Fetal cardiac rhabdomyoma with maternal tuberous sclerosis complicating pregnancy

INTRODUCTION: Tuberous sclerosis is an autosomal dominant disorder of hamartoma formation that can manifest as cardiac or central nervous system lesions and adversely affect maternal and fetal outcome. CASE REPORT: We report a case of maternal tuberous sclerosis with fetal cardiac rhabdomyoma detected in utero at 26 weeks gestation. CONCLUSION: To conclude, a pregnancy complicated by maternal or fetal tuberous sclerosis deserves careful vigilance and the fetus should undergo prenatal fetal Doppler echocardiography and if possible an MRI, so that parents can be counseled regarding its future prognostic implications.     

Non-cirrhotic portal hypertension and pregnancy outcome

OBJECTIVE: Non-cirrhotic portal hypertension (NCPH) is a common cause of portal hypertension in developing countries, especially Asia. Recent data have shown near-normal reproductive function and good pregnancy outcome for NCPH compared with cirrhosis. The aim of the present study was to evaluate complications during pregnancy and pregnancy outcome in women with NCPH. METHODS: Twelve pregnancies in five patients with NCPH were evaluated. RESULTS: In the present study, 20% of patients had hematemesis during pregnancy that was managed successfully. Four patients (80%) had severe anemia (hemoglobin 6-7 g%) and three (60%) had thrombocytopenia. There were no preterm births. Of a total of nine live births, six were vaginal deliveries and three were delivered by Cesarean section (performed for obstetric indications). Four babies (44%) were small for gestational age (SGA). CONCLUSIONS: The results show that patients with NCPH have normal fertility, no apparent increase in the incidence of hematemesis during pregnancy, an increased incidence of SGA babies and no indication for elective Cesarean delivery.     

Mixed epithelial and stromal tumor of the kidney in a puerperal woman

Puerperal pyrexia is still rampant, especially in third world countries, and is usually due to puerperal sepsis, urinary tract infections, upper respiratory infection, and breast infection. Rarely, in third world countries like India, it may be due to tuberculosis, malaria, typhoid, and so on, which are also rampant in the general population. Mixed epithelial and stromal tumor of the kidney (MESTK) is a recently recognized subset of renal tumors composed mainly of smooth muscle cells in which epithelial structures are embedded. It usually occurs in middle aged and older women. In the present case report, a 36-year-old woman presented with puerperal pyrexia, possibly due to tuberculosis and with an incidental mixed epithelial and stromal tumor of the kidney causing complex ascitis and fever, which required nephrectomy that was followed by full recovery. This case report highlights the importance of keeping MESTK in mind even in younger women with asymptomatic renal mass. It also highlights the importance of keeping renal tumors in mind as a possibility and to perform proper investigations for adequate treatment and recovery.     

Age and sex effects on brain morphology

1. 1. Brain morphology can be assessed readily in vivo using magnetic resonance imaging (MRI).

2. 2. In this study, the effects of age and sex on whole-brain morphology were examined using an operator-controlled computer-segmentation protocol.

3. 3. Results indicated that age was associated with gray-matter volume reduction.

4. 4. Brain-size differences between males and females were primarily attributable to whitematter volume.

5. 5. This study confirms the importance of controlling for age and sex in brain-morphology studies.

     

Hypoxia stimulus: An adaptive immune response during dendritic cell maturation

The 'injury hypothesis' in organ transplantation suggests that ischemia-reperfusion injury is involved in the adaptative alloimmune response. We previously found that a strong immune/inflammatory response was induced by ischemia during kidney transplantation in rats. We show here that immature dendritic cells (DCs) undergo hypoxia-mediated differentiation comparable to allogeneic stimulation. Hypoxia-differentiated DCs overexpress hypoxia inducible factor-1alpha (HIF-1alpha) and its downstream target genes, such as vascular endothelial growth factor or glucose transporter-1. Rapamycin attenuated DC differentiation, HIF-1alpha expression, and its target gene expression in a dose-dependent manner along with downregulated interleukin-10 secretion. Coculture of hypoxia-differentiated DCs with CD3 lymphocytes induced proliferation of lymphocytes, a process also neutralized by rapamycin. Furthermore, in vivo examination of ischemia-reperfusion-injured mouse kidneys showed a clear maturation of resident DCs that was blunted by rapamycin pretreatment. Our results suggest that hypoxia is a central part of the 'injury hypothesis' triggering DC differentiation under hypoxic conditions. Rapamycin attenuates the hypoxic immune-inflammatory response through inhibition of the HIF-1alpha pathway.