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.     

Epstein-Barr Virus-induced Lymphoblastoid Cell Lines from Patients with Primary Immunodeficiency Diseases

Peripheral lymphocytes from eight patients with congenital immunodeficiency diseases were infected with Epstein-Barr virus (EBV) in an attempt to establish B lymphoblastoid cell lines (LCL). These patients included three boys with congenital agammaglobulinaemia, two girls with hypogammaglobulinaemia, one boy with common variable immunodeficiency, one boy with severe combined immunodeficiency with adenosine deaminase deficiency, and one boy with DiGeorge syndrome. Five of the patients bore no surface immunoglobulins (slg) on their peripheral lymphocytes. LCL were established from seven of the eight patients. All the LCL established formed rosettes with EAC3 and had the ability to produce cytoplasmic immunoglobulins (clg) of various classes. Culture supernatants concentrated up to 100-fold developed precipitin bands by Ouchterlony's method with antisera to human Ig in all the established LCL. These results suggested that both slg^-, clg^- and C3⁺ cells and slg⁺, clg^- and C3⁺ cells might be the target cells for EBV and that slg^-, clg^- and C3⁺ cells might be the precursor cells of B lymphocytes.     

Effect of hypothermia on the in vitro potencies of neuromuscular blocking agents and on their antagonism by neostigmine

The effect of temperature on the potencies of neuromuscularblocking agents remains unclear. This study was undertaken toexamine the effects of different neuromuscular blocking agentsat 37 and 27 °C at a constant carbon dioxide content ( statprinciple). The effect of neostigmine 1 µmol litre^–1induced antagonism of these agents was also investigated. Phrenicnerve-hemidiaphragm preparations of rats were mounted in modifiedKrebs solution, maintained at 37 CC and aerated with a 5% carbondioxide-95% oxygen gas mixture, and at 27 °C with 4% carbondioxide to maintain the carbon dioxide content of the solutionconstant. Phrenic nerves were stimulated with 0.1 -Hz supra-maximalimpulses of 0.2-ms duration and the elicited tension of thediaphragm recorded. The potencies of the steroidal neuromuscularblocking agents (rocuronium, vecuronium, pancuronium and pipecuronium)increased significantly at 27 °C (P<0.05), while thepotencies of the benzyliso-quinolinium agents (tubocurarineand dimethyl-tubocurarine) did not change. Neostigmine-inducedantagonism of the steroidal agents did not differ significantlybetween each other but differed significantly from the benzylisoquinoliniumagents (P<0.05) at both temperatures. The ratios of IC₅₀(inhibitory concentration, 50%) with and without neostigmineat hypothermia were slightly higher for the steroidal agents,indicating slight enhancement of antagonism by neostigmine at27 °C. In contrast, the ratios were significantly greaterat 27 °C (P<0.05) for isoquinolinium agents, implyingsignificant enhancement of antagonism. Our results indicatethat at 27 °C the potency of all steroidal agents increasedand neostigmine-induced antagonism was slightly enhanced. Withthe isoquinolinium agents, hypothermia caused no change in potencyalthough neostigmine-induced antagonism was enhanced significantly.These findings suggest that the relative effects of steroidaland isoquinolinium agents on the neuromuscular junction aredifferent or that they have a different mechanism of actionon the neuromuscular junction.     

Intravenous glycerol therapy should not be used in patients with unrecognized fructose-1,6-bisphosphatase deficiency

BACKGROUND: In Asian countries, glycerol solution that contains fructose (5%) is often used for management of brain edema. However, glycerol and fructose may cause severe hypoglycemia and metabolic acidosis in patients with fructose-1,6-bisphosphatase (FBPase) deficiency, even under stable conditions. The aim of the present study was to determine whether glycerol solution was used for brain edema during acute metabolic decompensation of hypoglycemia and metabolic acidosis in patients with unrecognized FBPase deficiency in Japan and to examine a long-term prognosis of the patients who had this kind of severe metabolic decompensation with or without glycerol therapy. METHODS: A retrospective study of 20 children with FBPase deficiency was conducted, based on their medical records. RESULTS: Six of the 20 children were given glycerol solution for the presence or possibility of brain edema during acute metabolic decompensation of hypoglycemia and metabolic acidosis; two of the six patients administered with glycerol were given dialysis. In four patients treated with glycerol alone without dialysis, two had no brain edema before glycerol administration but it developed later after the administration. These four patients treated with glycerol alone died or developed severe neurological complications. Fourteen patients who were not treated with glycerol solution had no brain edema and showed good prognosis. CONCLUSIONS: Glycerol solution, which contains fructose in Asian countries including Japan, should not be used as an osmotic agent for treatment of brain edema in patients who have hypoglycemia and retention-type metabolic acidosis, until FBPase deficiency is ruled out by measuring blood concentration of lactate.     

Successful Coil Embolization of a Ruptured Basilar Artery Aneurysm in a Child with Leukemia: A Case Report

Ruptured intracranial aneurysms are rare in the pediatric population compared to adults. This has incited considerable discussion on how to treat children with this condition. Here, we report a child with a ruptured saccular basilar artery aneurysm that was successfully treated with coil embolization. A 12-year-old boy with acute lymphoblastic leukemia and accompanying abdominal candidiasis after chemotherapy suddenly complained of a severe headache and suffered consciousness disturbance moments later. Computed tomography scans and cerebral angiography demonstrated acute hydrocephalus and subarachnoid hemorrhage caused by saccular basilar artery aneurysm rupture. External ventricular drainage was performed immediately. Because the patient was in severe condition and did not show remarkable signs of central nervous system infection in cerebrospinal fluid studies, we applied endovascular treatment for the ruptured saccular basilar artery aneurysm, which was successfully occluded with coils. The patient recovered without new neurological deficits after ventriculoperitoneal shunting. Recent reports indicate that both endovascular and microsurgical techniques can be used to effectively treat ruptured cerebral aneurysms in pediatric patients. A minimally invasive endovascular treatment was effective in the present case, but long-term follow-up will be necessary to confirm the efficiency of endovascular treatment for children with ruptured saccular basilar artery aneurysms.     

Solitary psoas muscle metastasis after radical nephrectomy for renal cell carcinoma

Skeletal muscle is a very rare location for the metastasis of renal cell carcinoma (RCC) and only one case of solitary metastasis to the psoas muscle has been reported. We present a 63-year-old male patient with late recurrence (14 years) after left side radical nephrectomy for RCC. He first visited Chikushi Hospital, Fukuoka University, Japan in January 2000 for a postoperative follow-up because he had shifted residence to the area. Follow-up was by abdominal computed tomography (CT) and chest X-ray. In December 2001, a CT scan showed a 1.5 cm enhanced mass in the right psoas muscle without any other metastasis. The mass was resected that month and histological study showed RCC metastasis.     

Altered intrahepatic pathway of para-umbilical vein in portal hypertension

The object of this study was to determine the frequency and characteristics of altered paraumbilical vein in the hepatic parenchyma, developed from portal hypertension, using computed tomography (CT). Two hundred and ninety-two patients who presented with portal hypertension from 1986 to 1996 were studied retrospectively. The pathway of the dilated para-umbilical vein was demonstrated by contrast-enhanced CT. Thirty-one (11%) patients had a dilated para-umbilical vein arising from the left portal vein into the falciform ligament. In 24 (77%) of these patients, the para-umbilical vein followed the expected route, passing through the fissure of ligamentum teres hepatis. The remaining seven patients (23%) displayed the unusual pathway, with the vein arising from the left branch of the portal vein and passing into the hepatic parenchyma. In these seven patients, four had one collateral vein, and three patients had two collateral veins in the liver parenchyma. The dilated para-umbilical vein frequently passes through the hepatic parenchyma in patients with portal hypertension.     

Human T cell responses to recombinant mite antigens of Dermatophagoides farinae

We studied T cell responses to four glutathione S transferase (GST)-fused mite antigens prepared in our laboratory using peripheral blood lymphocytes from mite-sensitive patients with bronchial asthma. Of the four recombinant antigens, purified GST-Mag3 had the strongest ability to cause patients' lymphocytes to proliferate, and its potency was almost comparable to that of crude mite bodies (Dfb) and faeces (Dff) antigens and a purified major antigen, Der f 2. The responder lymphocytes were mainly T cells, because the proliferative response was depleted by the treatment of lymphocytes with anti-CD3 antibody and complement, but not with anti-CD20 antibody and complement. The responsiveness of lymphocytes to GST-Mag3 correlated with that to Der f 2, but GST-Mag3 displayed slightly higher activity to stimulate lymphocytes than Der f 2. Simultaneously, the levels of Dff- and GST-Mag3-specific IgE antibodies correlated with the responsiveness of lymphocytes to GST-Mag3. These results suggest that Mag3 is a new valuable antigen for the response of T cell proliferation in mite-sensitive patients.     

Neuropeptide Y regulates rat renal tubular Na,K-ATPase through several signalling pathways

Neuropeptide Y (NPY) has at least three receptors (Y₁, Y₂ and Y₃) through which it influences different mechanisms in many cell types. Previous data suggest that the Y₂ receptor may be divided into prejunctional and postjunctional subgroups. We have examined the intracellular signalling pathways of the postjunctional Y₂ receptor in rat renal proximal tubules. The results indicate that NPY regulates Na⁺,K⁺-ATPase through several signalling pathways: (1) In proximal tubule (PT) cells NPY increased intracellular calcium. The response was blocked by removing extracellular calcium and was also blocked by using nifedipine. This suggests that calcium was increased by influx from the extracellular space through L -type calcium channels. (2) NPY increased Na⁺,K⁺-ATPase activity in PT segments and this effect was also blocked by nifedipine. CaMKII-Ala²⁸⁶[281–302] a blocker of Ca²⁺/calmodulin-dependent protein kinase II (CaMKII) inhibited the NPY-stimulated Na⁺,K⁺-ATPase activity. This implies that increased intracellular calcium activates CaMKII which subsequently increases Na⁺,K⁺-ATPase activity. CaMKII thus appear to act similar to what has been proposed for protein phosphatase 2B. (3) Calphostin C, an inhibitor of protein kinase C (PKC), did not inhibit NPY-stimulated Na⁺,K⁺-ATPase activity. PKC is, therefore, unlikely to be involved. (4) Y₂ receptors are negatively coupled to the cAMP pathway. NPY attenuated forskolin-stimulated cAMP production in renal tubules and exogenous cAMP counteracted the NPY-stimulated Na⁺,K⁺-ATPase activity. This illustrated the importance of NPY for the regulation of renal sodium handling. We also propose that the renal tubule cell is a good model for studying the function and mechanisms of postjunctional Y₂ receptors.