This paper reviews the subject of tuberous sclerosis complex and presents data in eight cases of this condition, admitted to Hospital Universiti Sains Malaysia over a period of 8.5 years. The average age at presentation was 53 months. Seizures were the most common presenting feature. Male to female ratio was 3:1. Family history was present in four patients. All of the patients had one or more skin lesions. Six of them had retinal in six patients. Two patients had renal angiomyolipomas. A high index of hamartomas. Cardiac tumours were found in two patients. Multiple subependymal hamartomas were detected in six patients. Two patients had renal angiomyolipomas. A high index of suspicion and a detailed physical examination is required to diagnose this rare disorder.
Nocardiosis is an increasingly recognised opportunistic infection in immunologically incompetent hosts but diagnosis is often delayed. Between December 1975 to October 1988, our two Nephrology Units have encountered five cases of nocardiosis occurring in two post-renal transplant patients, two patients with systemic lupus erythematous (SLE) and one patient with mesangiocapillary glomerulo--nephritis. All were on immunosuppressants at the time. The first three patients presented with predominant pulmonary disease and were cured by combined trimethoprim-sulphamethoxazole (cotrimoxazole) and doxycycline therapy. The patient with limited skin involvement responded to cotrimoxazole alone. However, the last patient with lymphocutaneous disease initially responded to cotrimoxazole (+ chloramphenicol) but developed acute-on-chronic renal failure and relapsed with dose reduction of cotrimoxazole. Alternative treatment with amikacin and doxycycline was instituted with good response. We shall review potential clues that may suggest the diagnosis of nocardiosis and discuss other effective antimicrobial agents.
Consideration. is gzven to the recognition and prevention of carious types of mental retardation due to hazards of environmental origin. Observations are presented on congenital syphilis, congenital toxoplasmosis, congenital rubella, Singapore kernicterus, Japanese B encephalitis, and tuberculous meningitis. Appropriate preventiue measures have resulted in a significant reduction in Singapore of these conditions, and hence in a decreased frequency of environmentally determined mental retardation. and related disabilities.
Obesity is no longer just a "Western" problem, as evidenced by an increase in prevalence of up to 75% in parts of the developing world. It is important to transfer experience from the developed world to developing countries in an attempt to prepare for the inevitable health and economic problems. This case report highlights an unusual intraoperative complication that has medical and medico-legal implications. A simple apparatus designed to retract the panniculus of an obese patient might reduce complications when performing abdominal surgery in such cases.
We describe a patient with empyema thoracis that had eroded through the apical chest wall to the supraclavicular fossa and lung parenchyma, ultimately leading to a bronchopleural fistula manifesting as lobulated, localized subcutaneous emphysematous swellings over the chest wall and supraclavicular fossa. This is a rarely reported phenomenon and the reason why the lobulated subcutaneous emphysematous swellings remained localized is unknown.
A 17-year-old, sexually active, single, nulliparous young woman presented to us with one week history suggestive of nephrotic syndrome. She was found to have a benign hydatidiform mole confirmed by histopathological examination after suction and curettage. Renal biopsy revealed focal segmental glomerulosclerosis. The renal pathology was most probably due to molar pregnancy due to the close temporal relationship. To our knowledge, this is the first case of focal segmental glomerulosclerosis associated with a gestation trophoblastic disease described in the literature.
We report an unusual co-existence of Burkholderia pseudomallei and acid fast bacilli in a young Malay gentleman with liver abscess. He was treated with antibiotics and surgical drainage. This phenomenon has not been reported in previous literature and the dilemma of its management is discussed.
Pneumoperitoneum is almost always pathognomonic of a perforated abdominal viscus requiring urgent surgical intervention. Spontaneous or non-surgical pneumoperitoneum is a rare clinical condition arising secondary to abdominal, thoracic, gynaecologic or idiopathic causes. In addition to good clinical judgement, an important component in the management process is to rule out other causes of pneumoperitoneum by performing appropriate investigations. We describe a 60-year-old man who presented with clinical features of pseudo-obstruction, following an injury to his back which was compounded by hypokalaemia. Roentgenography revealed massive pneumoperitoneum and colonic distension. As there were no overt clinical features of peritonitis, the patient was managed conservatively with parenteral nutrition and close observation. A water-soluble contrast enema and computed tomography of the abdomen were of no help in identifying the cause of his pneumoperitoneum but were helpful in eliminating the presence of hollow viscus perforation or an obvious inflammatory focus. The aetiology of pneumoperitoneum in our patient was most likely due to dissection of air through the distended colonic wall, secondary to large bowel pseudo-obstruction. The diagnosis of spontaneous or non-surgical pneumoperitoneum is one of exclusion and we stress the importance of relying on clinical parameters when managing such patients conservatively.
An episode of acute upper airway obstruction was caused by a lingual haematoma, when a patient with end stage renal failure suffered a hypocalcaemic fit and bit his tongue. The large haematoma and profuse bleeding caused the patient to obstruct and become hypoxic, and rendered laryngoscopy and intubation impossible, requiring an urgent tracheostomy to secure the airway.
A case report of a missed appendicitis presenting with abdominal wall necrotising fasciitis which extended up to the right knee. This subcutaneous collection in the prepatella region of the right knee presented as a crepitus and mimicked an intraarticular pathology.
Thyrotoxic psychosis is an uncommon phenomenon and the presentation is usually of the affective disorders. Three cases manifesting psychotic symptoms akin to schizophrenia are described. The neurobiocheinistry and treatment are discussed.
Endometriosis and its complications are not uncommon in Malaysian women. Two cases of ureteric obstruction due to endometriosis are reported and the literature on ureteric obstruction and endometriosis reviewed. It is emphasized that the possibility of ureteric obstruction must be suspected when there is extensive endometriotic disease or if the patient presents with loin pain or pain on one side of the abdomen. Ultrasound scan of the kidneys and, if necessary, an intravenous pyelogram will have to be done on the slightest suspicion. Patients with endometriosis will have to be followed up until the menopause or even later if they are on hormone replacement therapy.
Varicella zoster infection is one of the self-limiting viral infections during childhood and dengue fever is an endemic infection in Malaysia, which commonly occurs in the form of nonspecific febrile illness at the initial stage. It is rare for the two viral infections to occur simultaneously. A case of dengue fever without warning sign in a five-year old girl was reported, with early symptoms of fever and vesicular rashes. She was clinically diagnosed with varicella zoster infection during the first visit. Surprisingly, she remained febrile even on day six of illness despite no new vesicular lesions on her skin. Due to suspicion of another infection, follow-up investigation was done and revealed isolated thrombocytopenia. This finding was confirmed with positive NS1Ag. A case of rare dengue fever concomitant with varicella zoster infection was reported.