A mild case of nesidioblastosis with diagnostic and therapeutic difficulty

A female infant with nesidioblastosis who showed mild clinical symptoms is reported. In this patient, insulin levels and insulin to glucose ratios (IRI/G) were often normal. Regular milk feedings supplemented with continuous glucose infusion (0.7-2 mg/kg per min) or oral glucose feedings (4.5 mg/kg per min) prevented hypoglycemia. As leucine-sensitivity was diagnosed at 2 months of age, she was started on diazoxide. This was, however, ineffective, and adverse effects appeared. Subtotal pancreatectomy (95%) was therefore attempted at 5 months of age, and persistent normoglycemia as well as normal growth and development followed up to 3 years after the operation. The pancreas showed characteristic signs of nesidioblastosis. The above clinical observation suggests that a patient with nesidioblastosis whose blood glucose level is easily controllable may develop an unexpected episode of hypoglycemia in the presence of a leucine sensitivity. In such a patient, diazoxide or, when it is of no avail, surgical intervention should promptly be instituted to prevent possible neurologic sequelae induced by hypoglycemia.     

Low-Dose Doxapram Therapy in Premature Infants and Its CSF and Serum Concentrations

ABSTRACT. The efficacy of low-dose doxapram therapy (0.2 mg/kg/h) in combination with methylxanthines was evaluated in 20 premature infants with idiopathic apnea unresponsive to methylxanthines alone, and in 13 premature infants with secondary apnea. The serum concentrations of doxapram and, in some infants, the simultaneous cerebrospinal fluid and serum concentrations were measured, and the correlation between cerebrospinal fluid and serum concentrations in the postnatal period was determined. The following results were obtained: 1) In idiopathic apnea of prematurity, low-dose doxapram therapy was as effective as a dose of 1.0-2.5 mg/kg/h and the side effects were few, mild, and reversible. 2) In premature infants over seven days of age, serum concentrations of doxapram were almost stable but were significantly lower than in infants within the first six days of life. 3) The ratio of the cerebrospinal fluid to serum doxapram concentration was 0.48 ± 0.13 (mean ± SD). There was a good correlation between cerebrospinal fluid and serum concentrations ( r = 0.933, p < 0.001). The initial doxapram dose can be set as low as 0.2 mg/kg/h in very young premature infants with idiopathic apnea of prematurity unresponsive to methylxanthines.     

A Case of a Patient with a Rectal Ulcer Who Had an Unusual Defecation Habit

Abstract: This case report describes a patient with a rectal ulcer who had an unusual defecation habit. Complete healing was recognized colonoscopically after the patient was instructed to break this habit. A polyp of the ascending colon was detected by a barium enema in a 37-year-old man complaining of anal bleeding. He was admitted to our division to undergo a polypectomy. At the time of the polypectomy, a round ulcer, measuring 1 cm in diameter, was detected on the right wall of the rectum 3 cm from the anal verge. A diagnosis of mucosal prolapse syndrome of the rectum could not be made because the patient did not exhibit the characteristic habit of excessive “straining” mentioned by patients with this syndrome, and no characteristic finding of fibromuscular obliteration was found on histological examination of biopsied specimens taken endoscopically from the lesion. Repeated history taking, however, revealed that the patient had the unusual habit of inserting his finger into his rectum after defecation. He broke this habit following instruction to do so. As a result, on colonoscopic examination 15 month later, the ulcer was found to have become a scar:     

The Preclinical Safety Evaluation of Human Monoclonal Antibody against Cytomegalovirus

The human monoclonal antibody against cytomegalovinis (Mab C23)was examined pharmacokinetically and toxicologically as partof the preclinical studies prior to approval for human use.Rats given repeated intravenous administrations of Mab C23 producedno antibodies against Mab C23 and maintained a blood Mab C23level in a dose-dependent manner. However, pregnant rabbitsproduced antibodies against Mab C23. The half-life of Mab C23in plasma was 15.9 days in rats, which was similar to that ofnormal human serum -globulin (NHSG). Neither behavioral effectsnor circulatory disturbance was found in mice, rats, and dogseven after a single intravenous injection of 100 or 200 mg/kg,which corresponds to 50 or 100 times the intended clinical dosage.The repeated doses of 2, 10, or 20 mg/kg of Mab C23 on six occasionswith 1- or 2-week intervals elicited a transient decrease inleukocyte counts in rats given 10 or 20 mg/kg, but no adverseeffects in cynomolgus monkeys. Mab C23 did not cause any reproductiveor developmental toxicity when administered to rats and rabbitsat dose levels of 20 mg/kg or less. However, pregnant animalsshowed lower plasma levels of Mab C23 than non-pregnant animals.The chromosomal aberration test disclosed no clastogenicityin human lymphocytes. An immunostaining for Mab C23 revealedno localizations in several tissues of cynomolgus monkeys givenintravenous doses of Mab C23. The preclinical safety evaluationin animals other than rabbits, which produced no antibodiesagainst Mab C23, showed that the behavior of Mab C23 is pharmacokineticallysimilar to that of NHSG and is as safe as NHSG, which has longbeen used as a biological agent. However, because there wasa difference in blood levels of Mab C23 between pregnant andnonpregnant animals, its clinical administration to pregnantpatients should differ from that to non-pregnant patients.     

Electroanatomical Mapping‐Guided Endocardial and Epicardial Ablation of Sustained Ventricular Tachycardia Originating From Alcohol Septal Ablation‐Induced Scar in a Patient With Hypertrophic Obstructive Cardiomyopathy

Ventricular Tachycardia After Alcohol Septal Ablation. A 76‐year‐old female developed 2 different ventricular tachycardias (VTs) 5 years after alcohol septal ablation (ASA) for symptomatic hypertrophic obstructive cardiomyopathy. VT#1 was a small macroreentry at the anterior border of the low‐voltage zone, suggesting the ASA‐scar and eliminated by endocardial ablation at a site recording fractionated potentials covering the mid‐diastolic and presystolic periods. VT#2 was a focal VT and eliminated by epicardial cryoablation at the basal posterior left ventricle, suggesting the posterior border of the ASA‐scar. Using the electroanatomical mapping, we demonstrated that the mechanism of the VTs was reentry at the edge of the ASA‐scar. (J Cardiovasc Electrophysiol, Vol. 21, pp. 1296‐1299, November 2010)     

Successful Catheter Ablation of Reentrant Junctional Tachycardia in a Patient with Asplenia Syndrome before Total Cavo‐Pulmonary Connection

Asplenia syndrome is commonly associated with complex structural cardiac malformations, and junctional tachycardia (JT), which may compromise hemodynamic status, has been reported in association with asplenia syndrome. 1 We report successful radiofrequency catheter ablation of reentrant JT in a patient with asplenia syndrome. (PACE 2010; e43–e45)     

Electron microscopic finding of eccrine sweat gland epithelial cells in a patient with Krabbe disease

A 13 month old boy was found to have severely reduced β-galactocerebrosidase activity suggesting infantile Krabbe disease. Clinically, the patient showed a progressive neurological deterioration with white-matter disease on radiological study. Axillary skin biopsy was performed to support the diagnosis. On electron microscopy, needle-like inclusions, which are the typical finding seen in the cytoplasm of astrocytes and Schwann cells in the classic infantile form, were present in eccrine sweat gland epithelial cells. This method is useful for diagnosis when nerve biopsy and biochemical analysis are not readily available.     

Postirradiation multiple minute digitate hyperkeratoses

We report a new case of multiple minute digitate hyperkeratoses after postmastectomy irradiation therapy for mammary cancer. This is the first case of multiple minute digitate hyperkeratoses in an Asian country as well as the third report of the disorder after irradiation. The characteristic eruption, the consistent trigger of irradiation and its occurrence in patients of different racial groups indicate that multiple minute digitate hyperkeratoses are a distinct clinical entity.