A description of eating disorders in 1932

This article consists of a shortened and annotated translation of a paper on "An Interesting Oral Symptom Complex and Its Relationship to Addiction" by M. Wulff of Berlin, which was delivered before the German Psychoanalytic Society on April 12, 1932. The article describes four cases of an eating disorder in women that was characterized by binge eating, hypersomnolence, apathetic depression, and disparagement of the body image. After the eating binges, all four patients manifested periods of fasting, and two of the three, about whom such information is available, vomited. Translations of large excerpts of the case histories are presented together with a summary of Wulff's discussion.     

Transient osteoporosis: transient bone marrow edema?

Ten patients with debilitating hip or knee pain were examined with magnetic resonance (MR) imaging. All had conventional radiographs that were either normal or showed nonspecific osteopenia. Nine patients had bone scintigrams that showed focal increased radionuclide uptake in the region of the painful joint. In each case, MR images of the affected joint showed regional decreased signal intensity of the bone marrow on T1-weighted images and increased signal intensity on T2-weighted images. Biopsy results of four patients excluded ischemic necrosis and metastases. The symptoms resolved spontaneously in all cases. The ten patients were followed up for 12-36 months, and there were no recurrences. The authors believe that the findings on MR images represent a transient increase in bone marrow water content. The focal findings on scintigrams confirmed the periarticular distribution of the process and provided evidence of accompanying hyperemia and increased bone mineral metabolism. For lack of a better term and to emphasize the generic character of the condition, the authors termed this condition "the transient marrow edema syndrome."     

Very low calorie diets: their efficacy, safety, and future

Very low calorie diets used to treat moderate and severe obesity produce average weight losses of 20 kg in 12 weeks. This paper reviews the development of very low calorie diets from research on fasting in the late 1950s and examines data on the amount of dietary protein needed to achieve positive nitrogen balance. The desirability of including carbohydrate in the diet, the choice of protein sources (formula versus animal protein), and the anorectic value of ketosis are discussed, as are patient selection and the clinical course of the diet. As contrasted to the earlier "liquid protein" diets that were associated with at least 60 deaths, very low calorie diets of high-quality protein appear safe when limited to 3 months or less under careful medical supervision. Evidence of this safety is provided by the results of 24-hour Holter monitoring and the fact that no diet-related fatalities have been reported in over 10 000 cases. The major problem to be resolved is the maintenance of the large weight losses achieved with these diets.     

Hyaline membrane disease and intraventricular haemorrhage in small for gestational age infants

19 small for gestational age (SGA) infants with gestational ages less than or equal to 32 weeks were matched with 19 appropriate for gestational age (AGA) preterm neonates with similar risk factors for intraventricular haemorrhage and hyaline membrane disease. Gestational age, 1- and 5-minute Apgar scores, type of delivery, survival rate, use of corticosteroids before delivery, sex, twinning, presence of premature rupture of membranes, and birth date were comparable in the two groups. Gestational age of both groups was 30 (+/- 1.8) weeks, and birthweights were 919 (+/- 202) g (SGA group) and 1268 (+/- 212) g (AGA group). The incidences of hyaline membrane disease and intraventricular haemorrhage were different: 74 and 42% respectively for AGA neonates, 5 and 11% respectively for SGA infants. We suggest that a stressful environment in utero may enhance maturation and prevent hyaline membrane disease and intraventricular haemorrhage.     

Pseudohypoparathyroidism. Variable manifestations within a family

A wide variety of clinical and biochemical manifestations have been reported in association with pseudohypoparathyroidism and associated disorders. This is illustrated by a family study in which the affected members show widely differing characteristics.     

Defect of platelet function associated with chronic hypoglycaemia

Two patients are described with chronic hypoglycaemia; the first having glucose-6-phosphatase deficiency (type I glycogen storage disease), and the second fructose 1:6-diphosphatase deficiency. Both cases were associated with a bleeding diathesis, a defect of platelet aggregation, and a deficiency of platelet adenine nucleotides. The effect on the platelet abnormalities of a period of normoglycaemia was studied in both patients. Correction of the platelet abnormalities occurred rapidly after stabilization of the blood glucose within the normal range. Normal function persisted for the duration of the normoglycaemia, facilitating diagnostic liver biopsy and surgical procedures. A biochemical explanation for the nucleotide deficiency is suggested.     

Recombinant human acid alpha-glucosidase: high level production in mouse milk, biochemical characteristics, correction of enzyme deficiency in GSDII KO mice

Glycogen storage disease type II (GSDII) is caused by lysosomal acid alpha-glucosidase deficiency. Patients have a rapidly fatal or slowly progressive impairment of muscle function. Enzyme replacement therapy is under investigation. For large-scale, cost-effective production of recombinant human acid alpha-glucosidase in the milk of transgenic animals, we have fused the human acid alpha-glucosidase gene to 6.3 kb of the bovine alphaS1-casein gene promoter and have tested the performance of this transgene in mice. The highest production level reached was 2 mg/ml. The major fraction of the purified recombinant enzyme has a molecular mass of 110 kDa and resembles the natural acid alpha-glucosidase precursor from human urine and the recombinant precursor secreted by CHO cells, with respect to pH optimum, Km, Vmax, N-terminal amino acid sequence and glycosylation pattern. The therapeutic potential of the recombinant enzyme produced in milk is demonstrated in vitro and in vivo. The precursor is taken up in a mannose 6-phosphate receptor-dependent manner by cultured fibroblasts, is converted to mature enzyme of 76 kDa and depletes the glycogen deposit in fibroblasts of patients. When injected intravenously, the milk enzyme corrects the acid alpha-glucosidase deficiency in heart and skeletal muscle of GSDII knockout mice.      

Potent expansion of human natural killer T cells using alpha-galactosylceramide (KRN7000)-loaded monocyte-derived dendritic cells, cultured in the presence of IL-7 and IL-15

Natural killer T (NKT) cells have an extremely restricted T-cell receptor repertoire, in man consisting of a Valpha24 chain preferentially paired with a Vbeta11 chain, and play crucial roles in various immune responses. Characterization of circulating Valpha24(+)Vbeta11(+)-T cells is hampered by their low frequencies. The alpha-galactosylceramide KRN7000 was reported to be presented by CD1d to NKT cells. Since dendritic cells (DC) are potent antigen presenting cells, and have been shown to express CD1d, we analyzed whether these cells could efficiently mediate expansion of Valpha24(+)Vbeta11(+)-T cells. During a 7-day co-culture of peripheral blood mononuclear cells and KRN7000-loaded mature monocyte derived DC (moDC) in the presence of interleukin-7 (IL-7) and IL-15, we observed up to 76-fold expansion of Valpha24(+)Vbeta11(+)-T cells. The expanded Valpha24(+)Vbeta11(+)-T cells expressed the cytotoxic molecule granzyme B, showed negligible expression of Fas ligand and could be induced to express high levels of interferon-gamma, while retaining the capacity to produce IL-4. B cells, expressing CD1d, could also present KRN7000, but Valpha24(+)Vbeta11(+)-T cell expansion was only observed in the presence of IL-7 and/or IL-15. Considering the low frequency of circulating Valpha24(+)Vbeta11(+)-T cells, the present method for expansion of Valpha24(+)Vbeta11(+)-T cells using KRN7000-loaded mature moDC will be of value for the further characterization of this unique T cell subset.