Autoimmune polyglandular syndrome type I. A case report

Calcineurin is a Ca2+ calmodulin dependent protein phosphatase which has an important role in the control of intracellular Ca2+ signalling. The protein is a heterodimer of one catalytic (CnA) subunit and one regulatory (CnB) subunit. As suggested by the protein sequence and confirmed by the crystallographic structure, the catalytic subunit of calcineurin (CnA) has high homologies with other protein phosphatases. The regulatory subunit (CnB) belongs to the EF-hand Ca2+ binding protein family. Despite its similarity with calmodulin, it has a different tertiary structure. Calcineurin is the target of two important immunosuppressant drugs: cyclosporin A and FK506. Subsequently, a detailed clarification of the role of calcineurin in the cytokine mediated activation of the T-cells has been possible. The understanding of the role of calcineurin in other cells, in particular neurons, is also progressing rapidly.     

Shprintzen-Goldberg syndrome. A case report

Major hemorrhage after pulmonary resection is infrequent. It is usually due to an unsecured vessel, and immediate reoperation to control hemorrhage is indicated. Diffuse bleeding from raw chest wall surfaces can complicate pleurectomy. Control of bleeding from this cause is difficult, and standard techniques may fail. Thoracic packing is occasionally needed. The authors describe a technique for thoracic packing. This technique permits removal of thoracic packing at the bedside, and simplifies subsequent management of an empyema, should one develop.     

Eye injuries during paintball games. The first Danish case with a summary of foreign experiences

Paintball shooting is played with increasing frequency in Denmark, and ocular injuries can cause serious damage. The first Danish case is presented and the literature is reviewed.     

Hantavirus pulmonary syndrome (HPS): report of first case in Louisiana

Since May 1993, 26 people have died of a "unique, previously unknown" viral illness that presents with flu-like symptoms and progresses rapidly to respiratory failure and death. Originally isolated in the four-corners region of the Southwest, the hantavirus responsible has now been isolated from patients in several other areas. In this article, we describe our experience with "the most puzzling case so far," an infection by a "cousin" of the Southwest hantaviral strain in a Louisiana bridgeworker. This case is unique not only because of the slightly different makeup of the virus, but also because of the absence of deer mice, the suspected carrier of the hantavirus, in the Louisiana region.     

Munchausen's syndrome: case report

A case report of a 31-year old butcher with haemorrhagica histrionica is presented. The patient went to great lengths to induce haematemesis and fake its symptoms to gain admission to hospital. The alarming frequency of admissions and dramatic behaviour of the patient prompted this report.     

The growing teratoma syndrome: report of a case

We report here the first case of a one-step endosonography(EUS)-guided pseudocyst drainage. A prototype large channel curved array echo endoscope (Pentax FG-38 UX) and a prototype delivery system for placement of an endoprosthesis was used for the procedure. The delivery system (GIP MedicinTechnik GmbH/Medi-Globe Corporation) consists of a handle part with a piston, a metal ring sheath, a plastic catheter with a diathermy needle and a double pigtail endoprosthesis (8.5 Fr). When mounted on the endoscope the endoprosthesis can be advanced out of the distal end of the endoscope. The introduction of the stent as well as the stent release can be monitored entirely by ultrasound. The procedure was tested in a 76-year-old woman with a pseudocyst measuring 60 mm in diameter located in the tail of the pancreas. The procedure was well tolerated by the patient, and there were no procedural complications. The advantage of a large channel echo endoscope and our new prototype delivery system is that the endoprosthesis can be inserted in to a pancreatic cyst guided exclusively by EUS without exchange of endoscopes, catheters or guide wires. Further studies are warranted.     

The Kocher-Debré-Semelaigne syndrome: a case report

High school sophomores residing in a community with elevated levels of sodium in the drinking water (107 mg/1) exhibited a marked upward shift in blood pressure distribution patterns for systolic and diastolic blood pressures in both males and females as compared with a similar group in the control community (8 mg/1). The students group from the high sodium community appears to exhibit a blood pressure distribution characteristic of persons several years older.     

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.     

Gluteal compartment syndrome: case report

A compartmental syndrome can occur in any space limited by fascia or skin. A case of a gluteal compartment syndrome is reported, apparently from prolonged pressure after a drug overdose. Clinical features were a painful expanding gluteal mass with sciatic nerve dysfunction. Fasciotomy of the tensor fascia lata and the overlying fascia of the gluteus maximus resulted in rapid relief of symptoms.     

The syndrome of painful legs and moving toes--a case report

The syndrome of painful legs and moving toes is an uncommon and distressing condition with pain in the feet or legs and involuntary movements of the toes. It can follow spinal cord or cauda equina trauma, lumbar radiculopathy, injury to the feet, peripheral neuropathy or without any preceding causes. Ephaptic transmission in damaged nerve roots or peripheral nerves with central reorganisation may be the underlying mechanism of the syndrome. Treatment is difficult. We report a case of this syndrome following peripheral neuropathy, with a good early response to the GABA agonists baclofen and clonazepam. The role of different GABA agonists in the treatment of this condition needs to be better defined.     

Morquio's syndrome. A case report and review of clinical findings

Morquio's syndrome is a genetic disorder caused by the lack of the enzyme responsible for the breakdown of the mucopolysaccharide keratin sulfate. The resulting buildup of keratin sulfate causes a specific pattern of abnormalities, including short stature, spinal deformity, cardiac abnormalities, corneal opacities, deafness and dental abnormalities. The specific dental findings in the patient described here are as follows: the posterior teeth were tapered and had pointed. cusps; the maxillary anterior teeth were spaced and flared, and on radiographs, the enamel was less than 25 percent of normal thickness but of normal radiodensity. Despite these findings, the patient exhibited a low rate of caries. This paper is a review of medical and dental findings in the Morquio's patient and how this may affect dental management.     

Sweet's syndrome associated with spinal surgical intervention. A case report

STUDY DESIGN: A patient with a medical history of Sweet's Syndrome, an acute neutrophilic dermatosis, was seen at the authors' institution for cervical pain. After undergoing a thorough history-taking and physical examination and after experiencing no relief with conservative therapy, the patient underwent cervical spine surgery. After the surgical procedure, the patient developed multiple cutaneous lesions that were consistent with the findings associated with an acute recurrence of Sweet's Syndrome. OBJECTIVES: To characterize the authors' experience with this unusual histologically documented dermatologic disorder. SUMMARY OF BACKGROUND DATA: Sweet's Syndrome is a rare form of neutrophilic dermatosis characterized by recurrent eruptions of painful, edematous, red, tender plaques that are found predominantly on the torso in middle-aged women. After an extensive literature search, it was noted that this rare and unusual disorder has not been reported previously in association with surgical intervention of any type, including spinal operations. METHODS: The patient's postoperative course was documented, and all medical records were reviewed retrospectively. RESULTS: The patient's rash resolved spontaneously. Solid fusion of C5-C6 occurred. The patient remained neurovascularly intact, and her axial cervical pain decreased significantly from its preoperative levels. CONCLUSIONS: Sweet's Syndrome remains a rare dermatologic disorder, which may complicate a routine postoperative course. Patients with Sweet's Syndrome have an exceedingly high rate of other serious medical illness. The effect of Sweet's Syndrome on physiologic bone healing is unknown. In this patient, there was nonunion of the cervical spine, with eventual solid bony union. Perioperatively, patients with this disorder are treated with oral prednisone and oral antibiotics to prevent secondary complications at the surgical wound.     

Spontaneous tibial compartment syndrome: case report

We report the case of an obese 34-year-old woman who spontaneously developed compartment syndrome in her right calf. The patient was treated with four-compartment fasciotomy and debridement of necrotic tissue. Four months after treatment she was ambulating without the use of aids and had full range of motion and muscle strength.     

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.