Anu Maheshwari

Adherence to antiretroviral therapy and its determinants in children with HIV infection – Experience from Paediatric Centre of Excellence in HIV Care in North India

The objective of this study is to assess adherence to antiretroviral therapy (ART) in HIV-infected children using the pill count method, and determine factors leading to adherence failure. Records of 106 children living with HIV (CLHIV) age <15 years and on ART for >6 months were reviewed. Average adherence to ART by pill count method over preceding six months was calculated and re-assessed by 3-day recall method. The caregivers of 105 children and one child himself were interviewed about the problems encountered while giving ART. Median age of enrolled children was 104 (inter-quartile range [IQR] 77.3–133.8) months. Median duration of ART was 25 (IQR 16–35) months. The desired adherence level of >95% during six months of review assessed by pill count was achieved in 95.3% children. The 3-day recall method yielded >95% adherence in 99% children (p ≤ .001). Caregivers of 59 children (56.2%) reported multiple problems while administering drugs. In most instances, problems encountered were related to family/caregivers, the commonest being multiple caregivers, job constraints and death/illness in the family. In conclusion, we found a very high level of adherence to ART in CLHIV. Poor adherence was mainly associated with issues related to the family/caregivers.

Rosai-dorfman Disease: A Case With Lymphadenopathy and Liver Involvement

Rosai-Dorfman disease (RDD), originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Formerly thought to be a disease process limited to lymph nodes, RDD has now been reported in many organ systems like bone, skin and soft tissue, central nervous system, eye and orbit, and upper respiratory tract. Here we report a case of RDD with hepatic involvement, which is even more rare.

Cumulative cardiac toxicity of sodium stibogluconate and amphotericin B in treatment of kala-azar.

Kala-azar or visceral leishmaniasis is a disseminated protozoal infection caused by parasites of the genus Leishmania (Leishmania donovani in India). Conventional therapy for visceral leishmaniasis continues to be pentavalent antimony (sodium antimony gluconate [SAG]). Amphotericin B is widely used for SAG-unresponsive cases and sometimes even as a first-line drug, especially in endemic areas. With the conventional regimen of SAG, cardiac toxicity has been reported in 8% to 17% of cases with 5% to 7% of them having fatal toxicity. Cardiac toxicity is uncommon with amphotericin B with only few isolated reports. We report some patients with kala-azar in whom coadministration of SAG and amphotericin B led to arrhythmia and sudden death.

Transalar transphenoidal meningoencephalocele: A rare cause of respiratory distress in a neonate

Respiratory distress in an infant is a common cause of admission in neonatal intensive care facility. Obstructive lesions of the airway constitute a minority of problems in the new born but present a diagnostic challenge. We present a 30-day-old male infant admitted with respiratory distress who was diagnosed to have an oropharyngeal cystic mass which on further evaluation by computed tomography and magnetic resonance imaging revealed a transalar transsphenoidal meningoencephalocele herniating into the oral cavity through a congenital split palate. The patient was operated successfully using a transpalatal approach leading to complete resolution of respiratory distress.

Clinical and laboratory observation systemic lupus erythematosus and acute lymphocytic leukemia: An unusual case

The association of systemic lupus erythematosus (SLE) and myeloproliferative and lymphoproliferative malignancies is widely reported. There is scarce information available with regards to the association of SLE and malignancy in children. Usually, SLE precedes the onset of lymphoproliferative disease, but the neoplasia can occur earlier or even simultaneously. There are only five pediatric cases of SLE and associated acute lymphoblastic leukemia (ALL) reported in literature. All of these except one satisfied the revised American College of Rheumatology Criteria for SLE. Three of these cases developed SLE several years after successful treatment of ALL. While two cases reported had simultaneous onset of SLE and ALL, one of the cases in this two-patient series, did not fulfill ≥4 criteria for diagnosis of SLE. We present a case of a 3-year-old boy with SLE and coexistent ALL. To the best of our knowledge, only two such cases of simultaneous presentation of both these diseases are present in literature.

Heat Stroke in an Infant With Hypohidrotic Ectodermal Dysplasia Brain Magnetic Resonance Imaging Findings

We describe the magnetic resonance imaging findings of a 4 month old infant with hypohidrotic ectodermal dysplasia who developed heat stroke. Magnetic resonance imaging of the brain revealed signal alteration with restricted diffusion in the splenium of corpus callosum, cerebral peduncles and cerebellar dentate nuclei.

Common Endocrine Problems in Children (Hypothyroidism and Type 1 Diabetes Mellitus)

Hypothyroidism and type 1 diabetes mellitus (T1DM) constitute two common endocrine disorders of children. Both these are chronic conditions which require long term follow-up for optimum control, can be missed on initial presentation unless a high index of suspicion is maintained and may be associated with adverse outcome if not properly managed. This article deals with basic pathology, recognition, initial investigations, and principles of treatment of these conditions from the perspective of a general pediatrician. It also describes the role of a practitioner in management of these disorders.

Dengue fever-like illnesses: How different are they from each other?

In tropical countries and possibly elsewhere, dengue fever can be confused with other common tropical infections like enteric fever, leptospirosis, typhus fever, malaria, etc. Many of these illnesses can present in significant numbers after rains, and because of similar early presentations, can cause confusion in decision-making. With global warming, these diseases can assume significant proportions even in non-endemic areas. Identifying these illnesses in a non-immune returning traveller is equally challenging. Recognition of these diseases is important to diagnose them and treat them early, in order to avoid potentially fatal complications. This review is an attempt to highlight important clinical and laboratory differences among dengue fever-like illnesses.

Myelofibrosis: a cause of increased transfusion requirement in a child with β-thalassemia intermedia.

A 10-year-old girl presented with gradually increasing pallor and abdominal distension for 1 year and fever for 6 months. She required 3 packed cell transfusions during this interval. Investigations done revealed that the child had thalassemia intermedia and parents were carrier of β-thalassemia gene. The increased transfusion requirement in this case prompted further investigations, which revealed the presence of myelofibrosis. Case reports of myelofibrosis and myelodysplastic syndromes with acquired hemoglobin disorders exist in literature, especially α-thalassemia. To the best of our knowledge, this is the first reported case of β-thalassemia intermedia in association with myelofibrosis.

Neonatal Kasabach-Merritt phenomenon

Kasabach-Merritt phenomenon (KMP) is a life-threatening consumptive coagulopathy in the presence of a rapidly enlarging vascular tumor. It usually presents in early infancy, but onset in early neonatal period, facial hemangioma, and vincristine use in neonates has rarely been reported. We, hereby, present a 6-day-old male child presenting with facial hemangioma and intracranial hemorrhage, and KMP responding well to steroids and vincristine. Pathophysiology of disease and various treatment options have been discussed.