Giant cementifying fibroma

A 36 year old female with giant cementifying fibroma is presented. The tumour had replaced one half of the body of the mandible. The management policy and brief review of the disease is discussed.     

Perinatal mortality-incidence and effect of various maternal factors,part 1

Of 12,716 consecutive briths, over a period of eleven years, perinatal mortality rate was 80.7, still birth rate 41.4 and early neonatal death rate 39.3. Perinatal mortality was very high in association with previous pregnancy wastages, obstetrical complications like toxemia and antepartum hemorrhage, acute infection and chronic illnesses during pregnancy, abnormal presentation, prolonged sedation and anesthesia. Teenage and elderly mothers, primi and grandmultipara, mothers with anemia and A or O blood group and consangunity also had high perinatal losses. Alcoholism and smoking tend to have adverse effects on fetus but require further studies to be conclusive. A low perinatal mortality rate of 29/1000 in properly booked cases is another highlight of this study.     

Physical growth of children with down syndrome in India

Anthropometric measurement of 139 Indian children upto the age of five years with Down syndrome, diagnosed by chromosomal studies, were recorded. The height curves of these children fell below the 50th percentile of normal in the first 9 months and subsequently below the 10th percentile. The weight curves remained below the 10th percentile from the beginning and advanced parallel to it with age. Head circumference curves of these children fell appreciably below the 10th percentiles at all ages, but the head continued to grow during the period of study.     

Clinical and molecular characterization of Indian patients with fructose‐1, 6‐bisphosphatase deficiency: Identification of a frequent variant (E281K)

Fructose‐1, 6‐bisphosphatase deficiency is an autosomal recessive disorder of gluconeogenesis caused by genetic defect in the FBP1 gene. It is characterized by episodic, often life‐threatening metabolic acidosis, liver dysfunction, and hyperlactatemia. Without a high index of suspicion, it may remain undiagnosed with devastating consequences. Accurate diagnosis can be achieved either by enzyme assay or gene studies. Enzyme assay requires a liver biopsy and is tedious, invasive, expensive, and not easily available. Therefore, genetic testing is the most appropriate method to confirm the diagnosis. Molecular studies were performed on 18 suspected cases presenting with episodic symptoms. Seven different pathogenic variants were identified. Two common variants were noted in two subpopulations from the Indian subcontinent; p.Glu281Lys (E281K) occurred most frequently (in 10 patients) followed by p.Arg158Trp (R158W, in 4 patients). Molecular analysis confirmed the diagnosis and helped in managing these patients by providing appropriate genetic counseling. In conclusion, genetic studies identified two common variants in the Indian subcontinent, thus simplifying the diagnostic algorithm in this treatable disorder.     

Intraoral removal of a giant submandibular sialolith obstructing Wharton's duct: a case report

Sialolithiasis is one of the most common pathologies of the submandibular gland; sialoliths account for about 80 percent of all salivary duct calculi. This report presents the unusual case of a large asymptomatic sialolith of the submandibular duct, initially diagnosed as a possible tumor. The giant sialolith was removed via an intraoral approach under local anesthesia. The etiology, pathogenesis, and management of such giant sialoliths are discussed.     

Organization of two independent kisspeptin systems derived from evolutionary-ancient kiss genes in the brain of zebrafish

Kisspeptins are new actors in the neuroendocrine regulation of reproduction. In vertebrates, the number of kiss genes varies from none to three. Zebrafish have two kiss genes, kiss1 and kiss2, and two kiss receptors (GPR54), kiss1r and kiss2r. To provide detailed information on the organization of the kiss systems in zebrafish, antibodies were raised against the C terminus of zebrafish preproKiss1 and preproKiss2. Immunohistochemistry fully confirmed in situ hybridization data, showing that kiss1-expressing neurons are only located in the habenular nucleus, while kiss2-expressing neurons are found in the dorsal and ventral hypothalamus. Kiss1-expressing cells project only to the interpeduncular and raphe nuclei and strongly expressed the kiss1r receptor. In contrast, kiss2-expressing cells are mostly present in the dorsal and ventral hypothalamus and project widely into the subpallium, the preoptic area, the thalamus, the ventral and caudal hypothalamus, and the mesencephalon. All these regions strongly expressed the kiss2r messengers. Kiss2 fibers profusely innervate the ventral forebrain and notably made close apposition with GnRH3 neurons. Estrogen treatment of juvenile fish with estradiol causes increase in kiss2 and kiss2r expression. In the pituitary gland, no proKiss2- positive fibers were detected, while positive cells were observed in the pars intermedia. In addition to proposing a successful strategy to develop antibodies to kisspeptins, these data indicate that the kiss2 systems of zebrafish are implicated in reproductive events, while the kiss1 gene would play other functions that remain to be established.