The "falcifolia" syndrome of Oenothera. First communication

Summary Reciprocal crosses between Oe. lamarckiana and Oe. suaveolens lead to an off-spring, part of which is crippled. According to Harte 1948 this characteristic malformation is called falcifolia (sickle-shaped leaf). It is restricted to certain sectors of the plant and appears in various plants at different stages of their development. For this reason the percentage of falcifolia-plants increases gradually during the vegetative period. Reciprocal crosses show a clear quantitative difference in regard to the percentage of crippled plants as well as to the degree of crippling. At maturity the percentage of falcifolia-plants is approximately as follows: Oe. lamarckianaxOe. suaveolens: 90–100% Oe. suaveolensxOe. lamarckiana: 20–40% Selfing of normal and crippled hybrids leads to an F₂ which consists of both types; the percentage varies. In general a crippled plant produces more falcifolia-progeny than a normal plant. Backcrossing with Oe. lamarckiana leads to an offspring with less than 20% falcifolia whilst backcrossing with Oe. suaveolens produces more than 50% crippled plants. This large difference appears in every backcross, regardless whether the F₁-plant is normal or crippled, and whether it functions as seed-parent or pollen-parent. Furthermore it does not matter whether the F₁ has been obtained with Oe. lamarckiana or with Oe. suaveolens as seed-parent. The peculiar inheritance appears to be independent of nuclear and plastid genes (genome and plastome), but is in fact a non-mendelian one. Falcifolia cannot be produced by grafting Oe. lamarckiana and Oe. suaveolens reciprocally. It seems to be caused by the interaction of different plasmotypes.

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Herrn Professor Dr. Dr. h. c. Friedrich Oehlkers zum 80. Geburtstag gewidmet.     

Sinus bradycardia in chronic neuroglycopenic syndrome. A case of "short intestine" syndrome

The clinical pattern of the chronic hypoglycemic disorders is different from that of the acute disorders. While in acute hypoglycemic syndromes tachycardia usually occurs, in chronic hypoglycemic disorders there is, even if seldom, sinusal bradycardia. The bradycardia occurs after months with glycemia under 60 mg/dl and may be correlated to the neuroglycopenic state. It is easily reversible when plasma glucose concentration approaches the normal range again. Recently we observed sinusal bradycardia set up during the chronic hypoglycemia state, lasting from more than 5 months, in a man submitted to subtotal intestinal resection, owing to wide extensive eosinophilic granuloma. The bradycardia disappeared quickly after the correction of the hypoglycemia, when the patient was submitted to parenteral hyperalimentation with a silicone rubber catheter in the superior vena cava. We suggest to set the bradycardia among the markers of undernutritional syndromes beside the known anthropometric, biochemical and immunological signs.     

New "pre-diabetes" category and the metabolic syndrome in Japanese.

BACKGROUND: Recently, impaired fasting glucose (IFG) was redefined as fasting plasma glucose of 100-125 mg/dl, and individuals with IFG and/or impaired glucose tolerance (IGT) were referred to as having "pre-diabetes". However, there is a lack of data using the new definition of IFG and "pre-diabetes". OBJECTIVE: The aim of this study was to examine associations of the metabolic syndrome components with the new "pre-diabetes" category in relatively lean Japanese. METHODS: Six hundred and sixty-one Japanese study participants underwent a 75 g oral glucose tolerance test. They were classified into three groups-normal (n=225), pre-diabetes (n=308), and diabetes (n=128). The metabolic syndrome was defined according to the National Cholesterol Education Program Adult Treatment Panel III, as modified for waist circumference criteria by the Regional Office for the Western Pacific Region of WHO. RESULTS: Prevalence of the metabolic syndrome in each group was 10.7%, 27.9%, and 53.9%, respectively. Of the metabolic syndrome components, the OR for prevalent pre-diabetes was 2.00 (95% CI, 1.73-2.31, p<0.001) for fasting glucose, 1.93 (95% CI, 1.54-2.42, p<0.001) for waist circumference, and 1.36 (95% CI, 1.10-1.68, p=0.005) for triglycerides. Similar associations were found in prevalent diabetes. Insulin resistance assessed using Stumvoll's index was significantly associated with both pre-diabetes and diabetes. CONCLUSION: Pre-diabetes and the metabolic syndrome frequently coexist in relatively lean Japanese. This association seems to link with abdominal adiposity and insulin resistance.     

The role of Branhamella catarrhalis in the "bloody-nose syndrome" of cynomolgus macaques.

During a 15-month period, 25 cynomolgus macaques (Macaca fascicularis) at the Johns Hopkins University were observed to have nasal discharge. Fifteen (60%) of these animals had positive nasal cultures for Branhamella catarrhalis. Clinical signs associated with infection by this bacterium were sneezing, epistaxis, and mucohemorrhagic nasal discharge. Treatment with antibiotics resulted in prompt resolution of clinical signs. Post-therapeutic nasal cultures were negative for B. catarrhalis. Two groups of clinically normal, culture-negative, cynomolgus macaques were inoculated with natural isolates of B. catarrhalis which had been passaged in culture for various amounts of time. Five of the eight animals inoculated became culture-positive and had mild nasal discharge. Presence of blood on nasal swabs was indicative of infection with B. catarrhalis. Three of the inoculated animals had post-swabbing epistaxis. This report documents the role of B. catarrhalis as an upper respiratory pathogen in the cynomolgus monkey which causes mild self-limiting disease reminiscent of the so-called "Bloody-Nose Syndrome." In addition to the obvious clinical significance of this finding to primate clinicians, development of an animal model for human disease caused by this organism may be possible.     

Galvanized caging as a potential factor in the development of the "fading infant" or "white monkey" syndrome.

Four rhesus females and their infants were kept in galvanized enclosures, and three were kept in stainless steel caging. All four offspring in the galvanized enclosures developed achromotrichia, alopecia, and weakness that varied from modererate to severe while the three infants in stainless steel cages were clinically normal. Plasma copper, zinc, and iron values as well as liver copper and zinc values of the infants were compared for the two types of caging. Plasma copper values were significantly lower (p less than 0.001) in the animals housed in galvanized cages. Plasma zinc and liver zinc levels were significantly elevated in these same animals (p less than 0.01 and p less than 0.05, respectively). Significant differences were not detected in liver copper values in the two groups of infants. Copper and zinc levels in the dams' plasma and milk were not statistically different between the two groups.     

The "lazy" NK cells of Chediak-Higashi syndrome

Natural killer (NK) function, measured in a short-term (4-hr) 51Cr-release assay, is profoundly depressed in circulating PBL of donors with Chediak-Higashi syndrome (CHS). In this study, we demonstrate that CHS NK cells can express relatively normal lytic function after prolonged exposure in vitro to high levels of activating as well as cytotoxic stimuli. After activation with the human cloned interferon (B1) for 24 hr, CHS NK cells have lytic activity comparable to unactivated normals in a 4-hr 51Cr-release assay. In addition, after 5 days of activation with mitomycin C-treated B cell lines, CHS NK cells have levels of activity similar to those of activated normals but are defective in generating cytotoxic cells capable of lysing the stimulator B cell. Even though CHS NK cells are defective in a 4-hr 51Cr-release assay, after 16 hr they enhance their killing capability 200 to 400-fold. In fact, after 16 hr of interaction with K562 target cells, CHS NK cells are capable of releasing NK soluble cytotoxic factors. These results are consistent with the hypothesis that CHS NK cells have all the necessary cellular structures and molecules required for them to function as lytic effector cells, but their lack of cytotoxic function is due to a relative refractoriness in initiating the post-binding lytic mechanism.     

A female with Coffin-Lowry syndrome and "cataplexy"

Coffin-Lowry syndrome (CLS) is an X-linked semidominant condition, caused by mutations in the gene encoding the ribosomal protein S6 kinase-2 (RSK-2), a growth factor regulating protein kinase, which is mapped to Xp 22.2. The syndrome is mainly seen in males. It is manifested by moderate to severe mental retardation and characteristic facial, hand and skeletal malformations. We present a female patient with fully manifested CLS, confirmed by molecular analysis, who experienced daily drop episodes, diagnosed as "cataplexy". The episodes were precipitated by emotional or auditory stimuli and were significantly reduced, by selective serotonine re-uptake inhibitors.     

The myelodysplastic syndrome: prognostic factors in relation to the "in vitro" clonogenic assay.

The object of this study was to determine whether the "in vitro" parameters of medullary and blood granulopoiesis in patients with MDS, furnish information of either prognostic or diagnostic value. This study covered 94 patients with MDS. All patients were studied at the onset of disease. In order to identify the factors related to patients' survival, Cox Multiple Regression analysis was performed by the BMD P2L program. When analyzing by means of actuarial curves the survival probability of patients with benign development versus those of malignant development (those who developed ANLL), the significance between both groups was p = 0.0001. Different variables of patients included in this study were analyzed and all showed great significances. Fab: p = 0.0022, disease evolution: p = 0.0001 and presence of blastic aggregates: p = 0.0011. Cox's regression analysis revealed that the only predictable survival variable is the presence of blastic colonies and/or clusters. Accordingly, two groups were constructed: favourable and unfavourable. In the favourable group, 40% of the patients belonged to the RA group, while in the unfavourable group, 55% belonged to the RAEB group. This study shows the validity of the elaboration of prognostic groups in MDS according to the presence of blastic colonies and/or clusters in CFUGM medullary and/or peripheral cultures. The "in vitro" myeloid progenitors culture techniques may therefore be advantageously applied in these disorders for formulating a diagnosis and predicting the patient's short term evolution.     

A new sublethal syndrome with multiple malformations associating diaphragmatic hernia, distal digital hypoplasia, and craniofacial anomalies. The "Fryns syndrome"

Prevalence, Clinical variability, Etiology, Survival and Prenatal Diagnosis--In this report, we summarize the actual data on the Fryns syndrome, a true MCA/MR syndrome with autosomal recessive inheritance and sublethal outcome. In addition to the diagnostic triad of diaphragmatic hernia--digital limb hypoplasia--coarse facies, multiple internal malformations are a constant feature.     

Neurochemical investigation of an endogenous model of the "hyperkinetic syndrome" in a hybrid dog.

A telomian-beagle hybrid has been recently proposed as a possible model for the hyperkinetic syndrome. They resemble hyperkinetic children in their poor ability to respond with the appropriate behavior in an inhibitory training test. Two groups of hybrids could be differentiated, the behavior of one of which improved with amphetamine (“Responders”) and of the other did not (“Non responders”). In the present study, the levels of HVA, 5-HIAA, MOPEG-SO were measured in CSF and the levels of NA, DA, HVA, DOPAC⁴, 5-HIAA were assayed in brain tissues from different regions, taken under basal conditions from beagles and telomian-beagle hybrids. Responder hybrids had lower levels of NA, DA, HVA in brain and low HVA in CSF. Therefore, they can be distinguished biochemically as well as behaviorally from non-responder hybrids and beagles and may prove to be useful as models for study of the mechanism and the therapy of this syndrome.