STROMAL-PREDOMINANT MESENCHYMAL HAMARTOMA OF THE LIVER WITH ELEVATED SERUM ALPHA-FETOPROTEIN LEVEL

Mesenchymal hamartoma of the liver (MHL) is an uncommon, benign, tumor-like lesion and is usually diagnosed in the first 2 years of life. Its pathogenesis remains unclear. Treatment of choice is radical excision. The authors report a case of solid stromal predominant MHL in a 12-month-old male infant who also had an elevated serum α-fetoprotein level. He also had hypospadias, which might represent a spectrum of developmental anomalies. It usually presents as an asymptomatic mass, however, as in the reported case, it may cause several complications due to the compression of surrounding structures. He was successfully treated with total excision of the pedunculated large tumor without any complication.     

Unusual presentation of infantile hemangioendothelioma

Infantile hemangioendothelioma is a rare benign hepatic tumor arising from mesenchymal tissue. Most of the cases present with congestive heart failure and asymptomatic abdominal mass were before 6 months of age. We described a patient with projectile vomiting, which is quite an unusual presentation. Following surgical resection, neither recurrence nor symptoms were seen in 2 years of follow-up.     

Clinical case series of pediatric hepatic angiosarcoma

Hepatic angiosarcoma is a rare, aggressive, malignant neoplasm with fewer than 50 cases reported in children. Prognosis is poor, with a minority surviving beyond 2 years after diagnosis. We report eight cases of pediatric hepatic angiosarcoma, diagnosed at a mean age of 3 years. Seven were initially diagnosed with an infantile hepatic hemangioendothelioma (IHHE) or hemangioma and the eighth with a “vascular tumor.” Two patients, who received liver transplant, survived. We suggest hepatic hemangiomas can rarely transform into angiosarcomas and a subset of IHHEs (Type II) are actually a low‐grade form of angiosarcoma rather than a benign lesion.     

A TERATOID WILMS' TUMOR WITH RAISED SERUM ALPHA-FETOPROTEIN LEVEL

Tumor markers are used to diagnose certain cancers and can be useful in monitoring the response to treatment. We describe a renal tumor with the features of a teratoid nephroblastoma associated with a raised serum level of alpha-fetoprotein (AFP). The serum AFP remained high during chemotherapy but returned to normal after nephrectomy. AFP was demonstrated by immunohistochemistry in cysts lined by enteric-type epithelium within the tumor. Cytogenetic examination of the tumor showed a clone of cells with trisomy 8. Raised serum AFP has not previously been described in teratoid Wilms' tumor.     

INFANTILE MYOFIBROMATOSIS WITH HEMANGIOPERICYTOMA-LIKE FEATURES OF THE TONGUE: A Case Study Including Ultrastructure

We report a case of an infantile myofibromatosis with hemangiopericytoma-like features arising in the tongue of a 5-month-old female infant. Many authors now classify neoplasms as infantile myofibromatosis that were previously called infantile hemangiopericytoma. The ultrastructural features of our tumor illustrate its biphasic nature and provide a possible explanation for its histogenesis. Infantile myofibromatosis, including those diagnosed as infantile hemangiopericytomas, rarely arise in any intraoral location. Despite the generally good prognosis associated with these neoplasms, complete surgical excision is recommended to avoid recurrences.     

婴儿型肝脏血管内皮细胞瘤并发心力衰竭2 例分析

目的探讨婴儿型肝脏血管内皮细胞瘤(IHHE)合并心力衰竭的临床特征、治疗及预后。方法回顾分析2例婴儿型IHHE合并心力衰竭患儿的临床资料,并复习相关文献。结果例1女性,产前超声示胎儿左腹部不均质回声团,生后第5天经磁共振成像、CT检查确诊,生后7天出现心力衰竭,于出生15天手术切除并经病理检查确诊IHHE,2月龄时复查无异常。例2男性,出生第15天时因新生儿黄疸入院,并很快出现心力衰竭,经磁共振、CT检查临床确诊IHHE。因病情进展迅速,于术前合并失代偿心力衰竭、呼吸衰竭死亡。结论婴儿型IHHE合并心力衰竭者病死率高,早识别、早干预、多学科干预可改善预后。     

LATE INFANTILE PROGRESSIVE ENCEPHALOPATHY WITH DISTURBED POLYUNSATURATED FAT METABOLISM

A case report is presented of a 6-year-old girl with a severe progressive encephalopathy of late infantile onset and with changes indicating marked disturbances in the metabolism of linolenic acid.     

COGNITIVE ABILITIES AND BEHAVIOURAL CHARACTERISTICS OF CHILDREN WITH IDIOPATHIC INFANTILE HYPERCALCAEMIA

An investigation of the psychological characteristics of 44 children aged 6-16 years with documented evidence of idiopathic infantile hypercalcaemia revealed cognitive abilities and behavioural patterns which distinguish these children from other mentally handicapped groups. The children were all mildly to severely mentally handicapped, but exhibited superior verbal skills compared to their visuo-spatial and motor abilities. They showed high rates of behavioural disturbance, particularly in terms of hyperactivity, social isolation, anxiety, and eating and sleeping difficulties. Other aspects of behaviour, spoken language and personality that were commonly encountered are also described and quantified. From these observations approaches to remedial teaching are suggested.     

HYDROPS FETALIS, HYDRAMNIOS AND HEPATIC VASCULAR MALFORMATION ASSOCIATED WITH CUTANEOUS HEMANGIOMA AND CHORIOANGIOMA

ABSTRACT. A premature baby presented with severe hydrops fetalis due to a multifocal angiomatous malformation of the liver. There were two other small vascular tumors: hemangioma of the skin and chorioangioma. Hydramnios and placental edema were also present. The association of severe hydrops fetalis and hydramnios with angiomatous malformation of the liver was not found in reviewing the literature.     

HYDROPS FETALIS IN A NEONATE WITH DOWN SYNDROME, TRANSIENT MYELOPROLIFERATIVE DISORDER AND HEPATIC FIBROSIS

Transient myeloproliferative disorder is a self limiting disorder characterized by leukocytosis with the presence of megakaryoblasts in the peripheral blood and bone marrow, anemia, thrombocytopenia, and organomegaly. It occurs in approximately 10% of newborn infants with Down syndrome. Hepatic fibrosis is seen in the severe form of transient myeloproliferative disorder with Down syndrome that is characterized by diffuse intralobular sinusoidal fibrosis and extramedullary hematopoesis. We describe a patient with hydrops fetalis, Down syndrome, and transient myeloproliferative disorder. We suggest that patients with the severe form of transient myeloproliferative disorder should be examined for hepatic fibrosis.     

SERUM LYSOZYME ACTIVITY IN CHILDREN WITH HEMATOLOGICAL AND MALIGNANT DISORDERS

ABSTRACT: Moe, P. J., Haneberg, B. and Finne, P. H. (Department of Paediatrics, University of Tromsø, Tromsø and Department of Paediatrics, University of Bergen, Bergen, Norway). Serum lysozyme activity in children with hematological and malignant disorders. Acta Paediatr Scand, 64: 830, 1975.–Pretreatment serum lysozyme activity was high in 2 children with myelomonocytic leukemia, 800 and 1000'/ig/ml, normal in all 10 cases of acute myelocytic leukemia and subnormal in 21 of 34 cases of acute lymphocytic leukemia. Normal values were found in all but one case of acute lymphocytic leukemia during complete remission including 8 cases after all therapy had been discontinued. All 8 are still in complete remission. Low serum lysozyme activity was found in 5 patients with acute lymphocytic leukemia in complete relapse, this could possibly be helpful in the diagnosis of early relapse. Activity was subnormal in 5 of 17 children with malignant tumours, and in 3 of 65 cases of various benign hematological disorders.     

婴幼儿肝血管瘤继发消耗性甲状腺功能减退症1例诊断及治疗

目的探讨婴幼儿肝血管瘤的诊断及治疗。方法回顾分析1例以反复贫血及先天性甲状腺功能减低症为表现的肝脏多发灶性血管瘤婴儿的临床资料,并进行文献复习。结果女性患儿生后2天诊断先天性甲状腺功能减低症,口服左旋甲状腺素片,疗效欠佳。患儿2月龄时血红蛋白68 g/L,为正细胞正色素性贫血,网织红细胞计数升高,总胆红素、游离胆红素、胆汁酸、甘氨胆酸及γ谷酰基转肽酶明显升高,促甲状腺素明显升高,游离T3明显降低。肝脏超声示肝脏实质内散在多个低回声团块,最大者位于肝右叶约8.9 cm×5.3 cm。腹部CT示肝脏体积明显增大,117 mm×145 mm×90 mm,肝实质内见类圆形异常强化,呈多发性大小不等的囊性病变,周边可见稍高密度分隔影,胆囊分辨不清。诊断为肝血管瘤,予加用普萘洛尔联合左旋甲状腺素片口服。治疗6个月后随访,腹部超声示肝脏血管瘤数量和大小都缩小一半,肝酶及胆汁酸水平降至正常范围。逐渐减量左旋甲状腺素,甲状腺功能恢复正常,目前生长发育同正常同龄儿。结论婴儿肝脏血管瘤可导致严重并发症,及时诊断及治疗可明显改善预后。     

SERIAL DETERMINATIONS OF SERUM FERRITIN IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA Evaluation of its Usefulness as a Prognostic Index

Abstract. Thirty children with acute lymphoblastic leukemia were monitored with serial serum ferritin determinations for up to 17 months. In children with acute lymphoblastic leukemia before initiation of therapy, or in relapse, the mean serum ferritin concentration was 636 μg/l. In children who went into primary remission, the mean serum ferritin concentration fell from 265 μg/l prior to start of treatment, to 161 μg/l after 3 months of treatment. Five patients relapsed. Their serum ferritin levels prior to the relapses ranged from 7 to 135 μg/l. At the time of relapse a further increase in serum ferritin was found in only 2 of the children. Thus, whereas high serum ferritin levels may signal disease activity in acute lymphoblastic leukemia, a normal serum ferritin level does not exclude disease activity or impending relapse.     

THE ROLE OF SERUM FACTORS IN THE LYMPHOCYTE TRANSFORMATION TEST OF CHILDREN WITH ACUTE LEUKAEMIA

ABSTRACT. Révész, T., Szigeti, R. and Schuler, D. (Second Department of Paediatrics, Semmelweis University Medical School, Budapest, Hungary). The role of serum factors in the lymphocyte transformation test of children with acute leukaemia. Acta Paediatr Scand, 63: 715, 1974.—PHA-induced lymphocyte transformation tests were carried out in 14 children who were in full remission of acute lymphoblastic leukaemia and in 12 controls. Stimulation was assessed on the basis of tritiated thymidine uptake. For an adequate testing of lymphocyte reactivity, dose-response curves were established for each child. No significant difference could be observed in either the maximum response, or the shape of the curve, between the leukaemic and the control groups. Supplementation of the culture medium with autologous plasma instead of AB serum did not result in any significant inhibition or stimulation of lymphocyte reactivity to PHA. The lymphocyte response of both leukaemic and control children, however, was greatly reduced when sera from untreated leukaemic children was used. Allogeneic sera drawn from patients in remission exerted little or no inhibitory effect, while that obtained in relapse was again more inhibitory. The observed effect was not due to cytotoxic antibodies, nor due to natural iso-antibodies, but is thought to be a phenomenon closely connected with the active phase of malignant diseases. The exact nature of the agent is still far from being clarified at present.     

SERUM INDOMETHAC1N CONCENTRATIONS AFTER INTRAVENOUS ADMINISTRATION TO PRETERM INFANTS WITH PATENT DUCTUS ARTERIOSUS

Abstract. Petersen, S., Christensen, N. C, Jensen, K. M. and Ryssing, E. (Departments of Neonatology and Paediatrics, Rigshospitalet, Copenhagen, and Dumex Ltd., Copenhagen, Denmark). Serum indomethacin concentrations after intravenous administration to preterm infants with patent ductus arteriosus. Acta Paediatr Scand, 70:729,.–Six preterm infants with PDA received 14 treatments with indomethacin 0.2 mg/kg intravenously. Auscultatory and echocardiographic assessment indicated closure of the duct in 2, partial closure in 2, and no effect in 2 infants. The mean serum concentration of indomethacin was: 15 min after the first injection 1314 ng/ml, after 1 hour 970 ng/ml, after 6 hours 718 ng/ml, and after 24 hours 388 ng/ml. The mean half-life of indomethacin in the serum was 20 hours (range 9–50 hours). Side effects in all infants were hyponatraemia, decreased urinary output, decreased urinary sodium excretion, and weight gain. One infant had transient thrombocytopenia and gastrointestinal haemorrhage. By intravenous administration of indomethacin in a dose of 0.2 mg/kg to preterm infants a sufficiently high serum concentration is obtained shortly after the injection. To maintain a high serum concentration for a longer period it is recommended to give a second dose of 0.2 mg/kg after 6 hours and if necessary a third dose of 0.1 mg/kg 24 hours after the first dose.     

INSULIN ANTIBODIES IN THE SERUM OF DIABETIC CHILDREN TREATED FROM THE DIAGNOSIS OF THE DISEASE WITH HIGHLY PURIFIED INSULINS

Abstract. Fitly-one diabetic children, treated with highly purified, porcine insulins from the diagnosis of the disease were studied for the development of insulin antibodies during the first years of treatment. Sera were obtained before the start of treatment and at repeated intervals thereafter. Serum insulin antibodies were measured by three methods in three laboratories. Twenty-nine children were treated with R.I. (rare immunogenic) insulins at one department and 22 with MC (monocomponent) insulins at the other department. Before the start of treatment, serum insulin antibodies were almost invariably zero or below the limit of detection. During the first year a considerable number of cases, more than that reported for adults, formed detectable amounts of antibodies, and more so in the R.I. than in the MC series. The levels were however definitely lower than those observed with the Same methods in diabetic children on conventional insulins. Analysis of variance showed no differences between the various age groups. No significant correlation was found between the highest titre of serum insulin antibodies on the one hand and insulin dose or glucosuria on the other hand. The results confirm the concept that children form antibodies to insulin more actively than adults.     

SERUM CHOLESTEROL CONCENTRATIONS IN NEWBORN INFANTS WITH GESTATIONAL AGES OF 28–42 WEEKS

Abstract. Ginsburg, B.-E. and Zetterström, R. (Department of Paediatrics, Karolinska Institute, St. Göran's Children's Hospital, Stockholm, Sweden). Serum cholesterol concentrations in newborn infants with gestational ages of 28–42 weeks. Acta Paediatr Scand, 69: 587, 1980.—Serum total cholesterol, HDL-cholesterol and VLDL-LDL-cholesterol were determined in 53 newborn infants with gestational ages of 28–42 weeks. In pre-term infants (gestational age < 37 weeks) the total cholesterol concentration in cord blood was higher than in term infants. Mean values were 2.4 and 1.7 mmol/l, respectively. The HDL-cholesterol/ VLDL-LDL-cholesterol ratio was 1.8 in pre-term and term infants. In 11 pre-term and 17 term infants a second determination was made 3–4 days after birth. Total cholesterol had increased more in term than in pre-term infants and the difference found at birth had already levelled out. Mean value was 3.0 mmol/I in pre-term and term infants. The HDL-cholesterol/ VLDL-LDL-cholesterol ratio had changed to 0.6 in pre-term and term infants. Six pre-term infants who received intravenous fluids only were also studied. Their values did not differ from those in pre-term infants fed orally. Free and esteritied cholesterol were determined in 26 infants of varying gestational ages. About one-third of the total cholesterol was in the free form in pre-term and term infants at birth and during the first days of life.