Background: Sickle cells are easily identified on the peripheral smear however quantification of sickle cells is seldom done. This study attempts to understand the importance of the sickling index in the reporting of peripheral smear in patients with SCD. Objectives: This study aims to standardize the methodology of quantification of sickle cells on the peripheral smear in paediatric SCD cases and study its relation to the patient characteristics like age and sex, SCD genotype, and treatment. Materials and Methods: A prospective study of 6 months duration was conducted at a tertiary paediatric referral hospital for 6 months. 30 known cases of SCD were admitted during this period. Routinely studied peripheral smears were used in the study. 30 normal smears of age-matched patients were also used for comparative purpose. The sickling index was calculated in both patient and age matched smears. The sickling index was compared in both the groups and also the index in the known SCD cases was studied in relation to the patient's age, SCD genotype, and treatment with blood transfusions or hydroxyurea. Statistical analysis: Descriptive statistics were used in the study Results & Conclusion: Hb SS was the commonest genotype seen in 69% of the cases. Higher sickling index was noted in older children, SS genotype and those on treatment with blood transfusions or Hydroxyurea. Sickling index is an interesting prospect which may provide helpful information regarding cases with increasing hemolysis and vasocclusive crises and also help in follow up of patients on treatment. Keywords: Paediatric, Disease severity, Sickling index
Background: Congenital obstructive nephropathy is the principal cause of end stage renal disease (ESRD) in children ( Beneldet al. 2003). Obstructive nephropathy leads to Hydronephrosis, dened clinically by an enlargement of the kidney as a result of urine accumulation in the renal pelvis or calyces. [1,2] Ureteropelvic junction (UPJ) obstruction is the most common cause of hydronephrosis with an estimated incidence of 1 in 1000–1500 newborns ( Changet al. 2004). [2,3] Aims: To evaluate the pathogenesis of congenital ureteropelvic junction obstruction (UPJO) by histopathology, IHC markers CD117 & S100 and special stain Masson's Trichrome. Methods And Materials: The study group consists of 50 Pediatric cases presenting with intrinsic ureteropelvic junction obstruction and 15 patients with Wilms tumor with no UPJO formed the control group. All the cases and controls were subjected to histopathological examination, immunohistochemical staining with markers CD117 & S100 and special stain Masson's Trichrome. Mann – Whitney U test and Pearson's chisquare test were used for statistical analysis. The cases had reduce Results: d CD117 and S100 expression, Irregularity of muscle bers, increased wall thickness & muscle thickness and submucosal collagen deposition compared to controls. Decreased ICC interpreted by Conclusions: decreased CD117, decreased neural innervation interpreted by decreased S100, structural changes - Irregularity of muscle bers arrangement, increased submucosal collagen deposition and increased smooth muscle thickness possibly cause disruption of the mobility of UPJ and lead functional obstruction.
BACKGROUND Choledochal cysts are rare congenital dilatations of the biliary tract with an incidence of approximately 1 in 1000 live births. Most cases present with jaundice or other obstructive symptoms and are treated by surgical resection of the cyst and re-anastomosis. A liver biopsy at the time of surgery is mandatory to study the histological changes including fibrosis and to grade these changes to determine prognosis. We wanted to study the histology of liver biopsies received along with choledochal cysts, grade the hepatocellular damage and fibrosis, and correlate these findings with clinical presentation of the patients. METHODS 40 liver biopsies received along with choledochal cysts in paediatric patients were assessed for histological changes using 7 parameters including liver fibrosis and scored semi-quantitatively according to established scoring systems. These scores were correlated with the clinical data, age at surgery, sex of the patient, type of choledochal cyst, clinical features, and the results were analysed for their role in predicting patient prognosis. RESULTS We found that all the cases showed histological variations in the liver biopsy. Age of children showed an inverse relationship with histological scoring. Infants showed higher scores in comparison to older children. Fibrosis of the liver was also higher in Infants. Most of the cases presented with clinical jaundice. Patients with jaundice, pain abdomen, fever and acholic stools showed higher scores. We found that the sex of the patient and type of choledochal cysts were independent of the scores. CONCLUSIONS Younger age and more severe clinical presentation warrant an urgent surgical intervention in paediatric choledochal cyst. Histological assessment of the liver is mandatory to grade the hepatocellular changes including fibrosis and suggest prognosis and follow up of patients. KEYWORDS Liver Histology, Grading, Paediatric Choledochal Cyst
Background: Vitellointestinal duct (VID) or Omphalomesenteric duct (OMD) is the embryonic connection between the yolk sac and the primitive midgut. Anomalies result from failure of the involution of the VID. Normally, it obliterates between the fifth and seventh weeks of fetal life. They include Meckel’s diverticulum (MD), fibrous band, fistula, sinus tract, cyst, and umbilical polyp. Symptomatic cases require surgical correction. They are characterised by different clinical presentations and histological appearances. This study gathers a single paediatric institutional experience of VID remnants, their demographic, clinical profile, varied histomorphology including the presence of ectopic tissues. Aims and Objectives: The objective of this study was to retrospectively review all the symptomatic cases of VID abnormalities at a tertiary pediatric referral hospital. The demographic profile, clinical presentation, and histopathology were reviewed and descriptively analyzed. We classified these anomalies based on embryology and histology. The findings of this study will provide insights to pathologists and treating clinicians less exposed to these rare lesions. Materials and Methods: Data was retrieved retrospectively from the pathology records of a tertiary paediatric referral institute for 3 years. Histological slides of VID anomalies were reviewed and a descriptive analysis of the findings was performed. A thorough review of the literature was also done on these very rare lesions and their findings compared to our results. Results: A total of 60 cases of VID anomalies were encountered during the study period. There was significant male preponderance. The most common age group at surgery was infancy. MD was the commonest VID anomaly. The least common was vitelline fistula and only a single case was encountered in the study period. Ectopic tissues were seen in 25% of the cases and included gastric and colonic mucosae and pancreatic tissue. Conclusion: The histopathology of VID anomalies provides an interesting diagnostic experience for the reporting pathologist. Awareness of the embryology, presentation and histology of these lesions aids in accurate diagnosis.
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