Techniques to avoid hypothermic circulatory arrest in the management of renal tumor with right atrium extension in children.

Clear cell sarcoma of the kidney is a rare variety of renal tumor accounting for less than 5% of all pediatric renal tumors. Cardiopulmonary bypass along with hypothermic circulatory arrest is frequently used for management of tumor thrombus extending into supra-hepatic inferior vena cava and right atrium. In this paper, we present a strategy of avoiding circulatory arrest and hypothermia and thereby fast-tracking the recovery in managing a case of clear cell sarcoma of the kidney in a 3.5-year-old child with tumor thrombus extending into the right atrium.

Single stage repair of anorectal malformation with rectovestibular fistula in adult.

Anorectal malformations are a wide spectrum of disorders, affecting both genders and rare adult presentation as with increased knowledge and advancements, the majority of cases are diagnosed and rectified at birth. This case is a classic example of delayed presentation caused by illiteracy and a lack of adequate health care in rural locations and highlights the uniqueness of this disease, presentation in adult age group and its management and effect on patient psychology and mental health. Because all low anorectal abnormalities can be treated in a single stage, neonatal assessment at birth and early referral to advanced health care centres are critical, with anterior or posterior sagittal anorectoplasty performed by the age of six months. Concerning the anomaly, parent education is an important aspect of patient care.

Genistein potentiates Centchroman induced antineoplasticity in breast cancer via PI3K/Akt deactivation and ROS dependent induction of apoptosis.

AIMS: Recently, strategies of cancer treatment using combination of agents with distinct molecular mechanism(s) of action are considered more promising due to its high efficacy and reduced systemic toxicity. The study is aimed to improve the efficacy of selective estrogen receptor modulator, Centchroman (CC) by combination with the phytoestrogen Genistein (GN). METHODS: Cytotoxicity was evaluated by Sulforhodamine B assay. Cell cycle analysis was done through flow cytometry. Further, Apoptosis was analyzed using Annexin V/PI staining, tunel assay and electron microscopic examination and verified using western blot analysis. In order to validate the in vitro results, in vivo analysis was performed using 4T1-syngeneic mouse model. KEY FINDINGS: In this study, we report that the dietary isoflavone genistein (GN) synergistically improved antineoplasticity of CC in breast cancer by arresting cells at G2/M phase culminating in ROS dependent apoptosis. The combination of CC plus GN caused dysregulation of Bax and Bcl-2 ratio inducing mitochondrial dysfunction, activation of Caspase-3/7, -9 and PARP cleavage. Further, combination significantly suppresses phosphorylation of PI3K/Akt/NF-κB, enhancing apoptosis. Additionally, combination markedly reduced tumor growth compared to CC and GN alone in mouse 4T1 breast tumor model. SIGNIFICANCE: Together, these studies suggest that GN represents a potential adjunct molecule whose role in CC induced apoptosis deserves attention.

Neem Seed Oil Induces Apoptosis in MCF-7 and MDA MB-231 Human Breast Cancer Cells

Background: In traditional Indian medicine, azadirachta indica (neem) is known for its wide range of medicinal properties. Various parts of neem tree including its fruit, seed, bark, leaves, and root have been shown to possess antiseptic, antiviral, antipyretic, anti-inflammatory, antiulcer, antimalarial, antifungal and anticancer activity. Materials and Methods: MCF-7 and MDA MB-231 cells were exposed to various concentrations of 2% ethanolic solution of NSO (1-30 µl/ml) and further processed for cell viability, cell cycle and apoptosis analysis. In addition, cells were analyzed for alteration in Mitochondrial Membrane Potential (MMP) and generation of Reactive Oxygen Species (ROS) using JC-1 and DCFDA staining respectively. Results: NSO give 50% inhibition at 10 µl/ml and 20 µl/ml concentration in MCF-7 and MDA MB-231 cells respectively and, arrests cells at G0/G1 phase in both the cell types. There was a significant alteration in mitochondrial membrane potential that leads to the generation of ROS and induction of apoptosis in NSO treated MCF-7 and MDA MB-231 cells. Conclusion: The results showed that NSO inhibits the growth of human breast cancer cells via induction of apoptosis and G1 phase arrest. Collectively these results suggest that NSO could potentially be used in the management of breast cancer.

Polymorphism of 3' UTR of MAMLD1 gene is also associated with increased risk of isolated hypospadias in Indian children: a preliminary report.

OBJECTIVE: To study MAMLD1 gene polymorphisms, serum LH and testosterone levels amongst Indian children with isolated hypospadias (IH) and controls. MATERIALS AND METHODS: Screening of the MAMLD1 gene was performed by PCR sequencing method in 100 Indian children aged 0-12 years presenting with IH and 100 controls. LH and testosterone hormone levels were also assessed (categorized in four age-wise groups). RESULTS: IH subjects had significantly higher incidence of MAMLD1 polymorphism as compared to controls (33 vs 15 %, p = 0.01). Of various genomic variants identified in this study, the noteworthy novel ones were missense mutation P299A and single nucleotide polymorphism c.2960C>T in 3' UTR of Exon 7. While p 299A was found to cause protein structural instability consequent to amino acid change, eighty percent subjects with c.2960C>T in 3' UTR of Exon 7 (corresponding to newly discovered currently non-validated exon 11) were found to have lower testosterone levels when compared with their age group mean. IH showed statistically higher incidence of c.2960C>T in comparison to controls (22 vs 10 %, p value 0.046) and about 2.5-folds higher risk of this anomaly. CONCLUSION: Occurrence of MAMDL1 gene polymorphisms, specially of c.2960C>T in 3' UTR of its exon 7 is associated with a higher risk of IH in Indian children, probably by lowering androgenic levels.

The Case of Sacrococcygeal Teratoma in an IVF Pregnancy: Is There any Association between Congenital Tumors and Assisted Reproduction Techniques?

BACKGROUND: Sacrococcygeal teratoma (SCT) is a rare congenital tumor and its association with IVF pregnancy is not clear. There are limited reports of congenital tumors in IVF pregnancy. The exact embryogenesis of SCT is not known but a genetic etiology has been reported. Whether these congenital tumors have any association with assisted reproductive techniques remains obscure. CASE PRESENTATION: In this study, a case of SCT in an IVF pregnancy with donor oocytes was reported. IVF was performed for bilateral tubal blockade and poor ovarian reserve. It was diagnosed antenatally by ultrasonography. Successful surgical treatment was performed in postnatal period and six months follow-up remained uneventful. CONCLUSION: The purpose of reporting this case is to emphasize on the possibility of association of congenital tumors with assisted reproductive techniques and hence, the need for screening in these pregnancies. An association could not be detected based on few case reports and therefore, large population based studies are required to elucidate the effect of these reproductive techniques on occurrence of congenital tumors.

Duplication cyst of oesophagus: a case report.

Duplication of the oesophagus is the second most common duplication of the gastrointestinal tract. Children with oesophageal duplication cyst usually present with dysphagia or as asymptomatic thoracic mass found o incidental chest x-ray. We report a case of oesophageal duplication cyst that presented with inspiratory stridor and dyspnoea in a 6 month old boy. Bronchoscopy revealed an external compression on the trachea. Duplication cyst arising from the oesophagus was suggested on CT and MRI. The cyst was surgically excised with resolution of symptoms.

Unusual complications of heroin abuse: transverse myelitis, rhabdomyolysis, compartment syndrome, and ARF.

INTRODUCTION: Heroin overdose can cause various rare neurological complications like spongiform leukoencephalopathy, seizures, stroke, toxic amblyopia, transverse myelopathy, mononeuropathy, plexopathy, acute inflammatory demyelinating polyradiculoneuropathy, rhabdomyolysis, compartment syndrome, fibrosing myopathy, and acute bacterial myopathy. We report here the simultaneous presentation of multiple complications of heroin toxicity. CASE REPORT: A young heroin addict was found unarousable lying in the lotus posture. Examination showed quadriplegia and left leg gangrene. He subsequently developed heroin-induced transverse myelitis, rhabdomyolysis, left leg compartment syndrome, and myoglobin-induced acute renal failure. DISCUSSION: This case leads us to consider a common linked or systemic mechanism of injury rather than a local mechanism when multiple simultaneous organ failure occurs complicating heroin abuse.

AIDS-related opportunistic mycoses seen in a tertiary care hospital in North India.

Sixty symptomatic confirmed human immunodeficiency virus (HIV)-positive adult patients, of both sexes, suspected of having a fungal infection were taken as a study population, and the clinicomycological profile was correlated with the immunological status of the patients with particular reference to CD4 counts. Relevant samples were collected and subjected to direct microscopy, fungal culture and serology. CD4 counts were determined by flow cytometry. Patients belonged to the age group of 17-65 years, with a male : female ratio of 4.8 : 1. Heterosexuality was the commonest mode of transmission. Candidiasis was the most common diagnosis (41.7 %), followed by cryptococcosis (10.0 %), and pneumocystinosis and aspergillosis (8.3 % each). Two cases of histoplasmosis were also diagnosed. A low mean CD4 count of <200 cells microl(-1) was seen with most fungal infections. A total of 73 % of patients belonged to World Health Organization (WHO) stage 4, while 23.33 % belonged to stage 3. Thirty one patients (51.67 %) belonged to Centers for Disease Control and Prevention (CDC) stage C3. Various fungal infections correlated well with the mean CD4 counts. It was difficult to correlate statistically WHO and CDC staging because of the small sample size. However, it was possible to assess to a limited extent the possibility of using clinical diagnosis to predict the status of progression of HIV infection in a resource-poor outpatient setting.

Acute demyelinating encephalitis after jequirity pea ingestion (Abrus precatorius).

INTRODUCTION: Castor and jequirity beans are uncommon causes of poisoning. The more common but less severe castor poisoning is well described, but jequirity bean (Abrus Precatorius) poisoning is rare. The toxicity is attributed to toxalbumins (ricin and abrin) that act by inhibiting protein synthesis. Their use as agents of biological warfare, mechanisms of action, and clinical features of poisoning are summarized. CASE REPORT: A 30-year-old previously healthy female presented with bloody diarrhea and deep coma following ingestion of 3-4 seeds of a plant called 'ratti.' Investigations, including an MRI brain scan, showed evidence of acute demyelinating encephalitis. The patient died three days later due to progressive central nervous system depression. DISCUSSION: This is a previously unreported manifestation of jequirity bean poisoning. Demyelination is immune-mediated, and Abrus is a well-known immuno-modulator and stimulator. A possible immunological pathogenic mechanism is hypothesized.

Antihypertensive and antioxidant action of amlodipine and vitamin C in patients of essential hypertension.

The etiology of essential hypertension includes increased oxidative stress. The role of antihypertensive drug amlodipine as an antioxidant and the benefit of addition of vitamin C, an antioxidant to antihypertensive therapy were studied. Forty male patients of essential hypertension were randomly divided into two groups and treated with 5 mg amlodipine. In addition one group also received 1000 mg vitamin C (as two 500 mg tablets) once daily for three months. Although blood pressure decreased in both groups, the systolic blood pressure in patients given vitamin C was less (126.4 +/- 7.47) compared to the other group (130.9 +/- 7.27). A decrease in malondialdehyde, an increase in erythrocyte sodium-potassium adenosine triphosphatase (Na(+) K(+) ATPase) and an increase in the superoxide dismutase levels were observed in both groups. The increase in SOD was statistically more in the patients given vitamin C in addition to amlodipine (0.1717 +/- 0.0150 compared to 0.152 +/- 0.0219 units/100 ml assay). In spite of the known antihypertensive, antioxidant activity, similarity in correcting endothelial dysfunction independently, giving the two drugs together and early introduction of vitamin C perhaps decreases oxidative stress and augments the antioxidant status. This may prevent further vascular damage due to oxidative stress, leading to a better prognosis in essential hypertension patients.

Effect of improvement in anemia on electroneurophysiological markers (P300) of cognitive dysfunction in chronic kidney disease.

Our aim is to study the effect of improvement in anemia on event-related potentials (ERPs; P300) as markers of cognitive dysfunction in predialysis and dialysis patients of chronic kidney disease (CKD). Thirty anemic patients of CKD (hemoglobin [Hb] < 9 g%), 15 in the predialysis group (Group A), and 15 patients on biweekly hemodialysis (Group B) were recruited for the study. Patients of uremic encephalopathy, dyselectrolytemia, and those with hearing problems were excluded. Both groups were given recombinant human erythropoietin (rhuEPO) 100 IU/kg biweekly for 6 weeks by the subcutaneous route. No intervention was performed in the third control group (Group C), which consisted of 30 normal healthy volunteers. The improvement in Hb was assessed every 2 weeks, and the amplitude and latency of the P300 component of the ERPs were studied before initiating treatment and after 6 weeks of rhuEPO administration. There was a significant increase in Hb in both the study groups without any significant alteration in kidney functions. A significant reduction in P300 latency was noted in both the study groups after intervention. Similarly, the amplitude of P300 also increased in both study groups, but attained statistical significance for the dialysis group only. No significant changes were observed in the control group. Administration of EPO in patients of anemia with CKD resulted in a significant improvement in the electrophysiological markers of cognitive function in the form of increased amplitudes and decreased latencies of P300 in both predialysis and dialysis patients.

Rare thoracic mass lesion--myofibrobastoma.

Mesenchymal soft tissue masses are uncommon tumours of the chest. Myofibroblastoma is a recently described entity consisting of cells with origin from the myoepethelial cell, mostly seen as benign well-circumscribed neoplasms of the breast tissue. Though usually classified as a benign lesion, rarely it can be multifocal and prone to recurrence. We describe below the case of a 26-year-old female who presented with exertional dyspnoea and evidence of a massive mass lesion in the left hemithorax causing mediastinal displacement to the opposite side. On histological and immunophenotypic analysis, a diagnosis of the very rare thoracic myofibroblastoma was made. The incidence, salient features, pathological differential diagnoses and treatment are reviewed.

Psoriatic nephropathy--does an entity exist?

Psoriasis is an immune-mediated chronic inflammatory disorder of the skin. Association with kidney disease has been debated for a long time. Secondary renal amyloidosis in psoriatic arthropathy and drug-induced renal lesions secondary to methotrexate or cyclosporine are accepted accompaniments of psoriasis. IgA nephropathy is also known to occur in psoriatics. We report three interesting cases of renal involvement in long-standing established psoriasis on topical therapy alone. The patients presented with hypertension, significant proteinuria, hypoalbuminemia, and dyslipidemia. Kidney biopsies revealed "mesangioproliferative glomerulonephritis with IgA nephropathy," "focal proliferative glomerulonephritis," and "membranous glomerulonephropathy." The former two had marked active urinary sediment. Patients improved on prednisolone and angiotensin-converting enzyme inhibitors. Contrary to the belief that renal involvement in psoriasis is coincidental, we propose that kidney disease may be a common accompaniment of psoriasis, which may be labeled as "psoriatic nephropathy" or "psoriatic kidney disease." The exact mechanism of this entity is yet to be elucidated.

Congenital cystic adenomatoid malformation of lung--report of three cases.

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare anomaly of fetal development of terminal respiratory structures. We report three cases of congenital cystic adenomatoid malformation (CCAM) of lung Stocker's type I. All the cases presented with progressive neonatal respiratory distress. One case developed hyaline membrane because of ventilator therapy. No other congenital malformation was found in any of our cases. Two of our cases were suspected on antenatal ultrasonography. Routine prenatal ultrasonography has increased the frequency of prenatal diagnosis of congenital cystic lung malformation including CCAM. The pathogenesis, management and prognosis of CCAM are discussed along with a review of literature.

Renal and systemic amyloidosis in systemic lupus erythematosus.

A young male presented with oral ulceration for two years; swelling face and feet of seven days duration; diffuse goiter without signs of thyroid disease; normocytic normochromic anemia, thrombocytopenia, deranged renal functions, albuminuria of 2.5 g/24h with active urinary sediment. ANA and anti-ds DNA were positive, sonography of abdomen suggested medical renal disease. Testing for HIV, HBV, VDRL, CRP, rheumatoid factor, p-ANCA and c-ANCA were negative. Thyroid hormone assays were normal. Kidney biopsy done to stage lupus nephritis did not show any evidence of lupus involvement but staining for SAA amyloid was positive. Subsequent biopsies from the liver and rectum also stained positive for amyloid. Diagnosis of "Systemic lupus erythematosus with renal and systemic secondary amyloidosis with euthyroid diffuse goiter" was made. The case is being reported and discussed because of the interesting and rare association between amyloidosis and systemic lupus erythematosus.