MOYAMOYA disease in an adolescent with HIV-1.

Moyamoya disease has been reported in both children and adults with HIV-1. Most cases reported in children were found to have unsuppressed viral loads and low CD4 counts. Although the aetiology of the disease is largely unknown, a few studies have postulated cytokine imbalance and immune activation as possible causes. Intimal staining of the involved cerebral arteries have revealed transmembrane glycoprotein of HIV-gp 41. We present the case of an 18-year-old boy with congenitally acquired HIV-1 who presented with right hemiparesis at the age of 12 years and was found to have Moyamoya disease on neuroimaging. His CD4 count has always been low (<100 cells/cumm) in spite of being virally suppressed. He was started on anti-retroviral therapy at 5 and half years of age and he was continued on the same. He was treated conservatively and he continues to have residual right hemiparesis.

SARS-CoV-2 Infection in an Adolescent With X-linked Agammaglobulinemia.

We present a case of a 17-year-old boy with X-linked agammaglobulinemia who had mild disease when initially infected with SARS-CoV-2 but after recovering from acute infection developed fevers and a raised erythrocyte sedimentation rate that persisted for several weeks without any ongoing respiratory symptoms. Multiple nasopharyngeal swabs were found to be negative for SARS-CoV-2 during the febrile period, but typical changes of COVID-19 on high resolution CT chest scan led to the detection of SARS-CoV-2 on RT-PCR in a sample from a bronchoalveolar lavage. His fevers completely resolved after a 5-day course of remdesivir.

Cephalosporin-resistant typhoid.

Typhoid fever is endemic in developing countries like India. An increasing prevalence of resistance to cephalosporins and fluoroquinolones by Salmonella isolates is seen. We present an 8-month-old boy with invasive Salmonella typhi disease. Blood culture showed S. typhi sensitive to ampicillin-sulbactam and cotrimoxazole but resistant to fluoroquinolones and third-generation cephalosporins. Cerebrospinal fluid examination revealed an aseptic meningitic picture. He was treated with intravenous meropenem and azithromycin following which his condition improved. This case highlights the need for improvement in environment sanitation and hygiene combined with early vaccination against typhoid fever and antimicrobial stewardship to help reduce the emerging resistance to cephalosporins and fluoroquinolones.

Neonatal cholestasis mimicking biliary atresia: Could it be urinary tract infection?

Cholestasis can occur in newborns due to infections. However, the manifestations of the underlying infections usually dominate the presentation. We present a 2-month-old infant who presented with jaundice and no fever or signs of systemic illness. Liver biopsy was suggestive of cholangitis. He was subsequently detected to have urinary tract infection with Klebsiella pneumoniae. The child was treated with appropriate antibiotics for 2 weeks following which the cholestasis resolved. Thus, neonatal cholestasis due to infections can also occur in the post-neonatal period without clinical manifestations of an underlying infection.

Toxoplasmosis with chorioretinitis in an HIV-infected child with no visual complaints-importance of fundus examination.

Central nervous system lesions are common in HIV-infected patients. In the combination anti-retroviral therapy (ART) era, Toxoplasma reactivation has been observed only in patients with unrecognized HIV infection or refusing therapy. We present the case of 10-year-old girl with AIDS who initially presented with pneumonia. She was treated for pneumonia and thereafter started on ART as her CD4 count was low. However, 5 days after starting ART she presented with left ptosis and right-sided monoparesis. She was diagnosed with neurotoxoplasmosis and responded successfully to pyrimethamine-sulfadoxine therapy. Though she had no vision difficulties, her fundus examination revealed chorioretinitis during the hospital stay. We emphasize the importance of routine fundus examination prior to starting ART to rule out chorioretinitis even in an older child with no visual complaints.

Opsoclonus-myoclonus-ataxia syndrome in an HIV-infected child.

Opsoclonus-myoclonus-ataxia (OMA) syndrome typically presents with chaotic eye movements and myoclonus with some patients exhibiting ataxia and behavioural disturbances. The pathogenesis may be inflammatory with an infectious or paraneoplastic trigger. We present a 13-year-old HIV-infected girl who was initially started on highly active antiretroviral therapy (HAART) in March 2013 with a CD4 count of 79 cells/cumm. Initially, the patient did not comply with treatment, resulting in a CD4+ count of 77 cells/mm3 in November 2015 and prompting a new HAART scheme comprising lamivudine, tenofovir and ritonavir-boosted atazanavir. Shortly after starting this scheme, she developed OMA syndrome in January 2016. She was treated with intravenous immunoglobulin and methylprednisolone followed by oral steroids along with oral clonazepam and gradually recovered. We suggest immune reconstitution inflammatory syndrome as a possible aetiology of OMA in HIV-infected children.

Frontal lobe tuberculoma.

Tuberculomas are usually infratentorial in children, and supratentorial lesions predominate in adults. We present a 4-year-old girl with multiple seizures, papilloedema and brisk reflexes. On investigation, she was found to have a large left parafalcine tuberculoma. She was treated with antitubercular treatment (ATT) and steroids. The child improved, seizures stopped and the papilloedema gradually disappeared. Follow-up magnetic resonance imaging brain after 8 months showed a mild reduction in the size of the lesion. Child is on regular follow-up.

Methicillin resistant Staphylococcus aureus meningitis.

Methicillin resistant Staphylococcus aureus (MRSA) meningitis is rarely known to occur in children. We report an 11-year-old girl with fever, headache and vomiting, right hemiparesis with left-sided upper motor neuron facial nerve palsy and bladder incontinence. On investigation, she was found to have MRSA meningitis with an acute left thalamo-corpuscular infarct. She was treated with vancomycin, linezolid and rifampicin. She recovered successfully with residual right-sided lower limb monoparesis. MRSA meningitis is rare but can occur in children.