Lesa Dawman

Evaluation of Risk Factors Associated with First Episode Febrile Seizure

INTRODUCTIONnFebrile seizure (FS) is the single most common type of seizure seen in children between 6 months to 5 years of age. The purpose of our study was to identify the risk factors associated with the first episode of febrile seizures, which would help in the better management and preventive measures in children at risk for FS episodes.nnnAIMnTo evaluate the risk factors associated with the first episode of febrile seizures in Indian children.nnnMATERIALS AND METHODSnThis was a hospital based, case control study. The purpose of this study was to identify the risk factors associated with the first FS episode in children. Seventy (70) children between age 6 months to 5 years with their first episode of FS were compared with 70 children with fever but without seizures based on various risk factors.nnnRESULTSnThe mean age was 24.90±16.11 months in cases and 26.34±16.93 months in controls. Male: female ratio was 2:1. A positive family history was found in 31.4% of first degree and 11.4% in second degree relatives. Mean maximum temperature was 102.06±1.1°F and URI (upper respiratory infection) was most common cause of fever. Antenatal complication was significantly higher in the case group. RBC (Red Blood Cells) indices like lower mean haemoglobin, MCV (Mean Corpuscular Volume), MCH (Mean Corpuscular Haemoglobin concentration) and higher RDW (Red Cell Distribution Width) values were seen in patients. Serum sodium, Serum calcium and random blood sugar values of the cases were significantly lower than those of controls (p<0.05).nnnCONCLUSIONnOur study shows that male gender, family history of febrile seizures, peak body temperature, underlying cause of fever, antenatal complications, low serum calcium, sodium, blood sugar and microcytic hypochromic anaemia are the risk factors associated with the occurrence of first episode of febrile seizure and, thus, preventive measures in removing these risk factors could lead to a decrease in incidence of FS.

Risk Factors for Steroid Dependency in Children with Idiopathic Nephrotic Syndrome in India

To the Editor: Minimal change nephrotic syndrome (MCNS) is the most common cause of idiopathic nephrotic syndrome (INS) with a 2:1 predilection for boys. INS has a more favourable prognosis and is characterised by steroid responsiveness in approximately 90 % of patients [1, 2]. The incidence of relapse rate is high and approximately half of them develop steroid dependency. Many predictors of steroid dependency have been proposed i.e., duration from initial steroid therapy to response, concomitant upper respiratory tract infection during relapse, relapse during the first half year after disease onset, low serum protein level, gender, age at onset, total cholesterol and steroid regimen used. We conducted our study to determine the predictive risk factors for development of steroid dependency in children with INS. The records of 125 children with steroid responsive INS were retrospectively studied; out of which 38 children (23.4 %) were steroid dependent. It was found that time duration of remission (<10 d) from start of therapy (p 0.00, OR= 1.1, 95 % CI=1.0–1.1) and steroid regime other than 6 wk daily (2 mg/kg)+6 wk alternate day (1.5 mg/kg) (p 0.01, OR= 5.6, 95 % CI=1.3–24.3) were found as significant predictors. The relapsing nature of the disease presents a major challenge, and 60 % of the patients have a high relapse rate [3], with a subsequent risk of steroid toxicity and complications and with half of those relapsing eventually becoming steroid dependent [4]. By early identification of those children who are likely to develop steroid dependency and its attendant morbidity, the attending clinician will be able to plan the long term management of these children and minimizing the steroid side effects.

Malignancy associated hemophagocytic lymphohistiocytosis in children

Aim of Study: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of immune dysregulation resulting in abnormal T-cell activation and inflammatory cytokine production which produces a constellation of clinical features unique to HLH. Pediatric secondary HLH is usually triggered by infection, malignancy, or rheumatological disorders. The diagnosis of malignancy-associated HLH (MA-HLH) poses a difficult challenge as clinical features may be attributed to the underlying disease or chemotherapy. Our study aimed to see the occurrence of this rare entity at our centre. Materials and Methods: Data were collected from all pediatric oncology patient treated at our center with the diagnosis of MA-HLH from January 2012 to December 2014. Data were collected for age, sex, underlying disease, treatment protocol, stage of chemotherapy, any underlying infection, treatment given for HLH, and outcome. Results: There were five patients with a diagnosis of MA-HLH in the study period. Age ranged from 18 months to 9 years. Of the five MA-HLH, two patients had acute lymphoblastic leukemia, two acute myeloid leukemia, and one had Hodgkin lymphoma. The three patients who had documented microbiological infection also did not improve after appropriate treatment. Two patients died during treatment. One patient improved completely on steroid alone. One patient received HLH 2004 induction. Conclusion: The delay in the diagnosis of MA-HLH in pediatric patients may be due to decrease awareness about the condition the timely diagnosis of MA-HLH is crucial for a better outcome. Herein, we discuss our experience with this rare entity in pediatric oncology patients with review of literature.

Localized lipodystrophy following single dose intramuscular gentamycin injection

1. Sammut S, Malden N, Lopes V. Facial cutaneous sinuses of dental origin ‐ A diagnostic challenge. Br Dent J 2013;215:555‐8. 2. Al‐Kandari AM, Al‐Quoud OA, Ben‐Naji A, Gnanashekhar JD. Cutaneous sinus tracts of dental origin to the chin and cheek: Case reports. Quintessence Int 1993;24:729‐33. How to cite this article: Pereira T, Shetty S. Pseudo-osteomyelitis. Indian Dermatol Online J 2017;8:372-3.

Primary angiitis of the central nervous system in a 7-month-old infant.

To the Editor-in-Chief: Primary central nervous system (CNS) vasculitis of childhood is a rare and serious but potentially reversible inflammatory brain disease. Effective treatment can be initiated as soon as possible with early recognition of this rare condition. Here, we are reporting primary CNS vasculitis in an infant, who presented with seizure. A 7-month-old previously healthy girl child presented with left focal seizure, which lasted for 10 min and self-aborted. There was no history of trauma, fever, rash, vomiting, ear discharge, and loss of consciousness or similar illness in the past or in other family members. At the time of admission, the child was active and alert with left upper motor neuron (UMN)-type hemiparesis and left UMN facial palsy. The rest of the systemic examination was essentially normal. On laboratory investigations, the child had normocytic normochromic anemia (Hb—11.2 g/dL) and leukocytosis (17,000/uL), with normal platelet count (3.67 lac/uL). Serum electrolyte was normal. Cerebrospinal fluid (CSF) examination revealed normal opening pressure with pleocytosis (cell counts—26/mm) and mild elevated protein (76 mg/dL). Brain MRI with contrast enhancement was suggesting of CNS vasculitis (Fig. 1a, b). In view of possibility of vasculitis, magnetic resonance angiography (MRA) was done which showed vessel stenosis (Fig. 2). Further work-up for vasculitis was carried out, which revealed an erythrocyte sedimentation rate (ESR) of 50 mm/h with elevated C-reactive protein (CRP—11.2 mg/L). Antinuclear antibody (ANA), rheumatoid factor (RF), and serum C3 and C4 were within normal limit. ANCA-PR3 (54.23 RU/mL) was elevated (normal < 20). Lipoprotein (a), homocysteine, protein C and S, and antithrombin III levels were normal too. No mutation was identified in factor V Leiden. Echocardiography did not reveal any structural heart disease. Tandem mass spectrometry and urinary organic acid profile were normal. On the basis of elevated ANCA, CRP, ESR, and typical angiographic findings with isolated central nervous system involvement, diagnosis of primary CNS vasculitis was made. The child was started on phenytoin (5 mg/kg/day) following which there was no recurrence of seizure along with prednisolone (2 mg/kg/day) and aspirin (5 mg/kg/ day). Focal neurological deficits were improved after 2 weeks of presentation. Dose of prednisolone was tapered after 6 weeks of full dose and subsequently stopped. No relapse neither seizure recurrence was recorded in the next 12 months, and the child was developmentally normal. CNS vasculitis is an inflammatory brain disease, which can present with devastating neurological deficits. It can be primary or secondary. Primary CNS angiitis is an idiopathic vasculitis usually involving large/medium or small vessels presenting with neurological symptoms. Large–medium-vessel vasculitis usually presents with headache and acute hemiparesis with sensory deficit. Subjects are usually diagnosed with help of conventional angiography [1]. Small-vessel vasculitis usually presents with intractable seizure, ataxia, and cognitive decline with behavioral abnormality. Angiographic finding in children with small-vessel vasculitis is usually negative and requires brain biopsy for confirmation of the diagnosis [2]. Typical MRA findings are vessel stenosis [3] in * Indar Kumar Sharawat sherawatdrindar@gmail.com

Mesenchymal Hamartoma of Chest Wall in an Infant: Mimicking Persistent Pneumonia.

Mesenchymal Hamartoma of the chest wall (MHCW) is a very rare benign tumour. They are usually discovered in infancy. Spontaneous regression is known to occur in this benign condition. Management is surgical removal of mass if respiratory compromise is present. Conservative management is preferred modality in asymptomatic children as malignant transformation is not reported. Herein, we present a case of MHCW in a 5 month old infant presenting with acute respiratory distress with history of respiratory problem at 3 months of age. Child was suspected to have persistent pneumonia in view of radiological findings. Childs respiratory distress improved with antibiotics and bronchodilators. Respiratory symptoms in MHCW are due to extrinsic compression of lung parenchyma. Present case had respiratory symptoms with persistent radiological findings leading to suspicion of persistent pneumonia. His respiratory symptoms and exacerbation on follow up was attributed to hyper reactive airway disease and MHCW was managed conservatively. The non-neoplastic nature, characteristic presentation, histopathology, imaging modality and management options of MHCW are discussed.