Asian Journal of Pediatric Research

Diagnosis and Treatment of Paediatric Septic Shock: A Critical Narrative Review of Recent Advances, Persistent Uncertainties and the Translation Gap

Xiaoli Li — 2026-09-28

Paediatric septic shock remains one of the largest contributors to childhood mortality worldwide, yet the field has changed more in how the condition is defined and detected than in how it is treated. The publication of the Phoenix sepsis criteria in 2024 replaced two decades of reliance on the systemic inflammatory response syndrome with a data-derived, organ-dysfunction-based construct, and the 2026 update of the international paediatric sepsis guidelines consolidated a management framework that nonetheless rests overwhelmingly on low-certainty evidence. This critical narrative review examines what has genuinely advanced, what remains contested, and why diagnostic refinement has not yet translated into measurable therapeutic gain. Literature was identified through structured searching of biomedical databases, scholarly indexes and citation tracking, with critical appraisal directed at study design, setting, outcome selection and generalisability rather than citation frequency. Four arguments are developed. First, the Phoenix criteria improve prognostic discrimination and cross-setting comparability, but they were derived to identify children already experiencing life-threatening organ dysfunction and perform substantially less well when repurposed as frontline screening instruments, particularly in intensive care populations where specificity falls sharply. Second, machine-learning and electronic screening systems demonstrate strong discrimination in retrospective evaluation yet have almost never been tested as interventions, leaving their clinical value unestablished. Third, host-response biomarkers, transcriptomic endotypes and latent-profile phenotypes have produced biologically coherent subgroups that remain unvalidated as treatment-selection tools, since no paediatric trial has yet randomised participants according to biological subclass. Fourth, the therapeutic evidence base has produced informative neutral and negative results, including a large pragmatic crystalloid trial showing no advantage of balanced fluid over saline, while the two adequately powered corticosteroid trials remain unreported. Across all domains, evidence is concentrated in high-resource settings, whereas mortality is concentrated elsewhere. Closing this gap requires trials designed around biological subclassification, functional outcomes and context-adapted delivery rather than further refinement of diagnostic labels alone.

Multiple Presumed Pyogenic Liver Abscesses Following Apparently Uncomplicated Acute Appendicitis in a Child: A Case Report

A. Lotfi — 2026-09-03

Aims: To describe multiple presumed pyogenic liver abscesses developing shortly after appendectomy for acute appendicitis reported as uncomplicated in a child, and to highlight diagnostic uncertainty, portal venous findings, and management when drainage is considered unsafe.

Presentation of Case: A 7-year-old boy was admitted five days after appendectomy with fever, abdominal pain, and deterioration of his general condition. Haemoglobin was 3.5 g/dL, requiring red blood cell transfusion; white blood cell count was 24,800/mm³ and C-reactive protein was 261 mg/L. Ultrasonography and computed tomography showed multiple hepatic and pericholecystic collections, peritoneal effusion, hepatosplenomegaly, and portal cavernoma. Blood and ascitic fluid cultures were negative, and no abscess material was obtained for culture. After one week of a third-generation cephalosporin, an aminoglycoside, and metronidazole, fever and inflammation persisted; repeat computed tomography showed enlargement of the collections with capsular disruption. Treatment was escalated to intravenous imipenem, vancomycin, and metronidazole; heparin and enteral nutritional support were also initiated. Drainage was considered unsafe by the treating team because of concern for hepatic rupture. Intravenous antimicrobial therapy was continued for four weeks, with clinical and radiological improvement and a decrease in C-reactive protein to 60 mg/L. Portal cavernoma was already present on the initial imaging, and thrombophilia screening later showed reduced protein C activity.

Discussion: Appendicitis-associated liver abscesses are rare in children and are usually reported with perforated appendicitis or pylephlebitis. In this child, the appendicitis had been reported as uncomplicated, making the temporal association unusual. Because no abscess material was obtained, the pyogenic nature of the lesions remained presumptive. The chronology and significance of the portal cavernoma could not be established in the absence of previous imaging.

Conclusion: Persistent postoperative fever and abdominal symptoms should prompt abdominal imaging even after apparently uncomplicated appendicitis. When drainage is considered unsafe, prolonged antimicrobial therapy with close clinical and radiological monitoring may be effective in selected patients. A temporal association in a single case does not establish causality.

Labial Actinomycosis in a Child in University of Uyo Teaching Hospital, Uyo Akwa Ibom State South-South Nigeria: A Case Report

Ikechukwu Agwu Francis — 2026-09-04

Background: Actinomycosis is a chronic suppurative infection caused by anaerobic Gram-positive Actinomyces species that normally colonise the oral cavity and digestive and urogenital tracts. Although cervicofacial disease is recognised, isolated labial involvement is uncommon, particularly in children, and may resemble other superficial or neoplastic lesions.

Case Report: An 11-year-old male was referred from a private paediatric clinic with a two-year history of recurrent mouth blisters and ulcers, associated skin lesions, low-grade fever, throat pain, and difficulty swallowing. Examination showed swollen lips and gums, multiple well-circumscribed ulcerative lesions with white-yellow pseudomembranous exudate, areas of necrosis, friable granulation tissue, and black eschar. No sinus tract was observed.

Diagnostic Assessment: Full blood count showed anaemia, neutrophilia, and thrombocytosis; HIV serology was non-reactive, and kidney function test results were normal. Direct Gram staining of lip aspirate demonstrated filamentous branching Gram-positive rods with sulphur granules. Actinomyces israelii could not be isolated because anaerobic diagnostic tools were unavailable. The patient received penicillin therapy and showed complete clinical resolution after eight weeks of treatment.

Conclusion: The case demonstrates the diagnostic value of direct microscopy in labial actinomycosis, particularly where anaerobic culture facilities are limited, and highlights the contribution of clinical microbiology to timely recognition and management of atypical lip lesions.

Cerebellar Ataxia as a Rare Presenting Manifestation of Juvenile Systemic Lupus Erythematosus: A Case Report

A. Lotfi — 2026-09-05

Aims: To describe cerebellar ataxia as a rare presenting manifestation of juvenile systemic lupus erythematosus (jSLE) and to emphasise the diagnostic value of associated systemic findings.

Presentation of Case: An 11-year-old previously healthy girl presented with a facial eruption and gait disturbance. Examination revealed a malar rash, frontal cicatricial alopecia, chilblain-like finger lesions, hypomimia, a predominantly left-sided resting tremor, broad-based gait ataxia, motor incoordination, and involuntary blinking. Laboratory investigations demonstrated normocytic normochromic anaemia, severe thrombocytopenia, marked inflammatory activity, hypocomplementaemia, a positive direct Coombs test, and positive ANA, anti-dsDNA, anti-Sm, anti-SSA, anti-ribosomal P, and anti-RNP antibodies. The infectious work-up was negative. Brain computed tomography was normal, while magnetic resonance imaging showed widening of the cerebral cortical and cerebellar sulci, suggestive of cerebral and cerebellar volume loss. The patient received intravenous methylprednisolone pulses followed by oral corticosteroids and antimalarial therapy, with subsequent improvement in cutaneous lesions, resting tremor, gait ataxia, motor coordination, and laboratory abnormalities.

Discussion: Cerebellar ataxia is an exceptionally uncommon manifestation of SLE, particularly as an initial presentation in childhood. Attribution to lupus requires exclusion of alternative causes and integration of neurological, systemic, immunological, and neuroimaging findings.

Conclusion: jSLE should be considered in children with otherwise unexplained cerebellar ataxia or movement abnormalities when accompanying mucocutaneous, haematological, or immunological features are present.

Shapiro's Syndrome (Spontaneous Periodic Hypothermia) in a Child: A Case Report

Hajar Belmkadem — 2026-09-11

Aims: To describe a paediatric case of spontaneous periodic hypothermia (Shapiro's syndrome) occurring in the absence of agenesis of the corpus callosum, and to review the relevant literature and diagnostic approach.

Presentation of Case: A 2-year-and-9-month-old boy, previously managed twice for intestinal intussusception, was referred after a one-month history of recurrent hypothermic episodes associated with transient alterations of consciousness. Seven episodes were documented by the medical and nursing staff, with axillary temperatures between 33 °C and 35 °C lasting two to seven hours. The episodes were frequently accompanied by profuse sweating, generalised pallor, and occasional shivering, and were sometimes preceded by a febrile peak of 39 °C. Throughout the hypothermic phases, the child remained haemodynamically stable, with preserved oxygen saturation and blood glucose. Biological work-up showed no evidence of infection or inflammation (C-reactive protein, procalcitonin, and cultures were negative), viral serologies were negative, and thyroid function, cortisol, electrolytes, renal and hepatic function were normal. Both brain magnetic resonance imaging (MRI) and electroencephalography (EEG) were unremarkable, with a structurally normal corpus callosum.

Discussion: After exclusion of metabolic, infectious, endocrine, and structural neurological causes, a diagnosis of a variant of Shapiro's syndrome without agenesis of the corpus callosum was retained. The clinical sequence observed in our patient is consistent with the prevailing hypothesis of a transient downward shift of the hypothalamic thermoregulatory set-point. Management was conservative, with no pharmacological treatment initiated; at the last available follow-up, three months after discharge, the frequency of episodes had markedly decreased, with three episodes over that interval.

Conclusion: Spontaneous periodic hypothermia, although rare, should be considered in children presenting with recurrent unexplained hypothermia once more common causes have been ruled out.

Early-Onset Seizures Revealing 1p36 Deletion Syndrome in an Infant with Craniofacial Dysmorphism and Optic Disc Coloboma: A Case Report

Safae Abdessadek — 2026-09-17

Aims: To describe an infant in whom early-onset seizures led to the diagnosis of 1p36 deletion syndrome and to emphasise the diagnostic contribution of whole-exome sequencing with copy-number variation analysis when the early phenotype is incomplete.

Presentation of Case: A 2-month-old male infant was admitted for generalised clonic seizures with gaze fixation in an afebrile setting. Examination showed hypotonia and craniofacial dysmorphism. Electroencephalography demonstrated diffuse interictal epileptiform abnormalities with right frontal predominance, whereas brain magnetic resonance imaging was normal. Ophthalmological examination revealed an inferior optic disc coloboma of the left eye. Echocardiography was normal, while abdominopelvic ultrasound showed testicular ectopia. TORCH serologies were negative and the metabolic work-up was normal. Whole-exome sequencing with copy-number variation analysis identified a pathogenic heterozygous deletion of at least 9 Mb involving chromosome 1p, consistent with 1p36 deletion syndrome. Seizures ceased under sodium valproate.

Discussion: 1p36 deletion syndrome has marked phenotypic variability, and epilepsy may begin in early infancy. A normal brain MRI or cardiac assessment does not exclude the diagnosis when seizures are associated with hypotonia and dysmorphic features. The optic disc coloboma in this patient should be considered an uncommon ocular finding rather than a specific manifestation. Exome-derived copy-number analysis can identify large deletions, but dedicated cytogenetic confirmation remains important for characterisation of the rearrangement and genetic counselling.

Conclusion: 1p36 deletion syndrome should be considered in infants with unexplained early-onset seizures, hypotonia, craniofacial dysmorphism, and associated congenital anomalies. Early genetic diagnosis supports appropriate multidisciplinary follow-up and family counselling.

Atypical Kawasaki Disease with Hepatosplenomegaly in a 6-Month-Old Infant: A Case Report

Lalnun Siami — 2026-09-19

Kawasaki disease (KD) is an acute, self-limiting systemic vasculitis that predominantly affects children younger than 5 years and may involve the coronary arteries. In young infants, incomplete or atypical presentations may complicate early recognition, particularly when classical clinical criteria are not fulfilled. This case describes a 6-month-old full-term male infant weighing 7.8 kg who presented with a 9-day history of fever and a generalised erythematous maculopapular rash that developed on the second day of illness. Only one principal clinical feature of KD, the polymorphous rash, was present. Laboratory investigations showed leukocytosis with lymphocytosis, thrombocytosis, an elevated C-reactive protein level, anaemia with reduced mean corpuscular volume and mean corpuscular haemoglobin, and proteinuria. Abdominal ultrasonography demonstrated hepatosplenomegaly, with liver and spleen measurements of 9.5 cm and 9.7 cm, respectively, while two-dimensional echocardiography showed no cardiac involvement or coronary artery aneurysms. Based on the prolonged fever, inflammatory findings, thrombocytosis, and hepatosplenomegaly, atypical Kawasaki disease was diagnosed. The infant received intravenous immunoglobulin, aspirin, methylprednisolone, and furosemide. Fever resolved and the clinical condition and inflammatory markers improved, and the infant was discharged after clinical stabilisation with follow-up advice. This case highlights the importance of considering atypical KD in infants with prolonged fever and unexplained hepatosplenomegaly despite incomplete classical features.