Xanthogranulomatous Pyelonephritis in a male child with renal vein thrombus extending into the inferior vena cava: a Case Report.

BACKGROUND: We present a case of Xanthogranulomatous pyelonephritis (XGPN) in a male child with renal vein thrombus extending into the inferior vena cava. This is a rare presentation. XGPN is a rare type of renal infection characterised by granulomatous inflammation with giant cells and foamy histiocytes. The peak incidence is in the sixth to seventh decade with a female predominance. XGPN is rare in children. CASE PRESENTATION: An 11 year old male child presented with a history of high grade fever and chills, right flank pain and progressive pyuria for two months. He had a history of vesical calculus for which he was operated four years back. In our case, a subcapsular right nephrectomy was performed. The surgical specimens were formalin fixed and paraffin embedded. The sections were stained with routine Hematoxylin & Eosin stain. Grossly; the kidney was enlarged with adherent capsule and thickening of the perinephric tissue. The pelvicalyceal system was dilated and was filled with a cast of pus. Histological evaluation revealed diffuse necrosis of the renal parenchyma and perinephric fat. Neutrophils, plasma cells, sheets of foamy macrophages and occasional multinucleate giant cells were seen. The renal vein was partially occluded by an inflammatory thrombus with fibrin, platelets and mixed inflammatory cells. The thrombus was focally adherent to the vein wall with organization. CONCLUSIONS: The clinical presentation and the macroscopic aspect, together with the histological pattern, the cytological characteristics addressed the diagnosis towards XGPN with a vena caval thrombus. Our case illustrates that the diagnosis of XGPN should be considered even in paediatric age group when renal vein and vena caval thrombi are present.

Bombay blood group: Is prevalence decreasing with urbanization and the decreasing rate of consanguineous marriage.

CONTEXT: Bombay blood group although rare is found to be more prevalent in the Western and Southern states of India, believed to be associated with consanguineous marriage. AIMS: To estimate the prevalence of the Bombay blood group (Oh) in the urban population of Puducherry. To find the effect of urbanization on consanguineous marriage and to establish whether consanguinity plays a part in the prevalence of Oh group. To compare Oh group prevalence with that of other neighboring states, where population is not predominantly urban. SETTINGS AND DESIGN: This is a descriptive study in a tertiary care hospital in Puducherry, over a period of 6 years. MATERIALS AND METHODS: All blood samples showing 'O' group were tested with anti-H lectin. Specialized tests like Adsorption Elution Technique, inhibition assay for determination of secretor status were performed on Oh positive cases. Any history of consanguineous marriage was recorded. STATISTICAL ANALYSIS USED: All variables were categorical variable and percentage and proportions were calculated manually. RESULTS: Analysis of the results of 35,497 study subjects showed that the most common group was 'O' group constituting 14,164 (39.90%) of subjects. Only three "Oh" that is, Bombay phenotype (0.008%) were detected. Consanguinity was observed in two cases (66.66%). CONCLUSIONS: This study shows the prevalence of Bombay blood group representing the urban population of Puducherry, to be high (0.008%) and associated with consanguineous marriage (66.66%). Thus, consanguinity is still an important risk factor present, even in an urban population in Southern India.

Xanthogranulomatous pyelonephritis in a male child--a case report with a brief review of literature.

Xanthogranulomatous pyelonephritis is a rare type of renal infection characterised by granulomatous inflammation with giant cells and foamy histiocytes.The peak incidence is in the sixth to seventh decade with a female to male ratio of 2:1. It is rare in children. A case of xanthogranulomatous pyelonephritis in a 11-year-old male child who presented with a history of high grade fever and chills for 15 days, right flank pain and progressive pyuria for two months is reported. He had a prior history of surgery for vesical calculus and anterior urethral calculus for which he underwent open cystolithotomy and urethrolithotomy four years back. Although Gram-negative bacilli were isolated from thick pus aspirated from the right renal pelvis under ultrasound guidance, urine and blood culture were sterile. Following a renal biopsy diagnosed as chronic pyelonephritis the patient underwent subscapsular right. nephrectomy.

Myelodysplastic syndromes/neoplasms: recent classification system based on World Health Organization Classification of Tumors - International Agency for Research on Cancer for Hematopoietic and Lymphoid Tissues.

The myelodysplastic Syndromes (MDS) are a group of clonal hematopoietic stem cell diseases characterized by cytopenia(s), dysplasia in one or more of the major myeloid cell lines, ineffective hematopoiesis, and increased risk of development of acute myeloid leukemia. The classification and the diagnostic criteria have been redefined by the recent World Health Organization Classification of Tumors - International Agency for Research on Cancer for Hematopoietic and Lymphoid Tissues. The myelodysplastic syndromes are now classified into the following categories - refractory cytopenia with unilineage dysplasia, refractory anemia with ring sideroblasts, refractory cytopenia with multilineage dysplasia, refractory anemia with excess blasts, myelodysplastic syndrome associated with isolated del (5q), myelodysplastic syndrome - unclassifiable, and childhood myelodysplastic syndrome. The clinicopathologic features, morphology, differential diagnosis, immunophenotyping, cytogenetics, prognosis and predictive factors are presented in the light of recent World Health Organization Classification of Tumors - International Agency for Research on Cancer.

Basal cell adenocarcinoma in the tongue: an unusual presentation.

We present a case of basal cell adenocarcinoma (BCAC) in the tongue in a 65-year old male. This is an extremely rare presentation. BCAC generally occurs in the parotid gland and rarely involves the minor salivary glands. Few cases have been reported in literature with a variable presentation. The biopsy was formalin-fixed and paraffin-embedded. The sections were stained with routine Hematoxylin and Eosin. Immunohistochemistry was performed. Hematoxylin and eosin staining showed tumour composed of variable sized and shaped, nests and sheets of basaloid epithelial cells having hyperchromatic to vesicular nuclei. Immunohistochemistry was positive for Pancytokeratin, Epithelial membrane antigen and p53. The clinicopathological features and the cellular immunophenotype addressed the diagnosis towards BCAC of the tongue. The goal of this report is to increase awareness of this rare disease and to review and discuss the differential diagnosis and important considerations in treatment.