1983年 · 第21卷第05期

Normal values of Hb, SF, FEP, FEP/Hb, SI, TIBC, TS were obtained statistically from 161 children under first two years of age who had been supplied with adequate iron. On the other hand, the status of iron nutrition was investigated in 239 children at the same age who had no supplement of iron. No evidence of iron depletion was found during the first 3 months and the level of iron storage (SF: X_G = 188.4 ng/ml) was high. After that time the decrease of iron storage was accompanied with increase of iron deficiency.

In four groups from 3 months to 2years the total incidence of ID, IDE and IDA was 40.0%, 39.3%, 82.3 % and 89.6% respectively. In control groups, iron nutrition was improved significantly by adding adequate iron for 3 months. It is necessary for each infant to be supplied with iron (or iron rich food) after first 3months.

SF, FEP and FEP/Hb were more sensitive indices to detect iron deficiency. SI, TIBC and TS were less sensitive in detecting ID and IDE. But they were still useful in the diagnosis of IDA especially in advaned IDA. After iron therapy (3 mg element iron/kg/d), the changes of Hb,FEP/Hb, SI and TS were more sensitive in contrast to that of SF, FEP and TIBC. So the formers are helpful in evaluating the efficiency of the therapy and the latters may indicate that iron deficiency is still present and further treatment is required.

The purpose of this paper is to observe the catecholamine metabolism in the healthy, the non-iron deficient anemia and the iron deficiency anemia children before and after iron therapy. The platelet monoamine oxidase (MAO) activity in children with iron deficiency anemia was abnormally lower than in the non-iron deficient anemia and the healthy children. Administration of iron could correct the MAO deficiency in iron deficiency anemia within one month. The urinary excretion of vanillyl mandelic acid (VMA) in 24-hour samples in the iron deficiency anemia was abnor mally higher than in the health children. The urinary excretion of VMA returned to normal within one month after the iron therapy. These changes of the catecholamine metabolism was related to the iron deficency rather than the anemia. These perhaps led the iron deficiency anemia children to behavior alterations.

During the past 27 years, 171 patients proved to be cases of histiocytosis X were admitted to Beijing children's Hospital. Among them 140 cases were followed up successfully. The survival rate was only 28.1% in 57 patients without specific therapy, whereas that of the 75 patients treated with combined chemotherapy was 73.3%. Three serious patients treated with thymodin got good effect.

Besides, there were other factors affecting the prognosis, namely, the age of the patient, the number of involved organs and the severity of functional disorder.

The main causes of death were respiratory failure, progressive wasting, hepatic failure and severe anemia. Among those survived 15 patients developed serious sequelae, such as diabetes insipidus, fibrosis of lung, mental retardation, epilepsy short stature and underdevelopment of mandible.

22 patients with ALL were encountered in 13 cases (59.1%) with Null-ALL, 4 (18.2%) with Pre B-ALL, 3 (13.6%) with T-ALL, 2 (9.1%) with B-ALL. our clinic from December, 1980 to June, 1982. Of which there were twelve kinds of cell markers were studied in these cases before treatment, after remission and during relapse. The changes of cell markers were found to be related to the clinical manifes tations of the disease.

The analysis of the cell marker revealed that E_37·c, E_4·c, HTLA, HBLA, SmIg and C-IgM were necessary in the classification of ALL.

Clinically, when Null-ALL became relieved, the cell markers E37. c, E_4·c and HTLA increased and C_3dR decreased as compared with before treatment, but when Null-ALL came to relapse, E_37·c, E_4·c and HTLA tended to decrease and C_3dR increased as compared with after remission. When Pre B-ALL became completely relieved, C-IgM, HBLA and C_3dR tended to decrease and E_37·c, E_4·c and HTAL increased as compared with before treatment;but when Pre B-ALL came to relapse, these cell markers underwent reverse changes.

The cell markers in 3 cases of T-ALL and 2 cases of B-ALL were found to change in different stages of the disease.

The study of the changes of cell markers in ALL was significant in the understanding of changes of the disease and the therapeutic effects of it.

A study on 70 children patients with acute lymphoblastic leukemia(ALL) classi fied immunologically with Es, EAC, EA, and SmIg assays was evaluated: 18 cases were T-ALL(25.7%); 52 cases were nonT non-B ALL(74.3 %) . 27 patients of this series were typed with antisera of AHTS, ATG, AHBS, and ACALLS: 9 cases were T-ALL; 14 cases were C-ALL; 4 cases were N-ALL.

66 patients with ALL were primarily treated, 54 of them were administered 2 or more than 2 weeks with a complete remission rate of 88.9%. The patient with C-ALL were well responsive to VP induction and remitted within a short period 3 types of reaction were obtained to VP induction in patients with T-ALL: common reaction, delayed reaction and rebounding reaction. The differences of the 4-6 week remission rates and the incidences of induction failure between these two groups were significantly apparent. It was shown that T-ALL should be treated with some chosen aggressive combined chemotherapy rather than VP regimen alone.

The increasing or decreasing of the level of platelet count during induction may be considered as an early indicator of being responsive to VP induction or a relapse of the disease.

The leukemic blast cells from 10patients with acute lymphocytic leukemia (ALL) were detected immunologically and classified as follows: T-ALL (2 cases) , BALL (1 case) and N-ALL (7 cases) . They were also studied by means of a scanning and transmission electron microscope and the correlation between the immunological types and the ultrastructural characteris tics were observed. Scanning electron microscopic examination showed no relation between the surface morphology of the cell and the immunological types in the ALL, but revealed significant relation between the surface morphology and the cell cycle. Transmission electron microscopic examina tion showed some specialities of ultrastruc tural characteristics in each immunological type. Cells from T-ALL exhibited a lot of dense granules clustering in the cytoplasm. In B-ALL, there were some masses of glycogen bodies, rough endoplasmic reticulum increased and centrosomes clearly displayed in many cells. Nothing particular was observed in N-ALL.

Combined rosette formation and surface membrane immunoglobulin markers were detected in a group of 17 patients with acute lymphoblastic leukemia (ALL) in childhood. The patients were divided into 3 groups:4 with T-ALL, 1 with B-ALL, and 12with N-ALL. E-rosette-forming blasts in 4T-ALL of these 17 patients for the presence of Fc receptors and the ultrastructure of malignant cells in 3 patients (2 T-ALL, 1N-ALL) of them were studied. The findings suggested that the heterogenicity of Fc receptor expression on leukemic blasts in these 4 patients demonstrated a malignant proliferation of T cell in different stages of differentiation or maturation. We found that the leukemic T-cell bound to SRBC by point contact; but several ultrastructural differences were demonstrated in different sources and the stages of differentiation. The correlation between the ultrastructural appearance of leukemic cells and surface im munological markers in ALL were discussed.

During the period of 1973—1982 30children were diagnosed as autonomic epilepsy in our clinic, including headache epilepsy-20 cases, abdominal epilepsy- 9cases and vomiting epilepsy-1 case. Criteria for dignosis were as follows: (1) other organic disease being ruled out after thorough physical examination, (2) typical paroxysmal symptoms of autonomic nerve system exist, (3) EEG finding abnormal, (4) good response to anticonvulsant therapy. After medication, 5 cases (16.7%) were free from episode and 15 cases (50%) undergood control, 3 cases (10%) had poor response and outcome of 2 cases (6.6%) was unknown. 13 cases were followed up with regular EEG examination for 1—9years. EEG became normal in 9 cases, re mained to have mild abnormalities in 3cases and no changes found in 1 case.

From September 1980 to June 1981 18 cases of hypomagnesemic convulsions were admitted. There were 10 neonates and 8infants. The chief clinical manifestation was convulsions and typical tetany was not seen. Signs and symptoms of hypomagnesemia are indistinguishable from those of hypo calcemia unless the serum magnesium is determined. Serum magnesium levels of 50normal children were determined. Accor ding to the average value—2 standard deviation = 2.17—2 × 0.34 = 1.49 mEq/L, We de fined hypomagnesemia as the serum mag nesium levels below 1.48 mEq/L. The serum magnesium levels of these patients varied from 0.65 to 1.46 mEq/L. Of 12cases serum calcium levels<7.5mg/dl.

2.5% MgSO₄ was administered intra venously by continuous infusion in a dose of 2—4 ml/kg every 12 hours. After the convulsions had been controlled a 25%MgSO₄ was given intramuscularly in a dose of 0.4 ml/kg twice daily. Convulsions usually ceased after 1一4 doses of MgSO₄, but the serum magnesium levels did not rise to the normal level until 6—13 days. The convulsions could not be controlled by re peated administrations of calcium gluconate in 6 patients who had both hypocalcemia and hypomagnesemia. Only after the admi nistration of MgSO₄ did the serum calcium levels rise to the normal level and the convulsions cease.

This paper reports 470 cases of histio cytosis X which were collected from 41hospitals of 20 provinces and two munici palities, According to their clinical, roentgenological and pathological findings, they were divided into 6 groups: (1) Letterer-Siwe syndrome 246 cases (52.3%);(2) Hand-Schüller-Christian syndrome 127cases (27.0%); (3) Eosinophilic granuloma of bone 34 cases (7.2%); (4) intermediate form 43 cases (9.8%); (5) Lesions limited to a single organ 6 cases (1.3%); and (6) Patients difficult to be classified 14 cases (3.0%).

Some rare expressions of histiocytosis X were observed, such as: lesions limited to lung or limited to liver; premature eruption of deciduous teeth at 2-4 month and early shedding at 6 month to 2 years;the intermediate form between LettererSiwe syndrome and malignant histiocytosis;occurrence of the disiase in monozygotic twins at the same time; and Letterer-Siwe syndrome occured in newborn or in elder child; etc.

Analyses show that the incidence of the disease tends some what to increase; the prognosis is related to the age at presenta tion and the kind, number and severity of involved organs. Classifing this disease according to the age and the involved organ of affected patient may be of value in determining the prognosis and treatment.