Blood product transfusion practices in pediatric critically ill patients at a tertiary care hospital, Pakistan.

Objective: To determine the frequency of inappropriate blood product transfusions in pediatric critically ill patients. Methods: We collected data for this descriptive study from January to December 2020 at the Pediatric Intensive Care Unit (PICU) of Dr. RKMP Civil Hospital, Karachi. We included all patients one month to 12 years of age, admitted to pediatric intensive care unit and received any blood product transfusion. We reviewed their medical records and noted the demographic and clinical variables, type of blood product transfused with its indication and determined whether transfusion was appropriate or not, as per the standard guidelines and consensus recommendations. Results: Number of patients who were transfused was n=39, out of which females were 51.3%. Mean age of the patients was 49.0 months ±50.9 months. Total number of transfusions done were n=135, with most common blood product transfused being Fresh Frozen plasma (FFP) in 44.4%. A total of n=117 (86.66%) transfusion were appropriate as per the standard guidelines, while 18 (13.33%) were inappropriate (P-value <0.5). The most common indication of FFP transfusion was INR >2.0 in 51.6%, for Packed red cell transfusion was hemoglobin between 5 and 7 gm/dl in 35.5% and for Platelets was <20,000 with risk of bleeding in 36.6%. Conclusion: This study highlights the occurrence of inappropriate transfusions in critically ill pediatric patients. We need to reinforce the knowledge of our health care workers and display the guidelines in intensive care units.

Intermediate Autosomal Recessive Osteopetrosis With an Unusual Absence of Fractures.

Background: Osteopetrosis includes a variety of rare inherited skeletal disorders characterized by increased bone density and thickness. It has different clinical forms, including infantile autosomal recessive, intermediate autosomal recessive, and late-onset autosomal dominant forms. Intermediate autosomal recessive osteopetrosis (IARO) displays high variability. Case Report: A 10-year-old male presented to our pediatrics emergency department with abdominal distension, low-grade fever, and swelling of the right maxilla with associated discharge. His local physician had treated the lesion with drainage and aspiration of pus without improvement. Examination revealed pallor, hepatosplenomegaly, poor dentition, and dental caries. Eye examination showed reduced visual acuity, absent color vision, nystagmus, and bilateral optic nerve atrophy. Laboratory investigations showed anemia and thrombocytopenia. Radiography yielded classic features of osteopetrosis. Detailed intraoral examination revealed an area of exposed necrotic bone in the alveolar region of the right maxilla, leading to a diagnosis of IARO with underlying osteomyelitis. The intraoral wound was treated with bismuth iodoform paraffin paste dressing, and the infection was treated with antibiotics. Anemia and thrombocytopenia were managed supportively by transfusion of packed red blood cells and platelets. Conclusion: IARO commonly presents with multiple fractures, so the absence of fractures in our patient was unusual. Studies evaluating the intermediate variant are meager; hence, documenting its various presentations is essential to aid physicians in making early diagnoses. Osteomyelitis of the jaws is a feared complication in these patients. Therefore, practitioners need to be cautious of infections of dental origin.

Sclerosing angiomatoid nodular transformation of spleen in a 8-year old girl.

Sclerosing angiomatoid nodular transformation of spleen is a rare, benign vascular lesion with an uncertain pathogenesis. It has been described as a separate entity through specific histopathological characters. It is usually asymptomatic, occurring commonly in adult females. Only a few cases of paediatric cases have been reported which have been commonly symptomatic. This disease has excellent prognosis after splenectomy, which is the only treatment. We report the case of an eight-year-old girl who presented with distended abdomen and history of bleeding from the nose following a road traffic accident. Examination revealed stunted height, decreased weight, tachypnoea, tachycardia, anaemia and a firm, massive spleen. Lab investigations further revealed microcytic anaemia, thrombocytopenia, deranged platelet profile and low vitamin B12 and folate levels. Computed tomography confirmed enlarged spleen. Therefore, a diagnostic biopsy was planned which confirmed sclerosing angiomatoid nodular transformation of spleen. Splenectomy was successfully performed soon after and the child is now healthy with no remissions of previous symptoms.

Seizures and Basal Ganglia Calcification Caused by Hypoparathyroidism.

Seizures, not a typical feature of hypoparathyroidism, may present along with myalgia and tetany in patients of hypoparathyroidism. Thorough history and examination, derangement of biochemical parameters such as hypocalcemia, hyperphosphatemia, and inappropriately low levels of parathyroid hormone, along with basal ganglia calcification as seen on imaging, lead to the diagnosis of hypoparathyroidism in a 10-year-old child who presented to us. Treatment with calcium and active vitamin D significantly improved his condition. In this report, we discuss the presentation and treatment of hypoparathyroidism. Early detection and follow-up at clinically appropriate intervals is important to avoid complications.

Pierson Syndrome Associated with Hypothyroidism and Septic Shock.

Pierson syndrome is caused by mutations in the laminin ß2 gene causing absent ß2 laminin, which is a normal component of the basement membranes of the mature glomerulus, structures in the anterior eye and neuromuscular junctions. The mutations manifest as congenital nephrotic syndrome and microcoria which are characteristic ocular features of this disease. These mutations may also result in neurological abnormalities such as hypotonia and psychomotor retardation. We report a two-month old boy who presented to the Pediatrics Department of Dr. Ruth K. M. Pfau Civil Hospital, Karachi, Pakistan, in 2015, with the typical features of microcoria and congenital nephrotic syndrome. The hypocalcaemia, hypoproteinaemia and probable immunocompromised state consequent to nephrotic syndrome resulted in seizures, hypothyroidism and urosepsis. Despite being treated aggressively with high dose antibiotics, ionotropic support, angiotensin-converting enzyme inhibitors, thyroxine replacement and nutritional support, the infant died due to significant multiorgan disease including renal failure and septic shock.

Congenital Cytomegalovirus Infection and Tetralogy of Fallot: An Unusual Association in a Three-month-old Baby.

Congenital cytomegalovirus (cCMV) infection is the leading cause of infant morbidity and mortality worldwide. Despite being associated with significant neurological sequelae in infected infants, it remains an under-recognized public health entity. Symptomatic newborns most frequently display hepatosplenomegaly, petechiae, jaundice, microcephaly, intrauterine growth restriction, chorioretinitis, purpura, and seizures. Progressive sensorineural hearing loss is the most prominent adverse outcome of both symptomatic and asymptomatic CMV infections in infants. We report the case of a three-month-old baby who presented with complaints of progressive jaundice for three months and a two days history of fever associated with one episode of fits. The baby was diagnosed with congenital CMV infection on the basis of positive CMV IgM and IgG and positive maternal serum CMV IgG. Finding a murmur on physical examination prompted echocardiography which revealed Tetralogy of Fallot (TOF). The child was managed with a 6-week course of ganciclovir after which his symptoms improved and he was referred to cardiology for the evaluation of his heart defect. Follow-ups at the clinic have shown normal growth and development. This is the first reported association of cCMV infection with TOF. This case highlights the need to consider the possibility of the presence of heart defects in all infants with cCMV infection in addition to neurodevelopmental abnormalities. Clinicians should maintain a high degree of suspicion for cCMV infection in all neonates to ensure timely intervention and to prevent long-term neurological sequelae.

The First Case of Rowell Syndrome with Lupus Nephritis and Lobar Pneumonia in a Male Child Reported in Pakistan.

Rowell syndrome (RS) is a rare entity that presents with lupus erythematosus (LE), erythema multiforme (EM) like lesions and characteristic immunological findings including positive rheumatoid factor (RF), speckled antinuclear antibody (ANA) and positive anti-Ro or anti-La antibodies in the serum. Only a few cases have been reported in the literature, mostly in middle-aged women. Our case is about an 11-year-old male child who had a history of pustular rashes over the whole body with scattered targetoid lesions for the past few months and later developed respiratory distress and swelling of the body. He was extensively investigated and confirmed on specific positive immunochemistry markers as RS along with lobar pneumonia (LP) and lupus nephritis. The co-existence of lobar pneumonia in our patient was a unique feature that has not been previously reported.

Maculopapular Cutaneous Mastocytosis in a Six-month-old Boy Who Presented with Respiratory Distress.

Mastocytosis is a diverse group of rare disorders characterized by mast cell proliferation and its aberrant accumulation within various organs including respiratory, gastrointestinal, genitourinary mucosa and most commonly the skin. The spectrum of presentations ranges from torpid disease (cutaneous mastocytosis) having normal life span with transient sign and symptoms to highly vigorous disease (systemic mastocytosis) and life-threatening malignant conditions. Prevalence of the disease in general population is unknown. It occurs in all races and there is no sex predilection. Peak incidence is during infancy and early childhood with second peak occurring in middle age. We report a case of maculopapular cutaneous mastocytosis (MCM) in a six-month-old male child who presented with respiratory distress. According to our literature search it is one of the least frequent cases reported in our country, Pakistan.

Non-pseudomonal Ecthyma Gangrenosum and Idiopathic Myelofibrosis in a Two-Year-Old Girl.

Ecthyma gangrenosum is a skin lesion consequent to bacteremia, mostly due to Pseudomonas aeruginosa, although it may develop secondary to other organisms as well. The disease is often witnessed in patients with leukemia; however, a few cases of ecthyma gangrenosum in adults were reported to be associated with myelofibrosis. We report a case of ecthyma gangrenosum due to Escherichia coli (E. coli) in a two-year-old girl with idiopathic myelofibrosis.

Do We Need To Give Measles Vaccine To Children Earlier Than The Currently Recommended Age?

BACKGROUND: Measles is a leading cause of death among children. No specific drug has yet been discovered to treat measles but an available vaccine can effectively prevent the infection. In Pakistan children are vaccinated against measles by two doses given at age of nine months onward. The last few years have witnessed an increasing number of measles cases at age lower than nine months. METHODS: Secondary data analysis of the records of Expanded Program on Immunization from all districts of Sindh was performed from January-April 2016. Data included all patients of any age or gender, fulfilling the World Health Organization case definition of measles, along with positive IgM antibodies for measles in their blood. Data was analysed using windows SPSS version 21.0. RESULTS: Analysis of 658 confirmed measles cases showed an age range of 3 months to 336 months with a mean of 32.82. Most patients (41.4%) belonged to age group 10-24months. Some noteworthy18% of cases were ≤9 months old including 1.8% patients who were ≤6 months age. An unexpected 76 (11.6%) were >60 months of age. 50.6% of the cases were male while 49.4% were female. Most of the patients (73.9%) belonged to urban areas. CONCLUSIONS: We conclude that a sizeable number of children are infected by measles before reaching the age of first recommended inoculation against measles. It is affecting people at both extremes of life ranging from as young as three months up to 28 years of age.

HEMATOLOGICAL CHANGES IN COMPLETE BLOOD PICTURE IN PAEDRIATRIC PATIENTS OF MALARIA CAUSED BY PLASMODIUM VIVAX AND FALCIPARUM.

BACKGROUND: Malaria is a major health problem and one of the major killers in paediatric population particularly in the developing world. High mortality is usually compounded by various haematological complications if left untreated. Their identification as risk factors for progression to severe disease may make the basis for optimal management of malaria. This study was conducted to determine various changes in the complete blood picture caused by malaria and to compare the severity of these changes among the prevalent species of plasmodia. METHODS: It was cross sectional study conducted in paediatric ward of Civil Hospital, Dow University of Health Sciences, Karachi over a period of six months. Children aged >2 months to 15 years, of either sex, with fever above 101 degrees F in the preceding 72 hours with positive malaria parasite on peripheral blood smear were included in the study. Children already on anti-malarial. treatment and long standing antibiotics, having co-morbidities like immune-compromised states, haemolytic disease or with any other haematological disorder were excluded from the study. Blood was tested for anaemia, leukopenia, leukocytosis, and thrombocytopenia. Data analysis was done via SPSS-15.0. RESULTS: Out of 374 children half were under 5 years of age with mean age of 66.7 +/- 46.8 months, 50.8% were female with male to female ratio of 1:1.03. Overall 364 (97.3%) children had anaemia with mean haemoglobin level of 11.7 +/- 6 g/dl. Overall mean WBC count was 10443 +/- 154 per cubic millimetre. Leukopenia was found in 39% cases. Mean platelets count of enrolled children was 69451 +/- 648 cubic millimetre and 51.3% cases had mild thrombocytopenia. Anaemia (p=0.012), leukopenia (p=0.001) and thrombocytopenia (p=0.004) were significantly more common in falciparum as compared to vivax malaria. CONCLUSION: We concluded that malaria frequently causes severe anaemia, leukopenia and thrombocytopenia in children. P. falciparum is the species more responsible for these changes.

Multiple autoimmune syndrome: Hashimoto's thyroiditis, coeliac disease and systemic lupus erythematosus (SLE).

Various autoimmune diseases have association with each other but it is very rare to see multiple autoimmune diseases in one patient. Presence of more than two autoimmune diseases in one patient is known as multiple autoimmune syndrome (MAS). We report the case of an 11 years old girl who presented with history of swelling in front of the neck along with constipation, anorexia, weight gain and increasing pallor over a period of six months. Additionally she had an episodic history of joint pains and abdominal pain with no specific relation to diet, time, other gastrointestinal or genitourinary symptom. Hypothyroid goiter (Autoimmune thyroiditis, Hashimoto's thyroidits) was diagnosed by raised thyroid stimulating hormone (TSH), low T4 and presence of thyroid specific antibodies in blood. Patient was discharged on tablet Levothyroxine to which she responded well with reduction in size of the swelling and relief of the symptoms except for the joint pains and abdominal pain. To evaluate the persistent symptoms she was investigated further for other autoimmune diseases and was diagnosed to be having systemic lupus erythematosus (SLE) and Coeliac disease also. The final diagnosis was multiple autoimmune syndrome (Hashimoto's thyroiditis, Coeliac disease and SLE).

Intestinal amoebiasis in children and its effect on nutritional status.

OBJECTIVE: To determine the frequency and clinical presentation of amoebic diarrhea in children and its effect on the nutritional status of the affected children. STUDY DESIGN: Descriptive. PLACE AND DURATION OF STUDY: Department of Diarrhea Treatment Unit (DTU), Dow Medical College and Civil Hospital, Karachi, from November 1998 to April 2001. METHODOLOGY: Paediatric patients between the ages of > 1 month to 15 years were included, who visited the DTU of the department with diarrhea. Stool samples of all these patients were checked under microscope for the vegetative forms of Entamoeba Histolytica (E.H.). Patients who were positive for E.H. were evaluated for the symtomatology including the type of diarrhea {acute watery diarrhea (AWD) or dysentery}, abdominal pain, tenesmus and nausea/vomiting. Examination carried out was assessed for state of hydration, fever and anaemia and nutritional status of those less than 5 years of age. The patients were divided into three age groups to assess the association of the age with severity and type of clinical presentation. Chi- square test was applied to calculate the p-values. P-values of 0.05 or less were considered statistically significant. RESULTS: The stool samples of 3870 patients with diarrhea were examined under microscope. Three hundred and twenty eight (8.47%) of these samples were positive for E.H. The difference between the number of patients presenting with acute watery diarrhea n=157, 47.86% and with dysentery n=171, 52.13% was found to be statistically non-significant (p=0.364). Two hundred and seventy seven (84.45%) patients had tenesmus, while abdominal pain was present in 287 (87.5%). Fever and malnutrition were present in 169 (51.52%) patients each. Malnutrition was significantly most frequent in 1-5 years of age groups (n=98, 57.98%, p=0.026). The nutritional evaluation of the 272 under-5 children with amoebic diarrhea showed a significantly normal status in 123 (45.55%, p-value < 0.001). All the signs and symptoms followed a similar trend with 1-5 years age group being the most affected group. However, the frequency of blood in stools increased in direct proportion to the increasing age. CONCLUSION: Although dysentery was the more common presenting complaint, almost half of the patients presented with watery diarrhea. Most of the patients with dysentery were under the age of 5 years. Fever was present in a high number of patients. The age group most affected by amoebiasis and malnutrition was 1-5 years.

Hereditary methemoglobinemia.

Methemoglobinemia is a rare congenital disorder of blood associated with central cyanosis. We report a case of 12 years old male child who had asymptomatic central cyanosis since birth in the absence of any cyanotic cardiorespiratory disease. He shared this cyanosis with seven other family members spanning over three generations. He was diagnosed as a case of methemoglobinemia on the basis of history and investigations, put on methylene blue and later ascorbic acid, to which he showed a very good response.

Renal and post-renal causes of acute renal failure in children.

OBJECTIVE: To identify the causes of acute renal failure (ARF) in pediatric population along with the identification of the age and gender most affected by the failure. DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: This study was carried out at the Pediatric Medical Ward of National Institute of Child Health (NICH), Karachi over a period of two years from 1996 to 1998. SUBJECTS AND METHODS: The study included children under the age of 12 years who presented with signs and symptoms suggestive of ARF (oliguria/anuria, vomiting, acidotic breathing etc.) along with raised blood urea nitrogen (BUN) serum creatinine and metabolic acidosis as shown by arterial blood gases (ABGs). Patients were divided into two groups on the basis of age; group A consisting of 0-2 years and group B from >2 years. Patients presenting with transient pre-renal azotaemia were excluded from the study. After providing initial emergency cover, detailed history, physical examination and investigations were carried out according to a proforma specially designed to ascertain the cause of ARF. Patients were managed for ARF as per standard recommendations and investigations completed or repeated as and when required. RESULTS: A total of 119 patients with ARF were admitted in the ward over a period of two years constituting 1.36% of the total admissions and 16.39% of the admissions due to renal pathology. Mean age of presentation was 4.5 years with 56.7% of the patients under the age of 5 years. Male predominance was noted in all ages with an overall male to female ratio of 2.3:1. Most common cause leading to ARF in younger age group was found to be hemolytic uremic syndrome [25(54.34%)] followed by septicemia [7(15.21%)]. In older patients renal calculus disease was the most common [22(30.13%)] underlying pathology followed by pre-existing, undiagnosed chronic renal failure [16(21.91%)]. More than 90% of cases were related, directly or indirectly, to infections. CONCLUSION: ARF is fairly common in children especially under the age of 5 years showing a male predominance. More than 90% of the cases can be prevented by improving primary health care and by early and prompt treatment of infections.