Alka Khadwal

Hypocalcemia nutritional rickets: a curable cause of dilated cardiomyopathy.

Dilated cardiomyopathy is an important cause of heart failure in children. Often it requires transplantation, but on rare occasions it is curable by micronutrient supplementation. Hypocalcemic nutritional rickets was found to be a cause for dilated cardiomyopathy in a 15-month-old child. The patient responded to calcium and vitamin D supplementation promptly and left ventricular systolic function normalized after 3 months of treatment. Nutritional rickets must the considered as an important curable cause for dilated cardiomyopathy among children especially in regions where nutritional rickets is still common.

Accidental Inorganic Mercury Chloride Poisoning in a 2-Year Old Child

Inorganic mercury poisoning is uncommon, but when it occurs it can result in severe, life threatening features and acute renal failure. A 2-year old well thriving child presented with alleged history of accidental ingestion of inorganic mercury chloride. He presented with evidence of corrosive trauma to the gastrointestinal tract mucosa, but with normal renal function at admission, which was managed with BAL and other supportive treatment. But he developed non-oliguric renal failure after admission, which also improved gradually. On follow-up, two months later, the patient’s renal function was normal; indicating that renal failure caused by acute inorganic mercury poisoning produced no permanent renal damage. We have hereby presented a case of mercury intoxication in a 2-year old child, with an excellent clinical improvement and normalization of laboratory results.

Factors Affecting Early Molecular Response in Chronic Myeloid Leukemia

OBJECTIVES There is controversy about whether 3- or 6-month molecular assessment predicts progression-free and overall survival in those with chronic myeloid leukemia (CML). The factors predicting molecular response at 3, 6, and 12 months have not been studied extensively. The study objective was to study the factors affecting molecular response at 3 and 6 months in patients with CML who are receiving imatinib mesylate. METHODS We prospectively enrolled patients with newly diagnosed CML who were receiving imatinib mesylate as the initial therapy for CML. The diagnosis of CML was based on clinical examination, bone marrow, and demonstration of BCR ABL(IS) transcripts by polymerase chain reaction. The molecular response(IS) was assessed at 3, 6, and 12 months by GeneXpert (Cepheid, Sunnyvale, CA) and co-related with various baseline characteristics of patients. We also looked at whether early achievement of a complete hematologic response within 6 weeks predicts molecular response at 3 or 6 months. The study took place at a tertiary care hospital in Northwest India catering to patients belonging to low-middle socioeconomic status. RESULTS We enrolled 131 patients with CML in the chronic phase from July 1, 2013, to August 31, 2014. The median age of the patients was 40 years (range, 13-67) with a male preponderance (61% were male). Most patients presented with symptoms of low-grade fever (52.7%) and abdominal fullness (26.7%). Spleen was palpable in 84.7% of patients. The median hemoglobin at presentation was 10.8 g/dL (range, 4.8-18.4 g/dL), white cell count was 138.3 × 10(9)/L (4.1-697 × 10(9)/L), and platelet count was 326 × 10(9)/L (85-1819 × 10(9)/L). The median number of peripheral blood basophils was 3% (range, 0%-20%), and blasts were 3% (range, 0%-10%). Myelofibrosis of more than grade 1 was present in 30% of patients. Most patients belonged to intermediate Sokal (45.8%) and Hasford (55%) scores and low EUropean Treatment Outcome Study (78.6%) score. Of 128 evaluable patients at 3 months, 96.9% achieved complete hematologic remission (CHR) and 82.3% achieved BCR ABL(IS) of less than 10%. None of the patients who had BCR ABL(IS) > 10% at 3 months achieved BCR ABL(IS) < 1% at 6 months or < 0.1% at 12 months. Early achievement of CHR (< 6 weeks), peripheral blood blast count of < 5%, and lactate dehydrogenase < 851 U/L were significantly associated with achievement of BCR ABL(IS) < 10% at 3 months and BCR ABL(IS) < 1% at 6 months. CONCLUSIONS We found that BCR ABL(IS) assessment at 3 months is superior to assessment at 6 months. Patients with CML in the chronic phase who achieve CHR within 6 weeks are more likely to achieve BCR ABL(IS) < 10% at 3 months and < 1% at 6 months than patients who achieve CHR between 7 and 12 weeks.

Immune Thrombocytopenic Purpura due to Mixed Viral Infections

An 11-year-old boy presented with epistaxis, petechial hemorrhages, easy bruising, and purpuric rash. He was diagnosed to have immune thrombocytopenic purpura and evidence of concomitant parvovirus B19 and dengue viral infection.

Anomaly of iliac veins: a rare cause of transient hepatic attenuation difference in a child

We report a case of transient hepatic attenuation difference (THAD) in a child caused by iliac vein anomaly. Iliac vein anomaly as a cause for THAD in a child is extremely rare.

Cost of Treatment of Multiple Myeloma in a Public Sector Tertiary Care Hospital of North India

Multiple myeloma (MM) is a neoplastic disorder, which accounts for 13% of all hematological malignancies globally. While, conventional chemotherapy used to be the mainstay treatment for the disease, the landscape of treatment witnessed a paradigm shift with the introduction of high-dose chemotherapy and autologous stem cell transplant (ASCT). In this paper, we present a cost analysis of various services provided to multiple myeloma patients, using either of the two modalities of treatments i.e. conventional chemotherapy or ASCT. Bottom-up costing methodology was used to collect data on all health system resources, i.e. capital or recurrent, which were used to provide various services to MM patients. Capital costs were annualized for their useful life using a discount rate of 5%. Out of pocket expenditure on treatment was also ascertained. Cost was assessed for various services, including outpatient consultation, bed day hospitalization in general ward, high dependency unit intensive care setting and bone marrow transplant unit. Unit costs were calculated from both health system and patient perspective. The overall cost per patient for ASCT (including high dose chemotherapy) and conventional chemotherapy from societal perspective was INR 395,527 (USD 6085) and INR 62,785 (USD 966) respectively. Estimates on cost from our study could be used for planning health services, and evaluating cost effectiveness of different modalities of care for multiple myeloma.

Evaluation of Bortezomib-Induced Neuropathy Using Total Neuropathy Score (Reduced and Clinical Versions) and NCI CTCAE v4.0 in Newly Diagnosed Patients With Multiple Myeloma Receiving Bortezomib-Based Induction

Background: Bortezomib‐induced peripheral neuropathy (BiPN) is a dose‐limiting adverse effect of bortezomib‐based therapy for multiple myeloma (MM). The reporting of BiPN is variable because of the use of different neuropathy scales. Most investigators use the National Cancer Institute Common Terminology Criteria for Adverse Events (NCI CTCAE). Patients and Methods: We prospectively evaluated the incidence of BiPN in treatment‐naive patients with MM receiving weekly cyclophosphamide, bortezomib, and dexamethasone (CyBorD) in 28‐day cycles using 3 neuropathy scores: Total Neuropathy Score—reduced (TNSr) and —clinical (TNSc), and NCI CTCAE v4.0. Results: Twenty‐six patients received CyBorD. Twenty patients completed follow‐up. The rates of occurrence of BiPN were as follows: TNSr ‐ 55% (n = 11), TNSc ‐ 40% (n = 8), and NCI CTCAE ‐ 45% (n = 9). All 3 scales showed worsening after treatment (P < .01). When compared to BiPN by TNSr, sensitivities for NCI CTCAE and TNSc were 77.8% and 88.9%, respectively. Specificity was 63.3% for both NCI CTCAE and TNSc. Among 12 patients who did not have BiPN by NCI CTCAE scale, 41.7% (n = 5) and 16.7% (n = 2) patients satisfied the criteria for BiPN by TNSr and TNSc, respectively. The higher detection rate of neuropathy by TNSr and TNSc is probably due to increment in scores that are allotted for increase in anatomic extent of sensorimotor involvement, unlike the NCI CTCAE scale, which requires functional limitation for increase in grade. Conclusion: NCI CTCAE may be suboptimal in comparison to TNSr and TNSc in assessment of BiPN because it may miss worsening neuropathy without functional limitation. &NA; The Total Neuropathy Score is ideal for evaluation of bortezomib‐induced neuropathy. We used the reduced (TNSr) and clinical (TNSc) forms as well as the National Cancer Institute Common Terminology Criteria for Adverse Events (NCI CTCAE) v4.0 to assess incidence of neuropathy in patients receiving cyclophosphamide, bortezomib, and dexamethasone as induction for multiple myeloma. Out of 20 evaluable patients, 55%, 40%, and 45% developed neuropathy when assessed by TNSr, TNSc, and NCI CTCAE, respectively. We found wide variation in TNSr and TNSc even when the NCI CTCAE scale reported no progression of neuropathy.

Genital vasculitis secondary to all-trans-retinoic-acid

Acute promyelocytic leukaemia is among the most curable haematological malignancies after the introduction of differentiating agents (arsenic trioxide (ATO) and all-trans-retinoic-acid (ATRA)). Despite excellent cure rates, approaching 85–95% in various series, APL is associated with significant early mortality and morbidity. ATRA-related side effects partly contribute to this morbidity, which commonly presents as differentiation syndrome, pseudo tumour cerebri, dermatitis, gastrointestinal disorders, liver dysfunction (raised transaminases) and dryness of skin/eyes. Rarely, ATRA can lead to hypercalcaemia, genital vasculitis, erythaema nodosum and Sweets syndrome. We present two cases of ATRA-associated genital vasculitis while being managed with a combination of ATO with ATRA therapy. Both patients developed these lesions despite being on prophylactic steroids (0.5 mg/kg). We also discuss the pathophysiology, clinical manifestations, differential diagnosis and treatment of genital vasculitis as a rare adverse event of ATRA.

Cortical cysts with hydrocephalus and ventriculitis: an unusual presentation of congenital toxoplasmosis at autopsy

We describe a case of congenital toxoplasmosis with absent serum serological markers in mother and baby, presence of periventricular cysts on radiology and jelly-like coagulum within the dilated ventricles at autopsy, extremely rare features of congenital toxoplasmosis which have not been reported in the literature. A 45-day-old female baby (first live born of non-consanguineous marriage delivered at 32 weeks) presented with generalised seizures and poor feeding. The mother had a previous abortion at 17 weeks with no cause identified. On examination, no dysmorphic features, pallor, jaundice, oedema or rash was noted. The occipito-frontal circumference was 32 cm (<−2SD) and weight 1.6 kg (below the third centile). Examination revealed wide, bulging anterior fontenella, and the eyes were normal. Blood counts, serum electrolytes, renal and liver function test were normal. The cerebrospinal fluid (CSF) examination revealed pleocytosis (520 cells), raised protein (2.5 g) and reduced glucose (10 mg/dl). IgG and IgM ELISA in the mothers and babys serum was non-suggestive on two occasions. IgG ELISA in patients CSF was non-suggestive, but IgM was high. The fungal and tuberculosis work-up was negative. Ultrasonography of the head showed increased echogenicity of ventricles with echogenic walls suggestive of ventriculitis and multiple hyperdense lesions in bilateral basal ganglia and cerebral hemispheres suggestive of infarct or abscess. The ventricular head size ratio varied between 58% and 71%. The cranial CT revealed gross dilatation of the ventricular system indicating communicating hydrocephalus. The ventricles showed much higher attenuation contents than normal CSF. Mild enhancement of the ventricular walls was seen, suggestive of ventriculitis. Additionally, large well-defined periventricular cysts were seen along thalami, basal ganglia and perimesencephalic cisterns (figure 1A,B). No …

Persistent coagulopathy in snake bite.

Bleeding diathesis is a cardinal feature of viperine bite, which has been thought to last not more than 24 hours. There is scarcity of literature about prolonged bleeding disorder in snake envenomation. Various explanations suggested in the literature include-temporary decrease in antivenin levels, rapid elimination of antivenin from circulation or continuous release of unneutralised venom from the envenomated site. Two children with prolonged coagulopathy lasting for more than a week, correction of which required more than 300 ml of antisnake venom are reported here.