Lipoblastoma and lipoblastomatosis in infancy and childhood: histopathologic, ultrastructural, and cytogenetic features

Lipoblastoma is a relatively rare tumor that occurs in infancy and early childhood and arises from embryonic white fat. Although a benign tumor, lipoblastomas tend to recur and may resemble myxoid liposarcoma. The authors report 26 cases over a 15-year period at Texas Children's Hospital. There was a slight female predilection (14F:12M). The most common symptom was a painless mass with or without increasing size. The trunk, extremities, head and neck, retroperitoneum, inguinal canal, peritoneal cavity, and lung were the tumor sites. Most tumors were circumscribed lipoblastomas and the minority were diffuse infiltrative lipoblastomatosis. Reexcision for residual or recurrent tumor was necessary more frequently in patients with lipoblastomatosis. Histopathologic examination and ultrastructural examination revealed cellular neoplasms composed of immature adipocytes with relatively well-defined septa, frequent lipoblasts, a fine vascular network, and often a myxoid appearance resembling myxoid liposarcoma. Cytogenetics was performed in 4 cases with chromosome 8q abnormality being most common. The major concern with lipoblastoma in children is to completely excise the tumor to avoid leaving residual tumor and to prevent recurrences. Confusion with myxoid liposarcoma, well-differentiated liposarcoma, and typical lipomas may occur. Although asymptomatic, lipoblastomas may cause dysfunction of other organ systems due to mass effect. Complete surgical excision with at least 2 years of follow-up is the preferred therapy.     

Über die Behandlung idiopathischer Ödeme mit Bromocriptin

Zusammenfassung Die Therapie idiopathischer Ödeme mit Diuretica ist problematisch, da sie die Symptome nicht beseitigt, sondern einen sekundären Hyperaldosteronismus induziert, der die Ödembildung unterhält. Die Beobachtung einer verminderten Dopaminausscheidung bei Patientinnen mit idiopathischen Ödemen läßt einen Behandlungsversuch mit dem oral wirksamen Dopaminagonisten Bromocriptin als sinnvoll erscheinen.Wir behandelten daher neun Patientinnen mit idiopathischen Ödemen mit typischer Symptomatik und mehrjähriger Anamnese mit 2 × 2,5 mg/die Bromocriptin (Pravidel®). Der Therapieerfolg wurde nach der Normalisierung der circadianen Gewichtsschwankungen und dem subjektiven Befinden beurteilt. Sieben Patientinnen zeigten einen guten, eine nur einen unsicheren Therapieerfolg, und eine Patientin brach die Therapie wegen Nausea ab.Vor und während Bromocriptinbehandlung sind Serumelektrolyte, Blutdruck, Plasmareninaktivität und Aldosteron normal. Bromocriptin normalisiert die circadianen Gewichtsschwankungen, ohne das morgendliche Körpergewicht zu senken. Die Ergebnisse der vorliegenden Pilotstudie legen nahe, daß Bromocriptin bei einigen Patientinnen mit idiopathischen Ödemen eine wirksame Alternative zur herkömmlichen diuretischen Therapie sein kann. Es bleibt offen, ob die Bromocriptinwirkung durch einen Dopaminmangel zu erklären ist, oder ob Bromocriptin eine rein symptomatische Behandlung darstellt.     

Association of dopamine D3-receptor gene variants with neuroleptic induced akathisia in schizophrenic patients: a generalization of Steen's study on DRD3 and tardive dyskinesia

Neuroleptic induced akathisia is a common and distressful extrapyramidal side effect of antipsychotic treatment. A significant proportion of the variability of its development has been left unexplained and has to be attributed to individual susceptibility. Since hereditary factors have been discussed in the etiology of acute akathisia (AA), part of the individual susceptibility might be of genetic origin. Moreover, AA is regarded as a forerunner of tardive dyskinesia, a drug-induced chronic movement disorder, which may be associated with homozygosity for the Ser9Gly variant of the DRD3 gene. Considering expression studies, which demonstrated functional variants of DRD3 polymorphisms, we investigated whether homozygosity for the Ser9Gly variant of the DRD3 gene is associated with AA. Homozygosity for the Ser9Gly variant of the DRD3 gene was connected to an 88% incidence of AA as compared with a considerably lower 46.9% incidence of AA in schizophrenic patients nonhomozygous for the 2-2 allele (exact P = 0.0223). Am. J. Med. Genet. (Neuropsychiatr. Genet.) 96:187-191, 2000.     

Naturally occurring ABO antibodies: long-term stable, individually distinct anti-A IgG spectrotypes.

Spectrotypes of naturally occurring IgG antibodies to histo-blood group substance A were analyzed by isoelectric focusing and a newly developed, non-radioactive affinity immunoblotting assay. Specificity was tested by preabsorption of sera on red blood cells and on four different types of blood group substance A. Clonotype-banding patterns of sera from 18 different blood donors were individually distinct, most of them showing an oligoclonal pattern with less than 15 visible bands. To analyze the influence of IgG subclass composition on spectrotype patterns, IgG subclasses were measured by ABO enzyme-linked immunosorbent assay (ELISA) and were shown to be mostly IgG1 and IgG2. Anti-A IgG1 spectrotypes could be detected with four sera and were also individually distinct. A series of six and seven serum samples, respectively from two donors was used to analyze the time-related changes of anti-A IgG spectrotypes. Banding pattern of both individuals remained stable during the whole observation period of 167 and 82 days, respectively, and no differences between follow-up samples could be detected. This long-term stability of individual anti-A IgG spectrotypes is backed by constant serum levels of anti-A IgG and -IgM antibodies during the observation period as measured by ABO ELISA.     

In vivo release of cisplatin from a needle-type copolymer formulation implanted in rat kidney

Cisplatin, cis-dichlorodiamine platinum (II), was incorporated in a needle-type copolymer formulation (0.8 mm diameter, 6 mm long) by radiation-induced polymerization. The copolymer used was copoly(diethylene glycol dimethacrylate/polyethylene glycol #600 dimethacrylate, 80/20 vol%). This copolymer, containing 6 mg of cisplatin, was implanted into the kidney of adult male Wistar rats (420 +/- 20 g). A total of 70 d was required for 100% release of cisplatin in vivo. The kidney tissue surrounding the formulation was strongly necrotized by the action of cisplatin. Two layers of necrosis could be distinguished: necrotic tissue surrounding the formulation and necrobiotic tissue surrounding the necrotic tissue. The amount of necrotic tissue changed markedly over time, but no change was apparent in the amount of necrobiotic tissue. The maximal amounts of necrotized tissue were observed 14 d after implantation: 3100 microns and 600 microns thick for the necrotic and necrobiotic tissues, respectively.     

Continuous and stepwise cystometry through suprapubic catheters--effect of infusion pattern and infusion rate on the cystometrogram of the normal human bladder

Continuous cystometry at two filling rates (50 and 100 ml min-1) and stepwise cystometry (successive rapid volume infusions followed by bladder wall relaxation) were performed in 12 healthy subjects. Suprapubic catheters were used for infusion and recording of perivesical and intravesical pressures. The continuous cystometrograms obtained at filling rates of 50 and 100 ml min-1, respectively, did not differ with respect to desire to void, transmural pressure increase or bladder capacity. Stepwise cystometry allowed the bladders to be filled to a slightly larger volume than during continuous cystometry, but with comparatively lower transmural pressures only at very large distension of the bladder. There was considerable inter-individual variation in transmural pressure at both continuous and stepwise cystometry. Stepwise cystometry did not appear to provide any important additional information about pressure-volume relationship in the normal human bladder than could be obtained at routine clinical cystometry.     

The painful diabetic shoulder

Different types of shoulder affection were studied in 62 diabetic patients with shoulder pain. Three groups of shoulder joint disorder were found: painful shoulder with restricted mobility (62%), tendinitis without mobility restriction (27%), and a small group with mixed diagnoses. Sixty per cent had hand symptoms and 38% had restricted mobility of their hip joints. High frequencies of retinopathy and neuropathy were found. Affection of the shoulder joint was seen with almost the same frequency in insulin-dependent as in non-insulin-dependent patients, but after a shorter duration of diabetes in the latter. A group of patients with the triad shoulder pain, hand symptoms and restricted mobility of the hip joints had a significantly higher frequency of proliferative retinopathy than patients with shoulder pain only. The long duration of diabetes, the high frequency of insulin treatment and classical late complications indicate that diabetic patients with painful shoulder and restricted mobility are suffering from clinically advanced diabetes mellitus.