Monday Blues - Rare Cause of Hypoglycemia in a Child with Leukemia

Hypoglycemia in a child with acute lymphoblastic leukemia (ALL) often makes the clinician think of sepsis or metabolic disturbances due to relative adrenal insufficiency with steroid withdrawal. We report a rare scenario of drug-induced hypoglycemia in a child on treatment for ALL. Recurrent symptomatic episodes of hypoglycemia in a 4-year-girl on treatment for high-risk ALL were analyzed and it was surprising to note that the episodes were noted on early hours on Monday and Sunday nights. Detailed evaluation for the etiology and the workup was not contributory. With the background of drug history for ALL maintenance and occurrence of episodes on Mondays, possibility of drug-induced hypoglycemia secondary to cotrimoxazole was considered. Dose alteration for trimethoprim-sulfamethoxazole was considered stopping the drug is not feasible. Malnutrition was attributed as the coexisting risk factor in our child

Primary antiphospholipid syndrome in children: experience from two tertiary centres in South India

Background: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by the presence of episodes of vascular thrombosis, recurrent fetal loss and other clinical features in the presence of antiphospholipid antibodies. The aim of the study was to analyze the clinical manifestations and immunologic profile of children presenting with APS.Methods: Authors did a retrospective case record study of patients admitted with thrombotic events between September 2013 and August 2018 and identified patients with positive antiphospholipid antibodies. Children who had clinical features of active lupus were not included.Results: The clinical and immunologic profile of 7 pediatric patients presenting with APS over 5 years from 2013 to 2018 were analysed. Symptoms secondary to vascular thrombosis were limb swelling, stroke, gangrene of toes and Budd Chiari syndrome.Conclusions:APS though rare should be considered in the differential diagnosis of children presenting with thrombotic events. They need long term anticoagulants to prevent further episodes.

Prognostic significance of receptor for hyaluronan acid-mediated motility (CD168) in acute pediatric leukemias - assessment of clinical outcome, post induction, end of treatment and minimal residual disease

ABSTRACT Introduction: The extracellular matrix protein hyaluronan acid plays an active in role in tumor cell proliferation and invasion. Hyaluronan acid receptors, namely CD168 or the receptor for hyaluronan acid-mediated motility (RHAMM) and CD44 have been implicated in promoting malignancy. There is a lacuna in data on the expression of the receptor in pediatric leukemias. Methods: Pediatric patients with acute leukemia who were diagnosed, treated and followed up in our center were enrolled. The bone marrow biopsies performed prior to treatment were subjected to immunohistochemical staining (54 biopsies: acute lymphoblastic leukemia - 45, acute myeloid leukemia - 9). Blast counts were carried out at diagnosis, end of the induction phase and end of chemotherapy, the minimal residual disease was assessed and follow up details were collected. Positivity was correlated with initial blast count, post-induction blast count, minimal residual disease and patient survival. Results: There was no correlation between the initial blast count and the percentage of blasts with RHAMM expression. The positive correlation between percentage of blasts expressing RHAMM and the post-induction blast count was moderate in acute myeloid leukemia (0.74) and mild in acute lymphoblastic leukemia (0.48). There was a statistically significant difference in RHAMM expression between the two minimal residual disease risk groups (p-value = 0.012) with a negative prognostic effect of RHAMM expression. Moreover, a negative prognostic effect of RHAMM expression was noted when patient survival was considered. Conclusion: This study shows that blasts in acute myeloid leukemia show more RHAMM positivity than those of acute lymphoblastic leukemia indicating the aggressive nature of this type of leukemia. In acute leukemias, patients with high percentages of RHAMM-positive blasts had more post-induction blasts, blasts in minimal residual disease and poorer prognosis.

Primary pulmonary diffuse large B-cell non-Hodgkin's lymphoma in a child.

A 3-year-old girl presented with a history of intermittent fever of six months duration associated with respiratory symptoms consisting of recurrent cough, fever, wheeze and a suspected history of contact with tuberculosis (TB). Chest radiograph revealed pulmonary infiltrates mimicking miliary TB. She was started on anti-tuberculous treatment, but in view of clinical deterioration, a further work-up including a lung biopsy revealed non-Hodgkin’s lymphoma (NHL). This case documents the extremely rare occurrence of pulmonary involvement and miliary infiltrates on the chest radiograph in NHL.

Propranolol Therapy for Infantile Hemangioma.

Context: There has been widespread interest surrounding the use of beta-blockers (i.e. propranolol, timolol, nadolol, acebutolol) in the treatment of infantile hemangiomas (IH). Objective: To review literature evaluating treatment of IH with propranolol. Evidence Acquisition: We conducted a literature search on PubMed and investigated for case reports, case series, and controlled trials by using search terms including “hemangioma” and “propranolol.” Results: Data suggest that beta-blockers are efficacious in cutaneous, orbital, subglottic, and hepatic hemangiomas and assist in the resolution of ulcerated hemangiomas. Improvement has also been documented in children with PHACE syndrome. Propranolol produces favorable results in children who do not respond to steroids and with no long-term adverse effects. Propranolol should be administered with caution due to rare but serious side effects including hypoglycemia, wheezing, hypotension, and bradycardia. Additionally, recurrence of lesions following the cessation of treatment has been documented. Conclusions: Although large-scale randomized controlled trials must be conducted in order to further evaluate the safety and the possible role of propranolol in the treatment of IH, the reviewed literature suggests that propranolol carries promise as a potential replacement for corticosteroids as first-line therapy or as a part of a multimodal approach.

Short stature and an interesting association.

Untreated hypothyroidism in children usually results in delayed puberty, but juvenile hypothyroidism causes isosexual precocious puberty in a rare syndrome called Van Wyk Grumbach syndrome, with a complete reversal to the pre pubertal state following thyroid hormone replacement therapy. We report here, a 7-year-old girl who presented with short stature, constipation and isosexual precocious puberty due to the long standing untreated severe hypothyroidism with this syndrome.

Hemophagocytic syndrome associated with Plasmodium falciparum infection.

Hemophagocytic syndrome (HPS) has been associated with infections, hematological malignancies and autoimmune conditions. Malaria is rarely reported to cause HPS. We report a case of an 11-month-old infant with fever, hepatosplenomegaly, pancytopenia, high serum ferritin, hypertriglyceridemia, and bone marrow hemophagocytosis, consistent with hemophagocytic syndrome. Gametocytes of plasmodium falciparum were identified on bone marrow aspiration. Rapid recovery was observed after treatment with antimalarials.

Caffey disease with raised immunoglobulin levels and thrombocytosis.

Infantile cortical hyperostosis (Caffey disease) is characterized by radiological evidence of cortical hyperostosis, soft tissue swellings, fever and irritability. We report a case of Caffey disease highlighting its presentation with thrombocytosis and high serum immunoglobulin level to alert physicians to use steroids cautiously in view of the known thrombocythemic effect of the drug. Raised Immunoglobulin also suggests that this syndrome could be infectious in origin.

Kenny-Caffey syndrome.

Kenny-Caffey syndrome is a rare hereditary skeletal syndrome characterized by dysmorphic features, severe growth retardation, classical radiological changes and hypocalcemia with hypoparathyroidism at an early age. We report an 8-month-old girl child with Kenny-Caffey syndrome who had most of the features of the syndrome. Any child with hypocalcemia who has typical facial features should raise a suspicion of this syndrome.

Paraneoplastic papilloedema in a child with neuroblastoma.

Non-metastatic neurological disease complicating neuroblastoma is well recognized. Gross papilloedema in the absence of intracranial disease as initial manifestation of neuroblastoma is reported in adults. We report for the first time a case of bilateral papilloedema in a child with neuroblastoma in the absence of intracranial disease and hypertension.

Intermittent clobazam therapy in febrile seizures.

OBJECTIVE: To evaluate the efficacy of intermittent clobazam therapy in preventing the recurrence of febrile seizures and to assess its safety. METHODS: The study was a prospective, randomized, double-blind placebo-controlled trial conducted in the Department of Child Health, Christian Medical College Hospital, Vellore between July 2001 and September 2002. Neurologically normal children between 6 months and 3 years of age with a history of febrile seizures and no evidence of acute CNS infection or EEG abnormality were included into the study. 19 children in a clobazam group and 20 in the placebo group were randomly allocated. Temperature reduction measures with paracetamol and tepid sponging were advised to all children. In addition the dispensed medication was to be administered at the onset of fever and continued for 48 hours irrespective of the duration of fever. The children were then monitored for seizures and adverse effects of clobazam. The children were followed up for a mean period of 9.9 months. The analysis was done on the number of febrile episodes in both the groups. RESULTS: There were a total of 110 episodes of fever during the study period. Mean number of febrile episodes in the clobazam group was 3.1 and in placebo group 2.56. Six (12.5%) of the 48 episodes in placebo group and one (1.7%) of 60 episodes in clobazam group had seizure recurrence. This was statistically significant (p = 0.01). Drowsiness and weakness were present equally in both clobazam and placebo groups whereas ataxia was present only in the clobazam group, the difference being statistically significant (p=0.04). CONCLUSION: Intermittent clobazam therapy is an effective measure in the prevention of recurrence of febrile seizures. The ataxia due to clobazam was much lower than that reported with diazepam.

Unusual cause for miliary lung mottling in a child.

A 12-year-old boy presented to us with a diagnosis of disseminated tuberculosis which was made based on a history of prolonged fever, multiple neck swellings and radiological findings of bilateral multiple micronodular opacities. Examination showed a diffuse thyroid gland swelling. He was diagnosed to have papillary thyroid carcinoma with distant metastases to cervical lymphnode on histopathology and to lungs.

Pulmonary aspergillosis in a child with hepatic failure.

Invasive aspergillosis is described more frequently as a complication of neoplastic disease and in immunocompromised patients. Hepatic failure is not a generally recognized risk for pulmonary aspergillosis. We report a 3-year-old boy who presented with hepatic failure and pneumonia and whose autopsy revealed liver cirrhosis and pulmonary aspergillosis.

An outbreak of echovirus meningitis in children.

An outbreak of aseptic meningitis in children as evidenced by increase in the number of admissions in a tertiary care hospital is described. Clinical data and stool samples were collected from 25 hospitalized infants and young children. The stool samples were subjected to virological investigations. Fever and vomiting were the commonest symptoms. Cerebrospinal fluid (CSF) showed lymphocytic pleocytosis in majority of cases. Of the 25 stool samples, 14 showed an enterovirus specific cytopathogenic effect (CPE) in rhabdomyosarcoma (RD) cell line. All the 14 samples were positive for enterovirus RNA by reverse transcription-polymerase chain reaction (RT-PCR). Partial sequencing of the Virion protein 1 (VPI) region of the enterovirus genome carried out on the first 7 isolates revealed 5 isolates to be echovirus serotype 4 and one each to be echovirus serotypes 3 and 30. All children showed a rapid recovery and were discharged within 3 days of admission.

Thrombocytopenic purpura as initial presentation of acute hepatitis A.

Extrahepatic immune manifestations are rare in hepatitis A virus infection. We report a 4 1/2-year-old girl who presented with thrombocytopenic purpura as initial manifestation of hepatitis A virus infection. She responded to steroid therapy.

Neonatal hypernatraemic dehydration and malnutrition associated with inadequate breastfeeding and elevated breast milk sodium.

The advantages of breastfeedng to both the mother and infant are well recognised. In rare instances if breasteding is inadequate or if the sodium content bast milk is high, malnutrition and hypernatraemia can result. A 15 days old exclusively breastfed baby presented with inadequate weight gain. On evaluation, he was found to have hypernatraemia and mother's breast milk showed high sodium concentrations. The infant needed parenteral fluid for correction of dehydration and hypernatraemia. His serum sodium and breast milk sodium of the other of the mother returned to normal gradually. He started gaining weight on exclusive breastfeeds. The present paper describes the case report and brief review of the literature.