Morquio's syndrome. A case report and review of clinical findings

Morquio's syndrome is a genetic disorder caused by the lack of the enzyme responsible for the breakdown of the mucopolysaccharide keratin sulfate. The resulting buildup of keratin sulfate causes a specific pattern of abnormalities, including short stature, spinal deformity, cardiac abnormalities, corneal opacities, deafness and dental abnormalities. The specific dental findings in the patient described here are as follows: the posterior teeth were tapered and had pointed. cusps; the maxillary anterior teeth were spaced and flared, and on radiographs, the enamel was less than 25 percent of normal thickness but of normal radiodensity. Despite these findings, the patient exhibited a low rate of caries. This paper is a review of medical and dental findings in the Morquio's patient and how this may affect dental management.     

MR findings of primary central nervous system lymphoma in a child. A case report

Huntington's disease is an autosomal dominant, inherited disorder that results in progressive degeneration of the basal ganglia (especially the neostriatal caudate nucleus and putamen) and other forebrain structures and is associated with a clinical profile of movement, cognitive and psychiatric impairments for which there is at present no effective therapy. Neuropathological, neurochemical and behavioral features of the disease can all be reproduced in experimental animals by local injection of excitotoxic or metabolic toxins into the neostriatum. All these features of the disease can be alleviated, at least in rats, by transplantation of embryonic striatal tissue into the degenerated striatum, which was the basis for commencing the first clinical trials of striatal transplantation in Huntington's patients. However, although rat striatal xenografts may temporarily reduce apomorphine-induced dyskinesias in monkeys, there has been no demonstration that allograft techniques that work well in rats translate effectively to the much larger differentiated striatum of primates. Here we demonstrate good survival, differentiation and integration of striatal allografts in the primate neostriatum, and recovery in a test of skilled motor performance. Long-term graft survival in primates indicates probable success for clinical transplants in Huntington's disease; in addition, our data suggest that graft placement has a direct influence on the pattern and extent of functional recovery.     

Atypical CT findings of cervical lymphadenopathy due to Hodgkin''s disease. A case report

We describe a case of Hodgkin's disease with cervical lymphadenopathy which may easily be confused with a granulomatous process both radiologically and pathologically.     

Localized prepubertal periodontitis--nonsurgical treatment of an adolescent patient: a case report

The treatment of localized juvenile periodontitis has been previously described in the literature, utilizing primarily a long-term (2 to 6 week) antibiotic regimen, notably tetracycline. This case report of juvenile periodontitis with extensive bone loss describes a short-term treatment (8 days), using a combination of two antibiotics and mechanical debridement. Clinical treatment included instruction of proper oral hygiene techniques. Initial scaling and root planing were performed to remove supragingival and subgingival accretions, followed by 2-month maintenance recalls. Pre- and postoperative radiographs, taken one year after the treatment, are used to document the evidence of natural bone regeneration. The learning objective of this article is to present an effective method of treatment-a debridement/antibiotic combination, followed by bone regeneration.     

Spondylitis in infancy. A case report

The authors report on a case of a spondylodiskitis in three year old child. Diskitis is an inflammatory process of the intervertebral disk space, not often described in pediatric age. The etiology is infective and generally presents with lumbar spine localization. Clinically it is characterized by lumbar pain at times radiating to a lower extremity. Symptoms are aspecific and radiological signs do not appear for several weeks after the onset of symptoms. Interest in this pathology derives from the fact that it may be more common than previously recognized. This case offered us the opportunity to review principal symptoms of diskitis and to describe the main diagnostic technics used.     

Subgingival microflora and treatment in prepubertal periodontitis associated with chronic idiopathic neutropenia

Prepubertal periodontitis affects both primary and permanent dentition. The purpose of this study was to examine the composition of subgingival microflora of the permanent dentition in an 11-year-old Caucasian female, who had premature exfoliation of her deciduous teeth on her 5th year of age, and the response of this condition to the antibiotic therapy and supportive periodontal care. Gingival tissues were highly inflamed and alveolar bone loss was detected radiographically. The girl had experienced frequent upper respiratory tract infections, tonsilitis and recurrent otitis media. Her mother had history of early onset periodontitis associated with chronic idiopathic neutropenia. Blood chemistry tests and immunological examinations were also performed. Subgingival plaque samples were collected from the proximal sites of permanent molars, incisors, canines and maxillary premolars. 27 different microbial species were isolated from the subgingival microflora. Among the predominant species were Porphyromonas gingivalis (17.6%-7.3%), Prevotella intermedia (12.4%-4.7%), Capnocytophaga sputigena (14.4%-10.4%), Capnocytophaga ochracea (13.2%-6.9%) and Actinobacillus actinomycetemcomitans (9.3%-5.5%). Periodontal treatment consisted of scaling, root planing in conjunction with antibiotic administration of Augmentin 312.5 mg and Flagyl 200 mg, each t.i.d. for 10 days. 3 weeks after the antibiotic therapy, bacterial samples were collected from the same sites. All the periodontal pathogens were recovered in lower levels and A.actinomycetemcomitans was almost eliminated in the 3-week period. The evaluation of clinical indices at 3, 6 and 12 months showed that periodontal treatment in conjunction with antibiotics was effective and rapidly followed by marked clinical improvement. The microbiological monitoring at 3, 6 and 12 months after antibiotic treatment and each time prior to supportive periodontal care, revealed that the periodontal pathogens fluctuated in low levels even 12 months after treatment and could be maintained at low level by supportive periodontal care at 3-month intervals.     

Castleman's disease. A case report

Angiofollicular lymph node hyperplasia or Castleman's Disease (CD) is a rare lymphoproliferative disorder that manifests itself as a local or generalized tumor-like condition affecting both lymph nodes and non-nodal tissues, usually in the chest and abdomen. Hyaline vascular and plasma cell types have been identified histologically. A new clinical form of CD with multisystemic involvement has been defined as multicentric Castleman's disease. It is very rare in childhood. In this paper we present an eight-year-old boy with multicentric Castleman's disease.     

Fatal exorcism. A case report

A five-year-old girl was killed by her mother when she tried to pull the devil out of the girl's mouth by means of her hands. In that way the enlarged tonsils were pushed back and caused together with the woman's fingers a temporary closure of the hypopharynx and at least the suffocation of the child. About two years ago the woman began to show paranoic ideas exacerbating up to the fatal event.     

Gallbladder torsion. A case report

INTRODUCTION: The unusual anatomical position of the organ, generally suspended by a mesentery, allows the rare possibility of its torsion with necrosis and even perforation. CASE REPORT: A woman, aged 78, who presented signs and symptoms similar to those of severe acute cholecystitis: abrupt onset of pain and a large palpable mass below the right costal margin. Immediate laparotomy with detorsion and easy cholecystectomy was performed. CONCLUSIONS: Should a greatly enlarged mass be palpable below the right costal margin within a few hours from the abrupt onset of unremittant abdominal pain the possibility of torsion should come to mind not to delay operation in the supposition of acute cholecystitis.     

Thymic carcinoid. A case report

A case of thymic carcinoid appearing in a 56-year-old man is presented. No endocrine disturbances were apparent. Mediastinal lymph node metastases were found at operation. The tumour was positive with Grimelius argyrophil silver stain. Ultrastructurally neurosecretory granules were seen.     

Stomach diverticulum. A case report

The author reports a case of gastric symptomatic diverticulum, not connected to other gastric-biliary-duodenal pathology. The author points out the rarity of diverticulum of the alimentary canal, and points out the symptomatic picture that, in association with a radiologically ascertained diverticulum staunching, represent indications for surgery.     

Morgagni-Larrey hernia. A case report

The authors report a case of Morgagni-Larrey hernia. It is anuncommon diaphragmatic hernia, often asymtomatic and occasionally found. The authors point out the disease's clinical, diagnostic and therapeutic aspects. The hernia's repair can be done both abdominal and thoracic via. The operation is always advisable as well as in asymptomatic pazients.     

Reno-colo-cutaneous fistula. A case report

Reno-colo-cutaneous fistula due to genito-urinary tuberculosis is extremely rare. We describe one such case which was successfully managed by single stage surgery (nephroureterectomy, fistulectomy and primary repair of colon) and anti-tubercular treatment.     

Neonatal listeriosis. A case report

The author describes a case of early neonatal listeriosis following by a positive outcome. The severity of this infection is underlined as well as the need to examine carefully the role of listeria monocytogenes as a cause of similar influenza-type syndromes in pregnant women.     

Orbital cellulitis. A case report

This study describes the case of a 6 years old child, male, with orbital cellulitis and underlines the importance of an early diagnosis and therapy to avoid severe complications often present in this disease. Swelling and redness of the eyelid, pain and ophthalmoplegia are the first sign of an orbital cellulitis and they require rapid diagnostic procedure such as ultrasound and TC scan of the orbital region to evaluate the integrity of the profound orbital tissues. The child was admitted at the Department of Pediatrics, University "La Sapienza" of Rome and underwent an ultrasound, TC scan and serum exams which demonstrated the elevation of the sedimentation rate, reactive C protein and WBC plus the interesting of the profound orbital tissues. The child was treated with antibiotic and antiinflammatory therapy showing a complete recovery within 7 days. An ultrasound performed 7 days later demonstrated a complete resolution of the inflammatory process. In summary, this study would like to stress the necessity of an early diagnosis and an appropriate therapy in order to avoid the severe complications often present in children with orbital cellulitis.