Relapsing insulin-induced lipoatrophy, cured by prolonged low-dose oral prednisone: a case report.

INTRODUCTION: Circumscript, progressing lipoatrophy at the insulin injection sites is an unexplained, however rare condition in diabetes mellitus. CASE PRESENTATION: We report a case of severe localised lipoatrophy developing during insulin pump-treatment (continuous subcutaneous insulin infusion) with the insulin analogue lispro (Humalog®) in a woman with type-1 diabetes mellitus. After 11 months of progressing lipoatrophy at two spots on the abdomen, low-dose prednisone (5-10 mg) p.o. was given at breakfast for 8 months, whereby the atrophic lesions centripetally re-filled with subcutaneous fat tissue (confirmed by MRI) despite ongoing use of insulin lispro. However, 4 weeks after cessation of prednisone, lipoatrophy relapsed, but resolved after another 2 months of low-dose prednisone. No further relapse was noted during 12 months of follow-up on insulin-pump therapy with Humalog®. CONCLUSION: Consistent with an assumed inflammatory nature of the condition, low-dose oral prednisone appeared to have cured the lipoatrophic reaction in our patient. Our observation suggests a temporary intolerance of the subcutaneous fat tissue to insulin lispro (Humalog®), triggered by an unknown endogenous mechanism.

Whole body MRI in type I Gaucher patients: evaluation of skeletal involvement.

BACKGROUND: Bone disease is a serious complication of type 1 Gaucher disease, which if untreated can result in pain, disability and reduced quality of life. MRI is the method of choice for assessing and monitoring bone involvement in Gaucher patients. MRI bone evaluation has been predominantly carried out on the lumbar spine and/or lower extremities using quantitative or semi-quantitative methods. We describe evaluation of skeletal involvement in Gaucher patients using whole body MRI scanning. METHODS: Whole body MRI was performed in 39 adult type I Gaucher patients using a 1.5 T superconducting magnet with total imaging matrix technology. A standard MRI protocol was performed in all patients using coronal T1-, T2-weighted (thighs) and STIR-sequences of the whole body, sagittal T1-, T2- (lumbar spine) and STIR-sequences of the entire axial skeleton. Bone marrow involvement was analysed using the Düsseldorf Gaucher score (DGS), bone marrow burden score (BMB), and vertebra-disc-ratio (VDR). Pelvis, humerus, legs and spine were also analysed using the pattern of marrow involvement described by homogeneous type A or non-homogeneous type B morphology. Avascular necrosis (AVN) of the humeral head was determined. RESULTS: Whole body MRI was well tolerated and of diagnostic value in all patients. Thirty one out of 39 patients (79%) showed bone involvement. In fifteen of these 31 patients (48%) humeral bone involvement was observed. The morphological appearance of bone involvement (type A or B) was consistent across the humerus, legs and pelvis. The infiltration pattern was also similar across cervical, thoracic and lumbar vertebral bodies. Humeral bone involvement was present in 89% of patients with type B morphology compared with 32% of patients with type A morphology (p<0.005). Humeral involvement was detected more frequently in patients with severe bone involvement as determined by higher DGS and BMB scores, than in patients with lower DGS and BMB scores (p<0.0001 and p=0.0016). AVN of the humeral head was detected in 6 patients (19%). CONCLUSIONS: In this group of patients, severe bone involvement in the lumbar spine and lower extremities and type B morphology was also associated with humeral involvement. The morphological infiltration pattern was consistent in the entire skeleton indicating the systemic character of bone disease. Whole body MRI presents a feasible means of assessing the entire skeletal system and could be a valuable diagnostic and monitoring tool in the management of patients with type 1 Gaucher disease.

Sudeck's disease stage 1, or diabetic Charcot's foot stage 0? Case report and assessment of the diagnostic value of MRI.

BACKGROUND: The diagnosis of Sudeck's syndrome stage 1 (nowadays termed complex regional pain syndrome I, abbreviated CRPS I) is based on clinical features, namely swelling and pain in a limb. Plain X-ray may be normal. In the absence of pain sensitivity, e.g. in diabetic neuropathy, CRPS I of the foot can be mistaken for Charcot's foot stage 0 (so-called neuro-osteoarthropathy). CASE PRESENTATION: The case of a type-1 diabetic woman is reported, in whom CRPS I following a calcaneal fracture was mistaken for Charcot's osteoarthropathy (because of bone marrow edema displayed by conventional MR imaging). In addition, a review is presented on 6 consecutive cases with CRPS I of the foot, and on 20 cases with Charcot's foot stage 0, with particular emphasis on MR imaging findings. The number of bones per foot affected with marrow edema was similar in either condition, with a tendency towards a more patchy, diffuse distribution of bone marrow edema in CRPS I. Bone marrow edema apparently regressed more promptly in response to treatment in Charcot's foot stage 0. CONCLUSION: Differentiation of CRPS I from Charcot's foot stage 0 remains a diagnostic dilemma in patients with pain insensitivity. Conventional MRI may be helpful, when repeated for monitoring the treatment response.

Routine MRI findings of the asymptomatic foot in diabetic patients with unilateral Charcot foot.

BACKGROUND: Imaging studies of bones in patients with sensory deficits are scarce. AIM: To investigate bone MR images of the lower limb in diabetic patients with severe sensory polyneuropathy, and in control subjects without sensory deficits. METHODS: Routine T1 weighted and T2-fat-suppressed-STIR-sequences without contrast media were performed of the asymptomatic foot in 10 diabetic patients with polyneuropathy and unilateral inactive Charcot foot, and in 10 matched and 10 younger, non-obese unmatched control subjects. Simultaneously, a Gadolinium containing phantom was also assessed for reference. T1 weighted signal intensity (SI) was recorded at representative regions of interest at the peritendineal soft tissue, the tibia, the calcaneus, and at the phantom. Any abnormal skeletal morphology was also recorded. RESULTS: Mean SI at the soft tissue, the calcaneus, and the tibia, respectively, was 105%, 105% and 84% of that at the phantom in the matched and unmatched control subjects, compared to 102% (soft tissue), 112% (calcaneus) and 64% (tibia) in the patients; differences of tibia vs. calcaneus or soft tissue were highly significant (p < 0.005). SI at the tibia was lower in the patients than in control subjects (p < 0.05). Occult traumatic skeletal lesions were found in 8 of the 10 asymptomatic diabetic feet (none in the control feet). CONCLUSION: MR imaging did not reveal grossly abnormal bone marrow signalling in the limbs with severe sensory polyneuropathy, but occult sequelae of previous traumatic injuries.

Combined esophageal injury complicated by progression to a second perforation: a case report.

INTRODUCTION: Intramural dissection of the esophagus is a rare disorder characterized by a lesion between the submucosa and mucosa dividing the esophagus into a false and true lumen. The etiology of esophageal dissection remains uncertain but it affects predominantly women in their seventies and eighties. Symptoms may include uncharacteristic ones such as retrosternal pain, odynophagia or dysphagia. Conservative management is thought to be adequate and surgery should only be performed if complications such as abscess formation or perforation appear. Here we report the case and surgical management of a combined esophageal perforation and dissection. CASE PRESENTATION: We report the case of a combined esophageal perforation and dissection in a 45-year-old Caucasian woman with a history of relapsing periods of dysphagia since her childhood. The clinical course in this patient was complicated by progression to a second perforation, which made a definitive surgical management by esophagectomy necessary. CONCLUSION: To the best of our knowledge, this is the first reported case of a combined esophageal perforation and dissection complicated by progression to a second perforation. This emphasizes that cautious and intensive observation is necessary in patients with esophageal dissection.

Myocardial delayed contrast enhancement in patients with arterial hypertension: initial results of cardiac MRI.

PURPOSE: In arterial hypertension left ventricular hypertrophy comprises myocyte hypertrophy, interstitial fibrosis and structural alterations of the coronary microcirculation. MRI enables the detection of myocardial fibrosis, infarction and scar tissue by delayed enhancement (DE) after contrast media application. Aim of this study was to investigate patients with arterial hypertension but without known coronary disease or previous myocardial infarction to detect areas of DE. METHODS AND MATERIAL: Twenty patients with arterial hypertension with clinical symptoms of myocardial ischemia, but without history of myocardial infarction and normal coronary arteries during coronary angiography were investigated on a 1.0 T superconducting magnet (Gyroscan T10-NT, Intera Release 8.0, Philips). Fast gradient-echo cine sequences and T2-weighted STIR-sequences were acquired. Fifteen minutes after injection of Gadobenate dimeglumine inversion recovery gradient-echo sequences were performed for detection of myocardial DE. Presence or absence of DE on MRI was correlated with clinical data and the results of echocardiography and electrocardiography, respectively. RESULTS: Nine of 20 patients showed DE in the interventricular septum and the anteroseptal left ventricular wall. In 6 patients, DE was localized intramurally and in 3 patients subendocardially. There was a significant correlation between myocardial DE and ST-segment depressions during exercise and between DE and left-ventricular enddiastolic pressure. Patients with intermittent atrial fibrillation showed a myocardial DE more often than patients without atrial fibrillation. CONCLUSION: In our series, 45% of patients with arterial hypertension showed DE on cardiac MRI. In this clinical setting, delayed enhancement may be due to coronary microangiopathy. The more intramurally localization of DE, however, rather indicates myocardial interstitial fibrosis.

Tenosynovial giant cell tumors as accidental findings after episodes of distortion of the ankle: two case reports.

INTRODUCTION: Tenosynovial giant cell tumors are benign tumors of uncertain pathogenesis. They occur in the joints, tendons and synovial bursas. Due to a high recurrence rate of up to 50%, some authors call a giant cell tumor a semimalignant tumor. To date, less than 10 cases of tenosynovial giant cell tumor of the ankle have been published in the international medical literature. CASE PRESENTATION: In this case report, we present two patients with localized tumors that were detected accidentally after the occurrence of ankle sprains with persisting pain in the joint. The tumors were resected by open marginal surgery and regular follow-up examinations were carried out. CONCLUSIONS: We present an unusual occurrence of a tumor along with a possible follow-up strategy, which has not been previously discussed in the international literature.

Mucinous cystadenoma of the appendix misdiagnosed as cystic hydatid disease of the liver: a case report.

INTRODUCTION: Primary neoplastic lesions presenting with a mucocele of the appendix are very rare and can be divided into benign variants of mucinous adenomas or cystadenomas, mucinous tumours of uncertain malignant potential or mucinous cystadenocarcinomas. Most of these tumourous mucoceles are asymptomatic and are found incidentally. The major complication of neoplastic mucinous appendiceal tumours is the development of a pseudomyxoma peritonei due to spreading of mucin-producing cells within the abdominal cavity. CASE PRESENTATION: A 44-year-old man presented with a history of non-specific symptoms of right upper abdominal pain. Abdominal ultrasound and computed tomography scan identified a cystic mass consistent with the morphological characteristics of an echinococcal hydatid cyst. After completing systemic albendazole therapy, an explorative laparotomy revealed a cystic tumour of the appendix. Ileocaecal resection was performed and pathology reports confirmed the diagnosis of a mucinous cystadenoma of the appendix. The postoperative course was uneventful. CONCLUSION: Here we present the case of a man with a mucinous cystadenoma of the appendix mimicking cystic hydatid disease. We discuss the importance of re-evaluation and differential diagnostic reflections in cases of appendiceal mucocele.

Nonoperative treatment of neuro-osteoarthropathy of the foot: do we need new criteria?

The worst manifestation of neuro-osteoarthropathy of the pain-insensitive foot is the Charcot foot with its devastating osteoarticular destructions and irreversible deformities. New diagnostic tools such as MRI have revealed that mechanical injury and overuse is the origin of the condition. Traditionally, only feet with bone and joint damage apparent on plain radiographs (fracture and dislocation injuries) have undergone nonoperative treatment with off-loading and immobilization; however, treating painless, seemingly asymptomatic nonfracture injuries (bone bruise or bone marrow edema) with off-loading and immobilization has proven highly effective in preventing the Charcot foot. Whether pharmaceutical treatment has a role in terms of prevention or healing of osteoarticular destructions remains to be demonstrated.

Portal vein embolization and autologous CD133+ bone marrow stem cells for liver regeneration: initial experience.

PURPOSE: To prospectively evaluate the effectiveness of portal vein embolization (PVE) and CD133(+) bone marrow stem cell (BMSC) administration to the liver, compared with PVE alone, to augment hepatic regeneration in patients with large hepatic malignancies. MATERIALS AND METHODS: The study was approved by the institutional ethics committee; informed consent was obtained. Thirteen patients underwent PVE of liver segments I and IV-VIII to stimulate hepatic regeneration prior to extended right hepatectomy. In six patients (three men, three women; mean age, 61 years; range, 46-72 years) with a future liver remnant volume (FLRV) below 25% and/or limited quality of hepatic parenchyma, PVE alone did not promise adequate proliferation. These patients underwent BMSC administration to segments II and III (group I). In seven patients (three men, four women; mean age, 69 years; range, 63-75 years) with an FLRV below 25%, PVE alone was performed (group II). Two radiologists blinded to patients' identity and each other's results measured liver and tumor volumes with helical computed tomography. Absolute, relative, and daily FLRV gains were compared by using the t test or the Wilcoxon test. RESULTS: The increase of the mean absolute FLRV in group I from 239.3 mL +/- 103.5 (standard deviation) to 417.1 mL +/- 150.4 was significantly higher than that from 286.3 mL +/- 77.1 to 395.9 mL +/- 94.1 in group II (P = .049). The relative gain of FLRV after PVE in group I (77.3% +/- 38.2) was significantly higher than that in group II (39.1% +/- 20.4) (P = .039). The daily hepatic growth rate in group I (9.5 mL/d +/- 4.3) was significantly superior to that in group II (4.1 mL/d +/- 1.9) (P = .03). Time to surgery was 27 days +/- 11 in group I and 45 days +/- 21 in group II (P = .057). CONCLUSION: In patients with malignant liver lesions, the combination of PVE with CD133(+) BMSC administration substantially increased hepatic regeneration compared with PVE alone.

Positive effects of nitric oxide on left ventricular function in humans.

AIMS: The myocardial effect of tonically released nitric oxide (NO) in humans is still not known. We tested the hypothesis that low-dose NO exerts positive effects on left ventricular (LV) function. METHODS AND RESULTS: Twelve healthy volunteers, 26+/-4 years, were enrolled in this study. Magnetic resonance imaging was used to precisely measure the direct effects of NO on stroke volume index (SVI). The NO pool was monitored by chemiluminescence. We reduced endogenous NO levels with intravenous infusion of the NO synthase-inhibitor N(G)-monomethyl-l-arginine. Replenishment of the NO pool was achieved with the NO donor S-nitrosoglutathione (GSNO) (0.5 micromol iv). To differentiate load-dependent from the direct effects of NO on LV function, changes in SVI in response to GSNO were compared with changes in the NO-independent vasodilator dihydralazine (2.5 mg iv) at matched arterial pressure and heart rate. Inhibition of NO synthesis was followed by reduction in SVI. Subsequent replenishment of the circulating NO with GSNO significantly increased SVI (39+/-8 to 54+/-7 mL m(-2); P=0.001), whereas no significant changes were observed with the NO-independent vasodilator dihydralazine (39+/-8 to 46+/-8 mL m(-2); P=0.0626). CONCLUSION: Inhibition of endogenous NO release reduces, whereas replenishment with exogenous NO increases LV function, pointing towards a positive effect of tonically released NO on LV function in healthy humans.

Superior effects of high-dose enzyme replacement therapy in type 1 Gaucher disease on bone marrow involvement and chitotriosidase levels: a 2-center retrospective analysis.

Dosing of enzyme replacement therapy (ERT) for Gaucher disease type 1 is still a subject of debate and varies from 15 to 130 U/kg/mo, making a huge economic difference of 70,000 US dollars to 380,000 US dollars(euro55,000-300,000) per patient per year. To investigate whether this difference in dosing ultimately translates into a different response, we retrospectively compared long-term outcome of ERT at 2 large European treatment centers, Academic Medical Center, Amsterdam, The Netherlands (n = 49, median dose, 15-30 U/kg/4 wks) and Heinrich-Heine University, Duesseldorf, Germany (n = 57, median dose, 80 U/kg/4 wks). These adult cohorts had a similar genetic background. All follow-up parameters were matched separately at baseline, to avoid bias with respect to disease severity. Improvement in hemoglobin, platelet count, and hepatosplenomegaly was not significantly different between both cohorts, whereas plasma chitotriosidase and bone marrow involvement by magnetic resonance imaging improved more quickly and was more pronounced in the higher-dosed group. Major bone complications rarely occurred in both groups. In conclusion, different dosing regimens of ERT do not affect outcome of hematologic and visceral parameters, but higher dosing leads to accelerated decrease of chitotriosidase and better objective bone response in adult type 1 Gaucher disease.

Portal application of autologous CD133+ bone marrow cells to the liver: a novel concept to support hepatic regeneration.

The liver has a large capacity for regeneration after resection. However, below a critical level of future liver remnant volume (FLRV), partial hepatectomy is accompanied by a significant increase of postoperative liver failure. There is accumulating evidence for the contribution of bone marrow stem cells (BMSCs) to participate in liver regeneration. Here we report on three patients subjected to intraportal administration of autologous CD133(+) BMSCs subsequent to portal venous embolization of right liver segments, used to expand left lateral hepatic segments as FLRV. Computerized tomography scan volumetry revealed 2.5-fold increased mean proliferation rates of left lateral segments compared with a group of three consecutive patients treated without application of BMSCs. This early experience with portovenous application of CD133(+) BMSCs could suggest that this novel therapeutic approach bears the potential of enhancing and accelerating hepatic regeneration in a clinical setting.

Follicular lymphoid hyperplasia simulating intussusception in a 6-year-old boy: clinical, radiological and histopathological findings.

We report the case of a 6-year-old boy who initially presented with recurrent abdominal pain. Diagnostic imaging, including ultrasound and CT, showed findings typical of an ileocecal intussusception with abdominal lymphadenopathy. Sonographically, the morphological appearance of the intussusception did not change during a 4-week follow-up period. Surgery was performed on the tentative diagnosis of a tumour versus lymphoma. Upon laparatomy, intussusception was ruled out and a large, broad-based tumour of the caecum was palpable. The histopathology after ileocecal resection revealed follicular lymphoid hyperplasia. Where there is radiological suspicion of an intussusception in children with no or insignificant symptoms, follicular lymphoid hyperplasia should be included in the differential diagnosis.