History of the Present Illness 5 wks PTA bipedal edema no other s/sx associated no consultation, no meds 4 wks PTA persistent bipedal edema consult w/ MD Imp.: Renal problem? u/a, abdominal uts: Normal Meds: Spironolactone
History of the Present Illness 3 wks PTA ankle, knee pain, weakness and edema no fever, cough or colds consult same MD Chest x-ray: cardiomegaly, 2d-echo: ? results referred to PHC 2 wks PTA seen at PHC: cardiac problem prob sec to Hypothyroidism Advised work-up
History of the Present Illness 11 days PTA fever, cough and colds, joint pain and edema Ffup at PHC: 2d-echo arterial thrombosis Meds: Aspirin 5 days PTA tachypnea, chest pain. Dx: ATP,Hypothyroidism Meds: Amoxicillin
History of the Present Illness 3 days PTA 4 pillow orthopnea, DOB, pallor, gen. weakness consult at PCMC Labs: dec T3, T4, inc TSH Advised admx but refused 1 day PTA above s/sx persisted, severe respiratory distress consulted PCMC ADMITTED
Review of Systems: No headache No seizure No abdominal pain No vomiting No constipation No dysuria No hematuria No oliguria No bleeding No heat/cold intolerance
Birth and Maternal History 23 y/o primigravid RPNCU at 3 mos AOG c/o LHC No maternal illnesses Delivered FT, NSD At home, by midwife No complications
Immunization History BCG DPT x 3 doses OPV x 3 doses Hepa B x 1 dose Measles x 1 dose No boosters doses given
Nutritional History Breastfed up to 1 yrs old Complementary foods at 6 mos. At present: picky eater
Developmental History 1 mo:regards 4 mos: head control 8 mos: sits w/o support 9 mos: cruises, utters ‘mama’ 1 y/o: walks w/ support 2 ½ y/o: runs fast -At present, has reached first year high school w/ average grades.
Past Medical History 7 y/o: Dx w/ Hypothyroidism Maintained w/ L-Thyroxine Poor compliance, no follow-ups Admitted at 14 y/o: DHF 2 Discharged after 5 days No previous accidents, surgeries No allergies to food or drugs
Family History 43 42 15 A maternal uncle was diagnosed of goiter and presently on medication. No family history for diabetes, CVD, hypertension and tuberculosis.
Psycho-Social History Lives w/ her family in the province. Closest to her maternal grandparents Stopped schooling after 1st year high school due to financial problems Enjoys watching TV and going out w/ cousins. She has no crushes or suitors at present. Never tried smoking, drinking liquor or using prohibited drugs No suicidal ideations, but is sad for her health condition
Physical Examination Gen. Survey: awake, pale, in respiratory distress Vital Signs: CR: 150/min RR: 50/min T: 38’c BP: 100/60 Wt: 53 kg (p50) Ht: 148 cm (p50), BMI: 24.1 No active dermatoses, diaphoretic Anicteric sclerae, pale palpebral conjunctiva, (+) alar flaring, (+) mucoid nasal discharge, no tonsilopharyngeal wall congestion, (+) CLAD, (+) neck vein engorgement Equal chest expansion, (+) SC and IC retractions, (+) decreased breath sounds left lung field, (+) rales bilateral lung fields, tactile/vocal fremiti dullness
Physical Examination Adynamic precordium, tachycardic, (+) muffled, distant heart sounds, no murmurs. Globular abdomen, NABS, (+) fluid wave, soft, (+) hepatomegaly, no masses palpated Tanner SMR stage 5 (+) grade 3 bipedal edema, no cyanosis, no clubbing, full and equal pulses, CRT: 2-3 secs.
Neurologic Examination: Cerebral: awake, conscious, oriented to 3 spheres Cranial nerves: I: intact II: 2-3 mm pupils, ERTL, no papiledema or hemorrhages III, IV, VI: intact EOM’s V: brisk corneals VII: no facial asymmetry VIII: intact gross hearing IX, X: good gag XI: good shoulder shrug XII: midline tongue
Neurologic Examination Motor: good muscle bulk and tone, 5/5 both upper extremities, 4/5 both lower extremities. Sensory: intact Reflex: +2 all extremities No Babinski, no clonus Supple neck Cerebellar: no nystagmus, good finger-to-nose test
Admitting Impression Congestive Heart Failure, Cardiac Tamponade probably secondary to Hypothyroidism Late Adolescent w/ Psychosocial Issues No Wasting, No Stunting
S> no seizure recurrence > no DOB > fair intake > joint pain 8th Hospital Day A> Pleural Effusion 2 to Myxedematous Pericardial Effusion sec to Hypothyroidism r/o Parapneumonic Effusion Seizure sec to Electrolyte Imbalance, resolved t/c SLE S > low to mod grade fever > tachypneic, tachycardic > painless, oral mucosal lesions > CXR: haziness R, consolidation vs pleural thickening P> Trans out to 121
S> no seizure recurrence > no DOB > cough > poor intake 10th-11th Hospital Day A> Myxedematous Pericardial Effusion s/p Pericardiostomy w/ Pleural Effusion prob sec to Hypothyroidism Sepsis Unspecified O> moderate to high grade fever > tachypneic, tachycardic > (+) rales BLF, distinct heart sounds > chest uts: bilat. Pleural fibroses, min pleural & pericardial effusion L. P> Oxacillin continued > Cefotaxime started > Amikacin shifted to Gentamicin
S> no seizure > no DOB > poor intake 12th-15th Hospital Day A> SLE Hypothyroidism Nosocomial sepsis O>mod-high grade fever > tachycardic, tachypneic, normotensive > oliguria, hepatomegaly > 2d-echo: pericardial effusion > ESR: 120 mm/hr > s. Albumin: decreased > s. Creatinine: normal P> Increased fluid intake encouraged > for ANA, anti-DS DNA
Arthritis Persistent knee and ankle pain Non-erosive, non-deforming 2 or more peripheral joints Tenderness, swelling, effusion Anti-inflammatory meds.
Mucocutaneous Involvement Beseler, Silvermann Pediatr Clin N Am 52 (2005) 443-467
Mucocutaneous Involvement Petechial rash, ecchymoses not as frequent r/o other lesions of platelet count abnormalities aggravated by sun exposure ‘..definite photosensitivity occurs in 16% of children…’ Downing, Mesina N Engl J Med 1992; 227:408-409
Mucocutaneous Involvement ‘rashes occur frequently in children w/ SLE but only 30-50% ever manifest the typical butterfly rash.’ Glidden, Mantzouranis, Borel Clin Immunol Immunopathol 1983; 29;196-210
Hematologic Involvement Pallor; normocytic/ normochromic anemia ‘ …the most common hematologic manifestation of SLE in children and adolescents is anemia…’ Wallace, Hahn2002
Cardiac Involvement Pericardial effusion cardiac tamponade ‘… Cardiac manifestations rarely are prominent in children and adolescents w/ SLE, but occasionally they are catastrophic…’ Auito, Stanbouly, Boxer Clin Pediatr 1993;32:566567
Cardiac Involvement Common Cardiac Pathology - Pericarditis - Myocarditis - Mild Valvular Involvement ‘…The most common form of cardiac involvement is pericarditis w/ pericardial effusion…’ Chan, Li, Tam Scand J Rheumatol 2003;32:306-308
Many children w/ SLE are anemic and develop few murmurs. Libman-sacks endocarditis may occur in childhood however, and this predisposes to bacterial endocarditis..
Wallace, Hahn 2002
Pulmonary Involvement Pleural effusion, bilateral ‘… Pleurisy and pleural effusion are the most common pulmonary manifestations…’ Delgado, Mulleson, Pine Semin Arthr Rheum 1990; 29: 225-293
Pulmonary Involvement Other Pulmonary complications: - pneumothorax - pneumonia - chronic restrictive lung dse. - pulmonary HPN - acute pulmonary hemorrhage Most fatal complication in children and adolescents: pneumonia
Pulmonary Involvement ‘…Pneumonia was the cause of death for 9 of 26 children w/ SLE coming to autopsy…pulmonary hemorrhage contributed to death of 5 others…’ Nadora, Landing Pediatr Pathol 1987: 7;118
GIT Manifestations Jaundice, hepatomegaly, abnormal LFT’s Other s/sx: - abdominal pain, anorexia, weight loss Often resolve w/ corticosteroid therapy
Infections in SLE Patient developed nosocomial sepsis due to her immunocompromosed state Major cause of mortality and morbidity Plat et al: 55 separate infections in 70 patients, over 9 years. >Results from: 1. combined effects of SLE 2. drugs used to mediate it
Infections in SLE ‘…The frequency of infection increases w/ increasing steroid dosage…’ Guizler, Deamond, Kaplan Arthr Rheum 1978; 21:37-44 Careful use and reduced dosage of corticosteroids decreases frequency of infections
Laboratory Evaluation ANA Anti- DS DNA Anti- Smith Ab Autoantibodies Hypergammaglobulinemia
Endocrine Involvement Thyroid Hypothyroidism>Hyperthyroidism ‘…Up to 35% of SLE patients have anti-thyroid antibodies, w/ 10-15% of patients developing overt hypothyroidism…’ Eberhard, Laxer, Eddy J Pediatr 1991;119:277-9
Treatment Depends on target organs and disease severity Pericardiostomy tube insertion, antibiotic coverage, immunosuppresive therapy
Cornerstone of Treatment of Children w/ Rheumatic Disease Accurate diagnosis and education of family Medications Physical medicine and rehabilitation Physical and psychosocial growth and development Coordination of care
Chronic Disease in Adolescents: Issues Involved 1.Compromised mental health may make management of the physical illness more difficult. - increased risk for psychiatric disorders and social adjustment problems. - non-compliance w/ medical treatment. 2. Risk taking in the areas of sexuality and substance use is an important contributor to morbidity. - management of high-risk pregnancies, w/c demands a high degree of compliance and medical regimentation. 3. Functional impairments increase the risk of academic and adjustment problems.
Chronic Disease in Adolescents:Trends in Management
Needs a biopsychosocial, multidisciplinary approach