Anand Prakash

Spectrum of purpura fulminans.

Purpura Fulminans is a severe disorder of acute onset with high morbidity and mortality. It is characterized by DIC with thrombocytopenia, hyofibrinogenemia, hypothrombinemia and anemia. It most often occurs in young with sudden appearance of symmetrical, tender, ecchymotic skin lesions usually involving the lower extremities. An infectious and noninfectious etiology has been proposed. Early recognition and early therapy with appropriate antibiotics and heparin is known to limit both morbidity and mortality. This article reports 5 cases of Purpura Fulminans treated at our centre with review of etiology, pathogenesis, clinical features and treatment.

BFHI in a Tertiary Care Hospital Does Being Baby Friendly Affect Lactation Success

To study the impact of Baby friendly policies on lactation success, a semi-structured questionnaire was administered to mothers of babies attending the well baby clinic of an urban tertiary care Baby Friendly Hospital. The feeding practices of outborn babies was compared to inborn babies(Baby friendly hospital). No major differences were found in the duration and success of lactation among the 2 groups. More intensive efforts and public awareness is required if the ideals of a baby friendly hospital are to be met.

Encouraging Treatment Outcomes of Pediatric Rhabdomyosarcoma: A Developing World Experience

We read recent paper on rhabdomyosarcoma (RMS) treatment and outcome at a multidisciplinary pediatric cancer center in Lebanon with interest. This study reports 5-year overall and disease-free survival rates of 83% and 64%, respectively, which are impressive [1]. It is challenging to treat children with cancer in the developing world especially when multimodality care is needed. Treatment abandonment is a significant challenge in India due to multiple and complex social issues [2]. Sepsis still remains a major barrier in improving outcomes as shown in a recent study on outcome of acute lymphoblastic leukemia [3]. There is lack of data on the outcome of RMS from India. We describe here the treatment outcome of RMS from our center giving a developing world perspective. This is a retrospective review of the clinical presentation, staging, grouping, therapy, and outcome of children with RMS. Chemotherapy was given according to Intergroup RMS Study-5 (IRS-V) protocol. Sixteen patients with RMS presented to our center between March 2005 and January 2011. Two patients refused therapy. Complete data were available for rest of the 14 patients. Median age was 4 years (1 month–12 years). M:F ratio was 2.5:1. Sites of presentation were head and neck—9 (orbit—4, parameningeal—2, other head and neck—3), genitourinary—2, others—3 (pelvis—2, retroperitoneal—1). Three patients had metastasis at the time of diagnosis. Clinical groups observed were group III in 11 and group IV in 3. TNM staging was Stage I—6, II—2, Stage III—3, and Stage IV—3. Histological distribution was spindle-cell variant—2, embryonal–5, alveolar—2, and undifferentiated—5. All patients were initially evaluated with CT scan of primary site, chest and abdomen, bone scan and bone marrow examination, and subsequently followed with the same for disease surveillance. Prognostic stratification included low risk—4, intermediate risk—7, and high risk—3. Surgical resection was possible in only two patients, while others had only biopsy at diagnosis. All low-risk patients had orbital primary Group III and received

Consensus Statement of the Indian Academy of Pediatrics in Diagnosis and Management of Hemophilia

JustificationDespite having standard principles of management of hemophilia, treatment differs in various countries depending on available resources. Guideline for management of hemophilia in Indian setting is essential.ProcessIndian Academy of Pediatrics conducted a consultative meeting on Hemophilia on 18th September, 2016 in New Delhi, which was attended by experts in the field working across India. Scientific literature was reviewed, and guidelines were drafted. All expert committee members reviewed the final manuscript.ObjectiveTo bring out consensus guidelines in diagnosis and management of Hemophilia in India.RecommendationsSpecific factor assays confirm diagnosis and classify hemophilia according to residual factor activity (mild 5-40%, moderate 1-5%, severe <1%). Genetic testing helps in identifying carriers, and providing genetic counseling and prenatal diagnosis. Patients with hemophilia should be managed by multi-specialty team approach. Continuous primary prophylaxis (at least low-dose regimen of 10–20 IU/kg twice or thrice per week) is recommended in severe hemophilia with dose tailored as per response. Factor replacement remains the mainstay of treating acute bleeds (dose and duration depends on body weight, site and severity of bleed). Factor concentrates (plasma derived or recombinant), if available, are preferred over blood components. Other supportive measures (rest, ice, compression, and elevation) should be instantly initiated. Long-term complications include musculoskeletal problems, development of inhibitors and transfusion-transmitted infections, which need monitoring. Adequate vaccination of children with hemophilia (with precautions) is emphasized.

Griscelli syndrome type 2: A silvery hair syndrome with hemophagocytosis

Griscelli syndrome is a very rare autosomal recessive disorder with only about 60 cases reported in the literature. It is characterized by reduced pigmentation of the skin, silvery discoloration of the hair and immunodeficiency. Patients can have an accelerated hemophagocytic phase. The accelerated phase commonly occurs following an infection and has a high risk of mortality. Griscelli syndrome has to be distinguished from Chediak-Higashi syndrome and Elejalde disease, which can also present with silvery hair. In this case report, we discuss the child with silvery hair who presented with hemophagocytic lymphohistiocytosis following varicella infection and was diagnosed to have Griscelli syndrome.

Oncology and Kidney

The pediatric nephrologist may at times be asked to evaluate renal dysfunction in a patient with cancer. This chapter gives an overview of various such scenarios. The section on renal tumors delineates common presentations, patient evaluation, and therapy plans in common pediatric kidney tumors. The section on “nephrology consults in oncology” deals with tumor lysis syndrome, chemotherapy-induced renal dysfunction, and secondary hypertension. These clinical scenarios require understanding and cooperation from both the nephrologist and the oncologist to optimize therapy. The last section deals with stem cell transplantation and renal issues arising in this setting. A short overview regarding the procedure is followed by a summary of renal concerns during and following a stem cell transplant.

Hodgkin lymphoma presenting with multifocal osseous lesions in a child.

Multiple osseous lesions in children are rare and suggest the possibilities of Langerhans cell histiocytosis (LCH), malignancies, enchondromatosis, multifocal osteomyelitis, and tuberculosis [1]. We present a 12-year-old boy with fever for 7 months and pain in the lower back and left hip joint for 5 months, with multiple sclerotic, lytic, and mixed osseous lesions in the axial and appendicular skeleton (see Figure 1). He had received antitubercular treatment for 5 months for suspected tuberculosis of the spine, with no clinical improvement. Aspirin and methotrexate prescribed for suspected juvenile rheumatoid arthritis had provided no relief of symptoms. He had no history of recurrent infections. His HIV was negative by ELISA and his serum immunoglobulin levels were normal. There was no palpable lymphadenopathy or organomegaly. Bone marrow examination was normal. Computed tomography (CT) showed enlarged mediastinal (largest 2.8 × 2 cm) and abdominal lymph nodes (largest 2.7 × 1.3 cm), sclerotic and lytic lesions of 5th rib, sternum, and various vertebrae. Endoscopic fine needle aspiration cytology (FNAC) of mediastinal lymph nodes, CT-guided FNA-biopsy of the 5th rib, and paravertebral mass as well as a laparoscopic biopsy of mesenteric and iliac lymph nodes were done in a staged manner, but all showed reactive changes. Finally, a bone biopsy from an occipital mass lesion, which appeared 3 weeks later, was suggestive of mixed-cellularity Hodgkin lymphoma (HL) (see Figure 2) with immunohistochemistry positive for CD30, CD15, and EBV. He was started on ABVD chemotherapy for stage IV-B HL. Fever, limp, and bone pain subsided dramatically after 2 weeks of chemotherapy. In view of residual disease after four ABVD cycles on positron emission tomography-CT scan, he received further four BEACOPP cycles. He has been disease free for 30 months.