Vishal Sondhi

Establishment of nomograms and reference ranges for intra-cranial ventricular dimensions and ventriculo-hemispheric ratio in newborns by ultrasonography.

Aim: To establish normal ranges for the lateral, third, and fourth ventricular dimensions and the ventriculo‐hemispheric ratio (VHR) in neonates using cranial ultrasonography.

Translating technology into patient care: Smartphone applications in pediatric health care

The latest generations of smartphones are increasingly viewed as handheld computers rather than as phones and the applications on these phones are becoming increasingly popular among the medical professionals. A large number of health care applications are available across various smartphone platforms. At times it may be difficult to identify most appropriate and reliable application for use at the point of care. In this review, we have tried to identify the applications relevant to Pediatrics and Childcare which when used at the point of care might be helpful in improving patient care.

Successful management of multi-focal hepatic infantile hemangioendothelioma using TACE/surgery followed by maintenance metronomic therapy.

Infantile hepatic hemangioendothelioma (IHE) is a rare angiogenic tumour with diverse clinical presentations and varied course ranging from spontaneous regression to life-threatening complications. The authors report a 2-year boy who presented with respiratory distress and was identified as a case of inoperable multi-focal hepatic IHE. He showed a transient response to trans-arterial-chemo-embolisation and liver resection but had progressive disease despite chemotherapy (prednisolone/vicristine/ifosfamide/cisplatin). Thereafter, he was successfully managed with metronomic therapy using cyclophosphamide and tamoxifen.

A unique association of unilateral idiopathic calcinosis cutis with ipsilateral porokeratotic eccrine ostial and dermal duct nevus.

An 11‐year‐old boy presented with complaints of multiple skin‐colored hard lumps on the right side of his body and progressive deformity of the right leg of 7‐years duration. His parents had also noticed multiple asymptomatic pits over his right arm, palms, and soles since childhood. Examination revealed skin‐colored nontender nodules on the right half of his body and shortening of his right leg. The multiple hyperpigmented pits over the right arm, palm, and sole raised diagnostic difficulties, but histopathologic, radiologic, and biochemical investigations confirmed the features of idiopathic calcinosis cutis and porokeratotic eccrine ostial and dermal duct nevus. Unilateral idiopathic calcinosis cutis has not been previously reported in the literature, and the association with ipsilateral porokeratotic eccrine ostial and dermal duct nevus makes this case unique. Diagnostic difficulties and limited options for treatment make this case interesting academically.

L-asparginase administration reduces white blood cell count and prevents tumor lysis syndrome in children with hyperleukocytic acute lymphoblastic leukemia.

Background: The management of hyperleukocytosis currently involves intensive supportive care for preventing tumor lysis syndrome (TLS)-associated metabolic abnormalities as well as cytoreduction procedures to reduce the white blood cell (WBC) count. These procedures are often equipment-intensive and may not be practised in developing countries with limited resources. Hence, it is not clear what would be the most effective strategy to manage hyperleukocytosis and prevent TLS. Procedure: All children ≤12 years, diagnosed with acute lymphoblastic leukemia (ALL) and hyperleukocytosis (WBC count >100 × 109/l) were administered L-asparginase (L-asp, 6,000 U/m2, i.m.) along with standard supportive care consisting of hydration, oral allopurinol administration and alkalization. The complete blood counts and biochemical parameters were monitored for 72 h. After 48 h, if the WBC count was >100 × 109/l, a repeat dose of L-asp was administered. Results: Twenty-one children (9 boys and 12 girls) with hyperleukocytic ALL were treated with L-asp. The median age of the children was 5.3 years (range 2-11 years). The median initial WBC count was 249 × 109/l (range 151-476 × 109/l). Twenty children received only one dose of L-asp. The mean reduction in WBC count achieved by treatment was 15.7, 42.0, 61.0, 76.4, 85.5 and 90.8% at 12, 24, 36, 48, 60 and 72 h, respectively. None of the patients developed TLS. Conclusions: Chemical cytoreduction by administering L-asp is an effective means of managing hyperleukocytosis and preventing TLS.

R542X mutation in SMPD1 gene: genetically novel mutation with phenotypic features intermediate between type A and type B Niemann-Pick disease

Niemann-Pick disease (NPD) is a heterogenous group of progressive neurovisceral disorder characterised by lysosomal accumulation of sphingomyelin. NPD types A and B are caused by mutations involving sphingomyelin-phosphodiesterase-1 (SMPD1) gene and are characterised by deficiency of acid sphingomyelinase activity. We present a case of a 9-month infant with clinical manifestations intermediate between types A and B NPD and genetically illustrating a novel R542X mutation in the exon 6 of SMPD1.

Procedural sedation associated displacement of sharp oesophageal foreign body

Oesophageal foreign body is a common clinical problem. The therapeutic intervention varies from endoscopic removal to observation for spontaneous passage of foreign body. The authors illustrate a case of sharp board pin ingestion by a child, which stayed impacted at C7–T1 level for 8 h with indentation of tracheal mucosa. However, upon administration of midazolam and ketamine for procedural sedation, the pin spontaneously advanced to stomach when muscle relaxation had set in. A few oesophageal mucosal erosions were noted on the endoscopy. The foreign body could not be retrieved by the procedure and was spontaneously egested impacted in faeces. This case presents a distinctive hazard associated with procedural sedation for a foreign body in aero-digestive tract, where the associated muscle relaxation can lead to complications due to spontaneous movement of foreign body.

Treating subglottic haemangioma with methylprednisolone and interferonα-2a

Subglottic haemangiomas are benign vascular malformations presenting in early infancy with respiratory distress due to progressive airway obstruction. The lesion, after undergoing proliferation during the first few months, naturally involutes by an age of 2–3 years. Due to high incidence of mortality, therapy should be initiated immediately. Multiple therapeutic options, such as steroids, interferon, surgery and laser ablation, are available but the best treatment is controversial. This report describes an infant presenting with respiratory distress and cutaneous haemangioma who was detected to have airway obstructive haemangioma from the subglottis to the carina. Due to the massive size, surgical and laser ablation could not be exercised. Also, the infant showed no response to dexamethasone, prednisolone or interferon. Following failure of these modalities, the infant was successfully managed with concurrent administration of daily interferon and pulse methylprednisolone. The regime resulted in rapid shrinkage of the haemangioma and resolution of symptoms, with no recurrence up to 24 months of age.