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1.
Indian Pediatr ; 2022 Nov; 59(11): 859-861
Article | IMSEAR | ID: sea-225268

ABSTRACT

Objectives: To determine the prevalence of delirium and its risk factors among children admitted to a Pediatric intensive care unit (PICU). Method: A descriptive study in which consecutive patients admitted to the PICU over a period of 12 months were screened daily for delirium using the Cornell Assessment of Pediatric Delirium (CAPD) score. Treatmentrelated and demographic variables were collected and analyzed. The statistically significant risk factors for delirium were analyzed by multivariable logistic regression for independent associations. Results: Among the 476 screened patients, 96 (20.2%) developed delirium. The independent risk factors associated with the development of delirium were respiratory failure (P<0.001), administration of benzodiazepines during PICU stay (P<0.001), and presence of multiple (?2) risk factors for delirium (P<0.001). The mean length of PICU stay was significantly higher among delirious subjects with P<0.001. Conclusion: Delirium is a frequent complication in critically ill children, and recognition of associated factors may assist in early diagnosis and focussed management.

2.
Article in English | IMSEAR | ID: sea-179923

ABSTRACT

Harlequin ichthyosis (HI) is the rarest and the most severe form of congenital ichthyosis. It is inherited in an autosomal recessive manner and associated with mutations in gene ABCA12. There have been reports of several families with siblings affected with harlequin icthyosis. Affected infants have severe ectropion, eclabium, diffuse decreased range of motion, thick, dry hyperkeratotic plates, over entire body and scalp and associated deep erythematous fissures. Infants with HI have historically succumbed in the perinatal period to sepsis, respiratory failure and infections, poor nutrition and electrolyte imbalances. It is a rare entity. We report here a case of harlequin Icthyosis infant born to consanguineous parents whose 3 siblings were also affected with the same disorder. Keywo

3.
Article in English | IMSEAR | ID: sea-173390

ABSTRACT

Sirenomelia, also known as “mermaid syndrome”, is a rare congenital deformity of uncertain etiology. Sirenomelia is characterized by complete or partial fusion of lower limbs, giving the appearance of the tail of a ‘mermaid’. This syndrome is almost always lethal due to associated congenital visceral abnormalities such as severe lung hypoplasia, and abnormalities of the kidneys, large intestines, and genitalia. We hereby, report a case of sirenomeila due to rarity of this condition and term live birth.

4.
Article in English | IMSEAR | ID: sea-173376

ABSTRACT

Hunter syndrome or mucopolysaccharidosis (MPS) type II is an X-linked recessive disorder caused by a defect in the metabolism of glycosaminoglycans (GAGs). We present a rare case of MPS with a typical presentation of coarse facies, short stature, mild mental retardation and absence of corneal clouding. His radiographic findings were suggestive of MPS and diagnosis was confirmed by demonstrating deficient Iduronate-2-sulphatase enzyme in plasma. We present this case to highlight the distinctive manifestations as well as radiological and definitive diagnostic findings of the Hunter syndrome.

5.
Indian Pediatr ; 2014 May; 51(5): 401-402
Article in English | IMSEAR | ID: sea-170622

ABSTRACT

Background: Liver abscesses in thalassemics are predominantly pyogenic. Case characteristics: 12-year-old thalassemic boy with abdominal pain and high grade fever for 15 days. Observation: CT abdomen revealed multiple liver abscesses. Pus culture and sensitivity showed multidrug-resistant mycobacteria. Patient was started on second line anti-tubercular drugs. Outcome: He responded well with symptomatic improvement and is doing well after 6 months of follow-up. Message: Liver abscess in patients with thalassemia rarely can be tubercular.

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