Isolated magnesium malabsorption in a 10-year-old boy

Isolated magnesium malabsorption (congenital hypomagnesemia) has been reported in approximately 30 patients worldwide. Patients typically present by 6 months of age with convulsions and diarrhea. We report an unusual case of isolated magnesium malabsorption in an older boy with no diarrhea.     

Posttraumatic priapism in a 6-year-old boy: a case report

A 6-year-old boy showed priapism after blunt perineal trauma. We diagnosed him with venous priapism mainly based on clinical symptoms, although we could not deny an arterial type. In fear of possible erectile dysfunction as a late complication of the venous type, we performed a shunt operation. The shunt was created between glans penis and corporacavernosa, resulting in disappearance of priapism and preservation of erectile function. We also reviewed 14 cases reported previously.     

Treatment of rhinocerebral mucormycosis with intravenous interstitial, and cerebrospinal fluid administration of amphotericin B: case report

IMPORTANCE: Rhinocerebral mucormycosis is extremely difficult to treat. Approximately 70% of patients are poorly controlled diabetics, and many of the remainder are immunocompromised as a consequence of cytotoxic drugs, burn injuries, or end-stage renal disease. Despite standard treatment consisting of surgical debridement and the intravenous administration of amphotericin B, rhinocerebral mucormycosis is usually a fatal disease. CLINICAL PRESENTATION: We describe the case of a 16-year-old male patient with juvenile onset diabetes mellitus who presented with fever, right-sided hemiparesis, and dysarthria. Axial view computed tomography revealed abscess formation in the left basal ganglia and frontal lobe, which was proven by stereotactic biopsy to contain Rhizopus oryzae. INTERVENTION: Intravenous administration of amphotericin B (30-280 mg/dose) was begun on the day of admission. On hospital Day 20, after the occurrence of frank abscess formation, the lesion was aggressively debrided. Despite these therapies, there was neurological deterioration characterized by the development of hemiplegia and aphasia. Sequential computed tomographic scans enhanced with contrast medium demonstrated progressively enlarging lesions. Ommaya reservoirs were placed into the abscess cavity and the frontal horn of the contralateral lateral ventricle. The patient was then treated with intracavitary/interstitial injections of amphotericin B during the course of 80 days and three doses of intraventricular amphotericin B. Clinical and radiographic improvement was achieved after treatment. Two years after the initial diagnosis, magnetic resonance imaging of the brain showed no evidence of disease and an examination revealed a neurologically intact and fully functional patient. CONCLUSION: We conclude that with an infection as morbid as rhinocerebral mucormycosis, it is advisable to use surgical debridement and all available routes for delivering amphotericin B to infected cerebral parenchyma, which include intravenous, intracavitary/interstitial, and cerebrospinal fluid perfusion pathways.     

A fatal influenza infection in a 10-year-old boy

A previously healthy 10-year-old boy died a few days after onset of septicaemia with non-specific clinical symptoms. Influenza B virus was isolated post mortem from pulmonary tissue. The histopathological findings did not indicate a virus disease. Specimens were taken for virus culture from other people in contact with the patient and affected with influenza-like illnesses. One other strain of influenza B virus was isolated. The strains could not be distinguished either serologically or genetically from other influenza B isolates of the season 1992/'93.     

Oesophageal intramural pseudodiverticulosis: a cause of dysphagia in a 10-year-old boy

We report a 10-year-old boy who presented with a piece of chicken stuck in his throat. He had similar episodes in the past that resolved spontaneously. The foreign body was removed and oesophagoscopy revealed no abnormality. Post-operative barium swallow showed oesophageal intramural pseudodiverticulosis.     

POEMS syndrome in a patient with diabetic ketoacidosis: case report and review

This article summarise the main data in the literature on the role of bacteriological contamination of the dialysate fluid in inflammatory reactions in hemodialysis. Pyrogenic substances of small molecular weight from Gram-negative bacteria grown in dialysate can pass across intact dialyzer membrane to stimulate cytokine production by peripheral blood mononuclear cells. Cellulosic hemodialysis membranes are more permeable to endotoxins than synthetic membranes. Polysulfone membranes and polyamide membranes are able to adsorb bacterial toxins on the dialysate side. The diffusive transfer of bacterial products across dialysis membrane from dialysate fluid was demonstrated. Transmembrane passage of cytokine-inducing bacterial products across reprocessed dialyzers is greater than across new dialyzers. Bacteriological contamination of the dialysate fluid is a problem which must be considered with much more care by nephrologists, especially as LAL test is unable to detect all the bacterial products which can contaminate the dialysate fluid.     

Carcinoma of the larynx in an 11-year-old boy with late cervical metastasis: report of a case with a ten-year follow-up

An 11-year-old boy with laryngeal carcinoma underwent a partial laryngectomy. Subsequently, a cervical metastatic node developed eight months following a tonsillectomy; a radical neck dissection was performed. The patient has remained free of disease over a ten-year follow-up period. In our review of the English literature, no similar case report was found. The potential relationship of tonsillectomy to alterations in local T cell-mediated immune response is discussed.     

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.     

Crohn's disease of the prepuce in a 12-year-old boy: a case report and review of the literature

We report a case of Crohn's disease with involvement of the foreskin in a 12-year-old boy. One year previously, on the basis of clinical features (diarrhea with blood, perianal fissures) and histologic examination, a diagnosis of Crohn's disease was made. Subsequently, he developed phimosis and balanitis and underwent circumcision. Sections submitted from the foreskin revealed noncaseating granulomatous inflammation consistent with Crohn's disease. Crohn's disease with involvement of the genitalia is unusual. Only 26 cases including our case have been reported in the scientific literature. We have analyzed these cases with emphasis on gender, age, clinical features, duration of Crohn's disease, and probable mode of spread to the genitalia. Careful examination of sections from genital lesions, including those submitted from the foreskin, is essential to detect small isolated granulomas that may then lead to the diagnosis of inflammatory bowel disease.     

Thoughts on a case of perforating flying-stone injury on a 10-year-old boy (author's transl)

After a left-sided perforating corneal injury caused by a stone, with iris prolaps and wound cataract suffered by a 10-year-old boy, retinal detachment or shrinking of the eye (phthisis bulbi) can occur. On the other hand vision with contact lenses excludes only a few occupations, e.g. in the armed forces, railways, ships etc. The possibility of retirement neurosis (money for suffering!) must also be considered.     

Cerebral mucormycosis after liver transplantation: a case report

A fatal case of cerebral mucormycosis occurring shortly after liver transplantation is described. The patient was a 32-yr-old male with advanced end-stage liver disease manifested by tense ascites, spontaneous bacterial peritonitis, deepening jaundice and anuria requiring hemodialysis. The 3rd day after successful liver transplantation the patient developed acute respiratory failure, then focal motor signs. Computed tomography showed fluid in the left maxillary sinus, partial opacification of the ethmoid and sphenoid sinuses, and diffuse low density lesions in both cerebral hemispheres. Despite treatment for cerebritis and cerebral edema, the patient's pupils became fixed and dilated, and brain death was declared. Autopsy revealed mucor sinusitis and cerebritis. Mucormycosis is an opportunistic fungal infection occurring in patients with diabetic ketoacidosis, malignancy, or immunodeficiency, and in those receiving wide-spectrum antibiotics, corticosteroids, or cytotoxic therapy. Mucor most frequently involves the face, rhinocerebral disease predominating. These infections are difficult to treat, but are curable with aggressive and frequent surgical debridement, discontinuation or reduction of immunosuppressive therapy and amphotericin. The diagnosis of mucormycosis is very difficult to make in cases such as the present one, in which the typical presentation and classical signs are not present. A high index of suspicion based on identified risk factors may assist in more rapid diagnosis of this life-threatening mycosis.     

Reversible blindness associated with alcoholic ketoacidosis: pseudomethanol intoxication

In Doppler sonography, the physiological meaning of Gosling's pulsatility index (PI) as a measure of downstream resistance is still under dispute. We deliver the theoretical derivation of its physiological significance. We present a mathematical model based on the linked theories of critical closing pressure (CCP) and cerebrovascular impedance, verified in preterm neonates. Mathematical transformation results in a series of equations interrelating several physiological parameters. Instead of indicating cerebrovascular resistance, PI is linked to the ratio of cerebrovascular impedances at the heart rate and at zero frequency. Next to arterial blood pressure, CCP is the principal determinant of PI. PI is identical to the ratio of the alternate and the direct component of the effective driving force. Thus, PI has no distinctive physiological meaning by itself. At present, our model is confined to physiological conditions where the lowest velocity is the end diastolic, and always more than zero.     

Local application of antimycotics in mucormycosis cerebri: a case report

A 65 year old man was found to have mucormycosis cerebri during immunosuppression after treatment of hairy cell leukaemia with 2-chlorodeoxyadenosine. Although mucormycosis cerebri has a poor prognosis, the patient survived after systemic administration of high dose amphotericin B, extensive excision of the abscess, and additional local application of amphotericin B with the help of an absorbable gelatin sponge.     

Orbital lymphosarcoma associated with reticuloendotheliosis virus in a peafowl

Escherichia coli biotin carboxylase was affinity labeled with adenosine diphosphopyridoxal to identify its ATP binding site. Lysyl endopeptidase digestion of the modified protein, followed by high performance liquid chromatography separation and amino acid sequencing allowed to identify lysine-238 to be the site of modification. Site-directed mutagenesis of this residue into alanine, arginine or glutamine resulted in mutants with much decreased activity. Lysine-238 seems to interact with the gamma-phosphate group of ATP but is not involved in catalysis.     

Transitory spinal cord swelling in a 6-year-old boy with Guillain-Barré syndrome

Adrenal enlargement was followed by serial ultrasonography in an infant with congenital lipoid adrenal hyperplasia (lipoid CAH) from day 12 until 2 years and 4 months of age, when they were no longer detectable. Contrary to other types of CAH in which the configuration changes soon after replacement therapy, this infant with lipoid CAH showed persistent adrenal cortex enlargement due to massive accumulation of lipids and cholesterol resulting in a damaged glandular cyto-architecture. CONCLUSION: ultrasonographically persistent enlargement of the adrenals after replacement therapy is suggestive of the lipoid form of CAH.     

Psychiatric illness and family support in children and adolescents with diabetic ketoacidosis: a controlled study

BACKGROUND: In the adult respiratory distress syndrome, nitric oxide (NO) inhalation improves oxygenation through reducing ventilation-perfusion mismatching, but detailed information on the pulmonary effects of NO inhalation in septic shock is scarce. The present study investigated the effects of inhaled NO on alveolar dead space (Vdalv) and venous admixture as well as on respiratory system compliance (Crs) and respiratory system resistance (Rrs) in a porcine model of septic shock. Protective effects of NO are discussed. METHODS: Thirteen anaesthetised and ventilated pigs were given an infusion of endotoxin for an observation time of 220 min to induce acute lung injury (ALI). In the NO-early group (n=6), an inhalation of 60 ppm NO was started simultaneously with the endotoxin infusion and continued for 190 min. In 7 control/NO-late animals, 60 ppm NO was administered for 30 min following 190 min of endotoxin infusion. Haemodynamics, single-breath CO2-, pressure-, and flow signals were recorded. RESULTS: Endotoxin induced haemoconcentration, pulmonary vasoconstriction, and a decrease in Crs, while venous admixture, Vdalv, and Rrs increased. In the NO-early group, the pulmonary vasoconstriction was attenuated, no increase in pulmonary venous admixture or in Vdalv was seen before cessation of NO, and the improvements in oxygenation outlasted the NO inhalation. In the control/NO-late group, the NO inhalation reversed the changes in dead space and venous admixture. NO had no effect on the changes in respiratory mechanics. CONCLUSION: In porcine ALI, 60 ppm NO diminishes pulmonary vasoconstriction and improves gas exchange by reducing pulmonary venous admixture and alveolar dead space, but does not prevent a fall in Crs. NO inhalation may help prevent long-lasting pulmonary failure.