Insulinomas in MEN-I patients: early detection and treatment of insulinomas in patients with the multiple endocrine neoplasia syndrome type-I

In the multiple endocrine neoplasia syndrome type I (MEN-I syndrome), periodic screening of patients and their close relatives may improve prognosis and life expectancy. Although there is diffuse involvement of the pancreas with microadenomatosis, insulinomas in the MEN-I syndrome usually occur as single tumours. This is illustrated here by two patients with insulinomas and the MEN-I syndrome. Preoperative localization of the tumours was achieved accurately by digital subtraction angiography combined with dynamic computerized tomography after a bolus injection of contrast medium. At present, two and three years after elective surgery both patients are asymptomatic. The early detection and treatment of insulinomas is extremely important because of the high risk of cerebral damage associated with late diagnosis. Periodic investigation of MEN-I family members can promote the early diagnosis and treatment of insulinomas, especially in young patients, whose life expectancy and quality of life may be improved.     

Non-invasive diagnosis and monitoring of chronic kidney disease by reverse iontophoresis of urea in vivo.

BACKGROUND: Reverse iontophoresis uses a small current to extract molecules and ions through the skin. The aim of the study was to determine whether reverse iontophoresis of urea can be used (i) to diagnose and monitor non-invasively chronic kidney disease (CKD), and (ii) to track urea levels closely during a hemodialysis session. METHODS: A current of 0.8mA was applied for 2h in 10 healthy volunteers, in 9 patients with CKD, and in 10 patients undergoing hemodialysis. Urea fluxes extracted by reverse iontophoresis and urea concentrations in the blood were measured. RESULTS: Extracted urea fluxes discriminated healthy volunteers from patients with CKD within 90min. A non-invasive measure of blood urea concentrations can be achieved after 120min. A urea reservoir in the skin interferes with the extraction and a pre-hemodialysis "depletion" period is required. Mild and transient sensation and erythema induced by iontophoresis were significantly lower in the CKD group. Gelling the formulation of the iontophoresis reservoir gave similar results to those obtained when using a simple aqueous solution. CONCLUSIONS: Reverse iontophoresis can be used to non-invasively diagnose individuals with CKD and to monitor urea concentrations in blood.     

Concomitant use of simvastatin and amiodarone resulting in severe rhabdomyolysis: a case report and review of the literature

Myopathy, including rhabdomyolysis, is a well-known, albeit rare complication of statin therapy. Predisposing factors include comorbidities and the concomitant use of cytochrome P-450 (CYP) 3A4 inhibitors. We report a case of severe simvastatin-induced rhabdomyolysis triggered by the addition of amiodarone to previously well-tolerated chronic statin therapy. Physicians should be aware of the risk of this potentially severe drug interaction. The dose of simvastatin should be reduced (to 20 mg daily) when concomitant treatment with amiodarone is required, or preference should be given to pravastatin, rosuvastatin or fluvastatin, which are not metabolised by the CYP 3A4.     

Live birth after allografting of ovarian cortex between genetically non-identical sisters

Aggressive chemotherapy generally results in the loss of both endocrine and reproductive functions. If the patient has not undergone previous oocyte, embryo or ovarian tissue cryopreservation, orthotopic allotransplantation of fresh ovarian tissue from a genetically non-identical sister may be considered. Here, we describe a case report. The patient, aged 15 years and presenting with homozygous sickle cell anemia, underwent chemotherapy (busulfan, cyclophosphamide) and total body irradiation before bone marrow transplantation, the donor being her HLA-compatible sister. HLA group analysis later revealed complete chimerism. When the patient was 32 years old, ovarian allografting was performed, with the ovarian tissue donor being the same sister who had already donated bone marrow. The goal was to restore ovarian activity and natural fertility. No immunosuppressive therapy was administered. No sign of rejection was observed. Restoration of ovarian function was achieved 3.5 months after transplantation, as proved by the first estradiol peak and follicular development detected by ultrasound. After 9 months of regular ovulatory cycles, IVF was attempted because proximal tubal stenosis (unknown at the time of grafting) could not be repaired by tubal reanastomosis. After stimulation, three oocytes were retrieved. Two embryos were obtained. One embryo was frozen and the other was transferred, resulting in an ongoing pregnancy. The patient delivered a healthy baby girl weighing 3.150 g at 37 2/7 weeks of gestation.     

Childhood chronic inflammatory demyelinating polyneuroradiculopathy--three cases and a review of the literature

BackgroundChronic inflammatory demyelinating polyneuroradiculopathy (CIDP) is an autoimmune disease of the peripheral nervous system, causing demyelination and even axonal degeneration. In children, abnormal gait as a first sign of muscle weakness is a frequent reason to seek medical attention. Diagnosis is made on the basis of clinical characteristics, electromyography and nerve conduction studies, and elevated protein in cerebrospinal fluid.AimsWe present three new cases of CIDP. The literature was reviewed in order to obtain more information on presentation, outcome and treatment strategies world-wide.ResultsThe course of disease can be relapsing–remitting or chronic-progressive. From case series it is known that first-line immunotherapy (intravenously administered immunoglobulin, corticosteroids or plasmapheresis) is initially of benefit in most children with CIDP. There is little evidence, however, on second-line therapies as azathioprine, cyclosporine A, mycophenolate mofetil, methothrexate, cyclophosphamide and IFN alpha. Although the outcome of children with CIDP is generally regarded to be good, disease related disability can be severe.ConclusionChildhood CIDP is rare. In general and in comparison to adults, children tend to have a more acute progressive onset, with more severe symptoms. Showing a higher tendency towards a relapsing–remitting course, children often show a better and faster improvement after therapy, and a more favorable outcome. Swift recognition of CIDP and empiric start of treatment are considered important to avoid potentially irreversible axonal damage and associated disability. Response to first-line therapies is usually favorable, however recommendations regarding the choice of second-line therapy can only be made on the basis of current practice described in case reports. Safety and efficacy data are insufficient. The cases described show that trial and error are often involved in finding an optimal treatment strategy, especially in those patients refractory to first-line treatment or with a prolonged course. Clinical experience with immunomodulatory treatment is paramount when treating children with CIDP.