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Biomedical subjects

J Ninane

Publications and source records attributed to J Ninane.

At least 55 records · Page 3Linked to original sources
PubMed1991

Bone marrow transplantation in sickle cell anaemia.

Sickle cell anaemia is still responsible for severe crippling and death in young patients living in developing countries. Apart from prophylaxis and treatment of infections, no active treatment can be safely proposed in such areas of the world. Therefore a bone marrow transplantation was performed in 12 patients staying in Belgium and planning to return to Africa. Twelve patients, aged between 11 months and 23 years (median 4 years), underwent a HLA identical bone marrow transplantation. The conditioning regimen included oral busulphan for four consecutive days (4 mg/kg) followed by four days of intravenous cyclophosphamide (50 mg/kg). In 10 patients the engraftment was rapid and sustained. A further patient suffered transient red cell hypoplasia and another underwent a second bone marrow transplantation from the same donor at day 62 because of graft rejection. All patients are alive and well with a follow up ranging from 9-51 months (median 27 months). In all cases a complete cessation of vaso-occlusive episodes and haemolysis was observed as was a change in the haemoglobin pattern in accordance with the donor's electrophoretic pattern.

Adolescent↗
PubMed1991

[Reversal of urinary diversion after radical surgery for rhabdomyosarcoma of the bladder].

The authors report a case of reversal of urinary diversion in a 14 year old boy who had presented, three years previously with an embryonal rhabdomyosarcoma of the base of the bladder. The initial treatment had consisted of preoperative and postoperative chemotherapy combined with radical cystoprostatectomy with urinary diversion via a colonic conduit. The principle of the technique of reversal of urinary diversion are discussed in the light of this unusual case.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1990

Familial phaeochromocytoma: successful treatment with 131I-MIBG.

We report the case of a family in which the mother died of hypertensive encephalopathy following the relapse of a phaeochromocytoma. Two of her children are still alive. Both children had malignant phaeochromocytomas that have been treated by surgery and 131I-MIBG. The first child presented with phaeochromocytoma of the right suprarenal gland at the age of 7 years. Surgery was performed. At the age of 14 years, he developed a tumour of the left suprarenal gland and two pulmonary metastases demonstrated by 131I-MIBG. The three tumours were removed, but new lesions occurred. The boy then was treated with 200 mCi (7,400 MBq) of 131I-MIBG given twice, and is now free of disease more than 2 years after treatment. His sister presented at the age of 12 years with phaeochromocytoma of the left suprarenal gland, the only lesion recognized by 131I-MIBG. The tumour was removed, but 5 months later, she developed phaeochromocytoma in the right suprarenal gland. She was treated with 200 mCi (7,400 MBq) of 131I-MIBG and surgery was performed 6 months later. Histology of the suprarenal gland could not demonstrate the persistence of phaeochromocytoma cells. The child is now free of disease more than 2 years after treatment.

3-Iodobenzylguanidine↗
PubMed1990

Parotid gland abnormality found in children seropositive for the human immunodeficiency virus (HIV).

Out of our series of 24 children seropositive for the Human Immunodeficiency Virus (HIV), parotid gland enlargement was noted in 4 children with AIDS-related complex (ARC) presenting also a Lymphocytic Interstitial Pneumonitis (LIP) on their chest radiographs. The ultrasound (US) aspect of the parotid gland suggests acinar enlargement (suggesting the presence of lymphocytic infiltration). The aspect displayed in the parotid mirrors the process developing in other areas (lungs, liver, spleen, lymph-nodes), i.e. a syndrome of lymphocytic (CD8) proliferation present at the stage of ARC.

Child, Preschool↗
PubMed1990

Major malformations in a case of infantile myofibromatosis.

A case of infantile myofibromatosis associated with oesophageal atresia, annular pancreas, additional sacral vertebra and hypoplatic right kidney in a male neonate is reported. The possibility of associated malformations in this rare disease is outlined.

Abnormalities, Multiple↗
PubMed1990

Treatment of immune thrombocytopaenic purpura in childhood. A review of 146 patients.

Between 1969 and 1988, 146 children with immune thrombocytopaenic purpura (ITP) were seen in the outpatient clinic. The diagnosis was based upon an isolated thrombocytopaenia, without disseminated intravascular coagulation, splenomegaly or systemic disease and a normal bone marrow. Patients who required treatment (116/146) received either steroids (105/116) or IV gammaglobulins (IV-Ig) (11/116) as initial therapy. The long term outcome was similar in both groups. IV-Ig had the advantage to give a rapid increase in the platelet count without major side effects and to be very useful in chronic ITP either as maintenance therapy or as preparation for splenectomy.

Adolescent↗
PubMed1990

Combined CO2-laser and alfa recombinant interferon treatment in five children with juvenile laryngeal papillomatosis.

Juvenile laryngeal papillomatosis is a rare and benign tumoral disease of childhood characterized by numerous relapses despite complete resection. The ENT treatment of choice is to vaporize the papillomas with a CO2 laser. Since the discovery of a viral etiology (Human Papilloma Virus), resection has been followed by medical attempts to control the disease by using various antiviral treatments. Among the latter, alfa interferon has proved effective during the first six months of treatment. In this article, we report on five cases of refractory juvenile laryngeal papillomatosis treated by excision (CO2 laser in four children, surgical resections in one child) and alpha-r IFN 1.5 x 10(5) U/kg daily. With this strategy, three of the five children are currently disease-free for periods ranging from 22 to 68 months. This series includes one remarkable observation of one child who responded only to double doses of alpha-r IFN, after initial failure at conventional doses. This therapeutic scheme reduced the frequency of relapses in a fourth child. In only one child the treatment did fail to modify the natural course of the disease. Side effects were tolerable and included anorexia (one case), palmar erythema (one case), a flu-like syndrome (two cases) and mild transient transaminase rise (three cases) not precluding further treatment. CO2-laser caused one laryngeal oedema and synechia of the anterior commisure of the vocal laryngeal cords in one other case.

Carbon Dioxide↗
PubMed1990

Familial essential thrombocythemia.

We report three siblings (2 F, 1 M) aged 9, 12, and 15 years with a thrombocytosis, above 650 X 10(9)/l for at least 18 months and a leukocyte alkaline phosphatase score below 37. The diagnosis of essential thrombocythemia was based upon the exclusion of a reactive thrombocytosis or another myeloproliferative disease. Cytogenetic examination of marrow cells was normal.

Adolescent↗
PubMed1990

Cyclosporine A in five children with severe aplastic anemia.

Five children with aplastic anemia (AA) were treated with Cyclosporine A (CyA) after failure or poor response to other immunosuppressive agents. A complete remission was achieved in 3 patients, a good partial response in 1, and a poor partial response in another. In 1 patient in whom a complete remission was obtained, a relapse occurred 4 months after CyA was stopped. The treatment was resumed and a complete remission was again observed. Four patients were still on treatment, and side effects were mild. CyA appeared to be an effective treatment in AA.

Administration, Oral↗
PubMed1989

Alkylating activity in serum, urine, and CSF following high-dose ifosfamide in children.

The pharmacokinetics of alkylating activity were studied in 17 children treated i.v. with ifosfamide (IF) at 3 g/m2 as a 1-h infusion for 2 consecutive days every 3 weeks, with mesna as a uroprotector. Two patients were treated for a newly diagnosed rhabdomyosarcoma according to the current SIOP (International Society of Pediatric Oncology) protocol. The other 15 patients were treated in a phase II study and presented with one of the following malignancies in relapse: neuroblastoma (7), osteosarcoma (3), soft tissue sarcoma (2), Wilms' tumor (1), non-Hodgkin's lymphoma (1), and acute lymphoblastic leukemia (1). Plasma alkylating activity levels determined by using 4(4'-nitro-benzyl)-pyridine showed considerable inter-individual and intercyclic variations and decreased biphasically, with mean alpha and beta half-lives of 60 min and 6-7 h, respectively. Probably as a result of liver mixed-function oxidase induction, on the 2nd day of treatment the terminal half-lives were shorter, the plasma exposures were lower, and the mean plasma clearances were higher. Renal excretion was almost complete after 24 h, accounting for a mean of 19% of the injected dose. The CSF alkylating activity levels, obtained in four children, were always lower than the plasma levels and ranged from 8 to 51 micrograms/ml, with a mean CSF/plasma ratio of 0.53 +/- 0.23 during the first 12 h. We conclude that IF alkylating activity was biphasically cleared from the plasma, with significant interindividual and intercyclic variability, that the renal contribution to the clearance was low, and that high levels of CSF alkylating activity could possibly contribute to the CNS toxic side effects observed in pediatric patients treated with high-dose IF/mesna.

Adolescent↗
PubMed1989

Itraconazole versus ketoconazole for the prophylaxis of fungal infection in neutropenic children: results of two consecutive nonrandomized studies.

Two consecutive nonrandomized studies were conducted in children with prolonged granulocytopenia to evaluate the prophylactic antifungal activity of ketoconazole and the new triazole itraconazole. The conditions were equivalent in both studies. The incidence of colonization was 10% in the ketoconazole group and 19% in the itraconazole group (this difference is not significant). For suspected and proven infections, the incidence was 5% for ketoconazole and 10% for itraconazole, but the incidences were too low for statistical comparison. Although the colonization rate was higher for itraconazole, there was no sign of Aspergillus, whereas in the ketoconazole group an autopsy, proven aspergillosis has been reported.

Adolescent↗
PubMed1989

[Deletion of the long arm of chromosome 18, primary hypothyroidism, Biermer's anemia and IgM hypogammaglobulinemia].

We report the case of an adolescent girl who presents with the 18q-syndrome, primary hypothyroidism, pernicious anemia and IgM hypogammaglobulinemia. Her karyotype was performed during infancy because of malformations and showed deletion of the long arm of chromosome 18. The patient had been treated with levothyroxine (Elthyrone) since age 13 when primary hypothyroidism was documented. A close hematological follow-up was then undertaken due to the presence of anti-parietal cell antibodies. A megaloblastic anemia of sudden offset led to the diagnosis of pernicious anemia by age 16, which was confirmed by a positive Shilling's test. Recently, the patient was found to have antimicrosome antibodies and moderate IgM hypogammaglobulinemia.

Adolescent↗
PubMed1988

Bone marrow transplantation in five children with sickle cell anaemia.

Five children with severe sickle cell anaemia underwent an HLA compatible allogeneic bone marrow transplantation. In four children the engraftment was rapid and sustained. The fifth child rejected the bone marrow graft and required a second bone marrow transplantation 62 days after the first one. The outcome was then uneventful. In all cases there was complete cessation of vaso-occlusive episodes and haemolysis. The haemoglobin electrophoretic pattern became similar to that of the donor (AA or AS) and cytogenetic studies in three patients confirmed the donor origin of bone marrow cells.

Anemia, Sickle Cell↗