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Biomedical subjects

N Kamani

Publications and source records attributed to N Kamani.

30 records · Page 2Linked to original sources
PubMed1988

Pediatric acquired immunodeficiency syndrome-related complex: clinical and immunologic features.

The long term clinical outcome for infants and children with the pediatric acquired immunodeficiency syndrome-related complex is unknown. This report describes our experience with 14 patients with acquired immunodeficiency syndrome-related complex who have been followed for 11 to 71 months since the onset of their symptoms. The most frequent clinical features at presentation were persistent generalized lymphadenopathy (14 of 14), hepatosplenomegaly (11 of 14) and a history of recurrent otitis media (7 of 14). Except for hypergammaglobulinemia (14 of 14) and reversed T4/T8 ratios (9 of 14), immunologic analyses, including in vitro responses to mitogens and antibody responses following immunization, revealed no consistent abnormalities. Over the course of follow-up, none of the patients have developed serious or opportunistic infections and 12 of 14 have shown catch up or age-appropriate growth. The T4/T8 ratios have remained stable in 8 of 11 and improved in 2 of 11 patients. Gradual regression of hepatosplenomegaly and lymphadenopathy has been noted patients. Although follow-up studies over a longer period are needed to confirm our observations to date, acquired immunodeficiency syndrome-related complex may represent a prolonged plateau in the course of human immunodeficiency virus infection in many infected children. Detailed immunologic evaluation of these patients may help to identify a subset of children that could benefit from periodic gamma-globulin or chronic antibiotic therapy.

AIDS-Related Complex↗
PubMed1988

Natural history of chronic granulomatous disease.

An analysis of the patient characteristics, clinical course, and therapy of 52 patients with chronic granulomatous disease (CGD) was carried out through a multinstitutional questionnaire survey in order to further define the natural history of this disorder of phagocyte microbicidal function. The results suggest that CGD is characterized by significant heterogeneity in clinical course and prognosis. The age at diagnosis, severity of disease, or early death in an affected sibling does not seem to be of value in predicting the prognosis for individual patients with CGD. A more detailed, large-scale multicenter study and the establishment of a patient registry is recommended.

Adolescent↗
PubMed1987

Eosinophilic cellulitis in a family.

Eosinophilic cellulitis (Wells' syndrome) is characterized by recurrent episodes of nonpruritic, indurative, cutaneous swellings. Three cases of this syndrome occurred in two male siblings and their mother. In all three family members, skin lesions appeared early in infancy. Unusual clinical manifestations included eosinophilic pleural effusions and pericarditis.

Adult↗
PubMed1987

Occult bacteremia with nontypeable Haemophilus influenzae.

A 2-year-old boy had occult bacteremia with nontypeable Haemophilus influenzae 6 weeks after receiving H. influenzae type b polysaccharide vaccine. Evaluation of his host defense was normal. As determined by outer membrane protein electrophoresis and Southern hybridization analysis, this strain was not related to type b strains. Its virulence in rats was similar to that of another nontypeable strain and less than that of a type b strain.

Animals↗
PubMed1987

Gallium scanning in lymphoid interstitial pneumonitis of children with AIDS.

Lymphoid interstitial pneumonitis (LIP) is a frequent pulmonary complication in the child with the acquired immune deficiency syndrome (AIDS) and human immunodeficiency virus (HIV) infection. We report the gallium scan findings in two children with AIDS and LIP. Gallium scintigraphy in both children demonstrated increased radionuclide concentration throughout the lungs, a pattern indistinguishable scintigraphically from that of Pneumocystis carinii pneumonia (PCP). This should alert nuclear medicine practitioners and referring physicians to another cause of diffusely increased gallium uptake in the lungs of patients with AIDS.

Acquired Immunodeficiency Syndrome↗
PubMed1985

Medical management of bilateral renal malakoplakia.

Renal malakoplakia is reported in an 11 year old girl with myelomeningocele and associated neuropathic bladder. She is the first reported child to have survived bilateral renal malakoplakia with full recovery of renal function after medical management.

Child↗
PubMed1984

Bone marrow transplantation in chronic granulomatous disease.

A 5-month-old Amish infant boy with chronic granulomatous disease underwent bone marrow transplantation from his 5-year-old, histocompatible brother after a preconditioning regimen of busulfan 2 mg/kg/day for 4 days, followed by cyclophosphamide 50 mg/kg/day for 4 days. At the time of bone marrow transplantation, he was free of infection, and remained so throughout the course of the transplant. He was engrafted promptly, with complete reversal of the neutrophil function defect and no sign of graft-versus-host disease. This was followed by loss of the erythroid graft and deterioration in neutrophil function over a period of 9 months. Sixteen months after transplantation, he is free of infection and growing normally, with essentially no evidence for neutrophil engraftment.

Bone Marrow Transplantation↗
PubMed1984

Bone marrow transplantation. Problems and prospects.

Over the last 15 years, human bone marrow transplantation has evolved from experimental therapy of last resort for patients with end-stage leukemia and marrow aplasia. The increasing success and use of marrow transplants has stemmed from important advances made in (1) the understanding and definition of the major histocompatibility complex in man, (2) the development of safe yet efficacious immunosuppressive and myeloablative preconditioning regimens, and (3) the development of techniques of intensive supportive care for these patients during their intra- and post-transplant course.

Adolescent↗
PubMedDate not supplied

Immune reconstitution after autologous purged bone marrow transplantation in children.

PURPOSE: Immune reconstitution was studied in 30 children who had received purged autologous bone marrow transplantation for neuroblastoma or acute myeloid leukemia (AML). METHODS: Patients with neuroblastoma received high-dose chemotherapy and total body irradiation, and patients with AML received chemotherapy alone. Marrows were purged ex vivo with either antineuroblastoma monoclonal antibodies (neuroblastoma) or 4-hydroperoxycyclophosphamide (AML). Lymphocyte subsets, mitogen stimulation studies, and immunoglobulin levels were studied every 4 months. RESULTS: There were no significant differences between the two groups of patients in lymphocyte number or subsets over time. In both groups, CD2+ and CD4+ cells were below normal in 33% of patients at 12 months. CD4+/CD8+ ratios were below normal for up to 8 months after transplantation and natural killer cells were elevated for up to 2 years in most patients. Median IgG and IgA levels were below the age mean even at 2 years after transplantation, although patients with AML had significantly higher IgG levels at 12 months compared with those with neuroblastoma. Lymphocyte proliferative responses to mitogens were markedly reduced at 4 months but returned to normal at 8 months. Despite the delay in immune reconstitution, there were no life-threatening infections. CONCLUSIONS: There appeared to be little difference in the overall kinetics of immune reconstitution between the children with neuroblastoma, who received total body irradiation and high-dose chemotherapy, and those with AML, who received high-dose chemotherapy alone as their pretransplant preparative regimen.

Acute Disease↗