Paraganglioma with a very rare presentation

Paragangliomas (PGLs) are rare tumors of neural crest origin, with a malignancy rate of approximately 10% and a 5-year survival rate of <50%. We present a case of malignant PGL arising from the porta hepatis with metastasis to the portal lymph node and bilateral ovaries. PGLs arising from the porta hepatis are very rare. As per our knowledge, only three cases of hepatic duct PGL have been reported. It is important to detect it earlier because the treatment modality and prognosis of benign and malignant PGL differs and defines the prognosis of the patient.

Placental histomorphology in unexplained foetal loss with thrombophilia.

BACKGROUND & OBJECTIVES: Acquired and genetic thrombotic conditions, both organ and non organ specific, are associated with increased foetal wastage. This study was carried out to examine the placenta from women with abnormal pregnancies and a history of unexplained foetal loss, and to associate with maternal thrombophilia status. METHODS: Placentas from eight women with history of unexplained foetal loss were analyzed for histopathological characteristics. All the women were simultaneously screened for the common acquired and genetic thrombophilia markers i.e., lupus anticoagulants ( LA), IgG / IgM antibodies for anticardiolipin (ACA), beta2 glycoprotein 1 (beta2GPI) and annexin V, protein C (PC), protein S (PS), antithrombin III (AT III), factor V Leiden ( FVL) mutation, prothrombin (PT) gene G20210A, methylene tetrahydrofolate reductase (MTHFR) C 677T, endothelial protein C receptor (EPCR) 23 bp insertion and plasminogen activator inhibitor ( PAI-1 4G/5G) polymorphisms RESULTS: Six of eight women were positive for one or more thrombophilia markers. The placenta in all the cases except one, showed the characteristic features of infarct fibrin deposition and calcification. Among two women who were negative for thrombophilia, one showed clear evidence of thrombus in the placental sections while the other did not show any characteristic infarcts in the placental sections. INTERPRETATION & CONCLUSION: Our findings showed that the histopathological examination of the placentas confirmed thrombophilia as the aetiological cause of thrombosis in 6 of the 8 women. The presence of thrombus in a negative thrombophilia woman suggests yet unidentified thrombophilia markers or probably non-haemostatic factors causing thrombosis.

Pulmonary manifestations of pediatric HIV infection.

Vertically acquired HIV infection is becoming increasingly common in India. The main clinical manifestations of HIV in childhood are growth failure, lymphadenopathy, chronic cough and fever, recurrent pulmonary infections, and persistent diarrhoea. Pulmonary disease is the major cause of morbidity and mortality in pediatric AIDS, manifesting itself in more than 80% of cases. The most common causes are Pneumocystis carinii pneumonia (PCP), lymphocytic interstitial pneumonitis (LIP), recurrent bacterial infections which include bacterial pneumonia and tuberculosis. The commonest AIDS diagnosis in infancy is PCP, presenting in infancy with tachypnea, hypoxia, and bilateral opacification on chest-X-ray (CXR). Treatment is with cotrimoxazole. LIP presents with bilateral reticulonodular shadows on CXR. It may be asymptomatic in the earlier stages, but children develop recurrent bacterial super infections, and can progress to bronchiectasis. LIP is a good prognostic sign in children with HIV infection in comparison to PCP. HIV should be considered in children with recurrent bacterial pneumonia, particularly with a prolonged or atypical course, or a recurrence after standard treatment. Pulmonary TB is common in children with HIV, but little data is available to guide treatment decisions. Much can be done to prevent PCP and bacterial infections with cotrimoxazole prophylaxis and appropriate immunisations, which may reduce hospital admissions and health care costs.

Internalization of dengue virus-induced suppressor cytokine during transmission of the suppressor signal via macrophage.

In dengue type 2 virus (DV)-induced suppressor T cell cascade TS1 cells secrete a suppressor cytokine (SF) which acts via syngeneic macrophages (M phi) to recruit TS2 cells. SF binds to both high and low affinity receptors (SF-R) on M phi. In the present study the fate of SF in M phi during transmission of suppressor signal is investigated. It was observed that SF bound to high affinity receptors internalized through receptor mediated endocytosis. This was inhibited by pretreatment of M phi with anti-SF-R-antiserum and didansylcadaverine, a potent inhibitor of endocytosis. Internalized SF was degraded by lysosomal activity as shown by inhibition of suppressor activity by pretreatment of M phi with monensin and NH4Cl. Degraded SF was transported to a site other than SF-R on M phi membrane for recruitment of TS2 cells. This was inhibited by anti-SF-antiserum. Transmission of suppressor signal is inhibited if M phi are treated first with H-2K-mAb and then with SF (shown earlier) but when M phi were treated first with SF and after 1 hr with H-2K-monoclonal antibody, the inhibition did not occur. As SF requires binding to H-2K and SF-R for mediation of suppression, the binding of H-2K occurred with degraded SF within the cell. Thus SF is internalized, degraded and binds to H-2K antigen before its recognition by native T cells.

Role of nitric oxide in transmission of dengue virus specific suppressor signal.

Production of NO2- was maximum when peritoneal M phi was incubated with SF (40 mg) for 45 min. Pretreatment of M phi with anti-SF-antisera inhibited production of NO2- Pretreatment of M phi with NG monomethyl L-arginine (L-NMA) or arginase, an inhibitor of L-arginine dependent pathway, inhibited production of NO2- and transmission of suppressor signal in a dose-dependent manner. This indicates that NO and Ca2+ serve as intracellular signal in transmission of DV-induced suppressor signal.

Transmission of dengue virus-specific suppressor signal depends on the presence of calcium.

The present study was undertaken to investigate the role of calcium in the production of dengue type 2 virus (DV) induced suppressor cytokine (SF) and in transmission of the suppressor signal via syngeneic macrophage (M phi) to recruit the second subpopulation of suppressor T(TS2) cell. Effect of calcium channel blocking drugs, Verapamil and Nifedipine, on the production and activity of SF was investigated. The production of SF was inhibited in a dose dependent manner by the calcium channel blockers. The suppressor activity of SF was also inhibited by calcium channel blocking drugs. SF could not be produced when spleen cells were cultured in a calcium free medium, the production being restored on substitution with calcium chloride. Treatment of M phi with the calcium channel blockers inhibited the transmission of the suppressor signal from TS1 to TS2 cells in a dose dependent manner. The influx of calcium during transmission of the suppressor signal was studied by measuring the 45Ca uptake. Calcium channel blocking drugs inhibited the 45Ca uptake by T lymphocytes. Thus, presence of calcium is obligatory for the production and suppressor activity of SF and it plays a critical role in transmission of the suppressor signal.

Determination of bone width in malnourished children under 5, and its inter relationship with mid arm muscle thickness, subcutaneous fat thickness and the mid upper arm girth.

The mid upper arm of 516 malnourished children (one to five years) were studied radiographically for changes in bone width, muscle mass and subcutaneous fat, of malnutrition at different ages. The data was studied by statistical analysis, determining the correlation coefficients of each of the factors. The findings indicate that previous assumptions about the components and the changes of the mid upper arm girth (MUG) in chronic severe malnutrition, were perhaps too simplistic.

Poisoning in children--analysis of 250 cases.

This study is an analysis of 250 cases over a period of 2 years, admitted to our hospital for various poisonings. The total incidence of poisoning was 11.9%, much higher as compared to the other series. Of these, 58.4% were seen in the age group of 1-4 years. Male to female ratio was 1.7:1. Incidence of food poisoning was 48.8% followed by that of kerosene (24%), pesticides (9.6%), chemicals and medicaments (8.4%), plant (3.6%) and animal bites (3.2%). Overall mortality was 0.8%.

Diagnostic and prognostic value of CT brain scan in term neonates with moderate and severe birth asphyxia.

Twenty term neonates with moderate (stage II) and 5 with severe (stage III) hypoxic ischemic encephalopathy (HIE) were prospectively studied to determine diagnostic and prognostic value of CT brain scan. Three neonates expired, 4 were lost to follow up while 18 were followed up to 18 months of age. Cerebral hypodensities were noted in 20 and intracranial hemorrhage (ICH) in 8, of which 6 had both ICH and hypodensity. Twelve of 14 infants with hypodensities and 5 of 6 with ICH who were followed up were handicapped at 18 months. Thirteen of 18 babies followed up were subjected to repeat CT scans between 9 and 18 months of age for assessing extent and severity of brain damage. Major abnormality noted on repeat CT scans was cerebral atrophy. All 6 infants whose follow-up scans were abnormal had neurological sequelae, while of 7 infants who had normal repeat CT scans, 5 had neurological sequelae. We do not recommend repeat CT scans in patients with HIE as a parameter to predict neurologic outcome.