Invasive group A streptococcal disease.

We report on two children who had presented in a poor clinical state after an initial bout of cough, sore throat and fever for a few days. Both of them had multisystemic involvement with fluid-refractory septic shock requiring ionotropic support, intubation and care in the paediatric intensive care unit. Recent significant rise in scarlet fever has led to a significant increase in the number of invasive group A streptococcal infections with increased mortality in paediatric patients. Both of them had co-infection with influenza, which could have led to an increased risk of invasive group A streptococcal (iGAS) infection. After prompt treatment, including early initiation of antibiotics, they both recovered well. To our knowledge, there are no reported cases of iGAS infection from the UK in any medical journal though the fatal cases have been reported to the health statistics department by various National Health Service trusts individually.

Co-occurrence of biphenotypic acute leukaemia, glucose 6-phosphate dehydrogenase deficiency and haemoglobin E trait in a single child.

Acute leukaemias occur as the result of clonal expansion subsequent to transformation and arrest at a normal differentiation stage of haematopoietic precursors, which commit to a single lineage, such as myeloid or B-lymphoid or T-lymphoid cells. Biphenotypic acute leukaemia (BAL) constitutes a biologically different group of leukaemia arising from a precursor stem cell and co-expressing more than one lineage specific marker. The present report describes a child with unusual co-occurrence of biphenotypic (B-precursor cell and Myeloid) acute leukaemia, haemoglobin E trait and glucose 6-phosphate dehydrogenase (G6-PD) deficiency. To the best of our knowledge, this constellation of haematological conditions in a single child has never been described before.

Cayler cardiofacial syndrome with situs inversus totalis.

Cayler cardiofacial syndrome is characterised by congenital unilateral hypoplasia of the depressor anguli oris muscle (DAOM) in association with congenital cardiac defects. Hypoplasia of this muscle causes inability to move one corner of the mouth downward and outward while crying or grimacing, giving rise to an 'asymmetric crying face' appearance. A variety of congenital cardiac defects have been described. Occasionally, other organ system anomalies may be additionally present. We present an instance of right-sided hypoplasia of the DAOM in a male newborn, which additionally had dextrocardia as a component of situs inversus totalis. To our knowledge, situs inversus totalis has not been previously documented as a part of this syndrome. Additionally, we reiterate that paediatricians need to be aware that this minor facial anomaly may be associated with severe internal organ system anomalies, with cardiac being most common.

Neonatal Brain Abscess due to Extended-Spectrum Beta-Lactamase Producing Klebsiella pneumoniae.

Klebsiella pneumoniae (K. pneumoniae) causing brain abscess in newborn infants is rare. Presented herein, is a 27-day-old male neonate who developed two frontal lobe abscesses in association with K. pneumoniae sepsis and meningitis. Antibiotic susceptibility testing utilizing the double-disk synergy method (Cefotaxime and Amoxycillin-Clavulanate) confirmed the extended spectrum beta-lactamase (ESBL) production by the isolate. He was treated simultaneously with antibiotics (Meropenem and Amikacin) and abscess aspiration through the anterior fontanelle, with less than satisfactory outcome. ESBL producing K. pneumoniae brain abscess in neonates is extremely rare in the English literature. Emperical carbapenems and aminoglycoside coverage in neonates with K. pneumoniae sepsis and brain abscess, especially in areas with high rate of ESBL producing bacteria may be warranted.

Necrotizing fasciitis following BCG vaccination.

We report a newborn with methicillin-resistant Staphylococcus aureus mediated necrotizing fasciitis after Bacilli-Calmette-Guerin vaccination. Radical debridement of the affected area coupled with twice daily surgical honey dressing and intravenous vancomycin and clindamycin resulted in satisfactory healing.

Transient unilateral oculomotor palsy and severe headache in childhood Kawasaki disease.

Transient affliction of the cranial nerves may at times be either the presenting feature or complication of otherwise uncomplicated Kawasaki disease (KD) in infants and children. The present report describes a 6 year 9 month old boy with classical KD who developed right-sided oculomotor nerve palsy (manifested by ipsilateral ptosis and medial rectus palsy) resulting in symptoms like severe nausea, intense frontal headache and double vision. The palsy resolved within 5 days of intravenous immunoglobulin therapy, with no residual ophthalmological abnormality at 6 weeks. Besides increased intracranial pressure, which commonly occurs during the course of KD in children, secondary to aseptic meningitis, intense headache in such children may have cranial nerve paresis as accentuating factors.

Childhood Plasmodium falciparum malaria complicated by acute pancreatitis.

Falciparum malaria is occasionally associated with multiple organ system complications. However, acute pancreatitis rarely occurs as a part of the spectrum. A 13-year-old boy presented with falciparum malaria complicated predominantly by acute pancreatitis. He recovered satisfactorily with supportive measures. To our knowledge, there are less than 10 such documented instances in the literature and they are mostly adults. Acute abdomen in Plasmodium falciparum infection may reveal pancreatitis which should be detected at the earliest.

Open and closed lip schizencephaly in Seckel syndrome: a case report.

Seckel syndrome (Online Mendelian Inheritance in Man database Number 210600) is the classic prototype of primordial bird-headed dwarfism. In addition to the characteristic craniofacial dysmorphism and skeletal defects, abnormalities of the cardiovascular, hematopoietic, endocrine, and central nervous systems are described. The full phenotypic spectrum of this clinically and genetically heterogeneous syndrome is yet to be delineated. Presented herein is a boy 2 years and 5 months old, with Seckel syndrome, born to second-degree consanguineous Muslim parents. In addition to the classic phenotype of the disorder, this patient had both, an open and a closed lip schizencephaly detected on cranial computed tomography (CT) scan. To our knowledge, the association of schizencephaly and Seckel syndrome is not described previously in the English language literature. In addition, presented briefly is a review of the anatomical cerebral cortical malformations associated with this syndrome.

Perinatal malaria and tuberculosis co-infection: a case report.

India remains endemic for both vivax malaria and tuberculosis. In spite of the high burden of tuberculosis in the country, reports on congenital tuberculosis in the literature are limited. We report herein an unusual instance of co-occurrence of perinatal falciparum malaria and tuberculosis in a 34-day-old female newborn, who presented with symptoms of sepsis. The diagnosis was based on the demonstration of Plasmodium falciparum on peripheral blood smear and tubercle bacilli in gastric aspirate samples. The maternal history for falciparum malaria was positive during her eighth month of pregnancy and the father was an open case of sputum smear-positive pulmonary tuberculosis. She responded dramatically to combined antimalarial and antitubercular chemotherapy. A search for combined etiologies in presumed 'sepsis' in the newborn, guided by history, physical examination, and laboratory investigations, is warranted.

Childhood cutaneous polyarteritis nodosa: an unusual presentation.

A 6-year-old boy presented with post-burn like cutaneous scars over the buttocks and the back of thighs, following skin ulceration. Cutaneous polyarteritis (CPA) was diagnosed based on the histopathological examination of the skin biopsy specimen from one of the scars. Such a presentation of childhood CPA, to our knowledge, is not documented in the English literature.

Childhood Plasmodium vivax malaria with severe thrombocytopenia and bleeding manifestations.

Thrombocytopenia of varying magnitude, usually without hemorrhagic manifestations, is commonly observed in Plasmodium falciparum and Plasmodium vivax malaria. However, severe thrombocytopenia associated with bleeding manifestations in malarial infection is distinctly unusual. The pathogenesis of this condition remains speculative till date, although several hypotheses have been put forward. We report a case of 4-year-old boy with P. vivax malaria, who developed severe thrombocytopenia during the course of his infection manifested by bleeding from skin and mucosal surfaces. He improved with antimalarial and intravenous immunoglobulin therapy. Clinicians caring for children should be aware of potential bleeding manifestations that may be the result of apparently innocuous infection like vivax malaria.