[Early childhood caries].

ECC was defined by the American Academy of pediatric dentistry at 2003 as the presence of 1 or more decayed (noncavitated or cavitated lesions), missing (due to caries), or filled tooth surfaces in any primary tooth in a child 71 months of age or younger. This is a virulent type of dental caries that start soon after the tooth erupts and progress rapidly. The prevalence is 1-12% in developed countries and 70% in developing countries, and changes in different cultures, communities, socioeconomic status, etc. The etiology of the disease is multifactorial like in any dental caries. The risk factors include high levels of SM and LB, enamel defects, oral habits, complication at pregnancy and birth, social and demographic factors and the child's age. The disease implications are: high risk of new caries defects in both permanent and deciduous dentitions, insufficient physical development, hospitalization and emergency room visits, loss of school days and increased days with restricted activity, increased treatment costs and time, diminished ability to learn, and diminished oral health-related quality of life. Due to the aggressive pattern of the disease, treatment should be specific for each individual patient, and should be given by an expert dentist with experience who could manage the young child and the process of the disease. Treatment options are: 1. Conservative approach which includes recalls and topical fluoride, 2. Aggressive restorative approach. In both we should first stop the carious habit and encourage prevention. Before choosing the type of treatment, we should consider the severity of the lesions, child's age, caries risk, child's behavior, and parents' cooperation. Prevention at home includes: 1. decreasing the mother's/primary caregiver's mutans streptococci levels, 2. avoid sharing the same utensils in the family, 3. implementing oral hygiene measures as the first primary tooth erupts, 4. dental home, 5. avoid inappropriate feeding practices of infants and toddlers. Prevention at clinic includes: 1. topical fluoride application, 2. proximal slices in appropriate cases.

Hormone-responsive myofibrillar protease activity in cultured rat myoblasts.

The effect of exposure to dexamethasone and serum-deprivation on myofibrillar protease activity was determined by following cleavage of [14C]globin by isolated myofibrils obtained from rat skeletal muscle in culture. Dexamethasone [10(-7) M] produced a 46% increase in protease activity, and serum-deprivation caused a 50% increase in activity over that of the enzyme in control cultures. The increases in proteolysis occurred concurrently with increased rate of overall protein degradation in these cells and were not associated with changes in cell viability. In cultured rat cardiac muscle cells dexamethasone failed to enhance myofibrillar protease activity, while serum-deprivation produced a 52% increase in the enzyme activity. Addition of insulin (50 mU/ml) to the cultures did not affect proteolysis or myofibrillar protease activity, but completely prevented the dexamethasone-induced increase of these activities. This effect of insulin suggests that the increase of muscle proteolysis in insulin-deficient diabetic animals reflects an enhanced response of the muscle to circulating glucocorticoids rather than a direct effect of insulin-deprivation on muscle proteolysis. Taken together, the present observations indicate that muscle cells in culture retain the ability to respond to catabolic stimuli by adaptive changes in the myofibrillar protease activity in a manner analogous to that of their parent tissue in the intact animal.

Torsion of the spleen with incomplete infarction: case report.

Torsion and infarction of a "wandering spleen" is a rare disease which is often confused with other acute abdominal crises. A correct preoperative diagnosis, when made, has usually been determined by arteriographic studies. A child is described in whom changes in the TcSC scan made a correct diagnosis possible by non-invasive methods.

Rectal mucosal biopsy in aganglionosis and allied conditions.

Rectal mucosal biopsies were performed in 146 patients suspected of having Hirschsprung's disease. The biopsies were serially sectioned and screened for the presence of ganglion cells. In the 101 cases in which ganglion cells were found, the diagnosis of Hirschsprung's disease was ruled out. In 45 patients, histologic examination revealed the typical features of Hirschsprung's disease, i.e., the absence of ganglion cells and the presence of numerous hypertrophied nerve bundles. The varied morphologic features of Meissner's plexus in patients of different ages and the diagnostic pitfalls are described in detail. Serial frozen sections stained with hematoxylin-eosin were examined in eight cases of neonatal intestinal obstruction and enterocolitis. This method was found to be useful and reliable in emergencies. In three of these cases ganglion cells were found and major surgical procedures were avoided. In 73 cases, staining for acetylcholinesterase activity was performed. The results matched the microscopic findings in all but three false-positive cases.

Persistent müllerian structures in infertile male.

Müllerian duct derivatives were identified in an infertile adult male patient who had long-standing azoospermia and was operated on for inguinal hernia. Persistent müllerian duct syndrome is reviewed, with special emphasis on the pathophysiologic and surgical considerations involved in the treatment of this abnormality.

Phenazine methosulfate induces a neurally-mediated contraction of the guinea-pig ileum.

Phenazine methosulfate (PMS) and related phenazines are widely used in biochemistry and histochemistry and act as anti-bacterial agents, however, there is little information on their pharmacological actions. In the present paper the guinea-pig ileum was used as a model for studying the effects of PMS on nerve cells. PMS was found to contract intestinal muscle. This action appeared to be mediated by the activation of muscarinic receptors since it was blocked by atropine. Neostigmine potentiated the response to PMS. The nerve blocker tetrodotoxin prevented the effect of PMS and it is concluded that PMS causes the release of acetylcholine from nerve elements. The action of PMS on nerves is not mediated by nicotinic receptors. Receptors for serotonin, substance P or cholecystokinin also appear not to be involved. Of all the phenazines tested PMS was found to be the most potent and reversible.

Nissen fundoplication in the treatment of children with familial dysautonomia.

Thirty-four children with familial dysautonomia (FD) underwent Nissen fundoplication and gastrostomy. The indications for operation were persistent cyclic vomiting that resulted in repeated aspiration pneumonia (94% of the patients), chronic dehydration (82%), failure to thrive (97%), and frequent hospitalizations (76%). There was no operative or early postoperative mortality. Long-term follow-up for up to 12 years was available. Eight patients died during this period from 7 months to 7.5 years postoperatively. In 5 patients (15% of the operated patients), the fundoplication ceased to function 16 months to 5 years postoperatively, which was attributed mainly to repeated severe dysautonomic crises with vigorous retching. Vomiting ceased in 85% of the symptomatic patients; pulmonary deterioration was halted, and the frequency of aspiration pneumonia was reduced in 68%; nutritional improvement was seen in 44%; the hydration status improved in 88%; and the frequency of hospital admissions decreased in 74%. These long-term findings resulted in a significant improvement in the quality of life for the majority of the patients. The absence of operative mortality and the low postoperative morbidity, together with the long-term beneficial results of this surgical procedure, should encourage early surgical intervention in selected FD patients.

How conservatively can postoperative small bowel obstruction be treated?

Although postoperative adhesion ileus is the most common cause of small bowel obstruction in adults, its management remains controversial. We retrospectively studied 297 admissions of 227 patients over a period of 14 years to evaluate our conservative approach in managing adhesion ileus. We found that nonoperative therapy of up to 5 days' duration can be used safely for the majority of patients who present with postoperative intestinal obstruction, including those with complete obstruction. In those patients, who responded to conservative treatment, the obstruction resolved within a mean of 22 hours and a maximum of 5 days. A trial of more than 5 days' duration proved ineffective. The conservative approach resulted in a 73% resolution of obstruction with no significant increase in mortality or in the rate of strangulated bowel.

Corrosive injury to the stomach due to acid ingestion.

Ingestion of acid often causes severe damage to the stomach. Ingestion of alkali, however, usually injures the esophagus and spares the stomach. Early complications of acid ingestion, e.g., massive gastric hemorrhage or perforation, are unusual. The absence of severe symptoms in most patients soon after ingestion of acid is often misleading. Gastric outlet obstruction is a common late result and may develop following an asymptomatic interval. The authors' experience with three patients with severe complications following ingestion of acid is presented. The initial treatment, as well as the surgical management, are discussed.

Max Sandreczky: a pediatric surgeon in 19th-century Jerusalem.

Until the mid-19th century, there had not been a single resident physician in the Holy Land. However, by the first World War in 1914, 19 hospitals had been established in Jerusalem. The reasons for this unusual phenomenon were religious, political, and sectarian. This does not apply to The Marienstift Kinderhospital, which was unique in 19th century Jerusalem. The hospital was founded and operated by Dr Max Sandreczky, a German pediatric surgeon. The standards of medical and surgical practice in the hospital not only compared favorably with those in western Europe, but, in certain aspects, they were more advanced. The Marienstift Kinderhospital was an "implant" of a western institution in a society that was medieval in character. The response of this society was studied as well as the natural history of this unique institution and its medical director, the pediatric surgeon, Dr Max Sandreczky.

Felix Wurtz: surgeon and pediatrician.

Felix Wurtz, a surgeon in Basel and Zurich, wrote a book on pediatrics, The Childrens Book, which was first published (after his death) in 1612. It is the first known book in western Europe to deal with pediatrics and surgery. The book discusses issues dealing with the care of children, also addressing problems in pediatric surgery, primarily congenital and acquired orthopedic malformations. The book, and the contribution of its writer to pediatric surgery, are reviewed. The accuracy of the first English language translation is also evaluated.

Low anterior resection with a long posterior anorectal myectomy and sphincterectomy for Hirschsprung's disease.

Nine patients with the severe form of Hirschhsprung's Disease (HD) underwent low anterior resection with posterior anal myectomy and sphincterectomy. Good results were achieved in six patients. Three patients had bouts of enterocolitis following the surgical treatment which was attributed to residual spasm of the aganglionic rectum and the internal anal sphincter. Myectomy had to be redone in two patients. One patient developed constipation which responded to toilet training. One patient died at home of an unknown cause, and one patient was lost to follow-up. The different methods of treatment of HD are discussed with emphasis on the role of the internal anal sphincter. The relatively high complication rate in this small group of patients does not justify its use in the severe form of HD.

Demonstration of rectourinary fistulas by pressure gastrografin enema.

Rectourethral and vesical fistulas in patients with high anorectal atresia can be demonstrated by using a pressure Gastrografin enema through the distal colostomy opening. A technique is described where contrast medium is forced by gentle pressure, filling the fistula and the urethra. This picture is of great value during the repair of these anomalies.

Hypoganglionosis of the myenteric plexus with normal Meissner's plexus: a new variant of colonic ganglion cell disorders.

A 16-year-old girl with a history of achalasia of the lower esophagus was investigated for progressive constipation since the age of five years. Normal ganglion cells of the Meissner's plexus were found in a mucosal rectal biopsy. Manometric studies showed absence of the normal rectosphincteric relaxation reflex. A long posterior rectal myectomy was performed in which no ganglion cells were found between the muscular layers. The patient underwent a rectosigmoid resection with a low anastomosis according to Duhamel. In the resected bowel, a normal Meissner's plexus was again found while marked hypoganglionosis of the myenteric plexus was evident. Intranuclear inclusion bodies in Schwann cells and scattered inflammatory cells were noteworthy. This case may be an example of an acquired hypoganglionosis localized to the myenteric plexus.

Congenital cricopharyngeal achalasia treatment by dilatations.

An infant with cricopharyngeal achalasia, recurrent aspiration pneumonia, and severe failure to thrive was fed for 5 months through a nasogastric tube. She responded promptly to dilatations of the spastic cricopharyngeal muscle. Normal oral feedings followed the treatment with progressive weight gain and no further pulmonary complications over a follow-up period of 1 1/2 years. Dilatations were used in the infant described here, in analogy to the well-known success of treatment by dilatations in infants with achalasia of the lower esophagus.

An experimental model of a submucosally tunnelled valve for the replacement of the ileo-cecal valve.

An enteric valve to prevent colonic content reflux and to prolong small bowel transit time was created by passing small bowel through a submucosal tunnel in the colon. Barium enema, Gl tract series, and iso- and antiperistaltic pressure measurements showed the valve to be of competence equal to the ileo-cecal valve. Late pathological examination revealed a patent valve with minimal scar formation. The optimal submucosal tunnel length was between 4 and 6 cm. This valve can, theoretically, increase the absorptive capacity in short bowel syndrome and may serve as a substitute for the critically important ileo-cecal valve.

Endesophageal resection of short benign strictures of the esophagus.

A relatively simple operation for the treatment of short esophageal strictures that do not respond to dilatations has been described. This operation was successfully carried out at all levels of the esophagus. The strictures treated were caused by lye burns, peptic esophagitis and previous anastomoses.

Nerve mediated responses to drugs and electrical stimulation in aganglionic muscle segments in Hirschsprung's disease.

The activity of isolated muscle strips from normal and aganglionic human large bowel was studied in vitro. The intrinsic nerves were stimulated electrically and by nicotinic agonists. The ganglionic preparations displayed a strong inhibitory response due to the release of both norepinephrine and a noncholinergic, nonadrenergic inhibitory neurotransmitter. In the aganglionic strips (obtained from patients with Hirschsprung's disease), nerve activation tended to evoke contraction, apparently due to enhancement in the release of acetylcholine. At the same time, the release of norepinephrine appeared to be less than normal. A particularly interesting finding in the aganglionic muscle strips was the presence of a substantial inhibitory response due to the release of a noncholinergic, nonadrenergic substance. These results provide further evidence for the importance of the innervation of the aganglionic segment in Hirschsprung's disease.

The surgical management of children with familial dysautonomia.

Familial dysautonomia (FD) is a rare incurable genetic disorder with multisystem involvement. Most of its clinical manifestations are related to disorders of the autonomic nervous system. The disease is associated with specific disturbances of the upper gastrointestinal tract: pharyngoesophageal dyskinesia, gastroesophageal reflux, and prolonged gastric emptying. About 40% of the dysautonomic children manifest repeat vomiting crises. In view of the extensive gastrointestinal symptomatology, children with FD are prone to repeated aspiration pneumonia and chronic respiratory failure, while inadequate calory and fluid intake may lead to a chronic state of hypovolemia and severe failure to thrive. Control of vomiting, prevention of aspiration due to abnormal swallowing, and the assurance of adequate calory intake are three major objectives in the treatment of the dysautonomic child. Medical treatment of the gastrointestinal disorders using different drugs has had limited success. This study reviews the surgical experience in ten children with FD. The type of the procedure used was determined by the severity of the upper GI disturbances. Nine children underwent gastroesophageal Nissen fundoplication and gastrostomy. In seven of them, a pyloroplasty was added. Gastrostomy alone was done in one patient only. Postoperative complications included transient dysphagia in four patients, gastric dilatation in four patients, and dumping syndrome in one. There has been no incidence of immediate postoperative death. One child died 6 months after operation from severe and irreversible respiratory failure. Following operation, the patients still suffered from dysautonomic crises but these were not associated with vomiting.(ABSTRACT TRUNCATED AT 250 WORDS)

Gastric devascularization--an emergency treatment for hemorrhagic gastritis in the neonate.

A newborn baby suffering from massive hemorrhage due to erosive gastritis, was treated successfully by devascularization of the stomach which is a rather simple procedure. Experience with adults as well as with this patient justifies the use of this operation when other measures fail to stop massive bleeding in hemorrhagic gastritis.