Venkatesh Chandrasekaran

Koebner's Phenomenon in Childhood Henoch-Schönlein Purpura: A Report of Two Cases.

Koebners phenomenon occurs rarely in connection with Henoch‐Schönlein purpura (HSP). We report two children with HSP who developed Koebners phenomenon on the second day after the onset of rash. The first was an 11‐year‐old girl with rheumatic heart disease who presented with abdominal pain for 1 month and subsequently developed rash and nephritis. The second patient was a 7‐year‐old girl who presented with rash and polyarthritis. To the best of our knowledge, Koebners phenomenon in childhood HSP has not been reported.

Maternal and birth risk factors for children screening positive for autism spectrum disorders on M-CHAT-R

This study was carried out to screen children aged 16-30 months, attending pediatric outpatient department of JIPMER, Puducherry, during June to August 2014, for ASD using modified checklist for autism in toddlers-revised (MCHAT-R) and to find association between maternal, birth and postnatal risk factors with risk of ASD. A total of 350 mother-child pairs with children aged between 16 and 30 months were recruited. M-CHAT-R was administered to all mothers to screen for ASD along with risk checklist. Based on screen result children were classified as ASD (high risk) and no ASD (low and medium risk) group. The association between risk factors and screen positivity for ASD was studied using odds ratio. According to our study, 33 (9.4%) screened positive for ASD. Mean age was 21 months. High mean paternal age at birth (P value 0.025), need for resuscitation at birth (OR 3.4, 95% CI 1.47-8.10), NICU stay >12h (OR 4.7, 95% CI 2.26-9.94), late initiation of breastfeeding (OR 3.9, 95% CI 1.83-8.39), neonatal seizures (OR 11.8, 95% CI 5.38-26.25) were associated with screen positivity for ASD. After adjusting for confounding, neonatal seizures, and maternal concern about child development were associated with increased odds of screening positive for ASD whereas exclusive breast feeding in the first 6 months of life is associated with decreased odds. Screening for ASD in children with above risk factors might help in early initiation of remedial interventions.

Basidiobolomycosis complicated by hydronephrosis and a perinephric abscess presenting as a hypertensive emergency in a 7-year-old boy

Abstract A 7-year-old boy presented with a chronic, indurated, tender left thigh swelling in association with a hypertensive emergency. He had a bilateral moderate degree of hydronephrosis and a left perinephric abscess, and MRI features of posterior reversible encephalopathy syndrome. Histopathological examination of the biopsy specimen demonstrated eosinophilic fasciitis with filamentous fungi. Basidiobolus ranarum was isolated from the culture. The fungus was also isolated from a perinephric fluid aspirate. Computerised tomography of the abdomen demonstrated features consistent with fungal invasion of the pelvic floor muscles and urinary bladder, leading to bilateral hydronephrosis. He required multiple antihypertensive drug therapy and was treated with intravenous amphotericin B, oral itraconazole and potassium iodide. Antihypertensive agents were discontinued after 2 weeks of antifungal therapy. At 6-months follow-up, the hydronephrosis had resolved completely. Perinephric abscess associated with basidiobolomycosis has not been reported previously.

Fulminant BCG Disease in a 7 Month Old Healthy Male Infant.

A seven month old healthy male infant was brought with papular skin lesions all over the body, which became ulcerative with increasing fever and redness within 1 week duration. On examination, Bacilli Calmette Guerin (BCG) scar was ulcerated with discharge; infant was irritable with tachycardia and tachypnea. Investigations revealed pancytopenia, and acid fast bacilli was positive in skin lesions and at BCG scar site. There was progressive worsening of infants condition, culminating in death.

Acute Motor Axonal Polyneuropathy Following Mumps Infection in a 9-Year-Old Girl

A 9-year-old girl presented with lower motor neuron type of paralysis involving limbs, trunk and multiple cranial nerves (7, 9 and 10) with preceding history of mumps 1 week before the onset of weakness. There were no features to suggest either a meningitis or encephalitis in the child. Cerebrospinal fluid showed hypoglycorrhachia and mild protein elevation; magnetic resonance imaging of the brain was normal. Nerve conduction study showed motor axonal neuropathy. Serology for mumps IgM was positive, consistent with a diagnosis of post-mumps acute motor axonal polyneuropathy. The girl made a complete recovery within 3 weeks.

Role of exclusive breastfeeding in conferring protection in children at-risk for autism spectrum disorder: results from a sibling case–control study

Background: Gut microflora influences neural development through complex mechanisms. Feeding practices, especially breastfeeding influence gut microbiome and thereby play a pivotal role in immune and neural development. Current understandings of the role of healthy distal gut microflora in the development of immune and neural systems provide insights into immunological mechanisms as one of the possible etiologies in autism spectrum disorder (ASD). Studies have shown that optimal breastfeeding is associated with lower odds of being at-risk for ASD and children with ASD are suboptimally breastfed. Methods: The feeding practices of children with ASD (n = 30) was compared to their typically developing siblings as matched controls (n = 30). Information regarding feeding practices was collected from mothers through a semi-structured questionnaire. Results: About 43.3% of children with ASD received exclusive breastfeeding, whereas 76.7% of their typically developing siblings were exclusively breastfed. Exclusive breastfeeding was associated with lower odds for ASD (odds ratio [OR] = 0.166; 95% confidence interval [CI] = 0.025–0.65), while early introduction of top feeds was associated with higher odds (OR = 6; 95% CI = 1.33–55.19). Difficulties in breastfeeding were attributed to child-related factors in 13.2% of the children. Conclusion: Children with ASD are suboptimally breastfed compared to their typically developing siblings. Exclusive breastfeeding may confer protection in vulnerable children. Further studies on larger prospective sample are required to establish the association.

Implications of comorbid ADHD in ASD interventions and outcome: Results from a naturalistic follow up study from south India

BACKGROUND Autism Spectrum Disorder (ASD) is a neurodevelopmental disorder commonly associated with Attention Deficit Hyperactivity Disorder (ADHD), the prevalence ranging from 14-70%. The current study attempted to assess the impact of comorbid ADHD in children with ASD, in terms of challenges in diagnosis, treatment, intervention outcomes and parental stress and coping through a naturalistic design. METHODS Fifty children aged 2-6 years with ASD were recruited, assessed and followed up for six months. Twenty children were found to have comorbid ADHD. Severity of ASD and ADHD was assessed by Childhood Autism rating scale and Connors abbreviated rating scale respectively. Parental stress and coping was assessed by Family Interview for stress and coping. RESULTS The diagnosis of ASD was apparently obscured by ADHD symptoms in about 22% of cases, as only diagnosis of ADHD was made at the time of referral to our centre. ADHD was the most common comorbidity followed by intellectual disability and seizure disorder. About 66% of children received combination of pharmacological and behavioral interventions. Clonidine was the most common medication to be used and was well tolerated. The improvement in ADHD symptomatology showed positive correlation with improvement with ASD-specific interventions as reflected by change in severity scores. Severity of ADHD significantly also predicted parental stress and coping, and thereby engagement in ASD-specific interventions. CONCLUSION The current study highlights the need for screening and early diagnosis of comorbid ADHD in children with ASD and vice versa considering the management challenges. In case of multiple comorbid neurodevelopmental disorders, early interventions for one disorder can improve the outcome of the other.

Severe acute kidney injury in children owing to infective endocarditis-associated immune complex glomerulonephritis: a report of two cases

Renal involvement in infective endocarditis (IE) may manifest with different clinical patterns, including diffuse proliferative glomerulonephritis and crescentic glomerulonephritis, which may lead to haematuria and/or proteinuria. However, severe acute kidney injury (AKI) in such cases is extremely uncommon and is reported mostly in adults. Two children with rheumatic heart disease and a peri-membranous ventricular septal defect, respectively, who developed haematuria, proteinuria and severe AKI in association with IE are reported. The first case had diffuse proliferative glomerulonephritis with 10% cellular crescents, and made a complete renal recovery with antibiotics and intravenous methylprednisolone followed by oral prednisolone. However, the second case had severe crescentic glomerulonephritis which led to residual renal injury despite intravenous methylprednisolone and cyclophosphamide in combination with antibiotics. The cases illustrate that crescentic glomerulonephritis or severe diffuse proliferative glomerulonephritis should be considered as possible complications in children presenting with haematuria, proteinuria and severe AKI. Renal biopsy along with antibiotic therapy and prompt immunosuppressive therapy should be considered for the management of this potentially life-threatening condition.

Chronic Pruritus in an 18-Month-Old Male Infant Due to Anicteric Cholestasis

An 18-month-old male infant was referred to us with pruritus from 6 months of age. He was being managed as atopic eczema with emollients and antihistamines without any response to treatment. On examination, he was found to have extensive scratch marks, mild hepatomegaly and no icterus. Blood investigations were suggestive of anicteric cholestatic liver disease. Liver biopsy was suggestive of progressive familial intrahepatic cholestasis type 3. He showed symptomatic improvement after starting ursodeoxycholic acid and fat-soluble vitamins and is under follow up.

Sub-conjunctival Hemorrhage Following a Bout of Cough: A Harbinger of Underlying Bleeding Diathesis

To the Editor:A five-year-old girl presented with red eyes for 15 d following a brief bout of cough illness. There was no pain, itching or watering of eyes or diminution of vision. There was no history of bleeding from any other site and no history of recent trauma. However, there was a history of prolonged bleeding from the umbilical stump in the neonatal period and also following trivial trauma in the past which had been forgotten. She was born out of a non-consanguineous marriage with no family history of bleeding diathesis. Examination revealed bilateral subconjuctival hemorrhage (Fig. 1). Clinical examination was otherwise unremarkable. Investigations revealed Hb of 9.3 g/dl , p la te le t count of 508,000/cu .mm, Prothrombin time (PT): > 240 s (INR >4); activated Partial thromboplastin time (aPTT): > 240 s; Thrombin time (TT): >180 s. Liver function test (LFT) was normal. A diagnosis of congenital fibrinogen deficiency was suspected. Serum fibrinogen level was 29 mg/dl (normal value 200–400 mg/dl). In the hospital she developed prolonged bleeding from the venepuncture sites which subsided with two units of fresh frozen plasma. At two weeks follow-up, the sub conjunctival hemorrhage had completely resolved. Inherited disorders of fibrinogen can either be a type 1 defect comprising afibrinogenemia and hypofibrinogenemia (quantitative deficiency) or a type 2 defect comprising dysfibrinogenemia (qualitative defect) [1]. Afibrinogenemia is inherited as an autosomal recessive condition presenting as umbilical cord bleeding, cutaneous, gastrointestinal and rarely, joint or central nervous system bleeds. Antenatal onset intracranial bleeds and spontaneous splenic rupture have also been reported [2, 3]. These children have prolonged PT, aPTT and TT. Hypofibrinogenemia is transmitted as an autosomal dominant or recessive condition and blood fibrinogen levels are usually above 0.1 g/L. Bleeding manifestations are less severe than afibrinogenemia [1, 4]. Rarely, they may be associated with intraosseous pseudotumors [5]. PT, aPTT and TT are prolonged. In dysfibrinogenemia, fibrinogen levels are usually between 1.5 and 3.5 g/L and thrombosis is more common than hemorrhage [1]. PT and aPTT are normal with prolonged TT. Treatment for all the above