Lymphocytic Infundibuloneurohypophysitis Diagnosis Owing to Positive Anti-rabphilin-3A Antibody Test Result in an 8-year-old Boy with Early-onset Central Diabetes Insipidus.

Childhood-onset lymphocytic infundibuloneurohypophysitis (LINH) has rarely been reported. Pathological evaluation via pituitary biopsy is necessary for a definitive diagnosis of LINH. However, pituitary biopsy is a highly invasive procedure. Recently, anti-rabphilin-3A antibody (RPH3A-Ab) has been reported as a promising diagnostic marker for LINH in adults; however, there are few such reports in the pediatric population. We report the case of an 8-year-old boy with central diabetes insipidus (CDI) who was clinically diagnosed with LINH based on RPH3A-Ab positivity. He was diagnosed with CDI using a water deprivation test combined with desmopressin administration. Serum and cerebrospinal fluid tumor markers were negative, and T1-weighted magnetic resonance imaging (MRI) revealed the absence of high signal intensity in the posterior pituitary gland and an enlarged pituitary stalk. Anterior pituitary function tests revealed no abnormalities. No pituitary biopsy was performed because of its invasive nature, and desmopressin treatment was initiated. Three months after CDI onset, the patient tested positive for RPH3A-Ab. MRI performed 9 months after CDI onset revealed amelioration of the pituitary stalk enlargement, and the clinical course corroborated our diagnosis of LINH. RPH3A-Ab may be useful as an early diagnostic tool for LINH in the pediatric population.

Hyperosmolar hyperglycemic syndrome induced by diazoxide and furosemide in a 5-year-old girl.

Hyperglycemia and hyperosmolar hyperglycemic syndrome (HHS) are the rare adverse effects of diazoxide. Furosemide has been reported to worsen glucose tolerance and cause HHS. A 5-yr-old girl presented to the emergency department with complaints of tachycardia, polyuria, and lethargy for 1 wk prior to hospitalization. She was treated with two diuretics for aortic valve reflux disease and diazoxide for congenital hyperinsulinemia. She was diagnosed with HHS based on her serum glucose level of 529 mg/dL and serum osmotic pressure of 357 mOsm/kg. There were no findings suggestive of new-onset diabetes mellitus. She had fever on admission and, was diagnosed with a urinary tract infection. The blood diazoxide level at the time of hospitalization was 25 µg/dL. Diazoxide use, even in patients with low diazoxide levels, may cause hyperglycemia. Patients on diuretics and diazoxide must be carefully monitored, considering the risk of developing HHS.

Transient Pseudohypoaldosteronism Secondary to Group B Streptococcus Pyelonephritis.

Secondary pseudohypoaldosteronism is a condition characterized by aldosterone resistance in renal tubules. It is highly associated with urinary tract infection and urinary tract malformations. Only a few cases of pseudohypoaldosteronism secondary to group B Streptococcus pyelonephritis have been reported to date. A four-month-old boy developed poor sucking and weight loss, and his laboratory test results revealed hyponatremia, hyperkalemia, renal dysfunction, high anion gap metabolic acidosis, pyuria, and hydronephrosis. Laboratory tests including urinalysis confirmed the diagnosis of pseudohypoaldosteronism secondary to group B Streptococcus. He was treated with intravenous normal saline and antimicrobial therapy. Electrolyte disorders were addressed and he was discharged on the 10th day of hospitalization without any sequelae. Voiding cystourethrography performed after discharge showed bilateral grade 5 vesicoureteral reflux and intrarenal reflux in the right kidney. Transient pseudohypoaldosteronism is an important consideration in the differential diagnosis in infants with hyponatremia and hyperkalemia. A thorough evaluation for urinary tract malformations should be performed, including early abdominal ultrasonography and systemic management.

A rare cause of acute kidney injury with chronic myelomonocytic leukemia.

An 80-year-old man with myelodysplastic syndrome developed acute kidney injury (AKI) and peripheral blood monocyte-dominant leukocytosis. Glomerular disease was suspected from urinalysis, which showed proteinuria and microscopic hematuria with red cell casts. Eventually, he died of respiratory failure, after which a postmortem was performed. In the glomeruli, the extracapillary space was filled with numerous mononuclear cells and erythrocytes. At first interpretation, the glomerular findings appeared to represent cellular crescents. However, immunostaining revealed that the extracapillary mononuclear cells were in fact leukemic cells. Furthermore, tubular injury due to marked accumulation of lysozyme was also recognized together with infiltration of leukemic cells in the interstitium. The diagnosis of kidney infiltration by chronic myelomonocytic leukemia (CMML) and lysozyme-induced tubular injury was eventually made. Our case is the first report showing extracapillary infiltration of leukemic cells by immunostaining. In addition, lysozyme-induced tubular injury is a forgotten cause of kidney injury in patients with CMML. This case teaches us the rare and forgotten causes of AKI with CMML.

The effect of elapsed time following alumina blasting on adhesion of CAD/CAM resin block to dentin.

The aim of this study was to evaluate the time elapsed bond strength of the CAD/CAM resin block (CRB) to bovine dentin after alumina blasting. CRB (KATANA AVENCIA BLOCK) slices were ground with #600-SiC paper and divided into three groups according to alumina blasting pressure -0.1 MPa, 0.2 MPa or untreated- and then divided into two subgroups according to the time elapsed after alumina blasting -same-day or one-week "dry-storage" at controlled laboratory conditions before cementation. The CRB slices were then cemented to bovine dentin with Panavia V5 (Kuraray Noritake Dental), and divided into two subgroups -light curing or chemical curing. After 24 h storage in distilled water at 37°C, the specimens were then subjected to micro-tensile bond strength (µTBS) testing. One-week group showed a significant decrease in µTBS. The µTBS values showed that CRBs must be cemented with light curing immediately after alumina blasting at 0.1 or 0.2 MPa to obtain a stable adhesion.

Effect of alumina-blasting pressure on adhesion of CAD/CAM resin block to dentin.

The aim of this study was to evaluate how alumina-blasting pressure affects the bond strength of CAD/CAM resin blocks (CRBs) to bovine dentin using two different types of resin cements. CRB slices were divided into three groups by alumina-blasting pressure, namely, untreated and 0.1 MPa and 0.2 MPa, and further divided into three subgroups by combination of CRBs surface treatment and types of cement: Scotchbond Universal and RelyX ultimate (RXB), Scotchbond Universal and RelyX unicem2 (U2B), ceramic primer and RelyX unicem2 (U2C). The CRB slices were then cemented to bovine dentin, microtensile bond strength test was performed and evaluated. Regardless of the alumina-blasting pressure, RXB group have the highest µTBS and bond strength tends to increase with increasing alumina-blasting pressure. Alumina-blasting to CRB surface by at an appropriate pressure and use of conventional resin cement were required to obtain strong adhesion with the tooth structure.

Polyarthritis caused by methimazole in two Japanese patients with graves' disease.

In many countries, methimazole (MMI) therapy is the first-line treatment in children with Graves' disease (GD). The rate of side effects of antithyroid drugs (ATDs) in children has been reported to range between 6% and 35%. Of these side effects, polyarthritis is uncommon but serious, and can also develop as a part of the antineutrophil cytoplasmic antibody-associated vasculitis that is induced by ATDs. Here, we describe two GD girl patients aged 15 years and 11 years who developed polyarthritis. The onset of polyarthritis in these patients was 24 days and 28 days after the initiation of MMI therapy, respectively. MMI was suspected of causing the polyarthritis in the two patients and was withdrawn. The symptoms of polyarthritis disappeared rapidly following cessation of treatment. Subsequently, one patient was treated with 131I therapy and the other patient was subjected to thyroidectomy. Although it rarely occurs in pediatric GD patients, severe polyarthritis is a serious side effect of MMI and is an indication for prompt cessation of treatment.