Jehovah's Witness and consent for blood transfusion in a child: The Indian scenario.

In India, the clinical guidelines and laws governing consent for blood transfusion in a minor are meager and vague. In an elective situation, whether the parents can make a decision for the child on his/her behalf or whether the doctor has the right to make the decision in the best interests of the child is not clear. We present the case scenario of a child belonging to Jehovah's Witness denomination diagnosed with Burkitt lymphoma. His parents were in a dilemma whether to opt for blood transfusion or not. In the absence of laws and guidelines in this context, and considering the complications that he developed during the treatment period, it was very challenging for us to manage the situation both medically and medico-legally. This situation highlights the need for framing consensus guidelines/laws regarding elective blood transfusion in a minor to make health-care delivery, smooth, transparent and flawless.

Comparative diagnostic test evaluation of serum procalcitonin and C-reactive protein in suspected bloodstream infections in children with cancer.

PURPOSE: To compute diagnostic test properties of C-reactive protein (CRP) and serum procalcitonin (PCT) levels in bloodstream infections in children with cancer and suspected sepsis, in comparison with blood culture as the gold standard. METHODOLOGY: Consecutive paediatric cancer patients, aged ≤14 years, with clinically suspected bloodstream infections were evaluated with blood culture and assay of PCT and CRP levels. Blood culture was taken as the gold standard for comparison. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), likelihood ratio (LR) and receiver operating characteristic (ROC) with area under ROC curve (AUC) were calculated to assess the diagnostic test performance for PCT and CRP.Results/Key findings. The ROC curve for PCT was better than that for CRP, with an AUC of 0.751 for PCT at a cut-off of 2.25 ng ml-1. The AUC for CRP was 0.638 at a cut-off of 8.0 mg dl-1. Among the three cut-off values of PCT selected from the ROC curve applicable to the patients under study, the cut-off value of ≥0.49 ng ml-1 had the maximum sensitivity of 81.4 % and an NPV of 94.67 %; ≥2.25 ng ml-1 had a sensitivity and specificity of 65.12 and 71.6 %, respectively, and ≥6.47 ng ml-1 had a maximum specificity of 82.10 %. For CRP, the cut-off value of ≥5.3 mg dl-1 had the maximum sensitivity of 72.09 %; ≥8.0 mg dl-1 had a sensitivity and specificity of 58.14 and 68.09 %, respectively, and ≥8.4 mg dl-1 had the maximum specificity of 70.04 %. CONCLUSION: PCT is a better serological marker for excluding bloodstream infections than CRP. The cut-off value of 0.49 ng ml-1 with a negative predictive value of 94.67 % will be ideal in a clinical setting of immune-compromised children with suspected sepsis.

Longitudinal CT and MR appearances of hemimegalencephaly in a patient with tuberous sclerosis.

CASE REPORT: A 3-day-old baby was referred to our institution with seizures since birth and a presumptive diagnosis of cerebral tumor detected by prenatal ultrasound. Computed tomography (CT) and Magnetic Resonance (MR) imaging performed immediately after birth revealed the presence of an enhancing mass in the left cerebral hemisphere. DISCUSSION: The possibility of a congenital malignant brain tumor was considered. Although surgery was advised, the parents opted for conservative medical management. The infant was under treatment for seizures with antiepileptic drugs. At 7-month follow-up, there was a noticeable increase in the seizure activity with signs of developmental delay and increased head circumference. Repeat CT and MR imaging showed features of hemimegalencephaly with a calcified mass involving the left hemisphere. The patient was diagnosed to have tuberous sclerosis. This case report illustrates how an enhancing mass (possibly a tuber) over a period of time diminishes in size and becomes calcified in a patient with tuberous sclerosis and associated hemimegalencephaly.

Testicular germ cell tumors in prepubertal children.

Pediatric testicular germ cell tumors are rare. Fifteen children, all less than 5 years of age, were evaluated and treated during February 1987 to July 1996. The median age was 18 months (range, 4-60 months). All were staged according to the Pediatric Oncology Group/Children's Cancer Study Group staging system. Seven patients had stage III disease. Histologically, 9 patients had pure endodermal sinus tumor, 1 had endodermal sinus tumor with embryonal carcinoma, 1 had embryonal carcinoma alone, 2 had immature teratoma, and 2 had mature teratoma. Six children were kept on surveillance. All others received chemotherapy with cisplatin, bleomycin, and vinblastine. The 10-year actuarial overall survival rate was 86.7%.

Langerhans cell histiocytosis in children less than 2 years of age.

OBJECTIVE: To study the clinical profile and outcome of langerhans cell histiocytosis in children upto 2 years of life. DESIGN: Retrospective analysis. METHODS: Medical records of Children upto 2 years of age with a diagnosis of langerhans cell histiocytosis (LCH) were analyzed. Their clinical pattern, treatment modalities and outcome were studied. The patients Were categorized into 2 groups according to their clinical presentation: (i) Subject without organ dysfunction; and (ii) cases with organ dysfunction. Treatment considered of surgical intervention, radiotherapy, chemotherapy or combination of all these modalities depending upto the extent of disease. RESULTS: There were 20 children upto 2 years of age with histiocytosis during the 12 year period (January 1983 - December 1994). The median age at diagnosis was 18 months (range 52 days - 24 months). Of the twenty patients,13 patients didn't have organ dysfunction and 7 had organ dysfunction. Out of the 13 children without organ dysfunction eleven patients received treatment and all of them are alive free of disease with a median follow up of 62 months. But all children with organ dysfunction succumbed to disease within a few weeks. CONCLUSION: Children under 2 years of age with localised and or multifocal LCH without organ dysfunction have a good prognosis and they should not be exposed to aggressive form of treatment. All children with organ dysfunction require multi-agent chemotherapy.

Non-Hodgkin's lymphoma in children: disease pattern and survival.

The use of intensive chemotherapy and incorporation of prophylactic treatment of the central nervous system have dramatically improved the outcome of children with non-Hodgkin's lymphoma (NHL). The authors analyzed retrospectively the disease characteristics and survival data of 34 children with NHL during a 7-year period. There were 26 boys and 8 girls with a median age of 8 years. The primary sites were the abdomen (41%) and peripheral node (41%). Histopathologically lymphoblastic and undifferentiated lymphoma (small nonclaved cell lymphoma) were equally distributed (41%). Thirteen patients had localized disease (stage I and II) and 21 patients had advanced disease (stage III and IV). Surgical removal of the primary tumor was done in 6 patients with localized gastrointestinal lesions. All 34 patients received chemotherapy, either cyclophosphamide, vincristine, methotrexate, and prednisolone (COMP) or adriamycin, cyclophosphamide, vincristine, and prednisolone (ACOP). Thirty patients achieved complete remission (88.2%). The 5-year event-free survival rate was 64%. The results indicate that most children with localized disease can be cured by COMP chemotherapy, but more aggressive chemotherapy is necessary to improve survival in advanced-stage disease.