Guillain-Barré syndrome associated with IgM anti-GM1 antibody followingCampylobacter jejuni enteritis

We report a 4-year-old girl diagnosed as having Guillain-Barré syndrome after infection by Penner serotype 19 ofCampylobacter jejuni. The patient had the HLA-B35 antigen. Neurological examination revealed distal-dominant weakness and intact sensation. Serial electrophysiological studies indicated that the predominant process was axonal degeneration involving motor nerves. An enzyme-linked immunosorbent assay revealed the presence of high titres of serum IgM antibodies to gangliosides G_M1 and G_M2. The IgM auto-antibody titres decreased concurrently with the clinical course of the illness and no switching from IgM to IgG secretion took place.     

IgG and IgM Antibody Responses to Pneumococcal Vaccination in Splenectomized Children and in Children Who Had Non-Operative Management of Splenic Rupture

ABSTRACT. The IgG and IgM type specific anticapsular pneumococcal antibody titres were studied with ELISA during one year following vaccination with a 14-valent pneumococcal polysaccharide vaccine in four different patient groups: 41 children splenectomized after a traumatic rupture, 16 splenectomized because of disease, 15 non-surgically managed after splenic rupture, and 19 healthy controls. Serum samples were obtained before vaccination and after 14, 60, 180, and 360 days. The IgG antibody responses were equally good in all of the patient groups, while the IgM antibody responses were less pronounced in the splenectomized. In the non-surgically managed patients, the IgM antibody titres after vaccination, as well as antibody responses per se, were significantly higher for all 12 pneumococcal types studied than in the patients who were splenectomized. Both the IgG and the IgM antibody titres were highest at 14 days after vaccination. However, while the IgG titres for most pneumococcal types remained elevated at 360 days, the IgM titres soon fell to prevaccination levels. The antibody response was less good for the types 3 and 6 A. The results indicate that the IgG antibody response to pneumococcal polysaccharide vaccination is unaffected in splenectomized individuals, but the IgM antibody response is decreased. In patients with a healed traumatically damaged spleen, both the IgG and IgM responses seem to be normal.     

IgG and IgM antibody responses to pneumococcal vaccination in splenectomized children and in children who had non-operative management of splenic rupture

The IgG and IgM type specific anticapsular pneumococcal antibody titres were studied with ELISA during one year following vaccination with a 14-valent pneumococcal polysaccharide vaccine in four different patient groups: 41 children splenectomized after a traumatic rupture, 16 splenectomized because of disease, 15 non-surgically managed after splenic rupture, and 19 healthy controls. Serum samples were obtained before vaccination and after 14, 60, 180, and 360 days. The IgG antibody responses were equally good in all of the patient groups, while the IgM antibody responses were less pronounced in the splenectomized. In the non-surgically managed patients, the IgM antibody titres after vaccination, as well as antibody responses per se, were significantly higher for all 12 pneumococcal types studied than in the patients who were splenectomized. Both the IgG and the IgM antibody titres were highest at 14 days after vaccination. However, while the IgG titres for most pneumococcal types remained elevated at 360 days, the IgM titres soon fell to prevaccination levels. The antibody response was less good for the types 3 and 6 A. The results indicate that the IgG antibody response to pneumococcal polysaccharide vaccination is unaffected in splenectomized individuals, but the IgM antibody response is decreased. In patients with a healed traumatically damaged spleen, both the IgG and IgM responses seem to be normal.     

Antibody‐mediated rejection after ABO‐incompatible pediatric living donor liver transplantation for propionic acidemia: A case report

We herein present the case of a four‐yr‐old boy with PA who developed AMR after ABO‐incompatible LDLT despite undergoing B cell desensitization using rituximab. Although the CD19+ lymphocyte count decreased to 0.1% nine days after the administration of rituximab, he developed a high fever which was accompanied by arthralgia due to a streptococcal infection 13 days after rituximab prophylaxis. After the clearance of the infection, he underwent ABO‐incompatible LDLT 36 days after the administration of rituximab. The CD19+ lymphocyte count just prior to LDLT was 1.2%. He developed AMR five days after LDLT, and the antidonor‐type IgM and IgG antibody titers increased to 1:1024 and 1:1024, respectively. He was treated by plasma exchange, IVIG, steroid pulse therapy, and rituximab re‐administration; however, his liver dysfunction continued. Despite intensive treatment, he died due to complicated abdominal hernia, acute renal failure, and ARDS. This case suggests that a streptococcal infection may induce the activation of innate immune responses; thus, additional desensitization therapy should be considered prior to ABO‐incompatible LDLT if B cell reactivation is suspected.     

Vertical transmission of hepatitis A

Two hospital delivered full term newborn babies were detected to have cholestatic jaundice in the first week of life. They had raised liver enzyme levels, which gradually declined over a period of one month. Both babies were anti HAV IgM positive on 6th day of life in Case 1 and on 7th day of life in Case 2 respectively. Both the mothers had jaundice 20 and 26 days before delivery and had anti HAV IgM positivity two and three weeks prior to delivery in Case 1 and 2 respectively. Hepatitis A virus is not transmitted vertically from mother to baby. However, there are 3 such case reports in literature stating vertical transmission of HAV infection. We are reporting it in two neonates for the first time in India     

Deep Candida infection in child liver transplant recipients: serological diagnosis and incidence

Nineteen children who received 22 orthotopic liver grafts on 20 occasions were studied with regard to Candida infection. Serum samples were analysed to determine Candida, IgA, IgM and IgG antibodies and detect free C. albicans glucoprotein antigen. Five children (25%) had a confirmed deep C. albicans infection (DCI) during the first 2 weeks after transplantation. In all children with DCI, serology was positive, a median of 6 days (range 2–9 days) before Candida infection was verified by fungal culture, direct microscopy and/or autopsy. The positive predictive values for Candida IgG, IgM and IgA antibodies in children with DCI were 100%, 78% and 100%, respectively, and for free C. albicans antigen, 45%. Pathological titres of IgM and IgA antibodies against Candida before liver transplantation were present in three of four children who later developed a DCI and in no child without infection. In conclusion, regular screening by Candida serology is recommended both before and after liver transplantation.     

Acute disseminated encephalomyelitis after allogeneic bone marrow transplantation for pure red cell aplasia – A case report and review of the literature

ADEM is a rare inflammatory demyelinating disease of the CNS, which usually presents after a viral infection or a vaccination. We report a 15‐yr‐old boy who was diagnosed with ADEM after an HLA‐identical sibling allogeneic BMT for transfusion‐dependent PRCA. His course was complicated with GVHD affecting the skin and lungs. Five months post‐BMT, he developed neurological symptoms including sudden mental status alteration, dysarthria, facial nerve palsy, and acute paraplegia. The MRI revealed multifocal hyperintense lesions mainly in the subcortical white matter of the cerebrum, the brainstem, the basal ganglia, and the thalami. CSF examination was normal. There was no laboratory evidence of infection. The typical MRI findings and an acute monophasic clinical course were consistent with the diagnosis of ADEM. Clinical and radiological improvement was observed after treatment with high‐dose steroids and IVIG. Complete neurologic recovery was achieved six months after the onset of symptoms. We present a rare case of ADEM post‐BMT and review of the literature.     

Leigh's subacute necrotizing encephalomyelopathy due to decreased activity of the pyruvate dehydrogenase complex

A new patient with Leigh's syndrome (subacute necrotizing encephalomyelopathy due to pyruvate dehydrogenase complex deficiency) is presented. A Turkish boy of consanguinously married healthy parents developed progressive muscle weakness since infancy. At the age of 3 years he was unable to sit, stand or walk. Clinical examination showed general muscle weakness, hypotonia, muscle hypotrophy, bilateral ptosis, partial bilateral external ophthalmoplegia, nystagmus, intention tremor and hypoactive tendon reflexes. The EEG showed diffuse slowing, the cerebral CT scan disclosed mild hydrocephalus e vacuo. Motor nerve conduction velocity was slightly decreased, the EMG revealed signs of neuropathy. In the biopsied muscle only a mild hypotrophy of type 2 fibres was found, no abnormal mitochondria could be detected. The sural nerve was slightly abnormal: loss of large myelinated axons, loss of unmyelinated nerves. CSF protein was elevated to 80 mg/dl, protein electrophoresis revealed the pattern of markedly impaired blood-CSF barrier. Serum lactate and pyruvate were permanently elevated. In the urine the excretion of alanine was raised. The clinical state deteriorated during intercurrent infections; somnolence, vomiting and Cheyne-Stoke's respiration occurred. At the age of 3 1/2 years the child died of pneumonia. In the liver tissue a decreased activity of the pyruvate dehydrogenase complex was found. Neuropathological examination of the brain demonstrated wide-spread changes of Leigh's spongiform encephalopathy. Several enzyme deficiencies have hitherto been associated with Leigh's syndrome: This patients confirms earlier findings that a subgroup of Leigh's syndrome is caused by pyruvate dehydrogenase complex deficiency.     

IgG and IgM antibodies to viral glycoproteins in respiratory syncytial virus infections of graded severity.

Serum antibodies to the fusion (F) and large glycoprotein (G) of respiratory syncytial virus in the serum of 57 infected infants were measured by enzyme linked immunosorbent assay (ELISA). Most serum samples taken at the time of admission to hospital contained antibodies to both glycoproteins, and overall there was no significant evidence of a selective deficiency of antibody to either viral antigen. Less than a quarter of the infants showed rising IgG antibody titres to either glycoprotein after infection, whereas over threequarters produced an IgM response. There was a significant correlation between IgG response to viral glycoproteins and the age of the infant. The correlation of age with the IgM response was less pronounced, and there was no correlation between serum IgG antibody derived transplancentally in the acute phase of infection and IgM response to either glycoprotein. Neither IgG or IgM responses correlated with a clinical assessment of the severity of infection in the infants. IgM responses, however, were weakly correlated with reduced secretion of infectious virus in the upper respiratory tract.     

Relevance of vesico-ureteric reflux in development of lipid a antibodies in recurrent urinary tract infections in children — a preliminary study

The serum titres of IgG and IgM antibodies to lipid A were measured in 24 children with chronic pyelonephritis (PN), 55 with recurrent lower urinary tract infections (LUTI), 13 with gram-negative sepsis (S), and in 50 control children using an enzyme-linked immunosorbent assay (ELISA). Children ranged in age from 1 month-17 years. Patients with PN were differentiated by the presence or absence of an acute infectious episode and/or vesico-ureteric reflux (VUR). During an acute episode in PN and LUTI, IgG titres were significantly higher than in controls, but only PN patients with an acute infectious episode also had significantly elevated IgM titres. Overall, children with LUTI showed a significantly lower frequency of detectable IgG lipid A antibodies (27%) than in PN (63%). In PN children with VUR not accompanied by an infectious episode, lipid A antibody was found at relatively low titres, while an episode not accompanied by VUR displayed significantly elevated IgG titres, and an episode accompanied by VUR showed elevation of both IgG and IgM anti-lipid A antibody titres.Abbreviations UTI urinary tract infections - LUTI lower UTI - LUTI₁ LUTI with acute infectious episode - LUTI₂ LUTI without acute infectious epsisode - PN pyelonephritis - PN₁ PN with VUR but no acute infectious episode - PN₂ PN with acute infectious episode but no VUR - PN₃ PN with VUR and acute infectious episode - VUR vesico-ureteric reflux - S sepsis - ELISA enzyme-linked immunosorvent assay - LPS lipopolysaccharide     

Nephrotic syndrome associated with Toxocara canis infection

This case report describes nephrotic syndrome in a 7-year-old boy coincident with Toxocara canis infection. This rare association was confirmed by elevated Toxocara-specific IgM titres. Treatment with corticosteroids resulted in remission of renal symptoms as well as abatement of the T. canis infection. The relationship between T. canis infection and glomerular disease is still unclear; nephrotic syndrome may be another manifestation of T. canis infection.     

Deep Candida infection in children with leukaemia: clinical presentations, diagnosis and outcome

Objectives : To analyse the clinical features associated with deep Candida infection (DCI) and the outcome in children with leukaemia, and to evaluate various diagnostic methods. Materials and methods : Serum samples were analysed to determine Candida IgA, IgM and IgG antibodies and detect free C. albicans glucoprotein antigen and C. enolase antigen in eight children who had nine episodes of DCI and six with suspected DCI. Results : DCI occurred shortly after the leukaemia diagnosis (median 40 days) or after the leukaemia relapse (median 30 days). Children with DCI had fever (100%), skin lesions/exanthema (45%), oral thrush (45%), oesophagitis (22%) and laryngo-tracheitis (22%). Candida endocarditis, arthritis and hepatic candidosis were diagnosed in one patient each. Two children with disseminated candidosis died in leukaemia relapse. In patients with C. albicans infections serology had a sensitivity of 83%. However, in patients with C. parapsilosis infection antibody detection was negative. As the patients were cured of their Candida infection, the IgG antibodies disappeared and the IgM and IgA antibodies fell within the normal range for age. Conclusion : DCI in children occurs shortly after the leukaemia diagnosis or shortly after relapse of leukaemia. The clinical features are many. Candida serology may help to diagnose or confirm DCI. The dynamics of antibody titres may help to establish the efficacy of antifungal treatment.     

Rapidly fatal acute bacterial myocarditis in a nonneutropenic child with acute lymphoblastic leukemia in remission

The authors report a fatal case of acute bacterial myocarditis in a nonneutropenic child with acute lymphoblastic leukemia. She was admitted to the hospital with a urinary tract infection resulting from and remained persistently febrile despite resolution of the infection. On hospital day 4 signs of acute cardiac failure developed. Despite aggressive resuscitation measures, she died. Pathologic examination revealed the cause of death to be bacterial myocarditis. In addition, she was found to have a generalized decrease in her serum immunoglobulin levels. Acute bacterial myocarditis in patients with malignancy has been rarely reported. The rapid clinical deterioration and death in the patient in this report is particularly interesting.     

Severe anemia due to B19 parvovirus infection in children with acute leukemia in remission

Two children developed severe anemia with reticulocytopenia while on maintenance chemotherapy for acute lymphoblastic leukemia. Bone marrow examination revealed marked erythroid hypoplasia and giant megaloblasts without evidence of relapse. One patient had evidence of B19 Parvoviremia at the time of severe anemia but failed to produce anti B19 antibodies. Despite the failure to mount an antibody response, the patient had no recurrence of viremia or anemia during the two years of follow-up after the infection. The other patient had no evidence of viremia or elevated anti-B19 antibodies at the time of anemia. However, when his serum was tested 16 months after the episode of anemia, he had elevated IgG and IgM antibodies against B19 parvovirus. The patient did not experience recurrent viremia or anemia over a two year period. Thus, patients with leukemia are at risk to develop severe anemia when infected with B19 parvovirus.     

Antibodies against some bacterial antigens in children

The prevalence of bacterial antibodies was determined in 173 children aged 0–15 years. The prevalence of IgG Borrelia burgdorferi antibodies in titres > 500 in children less than 8 years of age was 6% while none of the older children had these antibodies in titres > 400. IgG Helicobacter pylori antibodies were detected only in children older than 6 years of age, with a prevalence of 6.5%, as were IgA H. pylori antibodies, with a prevalence of 3.7%. The prevalence of high-titre IgG Campylobacter jejuni antibodies was 1.2%, that of IgA 1.8% and IgM 1.2%. The prevalence of high-titre (> 500 IU/ml) antistreptolysin O was 3%, that of antistaphylolysin-alpha (≥ 4 IU/ml) 2% and that of antiteichoic acid antibodies (titre 2) 2%. Low-titre Yersinia antibodies were detected in 2%. High-titre Bordetella pertussis antibodies were detected in 6% of recently vaccinated children and in 8% of children in their first years of school. In the latter, high-titre antibodies were mainly of the IgM and IgA classes. Altogether 35 children tested positive for bacterial antibodies other than Bordetella pertussis antibodies. Clinical evaluation revealed a possible infection, suggested by the antibody, in 5 (3%) of the children. Two (vaccinated) children had evidence of whooping cough. Eight of the 35 children with high-titre bacterial antibodies (23%) also had elevated levels of autoantibodies (but not autoimmune diseases).     

Immunglobulin concentrations in cerebrospinal fluid of children. Studies regarding age dependency and changes during inflammatory disease of CNS (author's transl)

75 children of different age groups were examined for immunglobulin concentrations in spinal fluid. No statistical significant differences were found between the age groups a) 1 month to 1 year, b) 1 year to 5 years and c) 5 years to 12 years. The average level for IgG was 1,41 +/- 0,8 mg%, for total protein 34,8 +/- 9,6 mg%. IgA and IgM were detectable not or only in small quantities even when the spinal fluid was concentrated. Furthermore 21 children with viral and 9 with purulent meningitis and 2 with encephalitis were examined. Patients with meningitis had a distinct increase in IgG, IgA and IgM. The increase was more pronounced in purulent than in viral meningitis. Children with encephalitis had an increase of IgG and in one case an increase of IgA. Continous examination of immunglobulins in spinal fluid from a child with inoperable hydrocephalus revealed a steady increase of all 3 immunglobulins which was probably a result of elevated vascular permeability.     

Myocardial fibrosis — a rare complication in patients with cystic fibrosis

We report a 10-month-old male infant who was admitted to our hospital with a history of failure to thrive and bulky stools. On examination, he was dystrophic and had a protruding abdomen, but he was well oxygenated and his lungs were clear on auscultation. A tachycardia of 145 beats per min and radiological evidence of cardiomegaly indicated involvement of the heart, but an ECG failed to show signs of myocarditis or cardiac hypertrophy. An elevated sweat chloride concentration of 141 mEq/l confirmed the diagnosis of cystic fibrosis (CF). Molecular analysis revealed heterozygosity for the common mutation delta F₅₀₈. He died unexpectedly of a sudden cardiac arrest 2 days later. Autopsy revealed scattered myocardial necrosis and fibrosis. Some 50 documented cases of myocardial fibrosis in infants with CF have been reported. Suggested causes such as malnourishment and hypovitaminosis remain speculative as systematic studies have yet to be done.     

Acute disseminated encephalomyelitis associated with herpes virus infection: a case report

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system, also called post-infectious encephalitis; it is triggered by an autoimmune mechanism and follows an infection or a vaccination after a free interval of 2 to 30 days. We report a case of ADEM in a 4-year-old girl, who was diagnosed based on the data from a brain MRI, which revealed multiple demyelinization foci in the periventricular white matter, the semi-oval centers, and the thalamic regions, both bilaterally and symmetrically. The clinical course was characterized by complete recovery 10 days after steroid therapy. In the literature, more than the half of the patients treated for ADEM had a good prognosis, with recovery and no sequelae. Clinical improvement is generally noted in the hours or days following the initiation of treatment. However, in the most severe cases of ADEM, the most frequent neurological sequelae consist in focal deficiencies of the limbs and ataxia or visual disorders. Cognitive and behavioral disorders are noted in 6 to 50% of pediatric patients.