Low-dose prophylaxis and its impact on the health of haemophilia patients.

BACKGROUND AND OBJECTIVES: There is convincing evidence to show that low-dose prophylaxis (LDP) results in reduction in annualized bleeding rate (ABR) and better health-related quality of life (HRQoL) compared with on-demand or episodic treatment (ET) in haemophilia patients. The aim is to review various LDP protocols practised for the treatment of haemophilia, specifically in resource-limited countries. METHODS: A literature survey was made of articles published in English language in PubMed and EMBASE without any time limit using keywords 'low dose', 'prophylaxis' and 'haemophilia' in different combinations. RESULTS: A total of 19 reports involving LDP in patients with haemophilia were included in this review. Almost all studies reported reduction in ABR, improvement in joint function, pain and HRQoL compared with ET, but this did not fully translate into significant improvement in structural arthropathy already caused by earlier bleeds, suggesting that LDP may be less or ineffective in either stopping or reversing the damage. Individualized dose escalation protocols based on pharmacokinetic (PK) or clinical parameters were found to be superior to fixed LDP protocols and cost-effective compared with standard dose protocols. CONCLUSION: The developing countries can initiate LDP as the first step of prophylaxis, but certainly this should not be the final goal of the health care system in any country. Due to the complex pathophysiological mechanisms underlying haemophilic arthropathy, long-term data on LDP in haemophilia patients are warranted.

Congenital Chikungunya--A Cause of Neonatal Hyperpigmentation.

A 12-day-old neonate presented with ill-defined dark pigmentation over the centrofacial area with flagellate pigmentation on the trunk and patchy pigmentation on the extremities. The mother had a history of fever starting a week before delivery and continuing for 3 days in the postpartum period. Together these led to consideration of a possible diagnosis of congenital chikungunya, which was confirmed according to the immunoglobulin M antibodies to chikungunya in the mother and child. The rare occurrence of cutaneous pigmentation was the only clue to the retrospective diagnosis of neonatal chikungunya. Chikungunya is an emerging viral disease that can be transmitted maternally during pregnancy and in the peripartum period. It can be added to the list of viral infections that can lead to fetal demise or, when present during labor and delivery, can cause neonatal disease with cutaneous signs.

Isolated intramuscular cysticercosis: a case report.

Human cysticercosis is caused by Cysticercus cellulosae, larvae of a tapeworm, Taenia solium. Cysticercosis can involve any tissue in the body; the most common affected sites are central nervous system, subcutaneous tissue, eyes, and muscles. A few cases of isolated intramuscular cysticercosis without any other tissue involvement have been reported in pediatric population. Here, we report a case of intramuscular cysticercosis diagnosed by ultrasonography in a 5.5 year-old boy who presented with the swellings over the calf and the scapular region, without any associated neurological or ocular involvement. The patient responded well to the course of steroids and Albendazole with complete resolution of both the swellings.

Mobile phone use by young children and parent's views on children's mobile phone usage.

Aims: This study aims to explore the prevalence of mobile phone use among young children aged 6 months to 4 years. We studied the usage patterns, optimal age for use, and the attitudes of parents toward their child's mobile phone use. Methods: We conducted a cross-sectional study in a pediatric OPD of a tertiary teaching hospital for a period of 2-months. Ethics committee approval and informed consent was taken before conducting the research. A predesigned and validated questionnaire was used to collect data. We calculated a sample size of 90 children at a 95% confidence level. Chi-square test and Fischer's exact test were used as a test of significance at 5% level of significance. Results: We observed that 73.34% of children were using mobile phones and mobile phone usage increased with age. Children used mobile phones for educational purposes (43.9%), and for less than an hour a day (57.6%). In the 3-4 year age group, 19% used mobile phones for 3 hours or more. While 93.3% of parents felt they shouldn't give their child a phone, 71.4% children of these parents still used one. Conclusions: Our study highlights a high prevalence of mobile phone use among young children aged 6 months to 4 years. Although parents aimed to limit their child's phone usage, the reality was different. We recommend that guidelines on mobile phone use be followed in India.

COVID-19 and preeclampsia: The unique and the mutually nonexclusive clinical manifestations.

Preeclampsia (PE) is a serious, unpredictable hypertensive disorder of pregnancy present in around 8-10% of all pregnancies resulting in high rate of maternal and fetal morbidity and mortality. With the pathophysiology partially known, delivery is the only cure for PE. The disease sets due to multiple pathologic processes involving endothelial cell activation, inflammation, multiorgan damage and syncytiotrophoblast stress.  Though the primary target organ is lungs in COVID-19, other systemic manifestations which include endothelial dysfunction, dysregulated angiogenesis, thrombosis, liver injury, thrombocytopenia, hypertension and kidney damage overlap with PE. COVID-19 patients show a higher incidence of PE as compared to their noninfected counterparts and vice versa. Similar pathophysiology and clinical features make differential diagnosis challenging. For effective and specific management, it is important to differentiate actual PE from COVID-19 with PE like features. There are contradictory reports about the accuracy of diagnostic tools in distinguishing PE from severe COVID-19 with PE like features. With the available data, it can only be stated that PE is a common adverse pregnancy event, which may be exacerbated by, or may exacerbate, COVID-19. Future research should focus on cohesive understanding of the pathophysiology of the clinical manifestations, and preventive strategies during pregnancy.

Maternal ingestion of diclofenac leading to renal failure in newborns.

BACKGROUND: The use of non-steroidal anti-inflammatory drugs, such as indomethacin, ibuprofen, and nimesulide, during pregnancy has been reported to cause nephrotoxicity in the fetus. However, neonatal renal failure following antenatal exposure to diclofenac has not been reported in the literature. We report three cases of neonatal renal failure, including a pair of twins, following ingestion of diclofenac by the mother during pregnancy. CASE-DIAGNOSIS/TREATMENT: Cases 1 and 2 involved a pair of twins born to a mother with oligohydramnios. The first twin had nonoliguric renal failure with incomplete recovery at day 17 of life. The second twin developed anuria and hyperkalemia on day 2 of life, for which peritoneal dialysis was initiated. After 20 days of peritoneal dialysis, the second twin remained oligo-anuric, developed peritonitis, and died. Case 3 involved a female infant born to a primigravida with severe oligohydramnios. The baby developed oliguria and renal failure after birth, which was managed conservatively. Creatinine normalized by day 15 of life and remained normal at 1 year of age. Ultrasonography in the first week of life showed that all three infants had normal-sized kidneys. Both mothers had been administered diclofenac during pregnancy. CONCLUSIONS: In utero exposure to diclofenac may be associated with neonatal renal failure that may be transient or irreversible. We recommend that the use of diclofenac during pregnancy be avoided.

Yoga in Children with Epilepsy: A Randomized Controlled Trial.

CONTEXT: Majority of epilepsy begins in childhood. Twenty to thirty percent of patients may not respond to antiepileptic drugs. Yoga as a complementary therapy has been found to be beneficial in adults, but has not yet been studied in children with epilepsy. AIM: To study the effect of yoga on seizure and electroencephalogram (EEG) outcome in children with epilepsy. SETTING AND DESIGN: A randomized controlled trial was conducted in the pediatric neurology outpatient department of a tertiary care teaching hospital. MATERIALS AND METHODS: Twenty children aged 8-12 years with an unequivocal diagnosis of epilepsy on regular antiepileptic drugs were enrolled. Yoga therapy was provided to 10 children (study group) and 10 children formed the control group. Yoga therapy was given as 10 sessions of 1h each. We compared seizure frequency and EEG at baseline, 3, and 6 months. Statistical analysis was carried out using standard statistical tests. A P value of <0.05 was considered significant. RESULTS: No children had seizures at the end of 3 and 6 months in the study group. In the control group, at 3 and 6 months, four and three children, respectively, had seizures. Eight children each in both the groups had an abnormal EEG at enrollment. At the end of 6 months, one EEG in the study group and seven in the control group were abnormal (P = 0.020). CONCLUSION: Yoga as an additional therapy in children with epilepsy leads to seizure freedom and significant improvement in EEG at 6 months.

Haemophagocytic lymphohistiocytosis: a cause of unresponsive malaria in a 5-year-old girl.

A 5-year-old immunocompetent girl presented with fever, jaundice, hepatosplenomegaly and pancytopenia. The peripheral blood smear demonstrated mixed malaria infection (Plasmodium vivax and Plasmodium falciparum). Fever was persistent despite antimalarials in the absence of any coexisting bacterial or viral infection. Laboratory findings included cytopaenia, hyperbilirubinaemia, hyperferritinaemia, hypertriglyceridaemia, hyponatraemia, deranged partial thromboplastin time, decreasing ESR and megaloblastic changes on bone marrow aspiration. A final diagnosis of haemophagocytic lymphohistiocytosis (HLH) with megaloblastic anaemia associated with severe mixed malaria was made. There was a dramatic response to corticosteroid treatment with improvement in her clinical condition. This report endorses the use of corticosteroids in malaria-associated HLH whenever there is no clinical improvement with antimalarials alone.

Concurrent malaria and dengue Fever: a need for rapid diagnostic methods.

Malaria and dengue fever are endemic in the South-East Asian region including India. Both the illnesses share similar symptomatology, but differ in certain respects such as different- causative organisms and mosquito vector with diverse habitat. Hence, concurrent malaria and dengue fever in the same patient is said to be unusual. There have been cases of concurrent malaria and dengue, but they are scarce from highly endemic region like ours. Here, we describe three unusual cases of Plasmodium vivax and dengue co-infection diagnosed by use of rapid diagnostic tests. Early diagnosis and timely intervention is crucial in managing such patients.

Hemophagocytic lymphohistiocytosis associated with cytomegalovirus infection in an immunocompetent infant: a diagnostic and therapeutic challenge!

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal syndrome that results from inappropriate activation of the immune system. Many viral agents are known to trigger HLH but cytomegalovirus (CMV) associated HLH is rarely described. We report a case of CMV related HLH in a 3½ month old immunocompetent male infant who presented with fever, respiratory distress and hepatosplenomegaly. He had fulminant sepsis like course in the hospital as he continued to have hectic fever spikes, progressive pneumonia, increasing hepatosplenomegaly and multiple episodes of generalized convulsions. Investigations revealed bicytopenia, biochemical hepatitis, hyperferritinemia and hypofibrinogenemia. CMV IgM serology was reactive in both infant and mother. Diagnosis of CMV-HLH was made as per HLH 2004 diagnostic protocol. Infant was successfully treated with intravenous ganciclovir along with dexamethasone and etoposide.

Haemophagocytic lymphohistiocytosis: a cause of unresponsive malaria in a 5-year-old girl.

A 5-year-old immunocompetent girl presented with fever, jaundice, hepatosplenomegaly and pancytopenia. The peripheral blood smear demonstrated mixed malaria infection (Plasmodium vivax and Plasmodium falciparum). Fever was persistent despite antimalarials in the absence of any coexisting bacterial or viral infection. Laboratory findings included cytopaenia, hyperbilirubinaemia, hyperferritinaemia, hypertriglyceridaemia, hyponatraemia, deranged partial thromboplastin time, decreasing ESR and megaloblastic changes on bone marrow aspiration. A final diagnosis of haemophagocytic lymphohistiocytosis (HLH) with megaloblastic anaemia associated with severe mixed malaria was made. There was a dramatic response to corticosteroid treatment with improvement in her clinical condition. This report endorses the use of corticosteroids in malaria-associated HLH whenever there is no clinical improvement with antimalarials alone.