Congenital dengue in neonate.

Congenital dengue should be considered as a cause of fever and rash in a neonate born to a mother especially in endemic areas.

Abnormal neurodevelopment outcome in case of neonatal hyperekplexia secondary to missense mutation in GLRB gene.

Hyperekplexia is an exaggerated startle to external stimuli associated with a generalised increase in tone seen in neonates with both sporadic and genetic predisposition. This is an uncommon neurological entity that is misdiagnosed as seizure. A 28-days-old infant was admitted to us with characteristic intermittent generalised tonic spasm being treated as a seizure disorder. The infant had characteristic stiffening episode, exaggerated startle and non-habituation on tapping the nose. Hyperekplexia was suspected and confirmed by genetic testing (mutation in the ß subunit of glycine was found). Initial improvement was seen with the use of clonazepam, which was not sustained. At the age of 4.5 years, the child is still having neurobehavioural issues like hyperactivity and sensory hyper-responsiveness. Usually, hyperekplexia is benign in nature. We report a case of hyperekplexia with non-sense mutation in the ß subunit of GlyR gene having abnormal neurodevelopmental findings at 4.5 years.

Latent iron deficiency at birth influences auditory neural maturation in late preterm and term infants.

BACKGROUND: In utero latent iron deficiency has been associated with abnormal neurodevelopmental outcomes during childhood. Its concomitant effect on auditory neural maturation has not been well studied in late preterm and term infants. OBJECTIVE: The objective was to determine whether in utero iron status is associated with auditory neural maturation in late preterm and term infants. DESIGN: This prospective cohort study was performed at Sir Ganga Ram Hospital, New Delhi, India. Infants with a gestational age ≥34 wk were eligible unless they met the exclusion criteria: craniofacial anomalies, chromosomal disorders, hemolytic disease, multiple gestation, third-trimester maternal infection, chorioamnionitis, toxoplasmosis, other infections, rubella, cytomegalovirus infection, and herpes simplex virus infections (TORCH), Apgar score <5 at 5 min, sepsis, cord blood not collected, or auditory evaluation unable to be performed. Sixty consecutive infants with risk factors for iron deficiency, such as small for gestational age and maternal diabetes, and 30 without risk factors for iron deficiency were enrolled. Absolute wave latencies and interpeak latencies, evaluated by auditory brainstem response within 48 h after birth, were measured and compared between infants with latent iron deficiency (serum ferritin ≤75 ng/mL) and infants with normal iron status (serum ferritin >75 ng/mL) at birth. RESULTS: Twenty-three infants had latent iron deficiency. Infants with latent iron deficiency had significantly prolonged wave V latencies (7.10 ± 0.68 compared with 6.60 ± 0.66), III-V interpeak latencies (2.37 ± 0.64 compared with 2.07 ± 0.33), and I-V interpeak latencies (5.10 ± 0.57 compared with 4.72 ± 0.56) compared with infants with normal iron status (P < 0.05). This difference remained significant on regression analyses after control for confounders. No difference was noted between latencies I and III and interpeak latencies I-III. CONCLUSION: Latent iron deficiency is associated with abnormal auditory neural maturation in infants at ≥34 wk gestational age. This trial was registered at clinicaltrials.gov as NCT02503397.

Immune complex reaction after successful treatment of meningococcal disease: an excellent response to IVIG.

The convalescence phase of severe meningococcal sepsis is complicated by immune complex reactions with arthritis being the commonest. No standard guidelines exist for management of such complications, but non-steroidal anti-inflammatory drugs and steroids have been used with varying success. We report excellent response to intravenous immunoglobulin in a child with immune complex reaction following meningococcal sepsis.

Unusual presentations of malaria in children: an experience from a tertiary care center in North East India.

OBJECTIVE: To identify cases of malaria with unusual presentations. METHODS: The medical record of all the cases of malaria admitted to PICU and pediatric general ward from Oct 2006 to Sep 2009, were reviewed and cases with unusual presentations were identified. The study design was retrospective descriptive study. RESULTS: Sixteen (10%) out of 162 malaria cases had unusual presentations - three had hemiplegia, two each with viral hepatitis-like presentation, acute abdomen, gastrointestinal bleed, generalized edema and hyperglycemia and one each with ptosis, severe headache and subacute intestinal obstruction-like presentation. Eleven cases had mixed parasitemia and two each with P. vivax and P. falciparum. One case was diagnosed on clinical grounds. CONCLUSION: Malaria is a common disease, but both typical and atypical presentations deserve attention for early diagnosis and management.

Macrophage activation syndrome in malaria.

Macrophage activation syndrome (MAS) is a clinical syndrome caused by an excessive proliferation of T lymphocytes and well-differentiated macrophages; an entity distinct from malignant histiocytosis. Although rheumatologic conditions are the common cause of MAS, a wide range of infections are also seen to cause MAS. We report an adolescent with severe Plasmodium falciparum malaria and MAS. He fulfilled six out of eight criteria required to diagnose hemophagocytic lymphohistiocytosis.