Hydrocephalus requiring urgent external ventricular drainage in a patient with diabetic ketoacidosis and cerebral edema: case report

OBJECTIVE AND IMPORTANCE: Neurological deterioration, typically attributed to cerebral edema, is a rare but life-threatening complication in the treatment of diabetic ketoacidosis (DKA). We report the case of a child with DKA who became comatose but demonstrated acute obstructive hydrocephalus, instead of cerebral edema. CLINICAL PRESENTATION: An 11-year-old male patient presented with new-onset insulin-dependent diabetes mellitus and DKA. He was initially responsive but, after several hours of treatment, became unresponsive, with dilated pupils and decerebrate posturing. Cranial computed tomographic scanning demonstrated obstructive hydrocephalus resulting from focal cerebellar and brain stem edema. INTERVENTION: The patient was initially managed with medical treatment but ultimately required urgent ventricular drainage to arrest a progressive herniation syndrome. To our knowledge, this report describes only the second such case reported and the first requiring urgent ventriculostomy. CONCLUSIONS: These observations emphasize the importance of recognizing hydrocephalus as a potentially reversible cause of coma in DKA and of initiating prompt neurosurgical intervention, if warranted.     

Intravenous immunoglobulin therapy in POEMS syndrome: a case report

One course of intravenous immunoglobulin was tried on a patient with the syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin lesions and Castleman's disease. No effect was noted.     

POEMS syndrome with vascular lesions: a role for interleukin-1beta and interleukin-6 increase--a case report

The authors describe the case of a 60-year-old man with POEMS syndrome associated with vascular lesions. The patient had osteosclerotic myeloma IgA (lambda), polyneuropathy, endocrinopathy, and skin changes. Subsequently, he developed gangrene of the lower limbs with no response to heparin therapy. The humoral study showed thrombocythemia, high levels of interleukin-1beta (IL-1beta) and IL-6 and of some coagulative/fibrinolytic and endothelial factors (von Willebrand factor, plasmin-antiplasmin complexes, plasminogen activator, and endothelial adhesion molecule ICAM-1). The authors suggest that these factors, induced by the increased levels of cytokines, could be responsible for microvascular damage, gangrene, and heparin resistance.     

Gastric cancer occurring in a patient with Plummer-Vinson syndrome: report of a case

We report herein the unusual case of a 59-year-old woman with Plummer-Vinson syndrome who developed gastric cancer. The patient had a longstanding history of dysphagia and iron deficiency anemia, for which she had sporadically taken iron supplements that improved the dysphagia to some extent, but not completely. Owing to her tolerance of the dysphagia, she had not been taking iron supplements for the past 17 years. On admission, she was in fair nutritional condition and not anemic. Blood chemistry results were all normal, including the serum iron level. Gastrointestinal radiographic series demonstrated cervical esophageal webs and advanced gastric cancer. Her dysphagia was successfully treated by endoscopic bougienage through the webs, and a distal partial gastrectomy with nodal dissection was performed. Histology of the resected stomach revealed atrophic mucosal change and, by chance, an adenomatous lesion in addition to adenocarcinoma. Her postoperative course was uneventful and she is now well, without any signs of recurrence. Although Plummer-Vinson syndrome is known to be associated with upper alimentary tract cancers, gastric cancer is extremely rare. A discussion on the etiology of Plummer-Vinson syndrome and its link with potential carcinogenesis follows this case report.     

Kenny syndrome: case report and literature review

A 34-month-old girl presented with a clinical picture of Kenny syndrome. The clinical manisfestations included growth retardation, persistent open anterior fontanelle, prominent forehead, mid-facial dysplasia, hypocalcemic tetany and characteristic radiologic skeletal abnormalities. Serum levels of immunoreactive parathyroid hormone (PTH) remained inappropriately low during hypocalcemic episodes in the neonatal period; indicating that hypocalcemia was a consequence of the hypoparathyroid state. This is the first reported case of Kenny syndrome in Taiwan. The literature on the pathogenesis, etiology and genetic basis of this disorder is reviewed in this paper.     

Case report: severe fibrosis in a patient with scleroderma and previous radiotherapy--a case report and literature review

Patients with scleroderma may develop marked local cutaneous fibrosis following radiation therapy. We describe one patient treated with wide local excision and radiation therapy, who subsequently developed scleroderma with severe fibrosis in the irradiated field.     

Nevoid basal-cell syndrome: literature review and case report in a family

The Nevoid Basal-Cell Carcinoma Syndrome (NBCC), or as it is also referred to, basal-cell nevus syndrome or Gorlin-Goltz syndrome, is characterized by multiple early-appearing basal cell carcinomas, keratocytosis of the mandible, and anomalies of the ocular, skeletal reproductive system. We describe four patients in the same family, all of them possessing a large number of skin tumors associated with other typical clinical and X-Ray anomalies of NBCC. The definitive treatment of NBCC has yet to be established, however, early diagnosis is very important as well as the periodical follow-up examination of ten patients, mainly due to the transformations in the skin lesions that may occur.     

Extramedullary plasmacytoma in a patient with AIDS: report of a case and review of the literature

We recently reported that the nonmetabolizable glucose analogue, 3-O-methylglucose, stimulates somatostatin secretion in the perfused dog pancreas. In this study, we report that 3-O-methylglucose also stimulates insulin secretion in the dog pancreas. The effect was present at 5.5 mM glucose (p < 0.001) but not at O or 2.7 mM glucose. The inhibitor of glucose metabolism, mannoheptulose, blocked the insulinotropic action of 3-O-methylglucose. In contrast, 3-O-methylglucose had no effect on insulin secretion in the perfused rat pancreas. We conclude that 3-O-methylglucose stimulates insulin secretion in the dog and that the effect requires the presence of stimulatory concentrations of D-glucose.     

Osseointegrated implants in a patient with osteoporosis: a case report

OBJECTIVE: To evaluate the performance of in situ piezolectric extracorporeal shock-wave lithotripsy (ESWL) in the middle part of the ureter. MATERIAL AND METHODS: 36 consecutive patients presenting with stones of the middle part of the ureter were treated by in situ piezoelectric ESWL (EDAP LT 02). The maximal dimensions of the stones ranged from 5 to 14 mm (m = 7.8). Moderate or severe ureterohydronephrosis was present in 19 cases (53%) and a double J stent had been previously implanted in 6 cases (17%). The ESWL sessions were performed in the ventral supine position without any anaesthesia or systematic premedication, but an IM injection of 100 mg of pethidine was administered during poorly tolerated treatments. RESULTS: The stone was located easily in 23 cases (64%) and with greater difficulty in 13 cases (36%), as an intraoperative intravenous injection of contrast agent was performed in 6 cases (17%). The number of sessions per patient ranged from 1 to 2 (m = 1.16). The complete sucess rate was 75%, with a 64% success rate after a single ESWL session. The performances were statistically independent of stone dimensions and the degree of obstruction of the urinary tract. The complication rate was 5.5%, but no ancillary endoscopic or percutaneous treatment was required. CONCLUSION: In situ piezoelectric ESWL allows effective management of most stones of the middle part of the ureter. However, the treatment of stones with a maximal diameter < 5 mm, especially poorly radiopaque stones, can raise problems of localization. Very large or impacted stones, especially when complicated by urinary tract infection, should be preferably treated by first-line ureteroscopy.     

Rhinolith in a patient with cleft palate: a case report

Dental anomalies are well documented in patients with cleft palate, although reports of intranasal teeth in these patients are extremely rare. This paper discusses the case of a rhinolith associated with tooth-like structures in a patient with a treated cleft palate.     

Serotonin syndrome: case report and review of the literature

We present the case of a 35-year-old woman who developed serotonin syndrome after receiving a single dose of the cyclic antidepressant imipramine (Tofranil). She was already being treated for depression with paroxetine (Paxil), a selective serotonin reuptake inhibitor. Two hours after receiving imipramine, the patient developed tachycardia, delirium, bizarre movements, and myoclonus, all classic findings of serotonin syndrome. Her antidepressants were discontinued and she was treated with intravenous fluids, sedation, and a short course of cyproheptadine, a serotonin receptor antagonist. All symptoms resolved completely within 24 hours. In this case report, we review the drug interactions that can precipitate serotonin syndrome, and give recommendations for the diagnosis and treatment of this potentially fatal disorder.     

Antley-Bixler syndrome: case report and review of the literature

Five patients requiring dialysis for acute pulmonary edema and uremia from severe renal artery occlusive disease underwent surgical revascularization. Three patients with oliguria had excellent outcomes and remain dialysis-independent as long as twenty-four months following operation (mean serum creatinine 2.0 mg/dl). The two patients who were anuric both had technically successful operations but remained dialysis-dependent. Diagnostic evaluation of the azotemic patient suspected to have renal arterial occlusive disease should include a history and physical examination, urinalysis, renal ultrasound, and duplex scan of the renal arteries. In appropriate patients, arteriography should then be considered if other diagnoses appear unlikely. This algorithm may help identify those patients who might benefit from renal revascularization. It appears that oliguria rather than anuria and the angiographic demonstration of a patent distal vessel and nephrogram suggest a better functional outcome after revascularization. Unfortunately, the response to surgery cannot be reliably predicted and patient selection remains a challenge, but retrieval of renal function can be achieved in some cases even if patients are already being hemodialyzed.     

Toxic shock syndrome: case report of a postpartum female and a literature review

1. 1alpha,25-dihydroxyvitamin3 (VD) is a nuclear hormone that has important cell regulatory functions but also a strong calcemic effect. EB1089 is a potent antiproliferative VD analogue, which has a modified side chain resulting in increased metabolic stability and a selective functional profile. Since EB1089 is considered for potential systemic application, it will be investigated to what extent its recently identified metabolites (hydroxylated at positions C26 and C26a) contribute to biological profile of the VD analogue. 2. Limited protease digestion analysis demonstrated that EB1089 is able to stabilize the high affinity ligand binding conformation of the VDR, starting at concentrations of 0.1 nM and affecting up to 80% of all receptor molecules. The metabolites EB1445 and EB1470 showed to be 100 fold less potent than EB1089, whereas the remaining three metabolites (EB1435, EB1436 and EB1446) showed a clearly reduced ability to stabilize the high affinity ligand binding conformation. Interestingly, at pharmacological concentrations all EB1089 metabolites stabilized a second, apparently lower affinity conformation to a much higher extent than EB1089. 3. In reporter gene assays all metabolites showed lower potency than EB1089. Moreover, the preference of EB1089 for activation of VDR binding to sites formed by inverted palindromic arrangements spaced by nine nucleotide (IP9-type VD response elements) appeared to be reduced (with EB1445 and EB1470) or completely lost (with EB1435, EB1436 and EB1446). The ranking of EB1089 and its metabolites that was obtained by limited protease digestion and reporter gene assays was confirmed by an analysis of their antiproliferative effect in breast cancer cells. . The potency and selectivity of the EB1089 metabolites in mediating gene regulatory effects was found to be drastically reduced in comparison to the parent compound suggesting that the contribution of the metabolites to the biological effect of EB1089 is minor. However, the compounds showed to be interesting tools for understanding the selective biological profile of EB1089.     

Mycobacterium genavense infection presenting as a solitary brain mass in a patient with AIDS: case report and review

Patients with AIDS are prone to developing infections with opportunistic pathogens. Recently, a new mycobacterium, Mycobacterium genavense, has been found to cause infection in patients with AIDS. Previously published reports indicate that patients who are infected with this organism present with the same clinical features as do patients with disseminated infection due to organisms of the Mycobacterium avium complex. We describe an unusual case of a patient with AIDS who presented with grand mal seizures and a mass lesion in his brain, which was found to be caused by infection with M. genavense. No evidence of disseminated infection could be found in this patient. We discuss the microbiology of this organism and review the literature on M. genavense infections. Clinicians should be aware of this organism so that efforts at culture and identification will be made.     

Orbital rhinocerebral mucormycosis associated with diabetic ketoacidosis: report of survival of a 10-year-old boy

Cerebral mucormycosis is a rare fungal infection that occurs mostly in association with diabetic ketoacidosis. This central nervous system infection is characterized by a rapid decline in clinical status, and has been recognized as a uniformly fatal event if aggressive therapy is not instituted. We report a diabetic child who presented with blurred vision, chemosis, and pain in the left periorbital region noted for about 1 week during an episode of ketocidosis. Neurologic examinations revealed that there was a decreased range of motion in the upward and lateral gaze, along with incomplete pupillary dilatation and papilledema of the left eye. Imaging studies demonstrated left-side orbital cellulitis, paranasal sinusitis, and a large lobulated abscess in the left frontal lobe. Two surgical procedures, including functional endoscopic sinus surgery for sinus debridement and a subsequent open craniotomy for abscess resection were performed. Pathologic specimens obtained from the abscess wall revealed necrotic inflammation and wide, nonseptate hyphae with right angle branching, which are typical characteristics of the family Mucoraceae. Postoperatively, the patient was treated with 1.5 g of amphotericin B over a 7-week period, and aggressive diabetic control for 2 months. Through the combination of medical and surgical treatment the child made an uneventful recovery.