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[Aspects of birth and breastfeeding from children with cleft palate].

Cleft lip and palate are the second most congenital malformations--with increasing incidence. Therefore it is even more important for the involved gynaecologist to be informed about the anomaly itself as well as its consequences according to children's nutrition. The aim of this article is to focus on this problem and to present help for breastfeeding and nutrition with human milk.

Breast Feeding↗

Experimental study on effects of vitamin B(6) on dexamethason-induced palatal cleft formation in the rat.

OBJECTIVE: To evaluate whether or not administration of vitamin B(6) (vitB(6)) has preventive effects on cleft palate formation. METHODS: On the 14, 15 day of gestation, the pregnant rats were treated with administration of vitB(6) (10 mg/kg weight im) before and simultaneously with dexamethason (DEX). Compared the palatal cleft incidence of their fetuses with those whose mother were given DEX only. RESULTS: Treatment with administration of vitB(6) before and simultaneously with DEX, the palatal cleft incidence of subject group fetuses (32.35%) was significantly less than that given DEX only (64.52%). The proportion of the palatal cleft type was also changed significantly. CONCLUSIONS: VitB(6) decrease both the incidence and the severity of cleft palate induced by DEX in rats, which indicates VitB(6) may have preventive effects on cleft palate formation.

Animals↗

Temporal and spatial expression of Pax9 and Sonic hedgehog during development of normal mouse palates and cleft palates in TGF-beta3 null embryos.

Transforming growth factor-beta (TGF-beta3) gene disruption causes cleft secondary palate. Pax9 and Sonic hedgehog (Shh) genes are involved in the patterning of vertebrate embryonic tissues, including the facial skeleton. We investigated the expression of Pax9 and Shh genes during normal mouse palate development and in the developing cleft palates of TGF-beta3 null embryos. Whole mount in situ hybridization was conducted with use of Pax9 and Shh riboprobes for TGF-beta3 null, heterozygous and wild type mice at E12.5-E16.5. Histological analysis was processed by section in situ hybridization. In the wild type, Pax9 and Shh were expressed in the palate between E12.5-E15.5. Shh expression in the secondary palate was restricted to the rugae and the soft palate. Pax9 expression was predominantly in the palatal medial edge between E14.5 and E15.5. These patterns suggest that Shh and Pax9 may have different functions during palate development. In TGF-beta3 null mice, both genes expression patterns in the palate were different to those in wild type mice. In TGF-beta3 null mice, Pax9 expression was much reduced in the palatal medial edge at the critical time of palatal fusion (E14.5-E15.5). Shh expression in the palates of TGF-beta3 null mice was reduced throughout E12.5-E15.5, whilst Shh expression in heterozygous did not appear down regulated compared with the wild type. These results indicate that Pax9 and Shh expression are altered when the TGF-beta3 gene is deleted and suggest that Pax9 and Shh may be involved in the TGF-beta3 regulation of normal palatal fusion.

Animals↗

[Palmoplantar epidermal atrophy with hypokeratosis, dys- and hypotrichosis, hypodontia, enamel and dentin hypoplasia, isolated cleft palate with cleft uvula, strabismus cryptorchism and other anomalies--an undescribed ecto-mesodermal dysplasia?].

A 15-year-old boy had suffered burning of both palms and all fingers during his 2nd year of life. Epitheliazation of the lesions did not occur. In his 6th year, erythema, desquamation and erosion-like alterations appeared spontaneously on the soles of both feet and on the plantar sides of the toes. These alterations were similar to those on the hands. Histologically the lesion on the right sole was covered with a very thin horny layer and thin epidermis. The cells of the epidermis had clear cytoplasm and probably derived from the ductal epithelium of the sweat glands. Under the atrophic epidermis, granulation tissue consisting of lymphocytes, plasma cells, histiocytes, and a few polynuclear cells and eosinophils was seen. The following symptoms were still present in the boy: dys- and hypotrichosis, oligodontia, enamel and dentin hypoplasia, cleft palate and uvula, concomitants strabismus (left eye), and cryptorchidism. Transplantation of the patient's skin from the right thigh to the lesion on the right sole was tried. The authors postulate a previously unreported form of ecto-mesodermal dysplasia in this case.

Abnormalities, Multiple↗

Sensitive stages and dose-response analyses of palatal slit and cleft palate in C57BL/6 mice treated with a glucocorticoid.

C57BL/6 mice were treated subcutaneously with triamcinolone acetonide in a single dose of 2.5-15.0 mg/kg once on days 6-15 of pregnancy (vaginal plug = day 0) and the palate of their fetuses was examined at term. The sensitive stages of palatal slit and cleft palate induction were studied and dose-response relations on days 9 and 12 of pregnancy were analyzed by the log-probit transformation method and compared. The test of significant increase in the frequencies of palatal slit and cleft palate showed that the sensitive period of palatal slit began earlier and ended later than that of cleft palate. On day 9 of pregnancy, the slope of the palatal slit response was similar to that of the cleft palate response, but the median effective dose of the former was lower than that of the latter. The slope of the palatal slit response was similar on days 9 and 12; however, the median effective dose was significantly greater on day 9. By contrast, the slope of the cleft palate response on day 12 was significantly different from that on day 9. The results of these dose-response analyses suggest that the underlying mechanism may be the same in palatal slit induction on days 9 and 12 and in cleft palate induction on day 9; the mechanism underlying the cleft palate response on day 12 is different from that of the other three responses, and there is more than one mechanism for cleft palate induced by triamcinolone.

Animals↗

Combination therapy with folic acid and methionine in the prevention of retinoic acid-induced cleft palate in mice.

BACKGROUND: During formation of the secondary palate, clefting may result when critical developmental events are altered. The purpose of this study was to reduce the incidence of retinoic acid (RA)-induced cleft palate (CP) in mice by the co-administration of folic acid (FA), methionine (ME) or a combination of both. METHODS: Four groups of time-pregnant Swiss Webster mice were injected intraperitoneally with 50 mg/kg RA on gestational day (GD) 10. Likewise, 4.0 mg/kg FA and 187 mg/kg ME were administered on GD 8-11. The experiment included a control group (RA plus H2O) and three experimental groups, (RA plus therapeutic doses of FA, ME, or FA + ME). Necropsies were carried out on GD 18 and pups were analyzed for teratogenic effects. RESULTS: Litters that received no therapy exhibited 100% CP with individual pups showing 76% susceptibility. Each therapy administered separately reduced the frequency of CP to approximately 6%, and the combination of FA and ME completely prevented the occurrence of RA-induced cleft palate (0%). A second experiment was conducted in which therapy levels were decreased by 25%. Litters that did not receive therapy experienced 100% clefting and individual pups exhibited CP at 86%. These therapies administered separately did not alter significantly the frequency of cleft palate. The combined doses of FA and ME, however, lowered significantly the frequency of cleft palate to 46%. Decreases in limb and tail defects with FA + ME therapy were also observed in both experiments. CONCLUSIONS: Although FA and ME, at appropriate levels, can reduce individually the frequency of RA-induced cleft palate and other defects in mice, the results from the present study suggest that there is an additive interaction between the two therapeutic agents that can reduce further the teratogenic impact of RA. Further studies are needed to assess the mechanism of action of concomitant doses of FA and ME in the reduction of drug-induced birth defects.

Abnormalities, Drug-Induced↗

Birth weight and gestational age of newborns with cleft lip with or without cleft palate and with isolated cleft palate.

The birth weight and gestational age of 1368 newborns with isolated cleft lip with or without cleft palate and 582 with isolated cleft palate were compared to those of matched healthy controls. The results indicate that fetuses with oral clefts are at elevated risk of having low and very low birth weight, but not of having a premature birth. Speculations on a relationship between these findings and the presence of oral clefts are presented.

Adult↗

Short stay after cleft palate surgery.

Although algorithms for the repair of soft and hard palatal clefts continue to be debated, the appropriate length of postoperative stay has not yet been defined. Recent reports of cleft palate repair advocate a 2- to 5-day hospitalization. The plastic surgery service at St. Joseph Hospital frequently uses same-day admission with 23-hour observation postoperatively, with no increase in complications from the reported 2- to 5-day stay. The authors inspected the records for all the cleft palate patients undergoing cleft repair at St. Joseph Hospital Cleft Clinic from August of 1988 through June of 1998. After excluding syndromic patients and secondary or revision surgical cases, 79 patients remained in the study. These 79 patients underwent 104 procedures; all procedures were performed by a single surgeon (E.D.C.) with resident assistance. Short-term morbidity, length of stay, and operation performed were studied. All patients were admitted the day of surgery. Mean age at the time of operation was 13.2 months, with a range of 6 months to 20 years. The length of operation averaged 1 hour and 37 minutes; 94 percent of patients stayed 24 hours or less postoperatively, and 97 percent stayed 36 hours or less. The longest stay was 72 hours, which was related to delay in resuming adequate oral intake. The overall complication rate was 3.8 percent for this cohort, which included two partial palatal dehiscences and two small fistulas. No blood transfusions were needed, and no infections were noted postoperatively. No patients required readmission postoperatively for bleeding, respiratory compromise, or inadequate oral intake. The authors do not advocate a 1-night stay for all cleft palate cases. However, they do think it is safe for a healthy group of patients undergoing routine cleft palate surgery. The decision to discharge a patient early must always be left to the treating physician.

Adolescent↗

[Orthodontic treatment in a cleft palate-cleft lip case with severe maxillary collapse].

The cleft lip and palate cases are the most frequently seen congenital deformities. Retardation of the maxillary development and collapse are the most significant characteristics in these cases. It is aimed to correct the functions like mastication, deglutition and speech which were disturbed due to anatomic deficiency and to obtain an acceptable occlusion and esthetics, by means of the therapy. As much as the preoperative orthopedic procedures applied just after the birth, the orthopedic and orthodontic appliances used in mixed and permanent dentition periods affect the prognosis in these cases. The purpose of the treatment in permanent dentition is to correct the malocclusion and to prepare the dentition to prosthetic restorations. In this article the orthodontic treatment of a case in permanent dentition, with cleft palate and lip characterized with severe maxillary collapse will be presented.

Adolescent↗

Bony or connective tissue union of operated experimental palatal clefts: implications on growth of the dentomaxillary complex. A biometrical study in the domestic cat.

Operated human cleft palates heal with formation of bone and/or connective tissue in the mended defect (Prydsö, Holm, Dahl & Fogh-Andersen, 1974; Enemark & Jörgensen, 1978). In domestic cats with experimental palatal clefts, reparation by hard or soft tissue appears to be conditioned by the type of palatoplasty applied (Freng, 1979a; 1979b;). Both tissue responses led to reduced transversal growth of the maxillary base (Freng, 1979c; 1981). As regards the concurrent dental arch growth, however, no difference from unoperated controls was found in individuals with bone postoperatively bridging the palatal mid-line (Freng & Voss, 1982). A submucous cleft led to a significant reduction in transversally directed arch development (Voss & Freng, 1982). The present study aimed to find out if these observations could be due to a relatively smaller width of the maxillary base in operated cleft palates healing with connective tissue. However, no significant difference in maxillary base width appeared when the two groups were compared. To some extent, the effect of the cat's scissor bite, on the dental occlusal relationship, may simulate the effect of orthodontic appliances in humans by transmitting mandibulary growth into laterally directed movement of the upper jaw's teeth. The difference in dental arch growth between the two groups might therefore be interpreted either as expressing a better preservation of obtained compensatory transversal maxillary teeth movements in palates healing with bone bridges, or a tendency for the upper dental arch to collapse in cases with submucous mid-palatal clefts.

Animals↗