Viral infections are an underrecognized problem in children on standard chemotherapy for acute ly... more Viral infections are an underrecognized problem in children on standard chemotherapy for acute lymphoblastic leukemia (ALL). In countries with high baseline seroprevalence of cytomegalovirus (CMV) such as India, it may be an important pathogen leading to fever, end-organ damage, and cytopenia. Data regarding the incidence and manifestations of CMV disease in pediatric ALL patients are scanty. The authors prospectively assessed all children on chemotherapy for ALL with prolonged febrile neutropenia (FN) for CMV disease over a 3-year period. Children with end-organ damage, including pneumonia, retinitis, and colitis, were also evaluated. Quantitative and qualitative polymerase chain reaction (PCR) from blood, body fluids, or tissue was done along with ophthalmologic evaluation. CMV disease was detected in 10% of the children with prolonged FN. In addition, other children were identified due to end-organ damage, lung and eye being the common organs of involvement. Time of CMV reactivat...
Relapses of acute lymphoblastic leukemia (ALL) in unusual sites can be challenging to diagnose. W... more Relapses of acute lymphoblastic leukemia (ALL) in unusual sites can be challenging to diagnose. We present unusual relapses occurring in children with ALL treated in a single institution over a 22-year period. Of 172 relapses, 9 (5.2%) were at unusual sites (nonmarrow, testes, central nervous system). The most common site of relapse was ocular (66%). The median symptom-to-diagnosis interval was 20 days. Two of 9 children attained second remission. A possibility of relapse should be considered when evaluating unusual symptoms in a child with underlying ALL.
Journal of Indian Association of Pediatric Surgeons, 2013
Paratesticular germ cell tumors are extremely rare. A 12-month-old boy with yolk sac tumor involv... more Paratesticular germ cell tumors are extremely rare. A 12-month-old boy with yolk sac tumor involving only the paratesticular tissue is reported. Pre-operatively raised alpha fetoprotein levels fell to normal levels after high inguinal orchiectomy. This appears to be the youngest and only the 3(rd) case reported in the English literature.
Viral infections are an underrecognized problem in children on standard chemotherapy for acute ly... more Viral infections are an underrecognized problem in children on standard chemotherapy for acute lymphoblastic leukemia (ALL). In countries with high baseline seroprevalence of cytomegalovirus (CMV) such as India, it may be an important pathogen leading to fever, end-organ damage, and cytopenia. Data regarding the incidence and manifestations of CMV disease in pediatric ALL patients are scanty. The authors prospectively assessed all children on chemotherapy for ALL with prolonged febrile neutropenia (FN) for CMV disease over a 3-year period. Children with end-organ damage, including pneumonia, retinitis, and colitis, were also evaluated. Quantitative and qualitative polymerase chain reaction (PCR) from blood, body fluids, or tissue was done along with ophthalmologic evaluation. CMV disease was detected in 10% of the children with prolonged FN. In addition, other children were identified due to end-organ damage, lung and eye being the common organs of involvement. Time of CMV reactivat...
Acute lymphoblastic leukemia arising from lymphoid precursor cells of the bone marrow, the lympho... more Acute lymphoblastic leukemia arising from lymphoid precursor cells of the bone marrow, the lymphoreticular system, and the soft tissue can present with medullary and extramedullary involvement. Extramedullary involvement has the propensity to affect a multitude of organs. Presentation with proptosis secondary to orbital mass in childhood acute lymphoblastic leukemia (ALL) is very rare. We report a child with pre-B cell ALL with an extramedullary soft tissue mass involving both orbits presenting with proptosis, and give a brief overview of the literature about this unusual entity. Rapid investigation and timely initiation of treatment are needed to salvage the eye and the vision. Orbital involvement is considered to confer a poorer prognosis to children with ALL.
Children infected with human immunodeficiency virus (HIV) have an increased risk of malignancies.... more Children infected with human immunodeficiency virus (HIV) have an increased risk of malignancies. Herein, we present a 16Â month old HIV infected child receiving antiretroviral therapy who presented with pain and swelling of both knees and ankles. This child was diagnosed as Langerhans cell histiocytosis (LCH) which is an uncommon proliferative disorder rarely reported in a HIV patient. The child was treated with chemotherapy and is well clinically. Infiltrative disorders like LCH should be kept among the differential diagnosis of bony pain and swelling in HIV infected children.
Superior mediastinal syndrome (SMS) is an uncommon manifestation of malignant neoplastic disease ... more Superior mediastinal syndrome (SMS) is an uncommon manifestation of malignant neoplastic disease in children. The commonest neoplastic cause of SMS is Non Hodgkin lymphoma. Acute myeloid leukemia (AML) as a cause of SMS is extremely uncommon in childhood. The authors hereby report a case of a child with AML who presented with SMS at their hospital. This report also highlights the importance of flowcytometry as a diagnostic modality in hematological malignancies.
Hyperleukocytosis is defined as peripheral blood leukocyte count exceeding 100,000/mm(3). Acute l... more Hyperleukocytosis is defined as peripheral blood leukocyte count exceeding 100,000/mm(3). Acute leukemia is the most common etiology in pediatric practice. Hyperleukocytosis is a medical emergency. The increased blood viscosity, secondary to high white cell count and leukocyte aggregates, results in stasis in the smaller blood vessels. This predisposes to neurological, pulmonary or gastrointestinal complications. In addition, patients are at risk for tumor lysis syndrome due to the increased tumor burden. Initial management includes aggressive hydration, prevention of tumor lysis syndrome, and correction of metabolic abnormalities. A red cell transfusion is not indicated in a hemodynamically stable child, as it adversely affects the blood viscosity. Leukapheresis is the treatment of choice for a very high count, or in patients with symptomatic hyperleukocytosis. The technical expertise required, a relative difficult venous access in younger children, risk of anticoagulation and possible non-availability of the procedure in emergency hours are limitations of leukapheresis. However, it is a rewarding procedure and performed with relative ease in centers that perform the procedure frequently. An exchange transfusion is often a practical option when hyperleukocytosis is complicated with severe anemia. The partial exchange aids in correcting both, without the risk of volume overload or hyperviscosity, which are the limitations of hydration and blood transfusion, respectively. Etiology and management of hyperleukocytosis in relevance to the pediatric emergency room is outlined.
Relapses of acute lymphoblastic leukemia (ALL) in unusual sites can be challenging to diagnose. W... more Relapses of acute lymphoblastic leukemia (ALL) in unusual sites can be challenging to diagnose. We present unusual relapses occurring in children with ALL treated in a single institution over a 22-year period. Of 172 relapses, 9 (5.2%) were at unusual sites (nonmarrow, testes, central nervous system). The most common site of relapse was ocular (66%). The median symptom-to-diagnosis interval was 20 days. Two of 9 children attained second remission. A possibility of relapse should be considered when evaluating unusual symptoms in a child with underlying ALL.
A child suffering from acute lymphoblastic leukemia on treatment with exclusive chemotherapy pres... more A child suffering from acute lymphoblastic leukemia on treatment with exclusive chemotherapy presented with vision-threatening cytomegalovirus (CMV) retinitis in 1 eye. Prompt diagnosis and treatment with 3 weekly doses of 2 mg/0.1 mL intravitreal ganciclovir resulted in successful healing of CMV retinitis with restoration of visual acuity. In children with acute lymphoblastic leukemia on exclusive chemotherapy without hematopoietic stem cell transplantation, CMV retinitis has been reported in only 1 case in literature. This child was treated successfully with intravenous ganciclovir. This report highlights the use of successful intravitreal ganciclovir in pediatric age group to avoid side effects of systemic ganciclovir.
Journal of Pediatric Endocrinology and Metabolism, 2000
Aromatase deficiency is a rare autosomal recessive disorder caused by mutations in the CYP19A1 ge... more Aromatase deficiency is a rare autosomal recessive disorder caused by mutations in the CYP19A1 gene and characterized by lack of conversion of androgens to estrogens. It presents with virilization of pregnant mothers during the antenatal period, and virilization of female fetuses at birth. Affected subjects of either gender later manifest with features of estrogen deficiency and androgen excess. We describe the clinical course of an Indian girl with aromatase deficiency from birth to 16 years of age. Estrogen replacement was begun at age 13.5 years. The child's growth, hormonal, radiological, and metabolic parameters were monitored throughout the course of treatment. The child presented with obesity, tall stature, delayed bone age, osteoporosis, hyperinsulinemia with acanthosis nigricans, and hypergonadotropic hypogonadism with cystic ovaries. Estrogen replacement resulted in a plateauing of height, improvement of bone maturation, and pubertal progression with the disappearance of ovarian cysts. However, hyperinsulinemia and acanthosis nigricans persisted despite estrogen replacement and metformin. Genetic analysis revealed a homozygous arginine to cysteine substitution at codon 435 in exon 10 of CYP19A1. This is the first case of aromatase deficiency reported from India. This case highlights the role of estrogen in skeletal maturation and mineralization and the effect of estrogen deficiency and androgen excess over glucose metabolism in adolescent females.
Viral infections are an underrecognized problem in children on standard chemotherapy for acute ly... more Viral infections are an underrecognized problem in children on standard chemotherapy for acute lymphoblastic leukemia (ALL). In countries with high baseline seroprevalence of cytomegalovirus (CMV) such as India, it may be an important pathogen leading to fever, end-organ damage, and cytopenia. Data regarding the incidence and manifestations of CMV disease in pediatric ALL patients are scanty. The authors prospectively assessed all children on chemotherapy for ALL with prolonged febrile neutropenia (FN) for CMV disease over a 3-year period. Children with end-organ damage, including pneumonia, retinitis, and colitis, were also evaluated. Quantitative and qualitative polymerase chain reaction (PCR) from blood, body fluids, or tissue was done along with ophthalmologic evaluation. CMV disease was detected in 10% of the children with prolonged FN. In addition, other children were identified due to end-organ damage, lung and eye being the common organs of involvement. Time of CMV reactivat...
Relapses of acute lymphoblastic leukemia (ALL) in unusual sites can be challenging to diagnose. W... more Relapses of acute lymphoblastic leukemia (ALL) in unusual sites can be challenging to diagnose. We present unusual relapses occurring in children with ALL treated in a single institution over a 22-year period. Of 172 relapses, 9 (5.2%) were at unusual sites (nonmarrow, testes, central nervous system). The most common site of relapse was ocular (66%). The median symptom-to-diagnosis interval was 20 days. Two of 9 children attained second remission. A possibility of relapse should be considered when evaluating unusual symptoms in a child with underlying ALL.
Journal of Indian Association of Pediatric Surgeons, 2013
Paratesticular germ cell tumors are extremely rare. A 12-month-old boy with yolk sac tumor involv... more Paratesticular germ cell tumors are extremely rare. A 12-month-old boy with yolk sac tumor involving only the paratesticular tissue is reported. Pre-operatively raised alpha fetoprotein levels fell to normal levels after high inguinal orchiectomy. This appears to be the youngest and only the 3(rd) case reported in the English literature.
Viral infections are an underrecognized problem in children on standard chemotherapy for acute ly... more Viral infections are an underrecognized problem in children on standard chemotherapy for acute lymphoblastic leukemia (ALL). In countries with high baseline seroprevalence of cytomegalovirus (CMV) such as India, it may be an important pathogen leading to fever, end-organ damage, and cytopenia. Data regarding the incidence and manifestations of CMV disease in pediatric ALL patients are scanty. The authors prospectively assessed all children on chemotherapy for ALL with prolonged febrile neutropenia (FN) for CMV disease over a 3-year period. Children with end-organ damage, including pneumonia, retinitis, and colitis, were also evaluated. Quantitative and qualitative polymerase chain reaction (PCR) from blood, body fluids, or tissue was done along with ophthalmologic evaluation. CMV disease was detected in 10% of the children with prolonged FN. In addition, other children were identified due to end-organ damage, lung and eye being the common organs of involvement. Time of CMV reactivat...
Acute lymphoblastic leukemia arising from lymphoid precursor cells of the bone marrow, the lympho... more Acute lymphoblastic leukemia arising from lymphoid precursor cells of the bone marrow, the lymphoreticular system, and the soft tissue can present with medullary and extramedullary involvement. Extramedullary involvement has the propensity to affect a multitude of organs. Presentation with proptosis secondary to orbital mass in childhood acute lymphoblastic leukemia (ALL) is very rare. We report a child with pre-B cell ALL with an extramedullary soft tissue mass involving both orbits presenting with proptosis, and give a brief overview of the literature about this unusual entity. Rapid investigation and timely initiation of treatment are needed to salvage the eye and the vision. Orbital involvement is considered to confer a poorer prognosis to children with ALL.
Children infected with human immunodeficiency virus (HIV) have an increased risk of malignancies.... more Children infected with human immunodeficiency virus (HIV) have an increased risk of malignancies. Herein, we present a 16Â month old HIV infected child receiving antiretroviral therapy who presented with pain and swelling of both knees and ankles. This child was diagnosed as Langerhans cell histiocytosis (LCH) which is an uncommon proliferative disorder rarely reported in a HIV patient. The child was treated with chemotherapy and is well clinically. Infiltrative disorders like LCH should be kept among the differential diagnosis of bony pain and swelling in HIV infected children.
Superior mediastinal syndrome (SMS) is an uncommon manifestation of malignant neoplastic disease ... more Superior mediastinal syndrome (SMS) is an uncommon manifestation of malignant neoplastic disease in children. The commonest neoplastic cause of SMS is Non Hodgkin lymphoma. Acute myeloid leukemia (AML) as a cause of SMS is extremely uncommon in childhood. The authors hereby report a case of a child with AML who presented with SMS at their hospital. This report also highlights the importance of flowcytometry as a diagnostic modality in hematological malignancies.
Hyperleukocytosis is defined as peripheral blood leukocyte count exceeding 100,000/mm(3). Acute l... more Hyperleukocytosis is defined as peripheral blood leukocyte count exceeding 100,000/mm(3). Acute leukemia is the most common etiology in pediatric practice. Hyperleukocytosis is a medical emergency. The increased blood viscosity, secondary to high white cell count and leukocyte aggregates, results in stasis in the smaller blood vessels. This predisposes to neurological, pulmonary or gastrointestinal complications. In addition, patients are at risk for tumor lysis syndrome due to the increased tumor burden. Initial management includes aggressive hydration, prevention of tumor lysis syndrome, and correction of metabolic abnormalities. A red cell transfusion is not indicated in a hemodynamically stable child, as it adversely affects the blood viscosity. Leukapheresis is the treatment of choice for a very high count, or in patients with symptomatic hyperleukocytosis. The technical expertise required, a relative difficult venous access in younger children, risk of anticoagulation and possible non-availability of the procedure in emergency hours are limitations of leukapheresis. However, it is a rewarding procedure and performed with relative ease in centers that perform the procedure frequently. An exchange transfusion is often a practical option when hyperleukocytosis is complicated with severe anemia. The partial exchange aids in correcting both, without the risk of volume overload or hyperviscosity, which are the limitations of hydration and blood transfusion, respectively. Etiology and management of hyperleukocytosis in relevance to the pediatric emergency room is outlined.
Relapses of acute lymphoblastic leukemia (ALL) in unusual sites can be challenging to diagnose. W... more Relapses of acute lymphoblastic leukemia (ALL) in unusual sites can be challenging to diagnose. We present unusual relapses occurring in children with ALL treated in a single institution over a 22-year period. Of 172 relapses, 9 (5.2%) were at unusual sites (nonmarrow, testes, central nervous system). The most common site of relapse was ocular (66%). The median symptom-to-diagnosis interval was 20 days. Two of 9 children attained second remission. A possibility of relapse should be considered when evaluating unusual symptoms in a child with underlying ALL.
A child suffering from acute lymphoblastic leukemia on treatment with exclusive chemotherapy pres... more A child suffering from acute lymphoblastic leukemia on treatment with exclusive chemotherapy presented with vision-threatening cytomegalovirus (CMV) retinitis in 1 eye. Prompt diagnosis and treatment with 3 weekly doses of 2 mg/0.1 mL intravitreal ganciclovir resulted in successful healing of CMV retinitis with restoration of visual acuity. In children with acute lymphoblastic leukemia on exclusive chemotherapy without hematopoietic stem cell transplantation, CMV retinitis has been reported in only 1 case in literature. This child was treated successfully with intravenous ganciclovir. This report highlights the use of successful intravitreal ganciclovir in pediatric age group to avoid side effects of systemic ganciclovir.
Journal of Pediatric Endocrinology and Metabolism, 2000
Aromatase deficiency is a rare autosomal recessive disorder caused by mutations in the CYP19A1 ge... more Aromatase deficiency is a rare autosomal recessive disorder caused by mutations in the CYP19A1 gene and characterized by lack of conversion of androgens to estrogens. It presents with virilization of pregnant mothers during the antenatal period, and virilization of female fetuses at birth. Affected subjects of either gender later manifest with features of estrogen deficiency and androgen excess. We describe the clinical course of an Indian girl with aromatase deficiency from birth to 16 years of age. Estrogen replacement was begun at age 13.5 years. The child's growth, hormonal, radiological, and metabolic parameters were monitored throughout the course of treatment. The child presented with obesity, tall stature, delayed bone age, osteoporosis, hyperinsulinemia with acanthosis nigricans, and hypergonadotropic hypogonadism with cystic ovaries. Estrogen replacement resulted in a plateauing of height, improvement of bone maturation, and pubertal progression with the disappearance of ovarian cysts. However, hyperinsulinemia and acanthosis nigricans persisted despite estrogen replacement and metformin. Genetic analysis revealed a homozygous arginine to cysteine substitution at codon 435 in exon 10 of CYP19A1. This is the first case of aromatase deficiency reported from India. This case highlights the role of estrogen in skeletal maturation and mineralization and the effect of estrogen deficiency and androgen excess over glucose metabolism in adolescent females.
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