Ho/01(P) incidence of iron deficiency in non anaemic children

HO/27(P) HAND SCHULLER CHRISTIAN DISEASE- A CASE REPORT

Dipankar Gupta, Megha Consul,Rajesh Mehta

Department of Paediatrics,Safdarjung Hospital and VMMC,New Delhi

Langerhans cell histiocytosis is a rare entity. A five yr old girl presented to the medical facility with complaints of polyuria, polydipsia and multiple depressed lesions on the skull noticed 3 months back. No history of trauma, drug intake, radiation exposure or any other significant illness in past was present. General physical examination revealed Rt. Sided exophthalmos with normal ocular function and movement, depigmented macular lesions on forehead were seen. Skull had multiple depressed bony lesions with irregular margins. Systemic examination was unremarkable except for mild hepatomegaly. On investigations all routine hematologic work up was within normal limits. X-ray skull showed multiple lytic lesions in both frontal and parietal areas.3D CT Skull showed irregular bony defect in both frontal, parietal and Rt ethmoidal region. Bone scan was consistent with increased tracer uptake from above areas only. Water Deprivation test was consistent with Central Diabetes Insipidus. MRI Brain showed absence of posterior pituitary bright spot and thickening of infundibulum at its origin (6x7x4.4mm) near the median eminence. Bone Biopsy showed features of Langerhan’s Cell Histiocytosis consistent with Hand Schuller Christian Disease variety. With the triad of exophthalmos, diabetes insipidus and lytic skull lesions and supportive imaging and histopathological findings a diagnosis of Hand Schuller Christian Disease was made.

HO/28(P) EOSINOPHILIC MENINGITIS: AN UNRECOGNIZED ENTITY!

Powlomi Pai, R.Begue, R.P.Warrier

LSU Health Sciences Center and Children’s Hospital ,New Orleans, LA

Introduction: Eosinophilic meningitis (EM) is a syndrome characterized by the presence of greater than 10 percent eosinophils in the cerebrospinal fluid. It is rare in the U.S. with outbreaks only in travelers returning from endemic areas and therefore remains a less recognized entity. However, this case demonstrates the importance of being aware of potential etiologies of EM. Case report: This is a 4 year old black male with a history of Sickle Cell disease presenting with headaches. This was treated as a pain crisis, but worsened with development of focal neurologic deficits. An evaluation included a MRI/MRA, showing cerebellar edema, and a lumbar puncture revealing 55 percent eosinophils. Serologies for Toxocara canis, Toxoplasma , Lymphocytic choriomeningitic virus, and Mycoplasma were negative. There was no history of travel, exposure to undercooked snails/mollusks or neurosurgical procedures. However, there was an exposure to rat and raccoon feces; therefore, serologies for Angiostrongylus cantonensis and Baylisascaris procyonis were sent and were diagnostic of Bayliascaris.. Empiric treatment with steroids and albendazole resulted in improvement of the patient’s symptoms and cerebrospinal fluid findings. Discussion: The most common cause of EM in the pediatric population is ventriculoperitoneal shunts. Parasitic infections are another cause . Angiostrongylus cantonensis is a rat lung worm endemic to Southeast Asia and the Pacific Basin. Its larvae infect intermediate hosts like snails and mollusks through fecal contamination. After ingestion of contaminated food by humans, the larvae invade the meninges and die, eliciting a eosinophilic response. , Baylisascaris procyonis is an ascarid parasite which is widely prevalent in raccoons and infects humans in a similar way. Clinical features include fever, headaches, visual disturbances, and neurologic deficits. Conclusion: EM is a syndrome with multiple etiologies, including parasitic infections. Although rare, the worldwide prevelance of these organisms should have clinicians maintaining a high index of suspicion.

HO/29(P) ACUTE LEUKEMIA AFTER GENE THERAPY FOR SCID

Malini Dave, Raj Warrier, Maria Velez, Lily Levia, Ricardo Sorensen.

LSU Health Sciences Center /Childrens Hopsital, New Orleans, LA

Severe combined immunodeficiency (SCID) is an inherited immune disorder characterized by T-cell lymphopenia and profound lack of cellular and humoral immunity.The treatment for SCID is immune reconstitution, which includes hematopoietic stem cell (HSCT) transplantation or gene therapy. The disadvantage of HSCT is the need of an HLA matched donor. Gene therapy with genetically modified autologous cells offers a cure for X-SCID with significant immune reconstitution. The disadvantage is the possibility of leukemogenesis. Of ten infants with X-linked SCID treated with gene therapy in Paris, France, three developed T-cell leukemia. Case report: A 3-year-old Caucasian male was diagnosed with X-SCID at birth by lymphopenia and an absent thymus. A missense mutation of IL-2R gamma C gene was identified at NIH. He received gene therapy at 3 months of age. Within 3 months, the lymphocyte count increased with normal number of T and B cells. Thirty-four months after treatment, the patient showed hyperleukocytosis, anemia, thrombocytopenia, and lymphoblasts on peripheral smear. Bone marrow biopsy confirmed T-cell leukemia with trisomy 10. He was treated with chemotherapy as per COG protocol and has been in complete, continuous remission for 3 years and is cuurently off treatment. He has not had any unusual episodes of sepsis or infection except one of Herpes Zoster controlled By Acyclovir after completion of chemotherapy. The development of leukemia in three children has raised concerns regarding the safety of gene therapy utilizing integrating vectors. Studies have been conducted using non-human primates and dogs. None developed abnormal hematopoesis or leukemia. This may suggest that retroviral integrations using replication-incompetent vectors are unlikely to result in leukemogenesis and that patient or transgene specific factors most likely contributed to the occurrence of leukemia. Gene therapy is clearly a feasible therapeutic option for SCID, though it has also raised important questions regarding the long-term outcome.
HO/30(P) ACUTE NECROTIZING ENCEPHALITIS IN SICKLE CELL DISEASE - THE ASSOCIATION WITH MYCOPLASMA PNEUMONIAE INFECTION AND ACUTE CHEST SYNDROME.

KL Nelson, MD, R Warrier, MD, C Arcement, MD. Departments of Pediatric Hematology/Oncology and Radiology, Louisiana State University and Children’s Hospital, New Orleans, LA.

Sickle cell disease has several vascular complications that are well known , with the most feared being a cerebrovascular accident or stroke. Unfortunately, many sickle cell patients who have suffered a stroke bear the the long-term sequelae, the most noted being hemiparesis. Symptoms that herald the onset of a stroke include headache, seizure, speech deficits, visual disturbances and hemiparesis. A stroke may occur spontaneously, but it may also occur in the setting of pneumonia, acute chest syndrome, aplastic crisis, priapism, other vaso-occlusive crises and even dehydration, which promotes sickling. However, sickle cell patients with acute neurologic change in the setting of a recent or concurrent acute chest syndrome without evidence of stroke on MRI should be evaluated for other causes of central nervous system disease. We present a 13-year-old who presented to the emergency room with diplopia beginning on the morning of presentation. He had also been experiencing intermittent frontal headaches and vomiting for two days prior to presentation. He had been hospitalized for an episode of pneumonia and acute chest syndrome ten days prior to the onset of these symptoms. The patient developed bilateral sixth nerve palsies soon after admission. A CT, MRI and MR angiography and venography of the cerebral vessels were all negative for stroke, as well as arterial and venous thromboses, respectively. A transcranial Doppler ultrasound was also normal, indicating a low risk for stroke. A subsequent repeat MRI revealed radiologic findings compatible with acute necrotizing encephalitis, a variant of acute demyelinating encephalopathy. Mycoplasma titers were positive for a recent infection, and had been negative just ten days prior. Acute necrotizing encephalitis with specific reference to the relationship to sickle cell disease, acute chest syndrome and Mycoplasma pneumoniae infection will be discussed with radiological and serological findings.
HO/31(O) THERAPEUTIC EFFICACY OF IRON POLYMALTOSE COMPLEX VERSUS FERROUS SULFATE IN TREATMENT OF IRON DEFICIENCY ANEMIA

Ankur Vikas Bopche, Navneetha S., Mahendra Jain, G.S. Patel, Rashmi Dwivedi

Tertiary Centre in the Department of Pediatrics, Kamla Nehru Hospital, Bhopal.
Objectives: To compare the clinical response and side effect of ferrous sulfate and iron polymaltose complex in young children in a randomized clinical trials. Material and Methods: The children with age group 1 to 6 year with iron deficiency anemia, hemoglobin level 7 to 10 gm were included. Diagnosis was confirmed by serum iron chemistry. Children were randomized into group-A (Iron polymaltose) and group-B (feuous sulfate). All children were given albendazole and advised to avoid phytates and tannins and were assessed after 1 month of treatment. Result: 138 Children were enrolled for the study of which 106 could be followed up. Of them 53 comprised group-A and rest comprised group-B. The difference of haemoglobin level at the start of the study of the two groups was not statistically significant. At the end of treatment the children of group-B were having higher hemoglobin level as compared to those of group-A (T test p<0.05). 26.3% of children had persistent complaints related to Iron deficiency anemia at the and of therapy in group-A of 3.77% in group-B. Loose motion, constipation, Nausea & black colour stool were observed in 3.77%, 0%, 0% 3.77% of children in group-A and 7.55%, 1.89%, 3.77% and 7.55% of children in group B. Conclusion: Ferrous sulfate has a better clinical response as well as more chances of side effect compared to iron polymaltose complex.