Whole-heart cine MRI using real-time respiratory self-gating.

Two-dimensional (2D) breath-hold cine MRI is used to assess cardiac anatomy and function. However, this technique requires cooperation from the patient, and in some cases the scan planning is complicated. Isotropic nonangulated three-dimensional (3D) cardiac MR can overcome some of these problems because it requires minimal planning and can be reformatted in any plane. However, current methods, even those that use undersampling techniques, involve breath-holding for periods that are too long for many patients. Free-breathing respiratory gating sequences represent a possible solution for realizing 3D cine imaging. A real-time respiratory self-gating technique for whole-heart cine MRI is presented. The technique enables assessment of cardiac anatomy and function with minimum planning or patient cooperation. Nonangulated isotropic 3D data were acquired from five healthy volunteers and then reformatted into 2D clinical views. The respiratory self-gating technique is shown to improve image quality in free-breathing scanning. In addition, ventricular volumetric data obtained using the 3D approach were comparable to those acquired with the conventional multislice 2D approach.     

Congenital disorder of glycosylation type 1a: three siblings with a mild neurological phenotype.

We report 3 siblings (1 male and 2 female) recently diagnosed with congenital disorder of glycosylation type Ia (CDG-Ia) in their mid-20s. They experience mild mental retardation but manage to function independently in society. Their professions are library assistant, professional artistic painter and secretarial work. All three siblings have cerebellar hypoplasia and ataxia, but are able to ambulate easily. Two of the siblings have required strabismus surgical repairs. All have antithrombin III deficiency, osteoporosis, and mild dysmorphic features. Hypergonadotrophic hypogonadism was a feature of the two female siblings. A type 1 sialotransferrin pattern and phosphomannomutase (PMM) deficiency have been demonstrated. They are compound heterozygotes for R141H and L32R mutations in the PMM2 gene. While there is clinical heterogeneity in CDG-Ia, we believe that our patients are among the mildest of intellectually affected CDG-Ia patients reported to date.     

Multifocal calcifying epithelial odontogenic tumor.

The calcifying epithelial odontogenic tumor (CEOT), or Pindborg tumor, is a rare and benign odontogenic neoplasm that affects the jaw. The most common manifestation of CEOT is a unifocal or localized lesion of the involved jaw, which may appear clinically as a hard tissue swelling and radiographically as a mixed radiolucent-radiopaque mass. In this article, we present a unique case of CEOT affecting multiple sites in the maxilla and mandible of a 51-year-old white man. Though biopsy samples from all involved sites revealed similar histopathologic features consistent with CEOT, the fact that there was a multifocal presentation is an unusual phenomenon for CEOT and has never been reported. Multifocal odontogenic lesions are not typical but have been observed in conditions associated with known genetic mutations. For example, multiple odontogenic keratocysts are the most common feature of the inherited condition known as nevoid basal cell carcinoma syndrome. This case, however, is the first one to demonstrate that there may be a multifocal variant of CEOT that has not been previously recognized.     

HIV Facial Lipoatrophy: Causes and Treatment Options

Background. Human immunodeficiency virus (HIV)-associated facial lipoatrophy is becoming epidemic and may seriously affect quality of life.
Objective. To review the possible causes and treatment options for HIV facial lipoatrophy.
Methods. This article is based on a review of the medical literature and the author's clinical experience in treating HIV facial lipoatrophy.
Conclusion. Although absorbable injectable fillers and implants are helpful in treating HIV facial lipoatrophy, they are limited by cost and the short duration of correction. Newer forms of longer-lasting fillers (poly-l-lactic acid [Sculptra]) and permanent injectable fillers (liquid injectable silicone) are proving useful for treatment of this condition.     

Endocrinology: Predictive value of serum oestradiol concentrations and oocyte number in severe ovarian hyperstimulation syndrome

Ovarian hyperstimulation syndrome (OHSS) is a serious complicationof gonadotrophin usage but it is difficult to accurately predictits occurrence. Previous investigators have identified the combinationof high oestradiol concentrations and oocyte number as beingpredictive in 80% of cases. In this study we sought to identifythe incidence of severe OHSS in patients with high oestradiolconcentrations and large numbers of oocytes and to evaluatethe importance of pregnancy in the development of OHSS. Between1990 and 1993, we studied 139 cycles using two assisted reproductivetechniques [oocyte donor, n =72; in-vitro fertilization (IVF),n = 67] in which either oestradiol (>4000 pg/ml), oocytenumber (>25), or both were elevated. OHSS was diagnosed bystandard criteria. There were no cases of severe OHSS in theoocyte donor group and six in the IVF group. Among 10 patientswith oestradiol concentration >6000 pg/ml and >30 oocytes,only one had OHSS (10%). The relative risk of OHSS with pregnancywas 12 (confidence interval 2.18–66.14). We conclude thatthe risk of OHSS even at high levels of stimulation is lowerthan previously believed. Secondly, donors have a very low riskof OHSS, probably because of the absence of pregnancy. As such,cryopreservation of all oocytes in IVF cycles is a reasonablealternative to cycle cancellation or use of adjunctive medication.     

Postirradiation malignant fibrous histiocytoma expressing cytokeratin. Implications for the immunodiagnosis of sarcomas

A malignant fibrous histiocytoma of the sacrum complicating the course of radiation therapy for endometrial carcinoma is presented. Although the tumor fulfilled the clinical, radiologic, and histologic criteria for a postirradiation malignant fibrous histiocytoma of bone, it also expressed cytokeratin. That this immunoreactivity reflected keratin synthesis by the tumor and not an unusual pattern of cross-reactivity with another intermediate filament such as vimentin is strongly suggested by the reproducibility of the immunoreactivity utilizing both polyclonal and monoclonal antibodies and extinction of the immunoreactivity following absorption of the primary antiserum with keratin proteins. This is the first reported instance of keratin expression by a malignant fibrous histiocytoma; it indicates that sarcomas apart from synovial sarcoma and epithelioid sarcoma may sometimes express this protein.     

Effect of hepatic artery chemotherapy on survival of patients with hepatic metastases from colorectal carcinoma treated with cryotherapy

Thirty-eight patients with unresectable multiple liver metastases from colorectal carcinoma were treated with either hepatic artery chemotherapy (HAC) and cryotherapy (n=27) or cryotherapy alone (n=11). Follow-up survival data were summarized using Cox regression. Allowing for the effect of the pathology of the primary tumor and the preoperative carcinoembryonic antigen (CEA) level, those patients who did not receive HAC after cytoreduction were three times as likely to die as those given HAC (RR 3.3, 95%; CI 1.2–9.3). The estimated median survival of patients treated with cryotherapy alone was 245 days, whereas for those given more than 3 months of HAC plus cytoreduction therapy it was 570 days. It is recommended that all patients who receive cryotherapy for multiple liver metastases from colorectal rectal carcinoma be given subsequent hepatic artery chemotherapy.

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Resumen En el presente estudio, 38 pacientes con metástasis hepáticas múltiples y no resecables de carcinoma colo-rectal fueron tratados con quimioterapia administrada en la arteria hepática (HAC) y crioterapia (n=27) o crioterapia sola (n=11). Los datos del seguimiento fueron resumidos según el método de regresión de Cox. Teniendo en cuenta el efecto de la patología del tumor primario y el nivel preoperatorio de antígeno carcino-embrionario, se halló que aquellos pacientes que no recibieron HAC luego de la citorreducción tuvieron una probabilidad de muerte 3 veces mayor que los que recibieron HAC (RR 3.3, 95% CI 1.2 a 9.3). La sobrevida media estimada de los pacientes tratados con crioterapia sola fue de 245 días, en tanto que aquellos que recibieron HAC por tres meses y terapia de citorreducción fue de 570 días. Se recomienda que todos los pacientes que reciben crioterapia para metástasis hepáticas múltiples de carcinoma colo-rectal reciban luego quimioterapia por vía de la arteria hepática.

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Résumé Trente-huit patients ayant des métastases hépatiques multiples non reséquables d'origine colorectale ont été traités soit par chimiothérapie par voie artérielle (CVA) associée à la cryothérapie (n=27) soit par cryothérapie seule (n=11). Les survies ont été analysées selon la méthode d'analyse du Modèle de Cox. En tenant compte de l'effet de la pathologie de la tumeur primitive et du niveau préopératoire de l'ACE, les patients n'ayant pas eu de de décéder que ceux qui en ont eu (RR 3.3, 95% IC 12 à 9.3). L'estimation de la survie médiane des patients traités par la cytoréduction seule a été de 245 jours, alors que celle des patients traités par les deux avec une CVA d'au moins trois mois, a été de 570 jours. On recommande que tous les patients ayant des métastases multiples du foie à partir des cancers colorectaux aient une CVA par la suite.

     

Assessment of the Severity of Asthma in a Family Practice

The objective of this study was to evaluate clinical history and self-perception of severity as predictors of asthma severity. A short-term longitudinal study was conducted in a family practice in Melbourne, Australia, utilizing peak flow monitoring, medication diary, and self-administered asthma severity questionnaire. Seventy-two asthmatic subjects with a positive bronchodilator or exercise test, aged between 6 and 79 years, were studied. Symptom and treatment items were correlated with peak flow variability and minimal peak expiratory flow rate (PEFR). An asthma severity scale was generated using the partial credit version of Item Response Theory and the participants' severity scores were validated against lung function tests and medication usage. Quantitative modeling procedures were used to investigate the interrelationships of factors associated with peak flow variability. Severity scores demonstrated significant relationships with peak flow variability (partial r = 0.34) and treatment items. Self-perceived severity of asthma in the preceding 2 weeks showed significant association with peak flow variability (partial rho = 0.46) and minimal PEFR (rho = -0.41). The severity module of the Monash Respiratory Questionnaire is a valid and reliable instrument. The most important symptoms appear to be the frequency of use of bronchodilator and frequency of nocturnal attacks. A carefully structured clinical history in conjunction with the peak flow criteria of variability and minimal peak flow rate would be appropriate in the evaluation of asthma severity. Patients' self-perception of the severity of their asthma needs further evaluation.