Adverse reactions associated with long-term drug administration in Mycobacterium avium complex lung disease.

SETTING: The number of patients with non-tuberculous mycobacterial lung disease (NTM-LD) worldwide has been increasing. Mycobacterium avium complex lung disease (MAC-LD) accounts for 90% of NTM-LD. MAC-LD necessitates long-term treatment, but adverse reactions with long-term administration of drugs are poorly understood. OBJECTIVE: To evaluate adverse reactions with long-term administration of drugs for MAC-LD. DESIGN: We conducted a retrospective single-centre medical chart review of 364 patients administered two or more drugs between July 2010 and June 2015. RESULTS: The prevalence and median time to onset of adverse reactions were as follows: hepatotoxicity 19.5%, 55 days; leucocytopaenia 20.0%, 41 days; thrombocytopaenia 28.6%, 61.5 days; cutaneous reactions 9.3%, 30 days; ocular toxicity 7.7%, 278 days; and increase in serum creatinine 12.4%, 430.5 days. Multivariate analysis showed that rifampicin use was independently associated with thrombocytopaenia, and ethambutol use was independently associated with increases in serum creatinine. CONCLUSION: The main adverse reactions appeared within 3 months after start of treatment. Most patients were able to continue treatment with liver-supporting therapy, antihistamine agents or desensitisation therapy; however, ocular toxicity must be monitored for up to 1 year after start of treatment.

Competitive polymerase chain reaction for the determination of N-myc amplification in neuroblastoma: report of clinical cases.

If an unfavorable prognosis is suspected in neuroblastoma, decision on a treatment protocol should be based on the N-myc copy number (12). We already demonstrated that the newly developed competitive polymerase chain reaction (competitive PCR) is a promising method for the determination of the N-myc copy number (6), and have started to use this competitive PCR procedure in neuroblastoma patients, together with fine-needle biopsy in selected cases. Seven children were studied. In one infant of 5 months of age whose tumor was diagnosed before undergoing mass screening for neuroblastoma, the competitive PCR procedure was performed with a fine-needle biopsy, and after obtaining a negative report on N-myc amplification within 48 hours, a regular protocol of treatment could be started without delay. We report that competitive PCR is a rapid and accurate method for the determination of the N-myc copy number, requiring only a small amount of material, and anticipate that competitive PCR will become the procedure of choice for the determination of N-myc copy number in neuroblastoma.

Management of prenatally diagnosed congenital cystic adenomatoid malformation of the lung.

We have treated four prenatally diagnosed cases of extensive congenital cystic adenomatoid malformation (CCAM) of the lung. The first case in 1982 was associated with severe fetal hydrops. After thoracentesis at 31 weeks of gestation abruptio placentae occurred, and a female baby was delivered by cesarean section. She underwent a right lower lobectomy, but soon died. The second baby without hydrops, diagnosed as having CCAM at 26 weeks of gestation, was followed conservatively until full term. After birth, it was necessary to treat the baby boy with extracorporeal membrane oxygenation (ECMO), but he survived. The third baby with fetal hydrops had an indwelling drainage catheter inserted into the CCAM at 27 weeks of gestation. The hydrops subsided and the baby was delivered at 37 weeks of gestation. He was allowed to breathe spontaneously, but was intubated 16 hours after birth. A right lower lobectomy was successfully performed 24 hours after delivery. The fourth baby without fetal hydrops was followed conservatively until delivery. He underwent left lower lobectomy successfully on the 4th day of life. Although management of prenatally diagnosed CCAM varies among patients, insertion of an indwelling catheter into the cyst appears to be the treatment of choice if indicated; the catheter can be maintained for as long as 10 weeks, as shown in Case 3. Cases of CCAM without fetal hydrops should also be treated carefully, because persistent fetal circulation may occur postnatally.

A new technique applicable to pediatric laparoscopic surgery: abdominal wall 'area lifting' with subcutaneous wiring.

BACKGROUND: Recently, the authors developed a unique method of laparoscopic surgery without pneumoperitoneum: "area lifting of the abdominal wall with subcutaneous wiring." METHODS: In this gasless procedure, the anterior abdominal wall is pulled upward by a pair of wires placed subcutaneously and held by thick sutures for "hanger lifting." Simultaneous lifting of a pair of subcutaneous wires across the abdomen, produces a wide, roof-shaped intraabdominal space sufficient for laparoscopic surgical procedures. The practical aspects of this gasless technique, as well as the authors' limited experience with this method in 24 children, ranging from 8 days to 15 years of age is presented. These children have had various pathologies including splenomegaly, rectal prolapse, ovarian cyst, gall stone, adrenal neuroblastoma, and abdominal wall abscess. CONCLUSIONS: Gasless laparoscopic surgery with double subcutaneous wiring is safe for children including neonates and those with respiratory compromise because all operative procedures are performed under normal abdominal pressure. Because of the highly elastic abdominal wall musculature inherent in children, this selective area lifting of abdominal wall creates a relatively larger peritoneal volume than in adults.

Histopathologic findings of advanced neuroblastoma after intensive induction chemotherapy.

BACKGROUND: Histopathologic findings of advanced neuroblastoma after intensive induction chemotherapy have not been studied well. METHODS: In the present study, all of the surgical specimens from 19 patients who had advanced abdominal neuroblastoma and were pretreated intensively with the protocol of the Study Group of Japan were reviewed. The authors found that dissection of the contralateral lymph nodes is mandatory in advanced neuroblastoma when the goal is the complete dissection of the abdominal disease. Effects of chemotherapy were graded histologically according to the ratio of viable residual neuroblastoma tissue to total areas of the tumor, including neuroblastoma, ganglioneuroblastoma, ganglioneuroma, hemorrhage, necrosis and fibrosis, in five ranks from ( ) to (-). CONCLUSIONS: The newly introduced, highly cytotoxic regimen of the Japanese protocol, designated "A3," appears to be more effective histologically than the conventional regimen, designated "A1" or "new A1." Effects designated ( ) or (++) were prerequisites for survival in stage IV disease, but some stage III patients with the (+) effect survived.

Primary chemotherapy for children with rhabdomyosarcoma of the 'special pelvic' sites: is preservation of the bladder possible?

Twenty-one children with rhabdomyosarcoma involving the "special pelvic" sites, defined as such occurring in the bladder, prostate, vagina and uterus, were treated with primary surgery between 1969 and 1977, and with primary chemotherapy beginning in 1978. Among 11 patients in the latter group who were placed in Clinical Group III (according to the classification of the US Intergroup Rhabdomyosarcoma Study (IRS), six showed partial response (PR) and underwent tumorectomy by radical surgery an average of 6 months after the start of treatment, and three showed complete response (CR) and were treated with further chemotherapy in the hope that cystectomy could be avoided. However, in the latter group, the tumor recurred (39 months, 35 months, and 27 months later), and all eventually underwent total cystectomy. Seven of the nine long-term survivors underwent total cystectomy and have premanent urinary-cutaneous stomas. Two had tumor-free bladders, but function was impaired in one because of the effect of irradiation. Normal function was preserved in only one patient in the series, whose tumor was located at the dome of the bladder. To preserve bladder function in children with rhabdomyosarcoma in these sites, more effective forms of chemotherapy will be required.

[Surgical treatment of tracheobronchial lesions in children].

A total of 16 cases of tracheal and bronchial lesions such as bronchial tumor (4 cases), aspirated foreign body (5 cases), and tracheo-bronchial stenosis (7 cases) were surgically treated at the National Children's Hospital from 1965 to 1990. A mucoepidermoid tumor was excised via bronchotomy. Pulmonary lobectomy was indicated for another 2 mucoepidermoid tumors which arose from the middle and lower lobe bronchus. Pneumonectomy was indicated for a leiomyoma located at the stem of the left bronchus and showed extensive invasion into the bronchial wall. It is important to accurately diagnose the location and extent of the tumor by performing intraoperative bronchoscopy, bronchotomy, frozen section examination in order to avoid unnecessary pulmonary resection. Three aspirated foreign bodies (2 marking needles and a peanut) were removed via bronchotomy. Right lower lobectomy was performed in a patient who received an accidental injection of barium sulfate into the right lower bronchus. The barium remained for more than one year and caused recurrent pneumonia. A radiolucent toy plate aspirated into the left main bronchus of a 3-year-old girl was overlooked for more than 3 years. Suppurative changes of the left lung necessitated pneumonectomy. Tracheal resection with end-to-end anastomosis was carried out successfully in a 5-year-old patient suffering from segmental fibrogranulous stenosis of the mid-trachea. Extensive congenital stenosis in 6 patients was repaired by costal cartilage autograft, with a 67% mortality rate. Treatment of the associated cardiac anomalies and the possible postoperative complications such as anastomotic leakage, restenosis, exuberant granulation, and tracheomalacia are the major problems associated with this type of tracheoplasty.

Spontaneous regression of patient omphalomesenteric duct: from a fistula to Meckel's diverticulum.

A newborn with a patent omphalomesenteric duct is described in this report. This anomaly is a very rare one. Regression of the omphalomesenteric duct occurs as a normal embryonic event in the intrauterine life between the fifth and ninth week of gestation; however, in this case regression occurred spontaneously in the neonatal period and resulted in a Meckel's diverticulum with no connection with the umbilicus. This is the first case to be reported.

Treatment of disseminated hepatoblastoma involving bilateral lobes.

A 30-month-old boy was investigated because of a huge abdominal mass in the right upper abdomen. A computed tomography scan and celiac angiography showed that the tumor involved bilateral lobes of the liver. At first, for this disseminated and "unresectable" tumor we did liver biopsy and hepatic arterial catheterization. Through this catheter we started chemotherapy using THP-Adriamycin and cis-platinum. After three courses of chemotherapy, a second-look operation was performed and trisegmentectomy was done to remove the main tumor and metastases, including the left lobe and the right anterior segment. The trisegmentectomy was performed with success, and the boy's serum alpha-fetoprotein (AFP) remains normal 37 months after the start of treatment, indicating a probable permanent cure. Such cases have rarely been reported in the literature. Our experience in treating this patient would seem to encourage aggressive management of advanced hepatoblastoma in other patients.

Determination of plasma total renin level by RIA with a monoclonal antibody: value as a marker for nephroblastoma.

Plasma total renin is a new, useful marker for nephroblastoma, but the conventional method for determining its level is sophisticated and requires large blood samples. To develop a simpler technique, a monoclonal antibody specific for both inactive and active renin (inactive + active = total) was raised, and a radioimmunoassay (RIA) system was established. This monoclonal antibody stains only the juxtaglomerular apparatus; values determined by this RIA did not change before and after activation. So far, the RIA system has been applied to 136 samples from 92 patients. Plasma total renin levels were also determined with the conventional method: samples were activated, then renin activity was assayed by measuring angiotensin I. The coefficient of the data obtained by these two different techniques was 0.921 (P less than .01). Plasma total renin levels in patients with nephroblastoma were significantly increased (546.5 +/- 297.8 pg/mL) over those in patients with neuroblastoma (218.6 +/- 46.5 pg/mL) and in controls (165.8 +/- 67.5 pg/mL, P less than .01). After removal of Wilms' tumors, the levels decreased to normal when sampled every 2 weeks. It was concluded that a newly developed RIA system is more useful in determining plasma total renin levels in patients with nephroblastoma.

Idiopathic gastric volvulus in infancy and childhood.

Gastric volvulus is a rare condition in childhood. Most of the reported cases have been acute and secondary in type with predisposing factors. Between 1965 and 1988, 49 patients were treated at this institution for chronic idiopathic gastric volvulus. Patients were divided further into two groups according to age at admission. The main symptom was vomiting in the neonatal and infantile group, whereas it was abdominal distension, vomiting, weight loss, or constipation in the older age group. This clinical feature was different from that of acute gastric volvulus in which the symptoms resulted from gastric obstruction. Plain film of the abdomen showed no characteristic findings. Contrast study of upper gastrointestinal series showed findings similar to those of the acute volvulus, but the extent or degree was less significant. Conservative treatment was successfully undertaken for the patients in the neonatal and infantile group just by keeping them in prone position. The rationale for this method was demonstrated by the clinical and radiological improvement. Fundic gastropexy was performed in all of 18 patients of the older age group and in one of 31 patients of the neonatal and infantile group. The operative results were satisfactory except for one patient with mental retardation. In Japan, neonates or infants are customarily nursed in the supine position. This is presumed to be a reason why the chronic idiopathic gastric volvulus is frequently noticed.