Acute intermittent porphyria: A critical diagnosis for favorable outcome.

Acute intermittent porphyria (AIP) is an inherited metabolic disorder characterized by the accumulation of toxic metabolites of the heme pathway. It rarely presents in the prepubertal age group. AIP often presents with nonspecific and nonlocalizing symptoms. Moreover, several commonly used medications and stress states are known to precipitate an attack. We present the case of a previously healthy 5 years female who was diagnosed as acute central nervous system infection/inflammation at admission. It was the presence of red flags that led to a correct diagnosis. Besides supportive management, a dedicated search for intravenous hemin (chemically heme arginate, aminolevulinic acid synthase inhibitor, and drug of choice) was attempted. Unexpected help was rendered by doctors from a medical college in Gujarat, and two ampoules could be obtained. The patient received three doses of intravenous hemin; however, she succumbed later. This case is presented for the diagnostic and therapeutic challenges faced in developing countries.

Rare treatment option for a common pediatric airway problem.

Laryngomalacia is a common cause of respiratory obstruction with stridor in infants. Although most cases resolve spontaneously, severe laryngomalacia needs surgical intervention. Tracheostomies have been the mainstay of treatment. However, this procedure was associated with high morbidity. At present, newer modalities of treatment are being tried. We discuss successful management of an infant having severe laryngomalacia (who had three failed extubation trials) with glossoepiglottopexy.

Body Image Perceptions and Body Image Dissatisfaction among Medical Students in Oman.

Objectives: This study aimed to examine body image perceptions and body image dissatisfaction and their relationship with body mass index (BMI) among medical students in Oman. Methods: This cross-sectional study was conducted among students of the College of Medicine and Health Sciences, National University of Science and Technology, Sohar, Oman, between September and December 2019. The data (including self-reported weight and height) were collected through a questionnaire-based survey. Body image perception and satisfaction were assessed using the Stunkard Figure Rating Scale. Results: A total of 351 medical students (response rate: 70.2%) participated in the study with a mean age of 21.6 ± 2.2 years and a mean BMI of 22.7 ± 4.3 kg/m2. Most students were within normal ranges of BMI (63%), 12% were underweight and 25% overweight/obese. Approximately, 66% of students perceived their body image correctly; students at both BMI extremes were more accurate about their body image perceptions. Body image dissatisfaction was high (80%) among the respondents with 73.5% of those dissatisfied desiring to be thinner. The association between self-reported BMI with perceived BMI and body image dissatisfaction was significant (P <0.001). Only 18.2% of students felt 'attractive'. The association between body image perception and body image dissatisfaction was statistically significant (P <0.05). Conclusion: A high degree of body image dissatisfaction and incorrect body image perceptions exists among medical students. There is an increased desire to be thinner and the majority of this study's participants feel unattractive. There is a need to foster healthy body image perceptions among students of health sciences as they are the future in healthcare and can influence the health beliefs in our society.

Parental knowledge and attitudes regarding asthma in their children: Impact of an educational intervention in an Indian population.

INTRODUCTION AND OBJECTIVES: Research shows positive effects of asthma education in improving parental knowledge, self-management skills, and reducing healthcare costs. Such studies are lacking in resource-limited countries. We studied the effectiveness of educational intervention in improving the knowledge and attitudes of parents/caregivers of asthmatic children. METHODS: The study was conducted in the pediatric chest clinic of tertiary hospital (India) over 21 months after ethics committee approval. Recruited parents were randomized into the interventional group (A) receiving education module and control group (B). Parents' asthma knowledge and attitudes were assessed at baseline and 5 months postenrollment using 25-item questionnaire. Detailed demographic data, clinical data, and exacerbations during study were noted. RESULTS: A total of 75 parents/guardians fulfilling inclusion criteria were analyzed (cases/group A: 37 and controls/group B: 38). 8.3 percent of parents/caregivers were illiterate. Around 36.9% of patients had a family history of allergy/asthma. Mean knowledge scores at follow-up were 12.24 and 9.89 for groups A and B, respectively (P < .05). Parents did better on knowledge items related to chronicity, family history, chronic cough, home administration of steroids in acute severe asthma, and maintaining records of clinical/medications for good control. Intervention group (A) showed significant improvement in most attitude-based questions postintervention as compared with the nonintervention group (B). There was no statistically significant difference in asthma severity and control between the two groups at follow-up. CONCLUSIONS: Small group education on asthma in parents/caregivers improves their knowledge and attitudes. Healthcare plans should invest in pediatric asthma education and identify key personnel/opportunities to impart the same in routine care.

Myxedema Coma in a Pediatric Patient with Down Syndrome.

Myxedema coma due to severe/long standing hypothyroidism is a known fatal endocrine emergency but is rare in children and unreported in pediatric Down syndrome. It mimics other conditions in the emergency room, making the diagnosis challenging. We present a 10-year-old-male child with global developmental delay and Down syndrome phenotype, admitted for altered sensorium subsequent to a febrile illness. The presence of myxedematous changes on clinical examination, on a background of altered sensorium and hypothermia, led to suspicion of myxedema coma, confirmed by laboratory testing. Due to nonavailability of triiodothyronine (T3), thyroxine (T4) was administered through nasogastric tube after an endocrine consult. Despite initial recovery in terms of improved consciousness, the child ultimately succumbed to refractory shock and terminal ventricular tachycardia. Our case highlights the need to consider myxedema coma as a differential diagnosis for altered mental status in the emergency room and use of screening tools for effective selection of patients.

Burden of respiratory illnesses in pediatric intensive care unit and predictors of mortality: Experience from a low resource country.

INTRODUCTION AND OBJECTIVES: Acute respiratory infections (ARI), a major cause of childhood mortality and morbidity, place substantial burden on health services worldwide. Due to scarce data regarding respiratory illnesses requiring Pediatric intensive care unit (PICU) admissions (especially in low income/developing countries), we studied the burden of respiratory illnesses in the PICU and the risk factors contributing to mortality. METHODS: This prospective observational study was conducted over 18 months. Children (aged 1 month to 12 years) admitted to the PICU for acute respiratory/cardiorespiratory illnesses were enrolled. Demographic and clinical details of the study population were recorded and tabulated. Risk factors contributing to mortality (severity of illness [PRISM III score], diagnosis/etiology, need for mechanical ventilation, immunocompromised status, malnutrition, and length of stay [LOS]) were analyzed (using the χ2 test or Fischer Exact test). RESULTS: Two hundred and ninety-three children were enrolled (median age: 5 months; range, 1-132 months; male/female: 181/112). Mean LOS in PICU was 5.25 ± 5.48 days and mean length of hospital stay of 14.82 ± 13.35 days. Mechanical ventilation was required in 62.8% cases. Pneumonia contributed to 66.89% of respiratory admissions and 91% of mortality. Ninety patients (30.7%) died; mortality being higher in cardiorespiratory cases (52.7%). PRISM III score and shock (respiratory cases) and age below 1 year (cardiorespiratory group) were significantly associated with higher mortality ( P < 0.05). CONCLUSIONS: Respiratory illnesses are a significant contributor to PICU admissions and are associated with significant mortality risk in presence of high PRISM III score and shock (respiratory group); and age below 1 year (cardiorespiratory group).

Acute Respiratory Distress Syndrome Caused by Mercury Inhalation: A Case Report.

Inhalation of mercury vapor is an uncommon cause of acute respiratory distress syndrome. It may go unnoticed if appropriate environmental/occupational history is not taken. A 2-year-old child presented with severe respiratory distress and progressed to develop acute respiratory distress syndrome, necessitating high-pressure mechanical ventilation. On detailed enquiry, history of exposure to mercury fumes was obtained (at home), during gold extraction by the father. Diagnosis of mercury poisoning was confirmed by blood levels, and despite treatment with dimercaprol (mercury chelator) and high-frequency ventilation, the child subsequently succumbed within 26 hours. This case reiterates the need for awareness regarding unusual environmental toxic exposures and need for stricter regulations for sale or use of toxic products.