Spirocyclopropanated bicyclopropylidenes: straightforward preparation, physical properites, and chemical transformations.

Perspirocyclopropanated bicyclopropylidene (6) was prepared in three steps from 7-cyclopropylidenedispiro[2.0.2.1]heptane (4) (24% overall) or, more efficiently, through dehalogenative coupling of 7,7-dibromo[3]triangulane (15) (82%). This type of reductive dimerization turned out to be successful for the synthesis of (E)- and (Z)-bis(spiropentylidene) 14 (67%) and even of the "third-generation" spirocyclopropanated bicyclopropylidene 17 (17% overall from 15). Whereas the parent bicyclopropylidene 1 dimerized at 180 degrees C to yield [4]rotane, dimerization of 6 at 130 degrees C under 10 kbar pressure occured only with opening of one three-membered ring to yield the polyspirocyclopropanated (cyclopropylidene)cyclopentane derivative 19 (34% yield), and at the elevated temperature the polyspirocyclopropanated 2-cyclopropylidene[3.2.2]propellane derivative 20 (25 % yield). Perspirocyclopropanated bicyclopropylidene 6 and the "third-generation" bicyclopropylidene 17 gave addition of bromine, hydrogen bromide, and various dihalocarbenes without rearrangement. The functionally substituted branched [7]triangulane 28 and branched dichloro-C2v-[15]triangulane 32 were used to prepare the perspirocyclopropanated [3]rotane (D3h-[10]triangulane) 49 (six steps from 6, 1.4% overall yield) and the C2v-[15]triangulane 51 (two steps from 17, 41% overall). Upon catalytic hydrogenation, the perspirocyclopropanated bicyclopropylidene 6 yielded 7,7'-bis(dispiro[2.0.2.]-heptyl) (52) and, under more forcing conditions, 1,1'-bis(2,2,3,3-tetramethylcyclopropyl) (53). The bromofluorocarbene adduct 33 of 17 reacted with butyllithium to give the unexpected polyspirocyclopropanated 1,4-di-n-butyl-2-cyclopropylidenebicyclo[2.2.0]hexane derivative 37 as the main product (55% yield) along with the expected "third-generation" perspirocyclopropanated dicyclopropylidenemethane 38 (21% yield). Mechanistic aspects of this and the other unusual reactions are discussed. The structures of all new unusual hydrocarbons were proven by X-ray crystal structure analyses, and the most interesting structural and crystal packing features are presented.

[Halitosis in two children caused by a foreign body in the nose]. / Halitosis door een corpus alienum in de neus bij 2 kinderen.

In two boys aged 4.5 and 1.5 years with halitosis a nasal foreign body was found. After removal the foetor disappeared. Little is known about the epidemiology of halitosis in children. Apart from specific odours with certain systemic disorders, local pathology such as chronic sinusitis, upper and lower respiratory tract infections and to a lesser degree gastrointestinal disorders may be the cause of the offensive smell. As in adults, bad breath in children is usually related to poor oral hygiene or disease of the oral cavity. The first-line treatment is proper oral hygiene and if necessary dental sanitization. In resistant cases further evaluation should be aimed at disclosing the causative pathology, in which case in children the possibility of a nasal foreign body should also be considered.

Tricyclopropylamine and Its Radical Cation.

A surprisingly high first vertical ionization energy (8.44 eV) and an unusually high oxidation potential are exibited by tricyclopropylamine (1)-in sharp contrast to triisopropylamine. These are attributed to the near-tetrahedral geometry of 1 at the N atom and the perpendicular orientation-with respect to the N-orbital axis-of the cyclopropyl groups. gamma-Irradiation of 1 led to the radical cation 1(.)(+), which, in accord with computations, has a planar C(3h)-symmetrical structure. The EPR-spectroscopic and computational results disclose a dramatic, previously not reported, conformational change on going from 1 to 1(.)(+).

The jaw index: new guide defining micrognathia in newborns.

OBJECTIVE: Newborns with glossoptosis due to micrognathia can suffer from life-threatening respiratory distress and severe feeding difficulties. These characteristic features are found in newborns with Pierre Robin sequence. In the relevant literature, no concensus concerning diagnosis for these children can be found. A guide defining micrognathia can be helpful in the diagnosis of glossoptosis and possible airway obstruction. METHOD: The jaw index is obtained in newborns by measuring three facial dimensions. In a series of 100 healthy neonates the standard measures were defined. RESULTS: The average jaw index in children with Pierre Robin sequence differs significantly from the reference group and was found to be over 3.6 times the normal value. CONCLUSIONS: Micrognathia can be defined with the jaw index.

[Ureterocele in children]. / Ureterokèles bij kinderen.

Two case reports of children with ectopic ureteroceles are presented. The embryologic development, symptoms, diagnosis and treatment are discussed. Hemi-nephroureterectomy is the treatment of choice. In instances, such as serious urinary tract infections, massive vesicoureteral reflux in other ureters and in single system ureteroceles, endoscopic incision of the ureterocele can be effective in terms of control of infection and decompression of other kidney parts. Definite surgery can be deferred until the child is in a better condition.

[A child with Wegener's granulomatosis]. / Een kind met Wegener's granulomatosis.

A 13-year old boy is described with painful joints, papular skinlaesions, malaise and haematuria. Impaired renal function and hypertension were found. In the past he frequently suffered of otitis media. Biopsies of kidney and skin lesions showed a vasculitis, furthermore the C-ANCA-titer (anti-neutrophil cytoplasm antibodies) was strongly elevated which justified the diagnosis Wegener's granulomatosis (WG). This disease is seldom seen in childhood. It is a necrotizing vasculitis with granulomas of mostly the respiratory tract and kidneys. Autoantibodies directed against intracellular antigens of neutrophils were first associated with vasculitis in 1982. Since 1988 two ANCA-subtypes have been recognized: C-ANCA and P-ANCA. The C-ANCA appear to be highly specific for diagnosis and follow-up of WG, and to differentiate of other forms of vasculitis (with P-ANCA). If children present with frequent and serious upper respiratory tract infections, with suspicion of a systemic disease it is important to perform the ANCA-test to differentiate between WG and other forms of vasculitis. The patient was treated with prednisone and cyclophosphamide, after which the symptoms and the ANCA-titer decreased.

Value of routine bone marrow examination for detection of bone marrow relapse in children with standard risk acute lymphoblastic leukemia.

The value of routine bone marrow examination (RBME) in children during and after treatment for standard risk acute lymphoblastic leukemia (SR-ALL) was investigated. The clinical symptoms and peripheral blood findings at the time of bone marrow relapse of 28 children were reviewed and compared with those of 28 matched controls in continuous complete remission. Five (45%) children with bone marrow relapse during maintenance therapy and six (35%) after cessation of cytostatic treatment were asymptomatic at the time of relapse. Signs indicative of relapse during treatment were lymphoblast cells in the peripheral blood, thrombocytopenia, hepatomegaly, anemia, or leukopenia in decreasing order of frequency. After cessation of treatment these signs were lymphoblasts in the peripheral blood, hepatomegaly, splenomegaly, thrombocytopenia, or leukocytosis. Except for one case with thrombocytopenia, no signs suspicious for relapse were found in the control groups. When each sign was evaluated separately only the presence of lymphoblasts in peripheral blood and hepatomegaly were significant symptoms for relapse after cessation of treatment. The mean percentage of lymphoblasts in the bone marrow at the time of relapse was significantly lower for patients with an unpredicted relapse (46.8%) than patients with clinical and/or laboratory evidence of relapse (79.5%). When lymphoblasts were present in the peripheral blood the percentage of lymphoblasts in the bone marrow was always more than 40%, both during and after cessation of treatment. These data suggest a relation between clinical and laboratory symptoms and progression of the disease. It is concluded that 46% of relapses are detected by RBME in the absence of clinical or laboratory symptoms. This early detection may have a positive prognostic influence with more effective treatment for relapsed ALL.

[Diagnosis and treatment of mega-ureters in children]. / Diagnose en behandeling van mega-ureters bij kinderen.

In connection with 4 cases of megaureter in children, the diagnosis and the therapy of this condition are described. The term megaureter describes a large wide ureter. It may be associated with reflux or obstruction or be present without reflux and/or obstruction. The diagnosis of obstruction by renography may be difficult because of diminished glomerular filtration rate, either physiological or due to a decreased number of glomeruli, a high compliance of the collecting system, diminished urinary output or a filled bladder. Clear cases of obstruction and/or reflux should be treated operatively. In the asymptomatic child with a primary obstructive megaureter and a good relative function conservative treatment may result in reducing the dilatation without loss of kidney function.