Performance of PRISM (pediatric risk of mortality) score and PIM (pediatric index of mortality) score in a tertiary care pediatric ICU.

Objective. To validate Pediatric Risk of Mortality (PRISM) and Pediatric Index of Mortality (PIM) score. Methods. All consecutive patients over a six month period were included in the study except patients with a PICU stay of less than 2 hours, those transferred to other PICUs, pediatric surgical cases, trauma patients and those dying within 24 hours of admission. The PRISM and PIM scores of all patients included in the study were computed and the outcome was noted in terms of survival or non-survival. Mortality discrimination was quantified by calculating the area under the receiver operating characteristic (ROC) curve. Hosmer and Lemeshow goodness-of-fit test was used to calibrate the scores. Results. Two hundred and thirty patients were enrolled with mean age of 40.6 months and male to female ratio of 1.2:1. There were 56 deaths (mortality rate 24.3%). The mortality in infants was higher (37.8 %) as compared to non-infants (16.2 %) (p = 0.011). The predicted deaths with PRISM score was 24.3%. The area under the ROC curve was 0.851 (95% CI 0.790 – 0.912). The Hosmer and Lemeshow goodness-of-fit test showed good calibration (p=0.627, chi square =1.75, degree of freedom = 3). The predicted deaths with the PIM score was 7.38%. The area under the ROC curve for PIM score was 0.838 (95 % CI 0.776- 0.899). The Hosmer and Lemeshow goodness-of-fit showed a poor calibration for PIM score (p = 0.0281, chisquare = 10.866, degree of freedom = 4). Conclusion. Both PRISM and PIM scores have a good discriminatory performance. The calibration with PRISM score is good but the PIM score displays poor calibration.

Kocher-debre-semelaigne syndrome with pericardial effusion.

We report a 10-year-old female child with hypothyroidism and limb muscle pseudohypertrophy (i.e. Kocher-Debre-Semelaigne syndrome) with pericardial effusion. The child presented with generalized swelling, breathlessness and difficulty in walking and in getting up from sitting position (of chronic duration). She had bradycardia, dull facies, marked hypertrophy of both calf muscles and nonpitting edema of legs. Pericardial effusion was detected clinically and confirmed on investigations. Muscle pseudohypertrophy was a striking feature and hypothyroidism was confirmed on thyroid studies. The response to thyroxine replacement was excellent, with resolution of the pericardial effusion and clinical improvement. The unusual presence of pericardial effusion in Kocher-Debre-Semelaigne syndrome is discussed in the report.

Pulmonary endarteritis.

Pulmonary arterial endarteritis is a rare event even in patients with congenital heart disease. In this paper, the authors report two cases of pulmonary endarteritis diagnosed at autopsy. The first was a 3-month-old male infant with absence of congenital heart defect (who had had neonatal sepsis). This patient presented with pneumonia and succumbed within 36 hours of hospital stay despite appropriate antimicrobial therapy and supportive management. The second patient was a 13-month-old male child with previously undiagnosed tetralogy of Fallot who had clinical presentation of acute meningitis with convulsions and succumbed within 12 hours of hospital stay despite adequate treatment. The main autopsy findings were chronic arteritis of the pulmonary trunk and right pulmonary artery in the former and rupture of the pulmonary trunk in the latter.

Incomplete monosymptomatic leptomeningeal angiomatosis.

Phakomatoses or neurocutaneous syndromes are an important cause of seizures in the pediatric age group. The Sturge-Weber syndrome may affect the eye, skin and brain at different times. The skin lesions need not always manifest. We report a case of isolated affection of the central nervous system in a case of Sturge-Weber syndrome in the absence of ocular or cutaneous manifestations. Our case qualifies to be called incomplete monosymptomatic leptomeningeal angiomatosis.