jeghers syndrome distinguishes it immediately from the other polyposis syndromes. Less widely appreciated than this most obvious manifestation are the pitfalls in management presented by the risk of cancer and by the performance of multiple laparotomies that also characterize this condition. An outline of these risks and an approach that minimizes them is presented.     

Sneddon syndrome: a case report

Doxorubicin is cardiotoxic and its use must be monitored carefully. Incidence of refractory cardiac failure is shown to increase once the cumulative dose exceeds 450 mg/m2. However, significant decline of ejection fraction (EF) may occur even at lower dose levels. EF was monitored using Multigated Radionuclide Angiography (MUGA) scan of all consecutive lung cancer patients, treated with Doxorubicin based regimens. Thirteen of 82 patients showed a significant (more than 15%) decline of left ventricular EF. The dose of doxorubicin producing this decline ranged between 91-180 mg/m2. Actual decline in EF ranged between 16-45%. Only 5 of 13 patients developed symptoms attributable to the cardiac disease. Doxorubicin can alter EF significantly in lung cancer patients at levels well below which are considered 'safe'. The reason for massive decline in ejection fraction in these patients has been hypothesized.     

Simultaneous mucosal and small bowel angioedema due to captopril

Although angiotensin-converting enzyme inhibitors (ACEIs) are well-known causes of orofacial angioedema, angioedema from these agents involving the bowel is not often considered. We report a case of simultaneous onset of small bowel and orofacial angioedema due to captopril. A 61-year-old black man with hypertension, coronary artery disease, and congestive heart failure had been treated with captopril for 5 years. He had sudden swelling of the lips, face, and tongue, followed by nausea, emesis, abdominal pain, and diarrhea. Other medications included aspirin, indomethacin, allopurinol, colchicine, and nifedipine. Examination showed swelling of the tongue, buccal mucosa, and neck; he also had midabdominal tenderness but no respiratory distress. Laboratory data were normal. A C1-esterase inhibitor level was normal. An ileus pattern was present on abdominal x-ray film. Angioedema was diagnosed, and all signs and symptoms resolved in 24 hours after captopril was discontinued. Clinicians need to be vigilant for bowel involvement from ACEI angioedema.     

Association of Moebius syndrome and hypopituitarism due to a midline anomaly. A case report

A 14 year old patient had both Moebius anomalad with arthrogryposis and growth hormone deficiency due to a midline defect. This combination suggests an abnormality in the organization of the neural crest and homologous neural tube segment.     

Carcinoid to cecal bowel localization: a case report and literature review

Data are submitted from long-term follow-ups over 78 patients with Gilbert-Meylengraht's syndrome (71 men and 7 women). The above syndrome was identifiable predominantly in those persons aged 15-30. A major proportion of the patients (75%) displayed different dyspeptic phenomena and asthenovegetative disturbances, 25% had no complaints. The principle diagnostic criterion for the syndrome is predominant or solitary elevation of blood serum levels of free (unconjugated, indirect) bilirubin without signs of affection liver, obturation of the biliary tract, and enhanced hemolysis. This form hyper bilirubinemia runs benign course. All above patients remain able bodied having enough capacity for work. In exacerbations, it is helpful to prescribe small doses of phenobarbital (0.05-0.15 gr daily), under the influence of which drug the serum level of bilirubin returns to normal, this effect lasting only for a period of the drug administration.     

Cerebral embolism due to a retained pacemaker lead: a case report

There are only a few reported cases of a pacemaker lead migrating inadvertently into the left atrium or ventricle. An unusual complication of unremoved, unwanted pacemaker lead is presented. The free tip of the lead caused cerebral embolism after perforating the interatrial septum.     

Scan findings in a case of splenic infarction due to amyloidosis: case report

Spleen images in a patient with monoclonal gammopathy showed numerous areas of decreased radiocolloid accumulation. Microscopic examination revealed areas of coagulation necrosis with diffuse amyloid infiltration in the spleen. In this patient, amyloidosis is considered to have caused the infarct.     

Charles Bonnet syndrome: a case report

Gastrointestinal lipomas are benign but rare lesions, mainly located in the colon. Depending on their size they are responsible for obstructive bowel symptoms. Diagnostic tools are either the endoscopy or computer tomography. It is recommended to investigate the entire large bowel as other pathologies are often found. Any lipoma found should be removed. Two cases with symptomatic lipoma are presented. In both cases the lipoma was removed by open surgery.     

Case report: sicca syndrome due to primary amyloidosis

Sicca syndrome consists of two major clinical findings: keratoconjunctivitis sicca and xerostomia due to destruction of the lacrimal and salivary gland parenchyma. Although it is most often due to Sj?gren's syndrome, a variety of other diseases causes sicca syndrome. We report the rare case of a patient with gland infiltration in primary amyloidosis. Sonographic, computed tomographic and magnetic resonance findings are presented.     

Retinoic acid syndrome: a case report

The objective of this study was to investigate the impact of allergy to hymenoptera venom on the occupational activities of patients undergoing immunologic treatment for insect sting anaphylaxis. The design was a cross-sectional study conducted in a sample of 500 out of 1,500 patients undergoing venom immunotherapy for insect sting reaction in 13 allergy clinics in Israel. A self-administered questionnaire was used to collect data about demographic characteristics of patients, severity of the allergic reaction and adverse effects on occupational activities. Of the 204 respondents who were part of the labour force, 48.5% reported adverse effects on routine occupational activities. The factors with a significant influence on the probability of adverse occupational effects were: (1) patient's type of work (blue collar vs. white collar: OR = 3.22, p < 0.001: army vs. white collar: OR = 5.28, p = 0.001); (2) severity of the allergic reaction (severe reaction vs. mild/moderate reaction: OR = 2.34, p = 0.007). Our findings suggest that severe insect sting allergy has an adverse impact on patients' occupational activities. This factor requires special attention by the medical community. Social workers and occupational physicians should collaborate in the assessment and management of these patients.