Case Report: Second case of a successful pregnancy in maternal isovaleric acidaemia

Summary: A female patient with isovaleric acidaemia had a successful outcome from pregnancy.     

Abnormal spinal interactions from hand afferents to forearm muscles in writer's cramp.

OBJECTIVE: Spinal reflexes from hand to wrist muscles were investigated in writer's cramp. METHODS: Stimulus-triggered rectified EMG averages after ulnar nerve and cutaneous stimulation, in wrist flexors and extensors during tonic contraction, were compared in 18 controls and 19 patients. RESULTS: On the patient dystonic side, ulnar-induced EMG suppression was decreased in wrist extensors, and facilitation in wrist flexors modified dependent on the dystonic wrist posture during writing. No change was found on the patient non-dystonic side. Cutaneous stimulation increased wrist flexor EMG on both sides of the patients with normal wrist posture during writing, but had no effect in controls and patients with abnormal wrist posture. CONCLUSIONS: Comparison between cutaneous and mixed nerve stimuli suggests that spindle afferents from intrinsic hand muscles may mediate patients' ulnar-induced EMG modulations. Abnormal proprioceptive control was only observed on dystonic side, while bilateral unusual cutaneous control was found in patients. Changes in spinal transmission were partly related to the dystonic wrist posture, suggesting that systems involved in sensory processing can be differentially altered in writer's cramp. SIGNIFICANCE: Changes in spinal transmission, probably related to peripheral and/or cortical inputs, might either take part in primary or adaptive mechanisms underlying writer's cramp.     

The first 1000 cases of Italian Endovenous-laser Working Group (IEWG). Rationale, and long-term outcomes for the 1999-2003 period.

AIM: The innovations for disease management need to be thoroughly evaluated so that their benefits and potential downsides can be compared with the already existing approaches. Endovascular laser (EVL) treatment for varicose veins offers today several advantages over surgical standard stripping. The Italian Endovenous-laser Working Group (IEWG) is a homogeneous group of surgeons and phlebologists who have been using EVL since 1999 and has undertaken to examine EVL in a multicenter study starting from a well defined rationale, with the benefit of a single protocol to use. METHODS: In a cooperative, multicenter, clinical study, 1076 limbs in 1050 patients, mean age of 54.5 years, 241 males and 809 females affected by chronic venous insufficiency (CVI) were considered eligible for surgery and stratified by CEAP classification in a four-year period (January 1999 December 2003). Inclusion criteria were insufficiency of the great and/or small saphenous vein at various levels, beyond those accessory saphenous trunks with incompetence in the saphenofemoral junction. In all cases truncular reflux apparead up on duplex scan examination, with or without associated varicosities. All the patients underwent a surgery on the basis of the clinical assessment. All the centres involved performed treatment in conformity with the Food and Drug Administration (FDA) validated procedure, using an endo-laser venous system kit with a 810-980 nm diode. Duplex scan was performed in all patients after 36 months with very few lost to follow-up cases. RESULTS: In the immediate postoperative period the results have been impressive, with a very effective closure of incompetent great saphenous vein and the other treated varicose veins (the early occlusion rate has been 99%). Major complications have not been detected: in particular, no deep venous thrombosis (DVT) evaluated duplex ultrasound. The patients' acceptability and satisfaction regarding the procedure, have been measured by means of a questionnaire on the quality of life, and the result was 96.7%. After 36 months, the total occusion rate of saphenous trunks has been 97%. CONCLUSIONS: The first important Italian experience with EVL based on preoperative, perioperative and postoperative duplex control and which is also based on the patients' satisfaction at mid/long-term has indicated some advantages over the standard treatment with the stripping method. In terms of reduced postoperative pain, shorter sick leave, a faster resumption of the normal activities, and, in particular, the total absence of DVT, we can conclude that EVL is a good solution for all patients with anatomic and hemodinamic patterns for saphenous vein surgery.     

The diagnostic utility of immunohistochemistry and electron microscopy in distinguishing between peritoneal mesotheliomas and serous carcinomas: a comparative study.

The histologic distinction between peritoneal epithelioid mesotheliomas and serous carcinomas diffusely involving the peritoneum may be difficult, but it can be facilitated by the use of immunohistochemistry and electron microscopy. D2-40 and podoplanin are two recently recognized lymphatic endothelial markers that can be expressed in normal mesothelial cells and mesotheliomas. The purpose of this study is to compare the value of these new mesothelial markers with those that are commonly used for discriminating between mesotheliomas and serous carcinomas, and also to determine the current role of electron microscopy in distinguishing between these malignancies. A total of 40 peritoneal epithelioid mesotheliomas and 45 serous carcinomas of the ovary (15 primary, 30 metastatic to the peritoneum) were investigated for the expression of the following markers: D2-40, podoplanin, calretinin, keratin 5/6, thrombomodulin, MOC-31, Ber-EP4, B72.3 (TAG-72), BG-8 (Lewis(Y)), CA19-9, and leu-M1 (CD15). All 40 (100%) of the mesotheliomas reacted for calretinin, 93% for D2-40, 93% for podoplanin, 93% for keratin 5/6, 73% for thrombomodulin, 13% for Ber-EP4, 5% for MOC-31, 3% for BG-8, and none for B72.3, CA19-9, or leu-M1. All 45 (100%) serous carcinomas were positive for Ber-EP4, 98% for MOC-31, 73% for B72.3, 73% for BG-8, 67% for CA19-9, 58% for leu-M1, 31% for keratin 5/6, 31% for calretinin, 13% for D2-40, 13% for podoplanin, and 4% for thrombomodulin. After analyzing the results, it is concluded that Ber-EP4 and MOC-31 are the best negative mesothelioma markers for differentiating between epithelioid mesotheliomas and serous carcinomas. The best discriminators among the positive markers for mesotheliomas are D2-40, podoplanin, and calretinin. From a practical point of view, Ber-EP4 and MOC-31, in combination with calretinin, and/or D2-40 or podoplanin allow the differential diagnosis to be established between mesothelioma and serous carcinoma in nearly all instances. As a clear distinction could be made between these two malignancies in all of the cases in which electron microscopy was performed, this technique can be very useful in establishing the correct diagnosis when the immunohistochemical results are equivocal or further support of a diagnosis of either mesothelioma or serous carcinoma is needed.     

Effect of high up front charges on access to surgery for poor patients at a public hospital in New Mexico

Background  

A public hospital in New Mexico required collection of 50% of estimated costs prior to elective surgeries for self-pay patients. This study assesses the impact of this policy on access to elective surgical procedures.