Reconstituting pancreas development from purified progenitor cells reveals genes essential for islet differentiation

Developmental biology is challenged to reveal the function of numerous candidate genes implicated by recent genome-scale studies as regulators of organ development and diseases. Recapitulating organogenesis from purified progenitor cells that can be genetically manipulated would provide powerful opportunities to dissect such gene functions. Here we describe systems for reconstructing pancreas development, including islet β-cell and α-cell differentiation, from single fetal progenitor cells. A strict requirement for native genetic regulators of in vivo pancreas development, such as Ngn3, Arx, and Pax4, revealed the authenticity of differentiation programs in vitro. Efficient genetic screens permitted by this system revealed that Prdm16 is required for pancreatic islet development in vivo. Discovering the function of genes regulating pancreas development with our system should enrich strategies for regenerating islets for treating diabetes mellitus.     

Aplasia cutis congenita. Report on 5 family cases involving the scalp.

Aplasia cutis congenita (ACC) is a rare malformation involving the scalp and sometimes the underlying tissues. It may occur as an isolated defect or be combined with congenital malformations and there is often a family background. The mortality rate is high, with infectious and vascular complications. We report our experience based on the treatment of 5 ACC of the scalp in children, where a family pathology was found.Controlled healing is the best approach for us, even for the serious forms.After the acute period, treatment is based on skin expansion and must be followed by genetic screening of the family.     

Impact of glycemic control on serum lipoprotein (a) in Arab children with type 1 diabetes

BACKGROUND: Lipoprotein (a) (Lp (a)) is an independent risk factor for coronary artery disease (CAD), a major cause of death in patients with type 1 diabetes mellitus. Both type 1 diabetes and CAD represent major problems in Kuwait. Data on the effect of metabolic control on Lp (a) in diabetic children are limited and this is particularly true for Arab children. The objectives of the present study were to analyze serum Lp (a) levels in patients with type 1 diabetes compared with non-diabetic children, taking into account the effect of glycemic control. METHODS: Circulating lipids, including Lp (a), were measured in serum samples from 60 prepubertal non-diabetic children and 58 prepubertal children with type 1 diabetes. Comparisons of Lp (a) concentrations were made between the non-diabetic and diabetic children with good to fair control (glycosylated hemoglobin (GHb) <11%) and a group of diabetic children with poor control (GHb > or = 11%). RESULTS: The mean serum Lp (a) level in all diabetic children was 187.62+160.43 mg/L, compared with 162.88+156.06 mg/L in the control group. The group of children with poor glycemic control had higher median Lp (a) levels (147.50 mg/L) than either the group of diabetic children with good to fair control (95 mg/L; P<0.028) or the group of non-diabetic children (125 mg/L; P<0.04). Moreover, 38.3% of poorly controlled diabetic children had elevated Lp (a) levels > or = 250 mg/L, compared with 12.5% of diabetic children with good to fair control and 16.7% of non-diabetic children (P<0.025 and P<0.039, respectively). No association was found between Lp (a), diabetes duration and insulin dose. CONCLUSIONS: In Arab children, highest Lp (a) levels are associated with poorest metabolic control. The prevalence of Lp (a) levels associated with cardiovascular risk is higher in poorly controlled diabetic children. Increased levels of Lp (a) may be another contributing factor to the high risk for CAD in diabetic patients.     

Potential of 1H MR spectroscopic imaging to segregate patients who are likely to show malignancy of the peripheral zone of the prostate on biopsy

Purpose

To evaluate the potential of MR spectroscopic imaging (MRSI) to segregate patients who, upon prostate biopsy, are more likely to show a malignancy in the peripheral zone (PZ) of the prostate gland.

Materials and Methods

Before biopsy, MRSI was carried in 123 men with elevated prostate specific antigen level or an abnormal digital rectal examination. After the MRSI investigation, all patients underwent systematic transrectal ultrasound guided biopsy and were categorized using standard random number tables into the following two groups: (i) Group I, a 62 member training set; and (ii) Group II, a 61‐member test set. The cutoff value for the [citrate/(choline+creatine)] ratio for patients in the training set was obtained using the receiver operating characteristic (ROC) curve method. This value was then applied to the test set of patients as well.

Results

The ROC method gave a cutoff value of 1.2 for the [Citrate/(Choline+Creatine)] ratio. When applied as a malignancy‐predictor to the test group of patients (Group II), the ROC method generated the following results: sensitivity, 77%; specificity, 83%; negative predictive value, 93%; positive predictive value, 55% and accuracy, 82%.

Conclusion

The results indicate that patients who are deemed as malignancy‐positive in the PZ by MRSI using the ROC cutoff may be subjected to prostate biopsy to confirm the diagnosis of cancer. J. Magn. Reson. Imaging 2009. © 2009 Wiley‐Liss, Inc.     

内窥镜胸交感神经链切断术治疗多汗症

目的 评价内镜下胸交感神经链切断术治疗多汗症的临床效果。方法 回顾性分析内镜下胸交感神经链切断术治疗58例多汗症的结果。结果 58例均治愈；出现气胸4例、代偿性多汗2例、肋间神经痛l例。结论 内镜下胸交感神经链切断术比较安全。     

维吾尔医诊断银屑病回顾性调查分析

目的回顾新疆5家维吾尔医医院10年以来诊疗银屑病临床资料,分析该病在人群中分布、发病规律、维吾尔医诊断技术等。方法对1998-2008年期间在新疆维吾尔自治区、喀什地区、和田地区、库尔勒市维吾尔医医院及新疆维吾尔医学专科学校直属医院5家医院确诊为银屑病并接受住院治疗的495例患者住院病历进行人口、诊断学资料为主的回顾性调查,分析总结维吾尔医银屑病临床诊断技术。结果 495例中,白里海密海日台比依（异常黏液质）最多,其次是赛非拉依海日台比依（异常胆液质）、赛危达依海日台比依（异常黑胆质）,胡尼海日台比依（异常血液质）比较少。结论单纯发生银屑病者,由于引起的合立体海日台比依（异常体液）种类,患者密杂吉（气质）不同,而表现出各种症状体征。通过维吾尔医辨证分析,正确判断致病合立体（体液）性质及异常气质类型,才能正确判断疾病性质,指导临床治疗。     

Architecture of the medial gastrocnemius in children with spastic diplegia

Ultrasound images were obtained of the medial gastrocnemius at different ankle joint positions with the knee extended. Fascicle length and deep fascicle angle were measured in five normally developing adults (mean age 33 years, age range 24 to 36 years) and in five normally developing children (mean age 7.8 years, age range 7 to 11 years), and in seven children with spastic diplegia (mean age 10 years, age range 6 to 13 years). These architectural variables were similar in the groups of normally developing adults and children. Importantly, no statistical difference could be found between the normally developing children and those with diplegia for fascicle length. Deep fascicle angles were reduced significantly in the clinical group at a particular ankle joint angle but not at the resting angles. The difference in deep fascicle angles is explained as a function of resting muscle length and is not attributed any clinical importance. Our results do not explain the structural origin of muscle contracture explicitly. However, they do indicate that most of the fixed shortness in the medial gastrocnemii of ambulant children with spastic diplegia is not due to reduced muscle fascicle length. We suggest that muscle contracture may be better explained in terms of shortness of the aponeuroses of pennate muscles, such as the medial gastrocnemius, through reduced muscle fascicle diameter.     

Acute Compartment Syndrome After Rupture of the Medial Head of Gastrocnemius in a Child

Rupture of the gastrocnemius muscle is an uncommon injury, with most cases occurring in athletes and, typically, presenting with the acute onset of focal calf pain and ecchymosis after injury. Although gastrocnemius ruptures are usually treated symptomatically with good results, we present an unusual case of a medial head of gastrocnemius muscle tear complicated by acute compartment syndrome in a 7-year-old boy whose right calf was crushed in a fall. After confirmation of the diagnosis of compartment syndrome, the patient underwent emergency fasciotomy with evacuation of hematoma, and, thereafter, he recovered unremarkably. Clinicians and surgeons need to maintain a high index of suspicion for compartment syndrome associated with gastrocnemius muscle injury, so that timely surgical decompression can be undertaken and complications related to delayed diagnosis and treatment can be avoided.     

Meckel''s Cave Tuberculoma with Unusual Infratemporal Extension

The authors describe a rare case of intracranial tuberculoma of the Meckel's cave and cavernous sinus with extension into the infratemporal fossa causing widening of the foramen ovale and adjacent bone destruction. The rarity of the lesion and the unusual extension of the lesion are presented with a brief review of literature.