A case of primary Addison's disease with hyperemesis gravidarum and successful pregnancy

We followed up a pregnant woman with Addison's disease diagnosed before conception. She presented with hyperemesis gravidarum. Throughout pregnancy, she received prednisone and the basic disease did not deteriorate during pregnancy. She was delivered by caesarean section due to breech presentation. The fetal prognosis was good.     

Assessment of digital ischaemia and evaluation of response to therapy by 99mTc sestamibi limb scintigraphy after local cooling of the hands in patients with vasospastic Raynaud's syndrome

AIM: Cold induced arteriolar constriction in patients with vasospastic Raynaud's syndrome (VRS) produces temporary digital ischaemia. The aim of this study was to ascertain whether 99mTc sestamibi scintigraphy is useful in the diagnosis and the monitoring of treatment in VRS. METHODS: Fifteen patients with VRS and 20 matched normal controls underwent examination. Twelve patients with VRS received therapy. For each patient, one hand was immersed in iced water for 30s while the other hand served as a control. Ten minutes after cooling, 99mTc sestamibi was injected and imaging was performed 60min later. The per cent decrease of the perfusion (%DP) was calculated by semiquantitative analysis to determine the severity of hypoperfusion. RESULTS: In all patients with VRS, moderate or marked hypoperfusion were seen in 99mTc sestamibi images after exposure to the iced water, while there was minimal or mild hypoperfusion in the control groups. Values for %DP were 46.86 +/- 19.04 and 7.85 +/- 4.53 for the VRS group and normal subjects, respectively. The difference between both groups was statistically significant (P = 0.0000). In 12 treated patients with VRS, pre-treatment and post-treatment %DP values were 51.16 +/- 18.42 and 33.58 +/- 17.83, respectively, and a significant difference was seen between both values (P = 0.001). However, there was still a statistically significant difference between control subjects and post-therapy values (7.85 +/- 4.53 vs. 33.58 +/- 17.83, P = 0.0000). The +/- 95% confidence interval of DP for control subjects was 5.7-10% (chi-squared, P = 0.000). When a DP of 10% was used as a cut-off point, sensitivity, specificity and diagnostic accuracy were 100%, 70% and 83%, respectively, for the 99mTc sestamibi scan. There was also a strong correlation between %DP and the duration of the disease (r = 0.80, P = 0.0003). CONCLUSION: The results of this study indicate that a 99mTc sestamibi scan is a valuable imaging method for the determination of digital ischaemia in vasospastic Raynaud's syndrome, and may play a role in evaluating the response to therapy.     

Upper tract surveillance in primary bladder cancer follow-up

OBJECTIVE: To determine if there is national variation in regimens of upper urinary tract surveillance in patients with primary bladder cancer. METHODS: A questionnaire was sent to 470 consultant urologists from a British Association of Urological Surgeons list in the UK; 301 anonymous replies were received. Two replies were incomplete and therefore the results of 299 questionnaires (64%) were analysed. RESULTS: Of the 299 surgeons, 19 (6%) use no form of upper urinary tract surveillance; 162 (54%) use surveillance in selected patients, i.e. those with carcinoma in situ (47%), multiple bladder tumours at first presentation (39%) and after cystectomy (70%), and 118 (39%) use upper tract surveillance on all patients with a history of bladder cancer. The median (range) screening interval was 24 (12-60) months and surveillance continued for a median of 10 (2 to indefinite) years, continuing for an indefinite period in 33%. CONCLUSIONS: Most urologists use upper tract surveillance in patients with bladder cancer but there is wide variation in the duration and interval for which it continues, and in the type of patient selected for surveillance. Some patients at high risk of upper tract tumour are not being screened. Asymptomatic upper tract tumours may not be diagnosed because the intervals between surveillance are too long, and the duration for which it continues inadequate. There is a need for multidisciplinary national guidelines to reduce variation in practice.     

Atypical meningioma and extensive calvarium defects in neurofibromatosis type 1

A 9-year-old girl with neurofibromatosis type 1 (NF1) presented with a massive atypical meningioma and calvarial defect. Skull radiographs and cranial CT showed an extensive lytic bone lesion at the vertex. MRI demonstrated a large mass invading the calvarium and sagittal sinus. The histopathological and immunohistochemical diagnosis of the resected mass was atypical meningioma. To our knowledge, this is the first case of NF1 associated with atypical meningioma and massive calvarial defect in a child.     

Inflammatory myofibroblastic tumor (inflammatory pseudotumor) of the maxillary sinus mimicking malignancy: a case report of an unusual location (is that a true neoplasm?)

The inflammatory myofibroblastic tumor (IMT) is a space-occupying lesion of unknown etiology and a distinctive but controversial lesion. This type of tumor is recently considered neoplastic rather than inflammatory. It is usually occurring during childhood, composed of fascicles of bland myofibroblastic cells admixed with a prominent inflammatory infiltrate consisting of lymphocytes, plasma cells, and eosinophils. IMT of the maxilla is very rare. The diagnosis of IMT can be made on the basis of histopathology and immunohistoche-mistry. Herein, we presented a patient who had IMT of the maxillary sinus that was initially misinterpreted as a malignant neoplasm upon clinical and radiographic examinations. We discussed the diagnostic and therapeutic procedures and may consider it a true neoplasm.     

Intravenous immunoglobulin and thymoglobulin facilitate kidney transplantation in complement-dependent cytotoxicity B-cell and flow cytometry T- or B-cell crossmatch-positive patients

BACKGROUND: The aim of this study was to investigate the effect of Thymoglobulin and intravenous immunoglobulin (i.v.IG) therapy on the clinical outcome of a putatively high-risk group of kidney transplant recipients who have positive B-cell complement-dependent cytotoxicity (CDC) along with positive T- or B-cell flow cytometry (FC) crossmatch results. METHODS: We prospectively studied the effects of i.v.IG and Thymoglobulin induction treatment in B-cell CDC, and T- or B-cell FC crossmatch-positive kidney transplant recipients (seven women and one man; mean age, 43+/-12 years). RESULTS: Mean peak panel-reactive antibody (PRA) was 47+/-32. Three patients had donor-specific antibody by flow PRA (two anti-DR4 and one anti-A2). Each recipient received induction treatment with i.v.IG 100 mg/kg for 3 days and Thymoglobulin 1.5 mg/kg for 5 days after transplantation. No acute cellular rejections occurred during a median follow-up of 15 months (range, 12-17 months). Only one acute humoral rejection occurred 8 days after transplantation, which responded to plasmapheresis, i.v.IG, and rituximab. One allograft was lost because of polyoma nephritis. Patient survival was 100% and allograft survival was 88%. CONCLUSION: Our results indicate that i.v.IG and Thymoglobulin induction treatment may facilitate kidney transplantation in B-cell CDC and T- or B-cell FC crossmatch-positive patients.