Acute lymphoblastic leukemia associated with Klinefelter syndrome.
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Child, Preschool↗
Biomedical subjects
A Kara
Publications and source records attributed to A Kara.
Congenital hypoplastic anemia in six patients: unusual association of short proximal phalanges with mild anemia.
Six congenital hypoplastic anemia (CHA) patients from five families who have been followed from 2 months to 28 years are presented. Mild hypoplastic anemia in a 13-year-old girl was associated with clinodactyly of the fifth finger on both hands, shortness in the proximal phalanges on all fingers, and syndactyly between the second and third toes and short fourth toe on the right foot. These abnormalities, except for clinodactyly, have not been reported previously in CHA. In one of the five families genetic transmission was thought to be autosomal-dominant since both the father and the son had the disease. Therapy with corticosteroids was initiated in all patients at the ages of 3.5 months to 13 years. Complete or near-complete recovery of anemia was obtained.
Adolescent↗
Toxic methemoglobinemia after injection of prilocaine in a newborn. A case report.
The acute onset of cyanosis in an infant is an alarming development. We report an infant who became cyanotic after injection of prilocaine. Prilocaine is a local anesthetic that is widely used in clinical practice, but usage in infancy easily causes methemoglobinemia. Acquired methemoglobinemia occurs in individuals exposed to an increased amount of agents that oxidize hemoglobin iron.
Anesthetics, Local↗
Profile of bone marrow iron stores in childhood iron deficiency anemia.
To demonstrate the importance of bone marrow iron stores, we examined the complete hemogram, serum iron (SI), serum iron-binding capacity (SIBC), transferrin saturation (TS), serum ferritin and bone-marrow-stored iron in 31 children with iron deficiency (ID). The ages of the patients ranged from one to 14 years (mean 3.7 +/- 3.9). Laboratory findings of the 31 patients were as follows: hemoglobin (Hb) 8.5 +/- 2.4 g/dl, hematocrit (Hct) 27.8 +/- 6.3 percent, mean corpuscular volume (MCV) 58.6 +/- 8.6 fl, red blood cell count (RBC) 4 +/- 0.8 10(12)/L, red cell distribution width (RDW) 19.3 +/- 4.9, SI 17.2 +/- 9.3 microg/dl, SIBC 311 +/- 50.5 microg/dl, TS 5.5 +/- 2.8 percent and ferritin 6.7 +/- 7.3 ng/dl. In the bone marrow smears with iron stains, all patients' scores were zero for iron stores, which shows that bone-marrow-stored iron in childhood is easily affected. Because of the traumatic effect of bone marrow aspiration, it is recommended that it not be done routinely. The diagnosis of ID could be especially difficult in patients with low SI levels but normal SIBC levels and in patients with chronic inflammatory diseases. In those conditions, illustration of bone marrow stores could be of particular assistance for diagnosis of iron deficiency.
Adolescent↗
Safety, tolerability and immunogenicity of a Haemophilus influenzae type b vaccine containing aluminum phosphate adjuvant administered at 2, 3 and 4 months of age.
The primary aim of this study was to assess the tolerability and immunogenicity of a new Haemophilus influenzae type b (Hib)/AlPO4 (CHIRON, SpA) vaccine, in two-month-old healthy infants. Twenty-three subjects were enrolled and administered the new Hib vaccine containing AlPO4 adjuvant at two, three and four months of age concomitantly with diphtheria-pertussis-tetanus (DPT) and hepatitis B vaccines according to the local program. Children were observed for 30 minutes after each immunization for any immediate local and systemic reactions. An active surveillance for side effects was performed on the 2nd and 7th days following each immunization by telephone. Families also filled out diaries for the first seven days. From the 2nd day to the next immunization only data about adverse events necessitating a physician's visit or about serious adverse events were collected. Blood samples were obtained before the first immunization and one month after the third dose for evaluation of anti-polyribosylribitol phosphate (PRP) antibody response. Local reactions at the Hib site were mild and less frequent compared to those observed at the DPT site. Systemic reactions noted after the three immunizations were fever in 70 percent, irritability in 48 percent, persistent crying in 26 percent, change in eating habits in 22 percent, diarrhea in 17 percent, sleepiness in 17 percent, vomiting in 9 percent, and unusual crying in 4 percent of the cases. There was no serious adverse event. One hundred percent and 95 percent of children achieved an anti-PRP antibody response over 0.15 microg/ml and 1.0 microg/ml, respectively. The geometric mean titer was 15 microg/ml and the geometric mean ratio 84. It was concluded that the new (Hib)/AlPO4 vaccine is safe and well tolerated, and induced a good PRP antibody response in healthy two-month-old infants.
Adjuvants, Immunologic↗
Salmonella septic arthritis in a patient with acute idiopathic thrombocytopenic purpura treated with steroid.
Salmonella has three clinical presentations: self-limiting gastroenteritis, a systemic syndrome (enteric or typhoid fever), and bacteremia with focal infection. Hematogenous infections can cause focal lesions, but unusual manifestations occur more often when predisposing factors such as T cell defect, hemolytic disorders (sickle cell disease, malaria) or trauma are present. Salmonella tend to invade bones and joints. There is no mention of acute idiopathic (immune) thrombocytopenic purpura as a predisposing factor for salmonella septic arthritis; however there are reports about the importance of platelets for the immune response. Here we present a case of Salmonella enteritidis septic arthritis following acute idiopathic (immune) thrombocytopenic purpura in a 15-year-old female patient who has been on steroid therapy for the last two weeks.
Adolescent↗
Screening for tuberculosis in a primary school in Ankara.
Tuberculosis is still an important health problem in developing countries. A screening program was conducted upon learning that one of the teachers of a primary school in Ankara was diagnosed to have active pulmonary tuberculosis. A total of 341 students in the same building with the index case were screened for tuberculosis. There were 109 students with positive tuberculin test reaction. A higher ratio of tuberculin test positivity among the students of the teacher with active tuberculosis versus students vaccinated with BCG one year previously according to the routine vaccination program was determined. Isoniazid prophylaxis was given to the students with positive tuberculin test. The study shows the importance of an urgent work-up of index cases and their environment to prevent the spread of tuberculosis.
Child↗
Giant cell pneumonia in a leukemic child in remission: a case report.
Giant cell pneumonia is a rare and uncommon type of lung infection developing as a complication of measles, especially in immunocompromised patients, whether their immune systems are affected primarily or whether they have acquired immune defects. As well as being uncommon, it is also atypical because of absence of the characteristic rash and of absent or low antibody titers against measles in most of the cases. It is known that cellular immunity is more important than humoral immunity in the host response to measles, so hypogammaglobulinemic patients with normal cellular immunity usually recover uneventfully from measles and also have the characteristic rash. We report a case with giant cell pneumonia that was confirmed by postmortem histopathological examination. We especially want to point out that even in the absence of rash, with the clinical and radiological features of pneumonia, measles should be considered in a patient, whether in remission or not, receiving immunosuppressive treatment.
Antineoplastic Combined Chemotherapy Protocols↗
Treatment of X-linked lymphoproliferative disease (Duncan disease) with high-dose methylprednisolone and etoposide (VP-16).
A five-year-old boy in the acute phase of X-linked lymphoproliferative (XLP) syndrome (Duncan disease) with high fever and hepatosplenomegaly was treated successfully with high-dose methylprednisolone and VP-16 for 15 months. He had been alive for four years after diagnosis as of this writing. We recommend high-dose methylprednisolone and VP-16 in patients with XLP who have to wait for a suitable donor before bone marrow transplantation.
Anti-Inflammatory Agents↗
Treatment of hemophilic pseudotumor with low-dose radiotherapy.
Hemophilic pseudotumor is one of the most serious complications of hemophilia and is usually treated with extensive surgery. A new treatment approach is radiotherapy. Patients with long-bone pseudotumors are usually treated with high doses of radiotherapy greater than 1500 cGy. We treated a 13-year-old hemophilic boy who had a pseudotumor of the tibia with low-dose radiotherapy (600 cGy). There was no complication during the two-and-a-half-year follow-up. Improvement of both the clinical and radiological status of the patient was noteworthy. We would like to suggest the use of low-dose radiotherapy in patients with hemophilic pseudotumors.
Adolescent↗