Young adult chronic patients: an analytical review of the literature

This article analyzes the periodical and "fugitive" literature concerned with an emerging psychiatric service entity, young adult chronic patients. The increasing prominence of a young adult patient population results from the confluence of two primary forces: deinstitutionalization policies and demographic factors. The author discusses the clinical diversity and program requirements of these patients. Young adult chronic patients are best served when their uniqueness as a patient population is acknowledged and heeded and when special services for them are integrated into the total system of care, the author concludes.     

Sweet's syndrome. Presentation of six cases and review of the literature

The acute febrile neutrophilic dermatosis or Sweet syndrome, initially described in 1964 by Robert Sweet (1). It is characterized fever, neutrophilic leucocytosis, abrupt appearance of erythematous, painful, cutaneous plaquets and dense dermal infiltrate consisting of mature neutrophils without vasculitis sings. Malignancy has been described in the 10-15% of the reported cases. We report our series of 6 patients diagnosticated of this illness in our department. One of this patients has Sweet syndrome associated with a malignancy disorder. All of them had diagnostic criteria of the described disease and had good response to corticotherapy. We also report a bibliographic review of this infrequent syndrome.     

Bone marrow transplantation in Shwachman-Diamond syndrome: report of two cases and review of the literature

Patients with Shwachman-Diamond syndrome (SDS) have an increased frequency of myelodysplasia and leukemic transformation. We described two patients who received allogeneic stem cell transplantation and developed multiple complications, including seizure, hyperglycemia and renal tubular acidosis. A review of the literature showed that patients with SDS appeared to have an increased incidence of various transplant-associated problems. These patients frequently have underlying organ dysfunction and should be managed with extreme caution when treated with allogeneic stem cell transplantation.     

Wallenberg syndrome: a review of 25 cases

INTRODUCTION: Wallenberg's syndrome is one of the most common clinically recognized conditions due to brain stem infarct, which can nowadays be identified thanks to modern neuro-imaging techniques. We present a retrospective study of 25 cases admitted to our hospital in the past eight years, to evaluate epidemiological aspects and clinical findings and to correlate these with magnetic resonance topography. MATERIAL AND METHODS: Patients diagnosed in the Neurology Section of Hospital Xeral-Calde in Lugo between January 1989 and December 1997 as having Wallenberg's syndrome. RESULTS: There was a predominance of middle aged men presenting at 55.06 years of age (range 30- 78). Arterial hypertension was the main risk factor (52%). There was a progressive form of onset in most cases. The commonest symptom was dysphonia followed by dysphagia. The commonest finding on physical examination was ataxic gait (24 patients) MR was positive in 22 of the 23 cases in which this was done. There were different clinical findings depending on the site of the lesion, whether rostral, caudal or medial. This is considered in the discussion. In most cases the prognosis was good. The commonest sequel was ataxia. CONCLUSIONS: The results are similar to those in the literature. We emphasize the excellent correlation of clinical data and neuroimaging findings.     

Megalourethra: a report of four cases and review of the literature

Megalourethra is a rare congenital anomaly characterized by severe dilatation of the penile urethra. Four cases of congenital megalourethra were seen at Sir Padampat Mother and Child Health Institute, Jaipur, during the last 10 years. Three cases of scaphoid megalourethra had no other associated congenital anomalies and were treat-ed successfully without any complications, while one patient with a fusiform megalourethra had severe associated congenital anomalies and died. These cases are reported with a review of the literature.     

Pfeiffer syndrome: report of a family and review of the literature

A 5-year-old boy and his father with Pfeiffer syndrome are described. They had acrocephaly, hypertelorism, antimongoloid slant of the palpebral fissures, protrusion of the eyes, large and broad nose, small mandible, irregularly placed teeth, additional upper canine, high-arched palate, partial syndactyly of fingers and toes, brachydactyly of toes, valgus deformity of hypertrophied triangular great toes, broad phalanges of the great toes and broad first metatarsals, accessory epiphyses lateral to the interphalangeal joint of the great toes, and normal intelligence. To our knowledge, this is the first family in which the syndrome is almost totally confined to the head and feet--it spares the upper limbs except for partial skin syndactyly between the fingers--and the third family showing inheritance through three successive generations suggesting an autosomal dominant mode of inheritance. The published papers are reviewed and the clinical and x-ray signs are tabulated.     

Carotid sinus syndrome. Report of five cases and review of the literature

Five cases of carotid sinus syndrome are presented. The syndrome is defined by spontaneous attacks of dizziness and fainting which can be reproduced by graded pressure on one carotid sinus. Three forms of the clinical syndrome, cardioinhibitory, vasodepressor, and cerebral, are discussed. The hyperactive carotid sinsu reflex, in which there is ventricular asystole lasting at least 3 seconds or a decrease of more than 50 mm Hg in systolic and diastolic blood pressure, should be differentiated from this syndrome. Treatment modalities include reassurance, drugs, radiotherapy, cardiac pacemakers, and surgical approaches. Carotid sinus syndrome should be considered in the differential diagnosis of unexplained syncope, arteriovenous block, or inappropriate sinus bradycardia.     

Leri's melorheostosis: three pediatric cases and a review of the literature

Three pediatric cases of melorheostosis are described. Growth disorders of the limbs are often the first signs in children. The radiological appearance consists of long tracks of increased cortical radiodensity in the long bones and of small dense islets in the epiphyses.     

Ureterovaginal fistula: a report of 12 cases and review of the literature

Ureterovaginal fistula is an uncommon complication of pelvic operations, seen most often after Wertheim's hysterectomy. We report 12 cases of ureterovaginal fistulas seen during a 20-year period, all of which followed operations for benign gynecologic conditions. Most patients had no urinary symptoms until the sudden onset of incontinence 1 to 4 weeks postoperatively. Diagnosis was established readily by a combination of excretory urography, cystography, cystoscopy, retrograde pyeloureterography and dye studies. In our series only 1 patient was treated by primary nephrectomy, while 11 underwent ureteroneocystostomy: 2 with a Boari flap and 9 by a direct method. Reconstruction failed in 2 patients, 1 of whom required a secondary nephrectomy.     

Parameatal cyst: presentation of two cases and a review of the literature

OBJECTIVE: To describe two additional cases of parameatal urethral cyst. The literature is reviewed and the etiology and treatment of this disease are discussed. METHODS: Two patients aged 20 and 24 years with parameatal urethral cyst are described. The patients were seen at the Gea González Hospital (México) during the period 1987-1988. The first case was asymptomatic and the cyst had been present since birth and in the other case, the cyst appeared at age 24 and caused irregular stream. Laboratory tests, surgical excision and pathological analysis of the cyst were performed. RESULTS: The laboratory tests were normal, no problems were encountered during surgical excision, the symptoms disappeared and there were no postoperative complications or recurrence. Pathological analysis demonstrated columnar epithelium in both cases. CONCLUSIONS: Parameatal urethral cyst is a rare benign condition that is asymptomatic in most of the cases. It may be present since birth or appear later and is prevalent in young males. Its etiology remains unclear and treatment is by complete surgical excision to avoid complications and recurrence.     

Atypical ameloblastoma: report of 3 cases and a review of the literature

The aim of our study was to test the hypothesis that the better absorption of sorbitol when ingested with glucose could be related to a delayed gastric emptying. We tested the effect of the ingestion of glucose and lipids on the gastric emptying and intestinal absorption of sorbitol in six healthy volunteers, using gastric scintigraphy and hydrogen breath test. After an overnight fast, subjects ingested in random order, on 48-h test periods separated by at least one week, the following solutions: (a) 20 g sorbitol alone; (b) 20 g sorbitol and 20 g glucose; (c) 20 g sorbitol and 9 g lipids. Isotopic acquisitions were taken for 3 h following the ingestion of sorbitol labelled with 111Indium. Hydrogen concentration was measured in end-expiratory samples during 5 h, and the areas under the breath hydrogen curve, reflecting the amounts of sorbitol unabsorbed in the small bowel, were compared between periods. Mean area under the curve was 397 +/- 159 when sorbitol was ingested alone, and this was significantly lower when ingested with glucose or lipids (313 +/- 181 and 337 +/- 135, respectively; P < 0.05). The three curves of sorbitol gastric emptying differed significantly from each other, the gastric emptying being the slowest for sorbitol plus lipids, and the fastest for sorbitol taken alone. We found a positive correlation between the half-emptying time and the hydrogen areas under the curve (r = 0.46, P = 0.05). In conclusion, our study demonstrates that adding glucose or lipids to a solution of sorbitol slows the gastric emptying of sorbitol, resulting in a better intestinal absorption of sorbitol.     

Gangliogliomas: a clinicopathological study of 25 cases and review of the literature

The histopathological, clinical, and radiological findings in 25 patients (median age 20.5 years; range 1.7-64.2 years) with gangliogliomas were assessed to correlate degree of astrocytic anaplasia and proliferative potential with recurrence or survival. Most patients (64%) presented with seizures (median Karnofsky Performance Score 90%; range 70-100%). Computerized tomography and magnetic resonance imaging showed nonspecific abnormalities. Neoplastic ganglion cells were defined as heterotopic, irregularly grouped, or having more than one nucleus of bizarre shape or size. The astrocytic component was moderately anaplastic in 15 cases and highly anaplastic (HAA) in 10. Eight patients had gross total resection, 11 had subtotal resection, and six underwent biopsy. Ten patients (five gross total resection, three subtotal resection, two biopsy) had no further treatment, 15 underwent external irradiation, and five had adjuvant chemotherapy. Twenty-four patients are alive 15-394 weeks (median 203.5 weeks) postoperatively; one with ganglioglioma-HAA died at 65 weeks. No tumor recurred after gross total resection. Duration of preoperative symptoms < 1 year, greater anaplasia, and age > 30 years at diagnosis may have increased the risk of recurrence after subtotal resection or biopsy by four, three, and two times, respectively (not significant). Bromodeoxyuridine labeling index (BUdR LI) was < 1% in eight non-recurring tumors and 1.3% in another recurring twice (second recurrence LI = 1.6%). Most patients with ganglioglioma have a good prognosis. After gross total resection, only observation is required. After subtotal resection or biopsy, recurrence is possible. BUdR labeling may guide further therapy.     

Pregnancy-associated Sweet's syndrome: world literature review

Sweet's syndrome (acute febrile neutrophilic dermatosis) is a steroid-responsive dermatosis characterized by pyrexia, neutrophilia, and painful erythematous plaques that histologically show a dense dermal infiltrate of neutrophils. Pregnancy-associated Sweet's syndrome is defined as the initial appearance or recurrent episode of Sweet's syndrome in a pregnant woman. Sweet's syndrome occurring during pregnancy has only been described in four women. The clinical characteristics of the women with pregnancy-associated Sweet's syndrome are reviewed and the differential diagnosis of pregnancy-associated dermatoses that clinically mimic Sweet's syndrome are discussed.     

Painless osteoid osteoma of the rib in an adult. A case report and a review of the literature

BACKGROUND: An asymptomatic rib lesion was discovered by means of a bone scan obtained during the clinical evaluation of an adult man with biopsy proven prostate adenocarcinoma. Clinically and radiologically considered to be a metastatic focus, on resection it proved to be an osteoid osteoma (OO). METHODS: A review of the English medical literature on OO was conducted with emphasis on the occurrence in older patients, costal location, and the absence of pain. RESULTS: OO in patients older than age 50 years is rare (1% - 2% of cases). Only 18 cases of painless OO were found. Of these, 8 (44%) occurred in the phalanges, and 3 (17%) in the cranial-facial bones, both uncommon sites for OO; only 7 (39%) arose in the long bones, the most common site for conventional OO. A disproportionate number of these patients (44%) were younger than age five years, a rarity for OO. Fourteen reported examples of costal OO were found, all of which were associated with pain. The possible mechanisms involved in the production of pain, including analysis of the effect of its site of origin and the presence of nerve fibers, is reviewed. CONCLUSIONS: Costal OO is distinguished from osteoblastoma and from what has been described as painless fibro-osseous lesion of the rib. The infrequency of metastases as a cause of solitary rib lesion is emphasized.     

Alstr?m syndrome. Report of 22 cases and literature review

OBJECTIVE: The authors report 22 cases of Alstr?m syndrome (AS), which is the largest series to date. Only 37 cases have been reported in the world literature since 1959. The authors review the clinical features and compare these with the overlapping condition of Bardet-Biedl syndrome. Their aim is to clarify the AS phenotype and to increase awareness of the early features. DESIGN: A retrospective case series. PARTICIPANTS: All patients (22) with a diagnosis of AS admitted to the authors' hospital in the past 10 years were included in this review. INTERVENTION: This is principally a review of ocular features, but other features are recorded and discussed. MAIN OUTCOME MEASURES: Features noted included age at onset of visual symptoms, presence of photophobia, visual acuity, and electroretinogram findings. Nonocular features recorded included cardiac status, weight and height, hearing, and presence of diabetes mellitus. RESULTS: Cardiomyopathy presenting in infancy has only been recognized recently to be a feature of AS. Of the authors' cases, 18 of 22 had infantile cardiomyopathy. In the authors' tertiary referral institution, there is an ascertainment bias toward younger patients and especially those with pathology that is other than ocular pathology. In addition, AS is difficult to recognize in childhood without the development of infantile cardiomyopathy. Alstr?m syndrome often is not recognized until diabetes mellitus develops in the second or third decade. Initially, a diagnosis of cone-rod dystrophy, achromatopsia, Leber's congenital amaurosis, or Bardet-Biedl syndrome may be made. In AS, there is a severe infantile retinal dystrophy. The electroretinogram is absent or attenuated with better preserved rod than cone function. The retinal dystrophy is progressive with the patient's visual acuity of 6/60 or less by 10 years of age and no light perception by 20 years of age. CONCLUSIONS: A diagnosis of AS should be considered in infantile cone and rod retinal dystrophy, particularly if the weight is above the 90th percentile (16 of 18 cases) or if there is an infantile cardiomyopathy (18 of 22 cases).     

Endobronchial lipoma: two cases and review of the literature

Endobronchial lipoma is a benign tumour of the large bronchi occurring in middle-aged men. To the 38 successfully treated cases in the English literature a further 2 are added. The symptoms are those of obstructive pneumonitis mimicking bronchogenic carcinoma, and the result of delayed therapy may be bronchiectasis. Treatment includes local resection through a bronchoscope or a bronchotomy incision, or removal, if necessary, of the obstructed lobe or lung at thoracotomy. Smoking may be important in the pathogenesis of this tumour.