COVID-19 associated renal artery stenosis in infancy - A report of two cases.

Organ-specific vasculitis is an uncommon, delayed complication of COVID-19 infection. It is usually seen in mildly symptomatic or asymptomatic patients. Underlying endothelitis is the most likely pathophysiological mechanism for such a manifestation. We report two infants with renal artery stenosis, most likely consequent to COVID-19 infection.

Successful balloon dilatation of an infolded polytetrafluoroethylene graft: An unusual cause of early Fontan failure.

Despite optimal medical management, an 8-year-old boy had persistent pleural drainage following total cavopulmonary connection. Detailed evaluation, including computed tomography angiography, confirmed obstruction at the lower end of the circuit due to infolding of the polytetrafluoroethylene graft. Balloon dilation of the obstruction resulted in prompt resolution of pleural effusion with sustained relief at 1-year follow-up. The case demonstrates the importance of careful assessment in the diagnosis and successful nonsurgical management of an unusual cause of obstruction in the Fontan circuit.

Hemophagocytic lymphohistiocytosis in an infant with multiple triggers!!

Hemophagocytic lymphohistiocytosis (HLH) is a disorder of immune dysregulation secondary to a massive unregulated cytokine storm and its downstream consequences. HLH is being increasingly recognized as a cause of pyrexia of unknown origin, unexplained cytopenias, and hepatic dysfunction. However, this potentially treatable condition is often missed due to lack of suspicion, variable, and nonspecific presentations, inability to fulfil all the diagnostic criteria and availability of diagnostic tests in resource limited settings. Both familial and acquired forms of HLH can be triggered by multiple factors in a susceptible patient. We report a 9-month old infant who developed HLH in association with Stevens-Johnson syndrome following massive blood transfusion.

Autoimmune Haemolytic Anaemia-A Spectrum of Presentation in Children.

Autoimmune Haemolytic Anaemias (AIHAs) are rare in children. They can be either a primary disease or secondary to/triggered by a host of other clinical conditions. We present five interesting cases of paediatric AIHA associated with infections (viral, bacterial and atypical) and autoimmune diseases {Systemic Lupus Erythematosus (SLE) and Autoimmune Hepatitis (AIH)}. The H1N1 influenza associated AIHA responded to oseltamivir and Intravenous Immunoglobulin (IvIg) while the cases secondary to Mycoplasma pneumoniae and pneumococcal bacteraemia required only treatment of the primary infection. AIHA with SLE responded well to corticosteroid therapy but the patient with AIH and AIHA succumbed to severe liver failure. Rest of the four cases with good response to therapy did not have any recurrence/relapse of AIHA during their follow up periods.

Is dengue emerging as important cause of acute liver failure in endemic regions?

Dengue virus infection continues to be major public health problem in large part of world. The epidemiology of dengue viral infection is becoming increasingly complex and has substantially changed over almost past six decades not only in terms of prevalent strains and geographical locations but also in terms of disease severity and atypical presentations. Though liver is the most common organ affected but is generally asymptomatic. We present a case of infant with severe dengue who died of fulminant hepatic failure and showed pan lobular necrosis on post mortem liver biopsy. The case is being presented to highlight life threatening complication of dengue in young children, and dengue viral infection as a cause of acute liver failure in endemic areas. Thus dengue fever should also be considered as one of the differential diagnosis in children presenting with fever and fulminant hepatic failure in endemic regions.

Delayed Treatment Response in a Neonate with Multisystem Langerhans Cell Histiocytosis Case report and review of literature.

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of the Langerhans cells, which are part of the mononuclear phagocytic system. The disorder varies in terms of the extent of the disease, its natural course and patient outcomes. While skin rashes are a common presentation of neonatal LCH, other systems or organs may also be involved. Delays in the diagnosis of neonatal LCH may occur due to its non-specific presentation and a lack of awareness of the condition among doctors. We report a two-month-old male neonate who presented to the Chacha Nehru Bal Chikitsalya hospital, New Delhi, India, in 2016 after the onset of pulmonary symptoms. He had been noted to have a generalised rash which had progressively worsened from 15 days of age. Following a skin biopsy and chest imaging, he was diagnosed with multisystem LCH with risk organ involvement. There was a delayed response to combined chemotherapy with no major side-effects.

Histoplasma capsulatum Infection with Extensive Lytic Bone Lesions Mimicking LCH.

Multiple lytic bone lesions in a child can be a manifestation of various diseases like Langerhans cell histiocytosis, metastatic neuroblastoma, leukemia, hyperparathyroidism, multifocal osteomyelitis and histoplasmosis. Disseminated histoplasmosis caused by Histoplasma capsulatum var. duboisii is well known to present with multiple osteolytic lesions in immunocompromised adults and is mostly restricted to the African subcontinent. Histoplasmosis seen in American and Asian countries is caused by Histoplasma capsulatum var. capsulatum, which presents with pulmonary and systemic manifestations and rarely bone involvement. We report a case of histoplasmosis, caused by H. capsulatum var. capsulatum with extensive lytic bone lesions in a 13 year old immunocompetent boy who presented with prolonged fever, weight loss and multiple boggy swellings. He responded to amphotericin and is currently on Itraconazole. This case is unique for extensive osteolytic lesions with H. capsulatum var. capsulatum infection in an immunocompetent child.