SEVERE POLYARTHRITIS FOLLOWING CAMPYLOBACTER ENTERITIS IN A 12-YEAR-OLD BOY

Abstract. Békássy, N. A., Enell, H. and Schalén, C. (Departments of Paediatrics and Medical Microbiology, University Hospital, Lund, Sweden). Severe polyarthritis following Campylobacter enteritis in a 12-year-old boy. Acta Paediatr Scand, 69: 269, 1980.—A severe attack of acute polyarthritis following a verified Campylobacter jejuni enteritis is described in a 12-year-old boy. The patient possesses the HLA-B27 antigen—often found in postinfectious arthritis following acute enteric infections. The ASO titre showed a significant rise, but other serological findings and the clinical course made streptococcal infection unlikely. Investigations to elucidate cross-reactivity between the two micro-organisms turned out negative.     

SUCCESSFUL TREATMENT OF ASPERGILLUS BRAIN ABSCESS IN A CHILD WITH ACUTE LYMPHOBLASTIC LEUKEMIA AND LIVER FAILURE

Invasive fungal infection continues to pose a significant threat to immunocompromised patients, with cerebral aspergillosis being among the most feared ones. The authors describe an adolescent girl with acute lymphoblastic leukemia (ALL) with subsequent acute liver failure, who developed an aspergillus brain abscess. The patient was treated with combined antifungal therapy using amphotericin B local instillation, prolonged systemic amphotericin B colloidal dispersion along with vinca alkaloids-containing chemotherapy, followed by neurosurgical débridement and oral voriconazole in the setting of ongoing antileukemic maintenance chemotherapy. Her ALL remains now in complete remission 30 months from diagnosis, with no evidence of fungal infection.     

LEGIONNAIRES' DISEASE COMBINED WITH ERYTHEMA MULTIFORME IN A 3-YEAR-OLD BOY

ABSTRACT. Andersen, R., Bergan, T., Halvorsen, K., Kallings, I. and ørstavik, I. (Departments of Anaesthesia, Microbiology, and Paediatrics, Aker Hospital; Department of Microbiology, Ullevål, Oslo, Norway; and the State Bacteriological Laboratory, Stockholm, Sweden). Acta Paediatr Scand, 70:427, 1981.–A case of Legionnaires' disease (LD) is described in a 3-year-old boy. He had fulminant disease with typical signs like bilateral pneumonia, gastrointestinal symptoms, and somnolence indicating involvement of the central nervous system. There was no premorbidity. An outstanding development was erythema multiforme, which has never previously been described in LD. The basic disease was caused by Legionella pneumophila . This is evidenced by specific serum IgM at admission and a subsequent significant rise in titers against L. pneumophila . At an early stage respiratory syncytial virus (RSV) was isolated from the patient's throat, although there was no antiviral serological response at the outset of erythema multiforme or 3 weeks after onset of disease; anti-RSV appeared later. The prolonged course of the disease can be explained by the successive occurrence of two infections. The possibility that the virus could have contributed to the development of erythema multiforme cannot be ruled out. The role of concomitant medication cannot be separated out, but on the basis of general knowledge of their immuno-genicity and the fact that immunosuppressive cortisone was given at the time it is less likely that the antibiotics contributed significantly.     

ECTOPIC CERVICAL THYMUS IN A 12-YEAR-OLD BOY: A Case Report

Thecase of a 12-year-old boywith ectopic cervical thymus is reported. This is a rare differential diagnosis in cervical tumors in childhood. The clinical symptoms might present as complications; in rare cases malignant transformations have been reported. The diagnosis ectopic cervical thymus can be achieved only histologically. Due to possible malignant transformation, it is mandatory to excise this thymus tumor totally.     

PRESUMED DISSEMINATED BCG IN A BOY WITH CHRONIC GRANULOMATOUS DISEASE OF CHILDHOOD

ABSTRACT. Verronen, P. (Department of Paediatdcs, Central Hospital, Tampere, Finland). Presumed disseminated BCG in a boy with chronic granulomatous disease of childhood. Acta Paediatr Scand 63: 627, 1974.—A male. child suffering from chronic granulomatous disease of childhood, confirmed by a Irucocyte bactericidal function test and a nitrobluc tetrazolium test, was given a BCG inoaulatlon in the neonatal pcrfod. He had draining lesions at the sites of vaccination and later miliary infiltration of the lungs, which responded favourably to antituberoulous therapy. Cultures for tb-bacilli were negative. An older sister is suspected to be a carrier of the trait responsible for chronlc granulomatous disease. Her sole clinlcal manifestation was miliary infiltration of the lungs at the age of 3 ⁹/₁₂ years.     

SPONTANEOUS RESOLUTION OF A SINGLE LESION OF MYELOID LEUKEMIA CUTIS IN AN INFANT: Case Report and Discussion

Though infantile leukemia has a historically poor prognosis, there may be a subset of patients with cutaneous disease whose disease will resolve without therapy. The authors report a case of infantile leukemia cutis who presented with a single subcutaneous chloroma that spontaneously resolved over the course of several weeks and who remains without evidence of disease nearly two years later. After reviewing the literature of congenital leukemia cutis, the authors conclude that withholding chemotherapy in infants with cutaneous myeloid leukemia in the absence of known negative prognostic factors (MLL or BCR-ABL translocations) or progressive disease is clinically indicated.     

SUCCESSFUL TREATMENT OF ASPERGILLUS BRAIN ABSCESS IN A CHILD WITH ACUTE LYMPHOBLASTIC LEUKEMIA

Cerebral aspergillosis carries a high mortality in immunocompromised patients. However, favorable outcome can be achieved by the prolonged use of antifungal agents and the maintenance of adequate drug levels. The authors report a 2-year-old girl who developed an aspergillus brain abscess during treatment for acute lymphoblastic leukemia. Predisposing factors for thefungal infection and details of the antifungal therapy are described. Prolonged treatment with AmBisome and 5-flucytosine successfully eradicated the lesion, but the girl's antileukemic therapy was compromised due to the infection. She developed a central nervous system and bone marrow relapse 9 and 15 months, respectively, after the initial presentation. The report emphasizes the need for further consideration of effective, long-term anti fungal prophylaxis and a careful balance between aggressive treatment for severe infection and antileukemic therapy.     

AN UNUSUAL CAUSE OF BREAST ENLARGEMENT IN A 5-YEAR-OLD BOY

A 5-year-old male developed painless enlargement of the left breast and was found to have a soft, mobile, and nontender swelling that was excised. Histological examination revealed a galactocele, which is a very rare cause of breast enlargement in male infants and children.     

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.     

COMPARISION OF THE APOPTOTIC EFFECTS ON LYMPHOBLASTS AND ON INCREASE OF MYELOID LINEAGE CELLS OF A SHORT-TIME,HIGH-DOSE METHYLPREDNISOLONE AND THE CONVENTIONAL-DOSE PREDNISOLONE TREATMENTS IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA

The authors compare the apoptotic effect on the lymphoblasts and the proliferative effect on the myeloid lineage cells of a short-course high-dose methylprednisolone (HDMP) and the conventional-dose prednisolone treatments in children with acute lymphoblastic leukemia (ALL). The patients were divided into 2 groups. Group I (n = 10) received HDMP (30 mg/kg/day for 7 days) in a single dose before 6 a.m. perorally. Group II (n = 10) received prednisolone (2 mg/kg/day for 7 days) in 3 doses. The apoptotic percentages of lymphpblasts and the percentages of blasts and myeloid lineage cells were determined after performing the bone marrow aspiration (BMA) at diagnosis on the 0th, 3rd, and 7th days of the treatments in all patients. The mean apoptotic percentages of the lymphoblasts on the 3rd day were significantly higher than those on the 0th and 7th days in both groups (p <. 05). The highest apoptosis was determined on the 3rd day in group I. The mean percentages of the blast cells on the 7th day were significantly lower than those on the 0th and the 3rd days in both groups (p <. 05). The lowest lymphoblast percentage was determined on the 7th day in group I. The mean percentages of the CD₁₃⁺ and CD₃₃⁺ cells on the 7th day were significantly higher than those on the 0th and the 3rd days in both groups (p <. 05). The highest percentages of the CD₁₃⁺ and CD₃₃⁺ cells were found on the 7th day in group I. Prednisolone and HDMP showed no proliferative effect on the CD₁₄⁺ cells. These findings indicate that a short-course HDMP treatment shows a more effective apoptosis on the lymphoblasts and on the increase of the myeloid lineage cells when compared to the prednisolone treatment. The authors suggest that HDMP may be used in the treatment of patients with ALL instead of prednisolone.     

ANALYSIS OF THE COMPONENTS OF THE HYPERCALCAEMIA IN A 14-YEAR-OLD BOY FOLLOWING PROLONGED IMMOBILISATION

ABSTRACT. A fourteen-year-old boy developed severe symptomatic hypercalcaemia following prolonged supine bed rest for major trauma. Treatment consisted initially of intravenous saline and frusemide together with oral phosphate, followed by intramuscular salmon calcitonin. Only after mobilisation and weight bearing was a sustained fall of plasma calcium to normal achieved. Plasma immunoreactive parathorntone levels using both N-terminal and mixed terminal specific antisera were always undetectable and urinary cyclic AMP levels were within the normal range throughout. However, before mobilisation, the tubular reabsorption of phosphate was reduced and that of calcium was increased thus indirectly suggesting increased parathormone activity. The hypercalcaemia was due to a combination of increased calcium release from bone and increased tubular reabsorption. We suggest that a factor other than parathormone is responsible for altered tubular handling of calcium and phosphate which develop following prolonged immobilisation in these patients.     

INVASIVE ASPERGILLOSIS OF THE SMALL BOWEL IN AN INFANT WITH ACUTE MYELOID LEUKEMIA AND INTESTINAL OBSTRUCTION

Acute myeloid leukemia was diagnosed in an infant with fever and pancytopenia. Intestinal obstruction was present at diagnosis and laparotomy performed after failure of conservative management demonstrated leukemic infiltration of the resected terminal ileum. Fever and intestinal obstruction persisted, necessitating a second ileal resection, which revealed invasive aspergillosis. Subsequent retrospective analysis identified occasional fungal hyphae in the initial resection specimen. With the combination of intensive chemotherapy and aggressive prolonged antifungal therapy the child survived. Invasive aspergillosis may unusually present at nonpulmonary sites at initial presentation of acute leukemia. Microbiological or histological diagnosis is needed to guide aggressive appropriate management.     

TEN CASES OF SO-CALLED LONG SURVIVAL IN CHlLDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA

Abstract. Armata, J. Cyklis, R. and Wyszkowski, J. (Institute of Paediatrics, Medical Academy in Cracow, Cracow, Poland). Ten cases of so-called long survival in children with acute lymphoblastic leukemia. Acta Paediatr Scand 63: 369, 1974.–Ten cases of acute lymphoblastic leukemia are presented, in children all of whom survived more than 4 years. WBC and blast cell counts were registrered at low levels at the onset of the disease and at subsequent bone marrow relapses. Extramedullary leukemia occurred after 2 years (average) in bone marrow remission, which did not predict early bone marrow relapse. From 1962 to 1970 three treatment regimens for acute leukemia were compared; the best results depended on the intensity of treatment during the first remission.     

THE USE OF A CARBOHYDRATE-FREE FEEDING FORMULA IN AUSTRALIAN ABORIGINAL CHILDREN WITH CHRONIC DIARRHOEA

A newly devised carbohydrate-free feeding fromula (CF1, Nestlé) was used in 48 Aboriginalinfants and children with malnutrition and diarrhoeal illnesses. It was effective in rapidly reducing diarrhoea in most patients, and in some requiring intravenous feeding it was a useful additional source of calories. Such a formula should prove useful in malnourished children with diarrhoea, and a simple, standardized regime is put forward for trial in ares where medical and nursing supervision are in short supply.     

GENITAL ULCERS AFTER TREATMENT WITH ALL-trans-RETINOIC ACID IN A CHILD WITH ACUTE PROMYELOCYTIC LEUKEMIA

All-trans-retinoic acid (ATRA) has been shown to improve the outcome of patients with acute promyelocytic leukemia (APL). However, various adverse effects of ATRA treatment have been noted, such as scrotal and genital ulcers in adult patients. The authors report genital ulcers that developed in a child with APL after ATRA treatment. An 8-year-old girl with APL was treated with ATRA for 21 days and after discontinuation of ATRA treatment she developed genital ulcers. Systemic and local antibiotic pomades were applied and the lesions improved within 15 days. In conclusion, genital ulcers may develop in children with APL as a complication of ATRA treatment and physicians should be alert to this possibility.     

MULTIPLE CONGENITAL ABNORMALITIES IN A NEWBORN BOY ASSOCIATED WITH MATERNAL USE OF FLUPHENAZINE ENANTHATE AND OTHER DRUGS DURING PREGNANCY

ABSTRACT. A boy was born with a short sloping forehead, wide metopic suture, persistent metopic fontanelle, telecanthus, ocular hypertelorism, nystagmoid eye movements, bilateral cleft lip and palate, imperforate anus, rectourethral fistula, bind scrotum and an unusual penis with hypospadias. The neutrophil polymorphs had numerous nuclear projections. The pregnancy was complicated by chronic maternal schizophrenia, severe toxaemia and maternal use of fluphenazine enanthate, dicyclomine hydrochloride, doxylamine succinate, pyridoxine hydrochloride and other drugs. Details of mother's illness, pregnancy and drug usage are presented with the clinical findings and investigations.     

BCG VACCINATION AS A CAUSE OF MULTIFOCAL OSTEOMYELITIS IN A 12-YEAR-OLD GIRL

ABSTRACT. A 12-year-old girl with multifocal skeletal sclerosis has been investigated. She was BCG vaccinated at birth and developed regional lymphadenitis in her left groin at two months of age. She was healthy until approximately 10 years of age, after which deficient height and weight gain occurred. BCG-itis was diagnosed in skeleton lesions. No evidence of immunodeficiency was found in the patient. After antituberculous treatment, the skeleton lesions, previously seen at bone scan, had disappeared. There has been a dramatic effect on her weight gain, growth and general well-being.     

OCCURRENCE OF OLIGOCLONAL GAMMAGLOBULIN IN THE CSF OF CHILDREN WITH PROLONGED and CHRONIC CNS-INFECTIONS

Abstract. Siemes, H., Siegert, M. and Hanefeld, F. (Department of Paediatrics, Free University of Berlin, F.R.G.). Occurrence of oligoclonal gammaglobulin in the CSF of children with prolonged and chronic CNS-infections. Acta Paediatr Scand, 70: 91, 1981.–CSF-proteins of 1770 children and adolescents with different neurological diseases and of 75 controls were examined by zone electrophoresis in agarose gel electrophoresis and by immuno-fixation electrophoresis. The quantitative evaluation of the phoretograms by an analog computer revealed oligoclonal changes of the gamma-globulin profile in 53 patients with subacute or chronic CNS-infections and in 5 children with a medulloblastoma. Seventeen of 37 children with congenital infections had 1–5 oligoclonal gamma-fractions consisting of IgG. Five to seven oligoclonal IgG fractions were detected in each of 16 children with SSPE. All 6 adolescents with multiple sclerosis had 2–5 oligoclonal IgG fractions. One to five oligoclonal gamma-bands occurred transiently in the CSF of 4 children with prolonged meningoencephalitis caused by different viruses, in 3 children with a prolonged non-bacterial meningitis of probable viral origin, in 2 infants with prolonged bacterial meningitis after corticosteroid therapy, and in 1 child with prolonged bacterial meningitis during cytostatic therapy. Four to six oligoclonal gamma-subfractions were found at different times during progressive viral encephalitis that developed during maintenance therapy of acute lymphoblastic leukaemia, and in 2 patients with chronic meningoencephalitis of unknown origin. Since oligoclonal gamma-globulin was detected almost invariably in the CSF of patients with prolonged or chronic neurologic infections, this finding implies persistence of antigens in the CNS and pathologic invasion of lymphocytes with selective proliferation of antigen-stimulated clones.     

ISOLATED RELAPSE OF ACUTE LYMPHOBLASTIC LEUKEMIA IN THE BREAST OF A YOUNG FEMALE

A 20-year-old female developed a relapse of B-precursor acute lymphoblastic leukemia (ALL) as a mass in her left breast after 6 years of maintained continuous complete remission. No leukemic lesions were identified in other sites such as the bone marrow or cerebrospinal fluid. The relapsed leukemic cells in the breast revealed the same immunophenotypes (CD10⁺, CD19⁺, CD20⁺, HLA-DR⁺, CD34⁺) as those of the onset ALL cells in the bone marrow. A literature survey found 10 other cases of ALL relapse in the breast without bone marrow involvement, mostly consisting of adolescent girls. Including the present report, a total of 11 cases were analyzed; the onset ages of ALL were a median of 16.5 (range 5–50) years old and the ages of relapse in the breast a median of 20 (range 12–51) years old. Data suggest that, although rare, the breast could become one of the extramedullary relapse sites of ALL developed in adolescent girls.