Persistent vacuoles in leukocytes: familial Jordans anomaly.

Multiple persistent vacuoles were seen in the neutrophils, monocytes and eosinophils of a 9 year old boy and his 10 year old sister. The siblings were both asymptomatic. In the bone marrow, the cytoplasmic vacuoles were also present in the promyelocytes, myelocytes and metamyelocytes, but not in the myeloblasts and they tended to be single and large in immature cells.

The molecular basis of beta-thalassemia in Turkey.

By using oligonucleotide hybridization, restriction endonuclease analysis and direct sequencing of amplified genomic DNA, we have been able to characterize 18 different mutations in the beta-globin genes of 161 beta-thalassemia homozygotes and 107 beta-thalassemia heterozygotes from Turkey (429 beta-thalassemia chromosomes). Previous studies dealing with beta-thalassemia in Mediterranean countries have shown that, in most Mediterranean populations, only a few mutations are prevalent. In contrast, beta-thalassemia in Turkey does not seem to be associated with a few predominant mutations.

Evaluation of growth hormone secretion and insulin-like growth factor-1 in prepubertal children with thalassemia.

Growth retardation is a clinical feature of patients with thalassemia major, and endocrine studies have frequently revealed the presence of normal growth hormone (GH) secretion. The present study was undertaken in 14 prepubertal thalassemic children (9 males and 5 females), aged 2(2/12) to 10(3/12) years, with the aim of evaluating GH response to i.v.

A rare entity among childhood malignant lymphomas (large anaplastic T-cell Ki-1 +).

A case of peripheral type T-cell lymphoma is presented to underline the difficulty in distinguishing the initial clinical findings of an inflammatory neoplastic disorder since the diagnosis could only be arrived at after several repeated lymph node biopsies. An 11 10/12-year-old boy admitted to the hospital with inguinal lymph node enlargement was diagnosed as having adenitis and periadenitis. The disease had progressed and the patient had remittent fever rising to 39 degrees C, and another biopsy was taken.

Intestinal effects of iron deficiency anemia in children.

A group of 11 children with iron deficiency anemia were studied with respect to intestinal structure and function. In six cases there were histological abnormalities of intestinal mucosa in varying degrees consisting of villous damage, increased activity in the crypts, increased lymphoplasmocytic infiltration and changes in the surface epithelium. Ultrastructurally, microvilli lesions, mitochondrial changes and an increase in lysosomes were observed.

Wilms' tumor: prognostic factors and survival.

A statistical analysis of 38 cases of Wilms' tumor treated and followed by the Pediatric Oncology Group of Cerrahpaşa Medical Faculty, from 1977 to 1984, is presented. The two-year survival rate was 67% and the five-year survival rate was found to be 63%. There was a strong correlation between the stage and age of the patients and prognosis.

Small non-cleaved follicular center cell lymphoma in Turkey. Burkitt's and non-Burkitt's types. A retrospective clinicopathologic analysis of 53 cases in the pediatric age group.

Fifty-three patients in the pediatric age group diagnosed as having small non-cleaved follicular center cell (FCC) lymphoma (40 Burkitt's and 13 non-Burkitt's type) have been re-evaluated. Although the histopathologic distinction between the two subgroups is fairly easy to determine there is a considerable degree of clinical overlap in regard to age, sex, anatomic presentation of the disease, the incidence of bone marrow and central nervous system involvement, and the survival. The most important determinant of survival in both subgroups is the stage of the disease.

[Deformity of the skull vault due to hypertrophy of red marrow in cases of anaemia ].

The shape of the head is often markedly changed in certain anaemic disorders, e.g. thalassaemia, congenital haemolytic anaemia and severe juvenile iron deficiency anaemia.

[The effect of changes in the bones of the skull due to Mediterranean anaemia on the shape of the skull and the formation of the cranial cavity (author's transl)].

The head and skull in patients with Mediterraneen anaemia show often a marked change of the external shape due mainly to the considerable enlargement of the cranial bones. From the abnormal external appearance the suspicion may arise that the form and space of the internal cranial cavity are likewise affected and even the basic structure of the skull is altered. However, a detailed investigation of the principal angles, distances and characteristic structures of the skull made on lateral roentgenograms of these patients, a comparison of the results between cases with different intensity of the osseous changes and a confrontation with the findings in normal individuals proved that there were no major discrepancies.